Combined Neuro

__ is a prokinetic agent used to tx N/V, gastroparesis. Pts taking this med should be monitored closely for the development of drug-induced extrapyramidal symptoms.

*Metoclopramide* = dopamine receptor antagonist - tx nausea/vomiting/gastroparesis - SE: agitation, loose stools, tardive dyskinesia, dystonic rxns, parkinsonism tx dystonic rxn: Benztropine or Diphenhydramine

Treatment of restless leg syndrome.

*Mild/intermittent sxs*: - Supplementation iron when serum ferritin < 75 - use supportive measures (leg massage, heating pads, exercise) - avoid aggravating factors (sleep deprivation, meds) *Persistent/moderate severe sxs*: *First line: Dopamine agonists* (pramipexole) Alternate: Alpha-2-delta calcium channel ligands (gabapentin enacarbil)

The majority of embolic stories are due to ___ that develop 2/2 to ___.

*Mural thrombi* *A. fib* * Pts with infective endocarditis can form septic emboli when parts of valvular vegetations break off and travel to the brain

Subdural hematoma are common in ___.

*Older patients* *Alcoholics due to brain atrophy + vessel fragility* Blunt or shearing trauma tears bridging veins, causing them to bleed into the subdural space

Pupils in ICH in Pons, thalamus, putamen

*P*ons: *p*inpoint, *T*halamus: non-reactive *T*owards, Putamen: dilated.

Enlargement of blind spot with momentary vision loss that varies according to changes in head positioning. Can lead to rapid permanent vision loss and requires urgent dx evaluation.

*Papilledema* - INC ICP transmitted to the optic nerve sheath, swelling of the optic nerve head - HA worse in the morning due to INC ICP Young age, obesity, due to idiopathic intracranial HTN (pseudo tumor cerebri) Next step get CT/MRI to exclude underlying mass lesion

parkinsons patient with orthostatic (and some dry skin, dry mouth ED)

*SHY-DRAGER*. MUTIPLE SYSTEM ATROPHY. parkinsonianism, autonomic dysfunction, widespread neuro signs TX: not anti-parkinson's drugs IVF, fludrocortisone, salt supplement alpha agonists, and constrictive garemnts to lower body? bulbar dyfxn and laryngeal stridor= fatal

Hemi-sensory loss of severe dysesthesia of the affected area is typical for a ___ stroke.

*Thalamic* (Dejerine-Roussy syndrome) - stroke in the VPL nucleus - CL hemianesthesia, transient hemiparesis, athetosis, or ballistic movements - dysesthesia in the sensory loss, thalamic pain phenomenon

treatment for herpes zoster (shingles)

*oral* acyclovir

25. 42yo M with 2-month history of staring spells that last 1 to 2 minutes each. Smacks his lips and picks at his shirt collar. Four years ago, he was comatose for 2 weeks after sustaining a head injury in a motorcycle collision; required 6 mo of rehab. Intermittent episodes of smelling burnt rubber that occur approximately every 2 weeks. Hears an intense hissing sound during these episodes. Dx?

+ hallucinations + large movements= complex partial seizure but what about lack of LOC?

44. 6yo boy with progressive visual loss over the past year. Deterioration of his hearing, speech, writing, and intellectual performance. Maternal uncle had similar symptoms. Visual acuity is 20/200 bilaterally. Funduscopic examination shows optic atrophy. Weakness and spasticity of all extremities. DRT are exremely hyperactive. Babinski's sign is present bilaterally. MRI shows marked symmetric white matter disease involving all lobes. Diagnostic studies will show?

- An excess of very long chain fatty acids

Clinical presentation: - HA - Focal neuro deficit - Solitary ring-enhancing lesion on brain CT scan - Fluid collection in the ethmoid sinus Dx.

*Brain abscess 2/2 ethmoid sinusitis* - Head and neck infections: *Viridans streptococcus* (most common) and other anaerobic (prevotella, bacteroides) - distant infections lung or endocarditis (staph aureus) tx: surgical draining and aspiration, prolonged abx therapy (4-8 weeks)

___ may result from hyperextension injuries, particularly in elderly patients with spondylosis.

*Central cord syndrome* - weakness in the UE - localized deficit in pain and T sensation

___ are benign suprasellar tumors that presents with visual defect, HA, and symptoms of pituitary hormonal deficiencies.

*Craniopharyngiomas* - Rathke's pouch common children, but can presents in adults - confirm MRI/ CT scan - Tx: Sx or radiotherapy

Normal pressure hydrocephalus thought to result from ____.

*DEC CSF absorption* or transient INC in ICP that cause permanent ventricular enlargement w/o chronically INC ICP Dementia, gait disturbance, and urinary incontinence

CT scan of ___ shows numerous minute punctate hemorrhages with blurring of grey-white interface.

*Diffuse axonal injury* - most significant cause of morbidity in patients with TBI - traumatic deceleration

B/L action tremor of the hands, usually w/o leg involvement. Isolated head tremor w/o dystonia. No other neuro signs.

*Essential tremor* - relieved with alcohol - Propranolol (useful also if pt has coexisting HTN) - second line: primidone, topiramate

Dx: - HA (worse at night) - N/V - Mental status changes - Papilledema - Focal neuro deficits Cushing reflex.

*Intracranial HTN* - *Cushing reflex* (HTN, bradycardia, respiratory depression) worrisome finding for *brainstem compression*

What does midbrain stroke present with?

*Ipsilateral Oculomotor Nerve Palsy *Ataxia (Damage to superior cerebellar peduncle) *Contralateral Hemiparesis

myotonic dystrophy

AD, CTG expansion in DMPK gene, or CCTG expansion of ZNF9 gene. probably due to RNA processing problem. leads to weakness, stiffness of distal muscles, action and percussion myotonia. proximal weakness comes later. also will have cataracts, ptosis, arrhythmias, dysphagia, insulin resistance, testicular atrophy, balding, changes in affect, personality, motivation, and cognitive dysfunction. Dx: CT is usually normal, myotonia on EMG, DNA tests, EKG drugs that decrease myotonia: mexilitine, phenytoin, carbamazepine

Tuberous Sclerosis, Sx

AD. Usu diagnose ~ 5-10 yrs. age 1. Ash leaf spots (hypopigmented) 2. Convulsive seizures (infantile) 3. Mental Retardation 4. Sebaceous Adenomas (butterfly nodules on face) 5. Shagreen patch (rough papule in lumobsacral area w orange peel consistency) 6. Renal angiolipoma (subcut nodule but involves BV - painful!!) 7. Cardiac rhabdomyoma 8. RETINAL HAMARTOMA

AIDP (acute demyelinating polyneuropathy) (GBS)

AI disorder where targets are gangliosides. will have anti GM1, GD1a, GD1b, GQ1b antibodies. most common is GD1b.

Labrynthitis looks like which arterial lesions

AICA + auditory changes

Labrynthitis looks like which stroke

AICA - lateral medullary stroke (wallenberg) - tons of sx

Parkinson's patient that develops a sudden onset of severe eye pain, nausea, vomiting, unilateral conjunctival injection and dilated pupil ...

Acute angle-closure glaucoma due to Anticholinergic medication .. such as Trihexypheidyl.

MS, best Rx

Acute attack - give IV Corticosteroids (decreases only ST course) Chronic: INF beta - best for relapsing remitting to decrease frequency of relapses and reduce LT progression of dz. Chronic Progressive MS: Immunosupp agents (Cyclosporine,MEthotrexate, etc) - works ST to slow rapidly progressive course. Muscle spasms - Baclofen or Dantrolene

"acute onset eye pain, global HA, blurred vision. red conjunctiva, midrange pupil, nonreactive" dx test tx

Acute closed angle glaucoma tonometry mannitol, acetazolamde, timolol, pilocarpine

Sudden orbital or eye pain in the face of nausea and vomitting, esp after an antichokinergic

Acute glaucoma

Polyneuropathy in multiple myeloma

Chronic distal symmetrical sensory or sensorimotor neuropathy

inferior olivary nucleus

Climbing fibers cross over and go to cerebellum. More active with novel activity.

Agents for RLS

Clonazepam, gabapentin, L-dopa, opiates

2nd generation antipsychotic with risk of agranulocytosis? higher risk of prolactin level? (amennorha, galactorrhea, gynecomastia) weight gain? QT prolongation? 2nd generation antipsychotics ... mechanism?!?

Clozapine (due to this, reservered for people who have failed atleast 2 antipyschotics!!) Risperidone Olanzapine. Ziprisadone! Seratonin 2A and Dopamine D2 antagonists.

high frequency unilateral headache with lacrimation, runny nose

Cluster Headache Tx: Triptan- abortive verapamil, lithium- prophylactic

Lennox-Gaustaut syndrome

Combination of retardation and slow spike and wave discharges on ECG, many children have history of infantile spasms

Sxs of cerebellar dysfunction.

Common among chronic alcohol abusers - gait instability - truncal ataxia - *ipsilateral * - difficulty with rapid alternating movements - hypotonia - intentional tremor

Basilar migraine symptoms

Complete blindness, frank psychosis, transient quadriplegia, stupor, syncope, coma

automatisms ((chewing, picking movements of hands, lip smack)) common of?

Complex Partial Seizures.

osmotic demyelination syndrome

Complication of aggressive correction of HYPOnatremia. Axonal demyelination of pontine white matter. "From low to high, your pons will die."

impaired repetition

Conduction Aphasia- arcuate fasciculus (white connection b/w Broca and Wernicke)

Risk of high load of phenytoin

Conduction block or cardiac arrhythmia

Rx BPPV

Confirm w Dix Hallpike 80% resolve w Epley manuever Can also give Meclizine

Sturge-Weber syndrome

Congenital disturbance that produces facial cutaneous angiomas with the distinctive and easily recognizable appearance along with intracranial abnormalities like leptomeningeal angiomas

Intractable temporal lobe seizures?

Consider anterior temporal lobectomy.

A typical facial pain

Constant deep usually bilateral, sensitive to antidepressant medication

Things to r/o with coma

Constriction intact - midbrain is okay (CN 3 intact) Anisocoria = asymmetric pupil size (might be uncal herniation, or medications/eye drops) Breathing on your own - brainstem is intact Oculochephalic Reflex (if C-spine okay) - look for ocular movt opp to cephalic/head movt. = brainstem intact Isoelectric EEG - flat line

Nucleus ambiguus

Contains the motor neurons that contribute to the ninth and 10th cranial nerves

The auditory pathway is primarily _____.

Contralateral (bilateral but majority contralateral)

Transection of Meyer's loop causes:

Contralateral upper quadrantanopia Meyer's loop = Pie in the Sky

ACA damage

C/L motor sensory loss in LE Cognitive/personality changes Abulia (lack of will, initiative)

Sympathetic innervation (of body, excluding eye) from spinal cord

C8-L3 (interomediolateral nucleus of lateral horn)

Contraindications for triptans

CAD, htn, hemiplegic or blindness as aura for their migraine

helpful labs for neuropathies

CBC, A1c, kidney, thyroid, serum/urine electrophoresis, CSR, CRP, ANA, Rh, B12. if none of those show anything then you can run second line labs: porphobilinogen, Pb, As, Hg, HepB, ANCA, CXR, CSF protein, CSF pleocytosis, HIV, anti-Hu, anti GM1, anti MAG (autoimmune neuropathy), EMG, NCS.

dementia, myoclonus, sharp tri-phasic discharges on EEG

CJD prion disease RAPIDLY PROGRESSIVE spongiform encephalopathy

6 month old, bilateral hearing loss, no other abnormalities - etiology

CMV

Bug associated with retinal findings of hemmorhages and fluffy or granular lesions/opicafication are retinal vessels that occur in the context of NO PAIN OCCURS WHEN CD4 count <50!!

CMV retinits

Spinal Cord

CN 11

Berry aneurysm, CN involvement?

CN 3 w pupillary changes

Midbrain

CN 3,4. Red nucleus

Pons

CN 5,6,7,8. Acoustic Neuroma sits at Cerebello-pontine angle

Medulla

CN 9,10,12

Consensual Pupillary Reflex Pathway

CN II, Edinger Westphal Nuclei (CN III nucleus), CN III, Ciliary Ganglion, pupilloconstrictor

Diabetic mononeuropathy often involved CN ___. Nerve damage is often ___, and only somatic nerve fibers are affected.

CN III - Ischemic - Parasympathetic fibers retain function - ptosis + down and out gaze

CNs with PARAsympathetic components?

CN III - ciliary ganglion CN VII - ptergopalatine and submandibular CN IX - otic CN X - terminal (intramural) ganglia

pt found by neighbor, winter, not seen for awhile, refurbishing new home. comatose, nl vitals, PERRLA, + babinski, Edema on ct.Dx?

CO poisoning

Caloric stimulation of horizontal semicircular duct

COWS Cold-Opposite Warm-Same (Warm water in left ear makes the brain sense head turning left. This results in LEFT nystagmus.)

caloric testing

COWS. Nystagmus is named for the direction of FAST movement.

Common cause of recurrent meningitis

CSF leak, usually through the nose or ear

CSF in Guillain-Barre syndrome.

CSF shows high protein with normal WBC (albuminocytologic dissociation) INC permeability blood-nerve-barrier Tx: IVIG or plasmapheresis

Screening test if you dx MG

CT chest for thymoma

Once confirmed the dx of myasthenia gravis what test should be ordered next?

CT chest look for thymoma if pt is younger than 60

TIA + fundus w/cholesterol deposits

CTA, MRA, carotid duplex, angiogram carotid endarterectomy (CEA) if pt is symptomatic then beneficial for pts w/ >70% stenosis antiplatelet meds too

Post-ictal state is seen in which seizures?

Can be seen in all! Even simple partial - even w/out LOC. Ex - Todd's paralysis can be seen. Resolves within 24 hrs - often confused w stroke.

Complex Partial vs Absence (aka Petit Mal)

Can look VERY similar. BUT Absence will disappear by adulthood whereas complex partial will NOT.

Pseudodementia pt presentation

Can look a lot like dementia, but also has depression sx. Treat depression and MMSE score should return to baseline.

B/L spread or limited to one area - neonatal head trauma

Caput Succ (2 words = B/L = both areas) Cephalohematoma (1 word + U/L)

Drug of choice for complex partial seizure without secondary generalization

Carbamazepine

Treatment for Trigeminal Neuralgia

Carbamazepine

Treatment for trigeminal neuralgia

Carbamazepine, phenytoin, gabapentin

Embolic Stroke

Carotid stenosis Mural thrombus in LA Paradoxical emboli *Need ECG & ECHO

hemiballismus

Caused by damage to subthalamic nucleus (possibly due to PCA lesion). Seen in Huntington's. Contralateral.

4 common lacunar syndromes.

Caused primarily by HTN.

Mucurmycosis

Cavernous sinus syndrome, preceding sinusitis + poorly controlled DM are clues it can infect arteries and cause infarction tx: surgical debridement, CT to assess tissue involvement

fever b/l eyelid edema and ocular movement restrictions fever forehead tender to light palpation and HA

Cavernous sinus thromobosis. no valves so skin infection extends into it dx. mr venoagraphy tx broad spectrum abx

Injury seen in elderly due to forced hyperextension causing burning pain and paralysis in upper extremities with relative sparing of lower extremities?

Central Cord Syndrome.

Retinal hemorrhage

Central Retinal V. Occlusion & Wet AMD Wet AMD (rapid) - also has subretinal fluid.

Ataxia, dysmetria, gait disorder, tremor increases as hand reaches target

Cerebellar Tremor

Location of medulloblastoma

Cerebellum

Multiple ring enhancing lesions that tend to occur in B. Ganglia and Cortical Grey-White Matter Interface?

Cerebral Toxo!

Most common cause of spontaneous lobar (e.g. parietal, occipital) hemorrhage

Cerebral amyloid angiopathy -B-amyloid deposition in the walls of small to medium size cerebral arteries and associated w/ Alzheimer dementia

contralateral weakness with UMN, aphasia or neglect

Cerebral lesion Brainstem- CN findings

Chronic ganulomatous inflammation of meibomian gland? Presents as hard, painless lid nodule Treatment?

Chalazion Incision and Curettage

ENG

Characterizes nystagmus and disturbances of eye movements that involve relatively fast I movements

Echinococcosis vs cysteicercosis

Children more likely to have echinocococis and the cycs have major cysts with multiple compartments

Conus Medullaris lesion vs Cauda Equina Syndrome

Conus Medullaris - where the spinal cord terminated (~L1-L2) --> part of SC still so see UMN and LMN lesions. - symmetric, hyperreflexia, EARLY onset bowel/bladder dysfxn. Usually no motor/sensory loss though. vs. Cauda Equina Syndrome - compression of spinal nerve roots below conus medullaris (L5-S1). Part of PNS (peripheral nerves) - so LMN sx only. - asymmetric, hyporeflexia, LATE onset bowel/bladder dysfxn - motor and sensory loss

Acute angle closure glaucoma presentation

Eye pain, redness, dilated pupil with poor response to light

Cold water irrigation in an unconscious person with brainstem intact

Eyes move towards water (no nystagmus)

holoprosencephaly

Failure of the left and right hemispheres to separate. SH problem? Associated with FAS. Accompanied by craniofacial abnormalities.

fat embolization

Fat embolization to the lung following MVA with multiple fractures of long bones. Frequently see fat and hematopoietic cells in embolus.Widespread petechiae in the cerebral white matter.

Hypercalcemia presentation

Fatigability, lethargy, generalized weakness, and areflexia

Sex that is more susceptible to symptomatic aneurysms after 40

Females

Acute otitis media Otitis Media w/ effusion chronic suppartive otitis media

Fever, Ear Pain, Bulging of Tympanic Membrane NO FEVER, bulging of tymp membrane but persistent effusion NO fever, bulging of tympanic membrane .. but because of chronice loss can lead to hearing loss, tymp memb perforation, and otorrhea >6 weeks

When can LP be falsely negative for xanthochromia?

First 6-12 hours and after 24-48 hours (Basically ideal window is 12-24 hrs)

Days from alcohol where alcohol withdrawal seizure is greatest risk

First day after drinking cessation

Why subacute subdural hematoma is not seen on CT

Fluid collection too small to produce substantial deformation of the underlying hemisphere

Meigs' syndrome

Focal dystonia characterized by blepharospasm, forceful jaw opening, id protraction , neck contractions, tongue thrusting

Jacksonian March

Focal seizure activity that is primarily motor and spreads

Mycotic aneurysm

Form over cerebral convexity with subacute bacterial endocarditis, from infected embolus lodging in the arterial wall

progressive ataxia, arms >legs, severe dysarthria, childhood

Friedreich Ataxia- AR

Renshaw cells

From neural crest cells, inhibit alpha motor neuron with GLYCINE.

Causes of paradoxical emboli

From venous system --> arterial system (not arterial to arterial) Need AVF, ASD, or PFO

Brain Lobes

Frontal lobe - movement, speech Parietal lobe - dominant (R) neglect; non-dom (L) aphasia Occipital - visual changes Temporal - understanding language, ST memory

Early personality changes, apathy, disinhibition, and compulsive behavior

Frontotemporal dementia

Early personality changes Apathy, disinhibition & compulsive behavior Which dementia?

Frontotemporal dementia: mental rigidity. lack of insight speech/behavioral changes short term memory is retained in FTD: so may remember the 3 word quiz they have pacify of speech so they wouldn't be able to list more than 10 animals or words that begin with F

"3 day hx of leg weakness & shooting pains, diff rising from chair, climbing stairs. decreased strength and DTR." thoughts?

GBS-LMN, acute onset

MCC contact-lens assoc Keratitis

Gram neg - usu Pseudomonas Medical emergency. *(but can also be due to gram +, fungi, etc)

Physical exam of pseudotumor cerebri

HA, blurry vision, papilledema, vision loss, and CN palsies (VI most common)

MC symptom of brain abscess

HA, develops within a few weeks

waxing and waning lucidity, difficulty with complex tasks, memory loss, apathy, IV drug use

HIV dementia

Primary CNS Lymphoma

HIV or immunocompromised pt progressive hemiparesis + HA that is worse lying down MRI-single enhancing periventricular lesion CSF + for EBV

Progressive Multifocal Leukoencephalopathy

HIV pt + insidious onset of focal signs/symptoms and mutiple areas of demylination on MRI that do not enhance or produce mass effect def: CSF JC virus PCR tx: HAART

Bug associated with severe, acute retinal necrxosis associated with pain, keratitis, uveitis, and peripheral pale lesions with central necrosis?

HSV VZV in HIV patients.

What is the most important risk factor for stroke?

HTN 4x the risk of stroke

hemorrhagic stroke

HTN risk. Basal ganglia commonly affected site.

stroke risk factors

HTN, Diabetes, Obesity, Hyperlipidemia, sedentary, smoking, cardiac disease, heavy alcohol use

Common AE seen early after starting Ldopa/Carbidopa

Hallucinations! (esp w older pts) After several years - start seeing EPS involuntary movts.

Drugs for treatment of Tourette's syndrome

Haloperidol, pimozide, trifluoperazine, and fluphenazine

Knee Flexion

Hamstrings Sciatic L5, S1, S2

Drug of choice for seizure prophylaxis in the hemorrhage

Phenytoin, Lamotrigene gene poor choice because it is slowly titrated over many weeks

What should you do for a patient with mild TBI? What qualifies as mild tBI?

Head CT w/out contrast or observe for 4-6 hours. GCS 13-15, loss of conciousness <5 minutes, headaches, loss of memory before/after injury for <24 hours, alternal in mental status at time of ijury

Vestibular nystagmus: fast movement is in the direction of ____.

Head movement (slow movement is opposite of head movement for compensation)

Cognitive manifestations of hypothyroidism

Headache, dementia, psychosis, and decreased consciousness

Low amplitude, acute onset w/ increased sympathetic activity such as drugs, hyperthryoidism, anxiety, cafeeine. Worse w/ movement and can involve face and extremities

Physiologic tremor

Talk to me about how to treat acute angle closure glaucoma

Pilocarpine- Open up Schlemm Canal Timolol, Acetazolamide- Decrease acqeous humor production

Tumors that can cause Parinaud syndrome

Pineocytoma's, germ cell tumors, teratomas , and gliomas

Tau protein seen with...

Intracellular. Neurofibrillary tangles. 1. Alzheimers Dz 2. Frontotemporal (Picks Dz) 3. CJD + white matter changes

Rx M. Gravis Crisis

Intubate if needed. Plasmapheresis or IVIG + Glucocorticoids *LT treatment = pyridostigmine +/- Immunotherapy [mycophenylate mofetitil, cyclosporine, azathioprine] **Crisis can be provoked by infection (i.e. URI) *** Pyridostigmine increases secretions, so hold in intubated pt so pt does not aspirate.

Shy-Drager syndrome

Involves preganglionic sympathetic neurons from the inetermediolateral cell column, characterized by orthostatic hypotention, anhidrosis, impotence, bladder atonicity

Restless Leg Syndrome

Irresistible urge to move legs that is exac by rest & at night (better w movement/daytime). + Daytime Sleepiness. Diagnosis of exclusion. R/O - Fe def anemia, DM, Uremia, ETOH use, MS, preggos, Parkinsons, etc. Rx - heating pads, massage, exercise. Sleep enough (less sleep can aggrevate). DA Agonists - Pramipexole Clonazepam Gabapentin

Convergence retraction nystagmus

Isaac Trichitt jerk back into the orbit unattended upgaze

Cocaine & Stroke.. which kind?

Ischemic AND Hemorrhagic

rhythmic twitching that spreads along body parts following the homunculus

Jacksonian March

Familial dysautonomia (Riley Day syndrome)

Jewish children. Autosomal recessive. Excess sweating, unstable pressure (orthostatic hypotension), difficulty feeding bc inadequate muscle tone in GI tract, progressive sensory loss. Due to loss of neurons in the autonomic and sensory ganglia.

Blood pressure guidelines for SAH

Keep < 150

mossy fibers

Major afferent input to CEREBELLUM from spinal cord, pontine nuclei, vestibular apparatus, trigeminal nuclei, reticular formation.

Pinealoma

Mass of the pineal gland (responsible for Melatonin secretion). Obstructive hydrocephalus (CSF blocked in aqueduct of Sylvius) --> HA / vomiting. + Accommodation; - Reaction Perinaud's sign - limited vertical gaze (pressure on pretectal region of midbrain) Collier's sign - eyelid retraction

Rinne test

Mastoid process (bone conduction), then next to ear (air conduction). CANNOT hear air conduction = conduction problem. CAN hear air conduction (a positive Rinne) = sensorineural problem (if indicated by Weber test, otherwise this is normal)

Basilar artery lesion

Medial inferior pontine syndrome. "Locked-in syndrome" with preserved consciousness and blinking.

Depolarization of hair cells in left ear (turning head left) activates:

Medial rectus (CN III) of left eye Lateral rectus (CN VI) of right eye To move eyes to the RIGHT. This is called the Vestibuloocular Reflex (VOR)

patient decline in past few months, now unable to walk or care for herself, MRI shows multiple small infractions of both cortices, on aspirin, hx of MI - additional anticoagulation med?

NO. Don't add anticoagulation meds even though she can't walk [maybe bcuz she had strokes before?]

vagal motor nucleus

NOT a motor nucleus--PSNS for CN X to organs

inattention to the left side

Neglect right frontal or parietal lobes w/ extinction

medulloblastoma

Neuroectodermal tumor. Common posterior fossa tumor in children. On midline of cerebellum.

Hypothalamic car Matoma presentation

Neuroendocrine symptoms; precocious puberty or acromegaly; cause gelastic seizures which are peroxysms of laughter

Cafe-au-lait spots, macrocephaly, feeding problems, short stature, feeding disabilities

Neurofibromatosis Type 1

musclular ridgitity, fever, autonomic lability, altered LOC, elevated CK, leukocytosis

Neuroleptic Malignant Syndrome (Drug- Dopamine blocking induced- Haloperidol/atypicals) Tx- removal, hydration, Bromocriptine or Dantrolene

Meds that cause AE of Parkinson Sx

Neuroleptic Meds (Chlorpromazine, Haloperidol) Metoclopramide (hits DA) Reserpine (anti-psychotic)

fluctuating proximal muscle weakness

Neuromucular Junction Disorder repetitive stim EMG/NCS Myasthenia Gravis, Lambert-Eaton

Conus medullaris level in newborn vs adult

Newborn - L3 Adult - L1

Dopamine Pathways

Nigrostriatal- coordination of movement; tics, Mesolimbic- euphoric; hallucinations Tuberoinfundibular- inhibit ant pituitary prolactin secretion; gynecomastia w/ antipsychotics

Drug use to prevent vasospasm after a bleed

Nimodipine

Treatment of tardive dyskinesia.

No definite treatment but clozapine might help!!

Cold water irrigation in an unconscious person with damage to the brainstem (damage to vestibular nuclei)

No eye movement with irrigation

1st time seizure, Rx?

None usually, as long as workup is negative.

Ataxia early in the disease Urinary incontinence Dilated ventricles on neuroimaging

Normal pressure hydrocephalus

Ataxia early in disease, urinary incontinence, dilated ventricles on neuroimaging,

Normal-pressure hydrocephalus

Rhine test

Normally AC>BC--will be normal with sensorineural loss. Abnormal (BC>AC) with conductive loss.

secondary vesicles

Note that the brainstem comes from 3 parts: mesencephalon (mid), metencephalon (pons), and mylencephalon (medulla)

stages of sleep

Notes: -REM exhibits beta waves just like awake relaxed state. -Body temp decreases in stage 2 -Sleepwalking, bedwetting occur in Stage 3 (delta) -REM paralysis, inc O2 use, varying HR & BP -(Remember: BATS Drink Blood at night.)

Direction of nystagmus when warm waters put into the ear canal

Nystagmus toward the ear that is stimulated, eyes deviate slowly away from the ear that is stimulated

Pickwinian syndrome

Obesity associated with hypersomnia; sleeping attacks

T or F, focal seizures are not provoked by hyperventilation? Focal seizures with impairment of concioiusness ... what can you say? How can you differ from abscence seizure?

T Becomes 2ndary generalizes and can be accompanied by automatisms Abscence seizure does not have post-ictal period!!!

Type of it MRI to view demyelination

T-2 weighted

T4 T10

T4 - nipples T10 - umbilicus

T or F CT with contrast/MRI is more useful than MRI without contrast for identifying structural abnormalities massed (masses, mets, abcess) than w/o contrast?

TRUE

T or F patients with MG have normal muscle tone, reflexes, and autonomic f(xn)

TRUE!

Sensory ataxia Lancinating pains Neurogenic urinary incontinence Associated with Argyll Robertson pupil.

Tabes dorsalis - INC incidence syphilis in men who have sex with men & HIV infected patients - Treponema pallidum spirochetes directly damage the dorsal sensory roots

Sensory ataxia, lancinating pains, reduced/absent deep tendon reflexes and Argyll Robertson pupils (normal pupillary constriction w/ accommodation but not with light). Dx and Tx:

Tabes dorsalis from Syphillis infection. Tx: IV Penicillin for 10-14 days.

Heat stroke definition

Temp>40 and AMS

new onset headache >50 yo, w/ tender scalp, claudication of jaw

Temporal Arteritis- granulomatous arteritis ESR, C Reactive Protein, Biopsy Visual loss Tx- steroids

41 yo periods of blank stare then drags leg and confused after

Temporal lobe epilepsy

recurrent holocranial squeezing/tightness headaches w/ neck muscle spasm

Tension Headache

Most common site for meningiomas

Tentorium cerebelli

How to interpret red glass test

The eye is not moving with a red image appears to be

Artery infarct that produces Wallenburg syndrome

Occlusion of the vertebral artery, PICA also possible

Herpes Simplex Keratitis

Occular irritation, photophobia +/- pain Dendritic ulcer on CORNEA; can cause irreversible vision loss. Rx - topical antiviral eye drops

Encephalitis + White,Fluffy lesions surrounded by retinal edema + vitritis

Ocular Toxo

Keratoconjunctivitis

Ocular allergy (~ atopic conjunctivitis but SEVERE/prolonged). - itching, tearing, mucus discharge, photophobia, blurred vision. - kerato = includes cornea (thickening of eyelids/surrounding skin can occur)

Irritative lesion of the uncus can cause

Olfactory and gustatory hallucinations

CN II

Only CN myelinated by oligodendrocytes. Part of the CNS (diencephalon) and ergo does not regenerate if damaged.

Cold water irrigation in an unconscious person with damage to MLF

Only the eye on the same side of irrigation abducts towards the irrigated ear

Optic disk edema

Optic Neuritis

Junction scotoma occurs when

Optic nerve is cut close to the chiasm: Ipsilateral blindness and junction scotoma, which = contralateral upper temporal quadrant defect

Tumors in an NF1

Optic nerve tumors, cutaneous and subcutaneous tumors

Initial tx for myasthenia gravis

Oral anticholinesterase (pyridostigmine)

Postural changes in heart rate/blood pressure after standing suddenly

Orthostatic syncope

Anterior Uveitis

PAIN, miosis, photophobia (sensit to light) + redness, tearing, blurring - Seroneg. arthritis (all 3) - Behcet's - IBD - Juv Idiopathic Arthritis - Sarcoidosis Slit lamp exam: flare and cells

Erythema Nodosum

PAINFUL, red, elevated, subcut nodules - usu on Anterior aspect of tibia. Self-limited MCC - GAS infxn (get ASO titer) IBD Sarcoidosis (get CXR) / TB Fungal - Histoplasmosis Behcet's Syphilis (do VDRL)

Posterior Uveitis

PAINLESS floaters decreased visual acuity (or asymptomatic) - HSV - TB, Sarcoidosis - Toxo

Weber syndrome

PCA and Circle of Willis lesion in medial midbrain. Involves CNIII (eye looks down and out) and CSTr. (Remember: A Weber grill is circular...this lesion is in the Circle of Willis)

Benedickt syndrome

PCA lesion in paramedial midbrain. Involves CNIII, ML, and red nucleus (causing involuntary mvmts). The red nucleus involvement distinguishes this from Weber.

ipsi ptosis, pupil diation, opthalmoplgia

PCOM aneurysm

What is the gold standard in dx herpes encephalitis?

PCR analysis of HSV DNA in CSF (highly sensitive and specific)

Vestibular neuritis looks like which arterial lesions

PICA aka Lateral Medullary aka Wallenberg Basically post viral infxn, see Labrythitis WITHOUT auditory changes.

Amourosis Fugax

Painless U/L curtain down over, TEMPORARY loss of vision - can be from emboli (KEY SIGN OF stroke/TIA) - look for hx of atherosclerosis OR - from retinal detachment Do Carotid U/S & endartectomy if needed. Get cardiac workup (lipid panel, EKG, etc)

What is amaurosis fugax?

Painless loss of vision from emboli.

What extremities does glucocorticoid-induced myopathy typically effect?

Painless proximal muscles weakness distal > proximal extremities (lower extremities)

Sensory distribution of the median nerve

Palmar surface of the thumb and 1st 4 digits

Enlarged blind spots, caused by?

Papilledema. Sx: INTERMITTENT vision loss w/ changes in head movement due to high ICP Ex: Idiopathic Intracranial HTN (Pseudotumor cerebri). *can lead to permanent vision loss!

daily frontal headaches, pain begins as dull ache in morning and gradually worsens, exacerbated by loud noise or stress, relieved by acetaminophen or sleep - diagnosis

- tension headache

ulnar nerve compression

- tingling in ring and small finger - weakness in interosseous, hypothenar, and flexor digitorum profundus or ring and small fingers

74 yo woman presents 1 hour after sudden onset partial blindness, low-grade fever, occipital headaches, pain in shoulders pelvic area and joints, morning stiffness for 2 months, generalized muscle tendrness, right central retinal artery occlusion - diagnosis

- tmeporal arteritis (GCA)

Features of midbrain stroke?

-> Ipsilateral Oculomotor Nerve Palsy -> Ataxia (Damage to Superior Cerebellar Peduncle) -> Contralateral Hemiparesis

anterior cord syndrome

-Cause: damage to anterior spinal a. -Also bilateral spacticity

Charcot-Marie-Tooth disease

-Cause: hereditary (most common) -Damage to dorsal columns and LMN in SC gray matter. -Marked by progressive weakness, primarily fibular and distal leg muscles

Friedreich ataxia

-Cause: inherited disease affecting dorsal columns, spinal cerebellar, and corticospinal -Loss of Purkinje cells and neurons in dentate nuc. -Sx: ataxia, Babinski sign, loss of DTR in legs, pes cavus, staggering gait, kyphoscoliosis "Friedrich is a frat brother (frataxin iron binding protein prob)--always staggering and falling but with a sweet (DM risk), big heart (cardiomyopathy)."

syringomyelia

-Damage to anterior SC white commisure, some MNs. -Bilateral loss of pain and temp and motor weakness

macular degeneration

-Dry: majority--gradual vision loss. Yellowish drusen. -Wet: minority--rapid vision loss Sign is central scotoma.

cerebral cortex

-FEF damage: transient paralysis of voluntary gaze to contralateral side (eyes look toward lesion) -Broca is in frontal lobe -Wernike in temporal lobe

hypothalamic nuclei

-Lateral: hunger (zap it and shrink laterally) -Ventromedial: satiety (zap it and grow ventrally and medially...also rage) -Anterior: cooling, PSNS (A/C) -Posterior: heating, SNS -Supraoptic/paraventricular: ADH/oxytocin release -Arcuate: releasing hormones

T or F: Patients with Polymyalgia Rheumatic have 1) Normal muscle strength 2) Elevated ESR? 3) Elevated CK? 4) Improves with glucocorticoids

1) True! 2) True! 3) False! Normal CK 4) True!

Tx for stress incontinence

1) alpha-agonists: pseudoephedrine; midodrine 2) estrogen 3) duloxetine

treatment for hypersensitive bladder/spastic bladder

1) anticholinergics--tolterodine, oxybutinin, trospium (does not cross BBB), solifenacin/darifenacin 2) TCAs 3) desmopressin (if nocturia) 4) intravessicular capsacin 5) b-3 agonists- *acetanilide.*

atonic bladder treatement

1) credes or valsalva maneuver 2) intermittent self-cath 3) bethanechol seldom effective

Detrussor-sphincter dyssynergia treatment

1) intermittant cath 2) suprapubic cath 3) sacral nerve stimulation 4) alpha-1 blocker (doxazosin)

Ways to detect PKU 1) early in life 2) later in life

1) newborn screening tandem mass spectrometry 2) quantitative amino acid analysis

What are different ways we can reduce ICP?

1. Increased venous outflow (Elevate Head) 2. Reduce Volume (LP) 3. Vasoconstrict (Hyperventilate -> reduce PCo2) 4. Reduce water content of parenchyma (mannitol) 5. REduce metabolic demand (sedation)

What meds can cause IIP?

1. Isoretinonin 2. GH 3. Tetracylcines

MS, what tests do you order?

1. MRI w gadolinium 2. LP - CSF w normal protein but high IgG 3. Evoked potentials - newly re-myelinated nerves will conduct more slowly.

First line treatment for narcolepsy? What drugs should be avoided?

1. Modafinil (Provigil, Nuvigil) Drugs that cause more daytime sleepiness such as benzos, opiates, antipsychotics, and alcohol.

Central vs Peripheral Vertigo

1. Nystagmus - bidirectional in central - only vertical in peripheral 2. *Both pts fall towards the side of lesion. 3. Tinnitus, hearing loss only in peripheral 4. Refractoriness only present in peripheral (tilt test, ex) 5. Brainstem sx / focal neur. deficits only in central

Temporal Lobe Epilepsy

1. Olfactory hallucinations as aura (not always) 2. Temporal Lobe - responsible for short term memory, so if Complex Partial (staring, altered speech, automatic movts) or Generalized Tonic-Clonic - might not rmbr.

Conductive hearing loss

1. Otitis Externa or media 2. Cholesteatoma- erosive and expansile growth consisting of keratininzing squamous epithelium. destruction of ossicles. Otoscopic exam shows white plaque on tympanic membrane 3. Trauma 4. Cerumen 5. Tympanic Membrane perforation

Types of Seizures

1. Partial --> Simple Partial or Complex Partial 2. Generalized --> Tonic Clonic or Absence

Lacunar stroke, types

1. Posterior limb of IC - pure C/L motor deficit 2. VPL of Thalamus - pure C/L sensory deficit 3. Anterior limb of IC - ataxic-hemiparesis 4. Pons - Clumsy hand dysarthria *remember, subcortical structures!! MCC of lacunar stroke = HTN

Sensorineural hearing loss

1. Presbycusis 2. Meniere- excessive endolypmh in membranous labyrinth 3. Barotrauma 4. Acoustic Neuroma(Schwann cells covering vestibular branch of 8th cranial nerve, tumor grows and causes vertigo, unilateral tinnitus as well) 5. Cerebrovascular ischemia 6. Drugs- damage to cochlear hair cells

Sensorineural Hearing Loss

1. Presbycusis (gradual, symm loss w aging - high frequency goes first) 2. Noise-induced (chronic exposure to db > 85) 3. Infxn (labrynthitis) 4. Drug induced (AG, Ab, Cisplatin, Quinidine, Furosemide) 5. Congenital TORCH 6. Meniere's (Rx - restrict salt; Meclizine) 7. CNS cause (acoustic neuroma, meningitis, MS, syphilis)

Rx M Gravis

1. Pyridostigmine 2. Corticosteroids if needed 3. Resp failure in Acute exacerb --> plasmapheresis, IVIG, intubation

Mononeuritis Multiplex

2 or more nerves are damaged in completely different parts of the body - usu seen w Vasculitis

Subacute Combined Degeneration

2/2 Vit. B12 dorsal column + corticospinal/spinocerebellar tract dysfunctionin and a stocking-glove neuropathy dx: check serum B12 tx: IM B12

Akathisia

2/2 adverse rxn to haloperidol or typcial antipsychotic restlessness-stop offending agent, give atypical antipsychotic

Tardive Dyskinesia

2/2 chronic neuroleptic use, takes months to years after drug usechoreiform movements of mouth/face mainly remove haldol and use atypical antipsychotic

Central vertigo

2/2 embolic stroke in posterior circulation expect other cranial nerves to be involved along with CN VIII eg. stroke sx of dysarthria, diplopia etc nystagmus in all gaze directions dx: CT or tPA if within window, MRI brain tx: acute stroke tx, antihistamines and benzos for vertigo, vestibular rehabilitation

Meralgia paresthetica

2/2 entrapment of the lateral cutaneous nerve as it crosses the inguinal ligament in pts with rapid wt gain or obese pts or tight clothes or preggo for non pregoo: NSAIDS, drugs for neuropathic pain, or local corticosteroid injections

Acute dystonia

2/2 neuroleptic use pt is stuck in a position, stiff muscles tx: anticholinergic meds (benztropine or diphenhydramine)

Myasthenic Crisis

2/2 trigger like an infection evaluate vital capacity and negative inspiratory flow to see if significant neuromuscular respiratory weakness prior to significant drop in O2 + intubation start plasmaphoresis or IVIG

NF2 chromosome mutation

22

Tabes Dorsalis

3 Syphillis - dorsal columns damaged only

allocortex

3 layers, olfactory cortex and hippocampus

EEG changes w Absence seizure

3 per second spike and wave discharges. *Induced w hypERventilation.

Cluster headache

occur around the same time each day for wks/mo. lasts 15mins-3hrs, autonomic sx on ipsilateral face (tearing, runny nose, flushing, horner's syndrome) tx: 100% O2 via nonrebreather mask for 15 min prophylaxis-prednisone short term or verapamil for chronic

headache beings sudddenly and peaks within seconds

Thunderclap Headache Subarachnoid Hemorrhage CT and LP

Ankle Dorsiflexion

Tibialis Ant Deep Peroneal L4, L5

rapidly progressive ascending paralysis (Can be asymmetrical), absence of fever and sensory abnormalities, and normal CSF examination.

Tick-Borne paralysis- due to neurotoxin release- Ticks feed for 4-7 days and removal results in spontaneous improvement

postictal hemiparesis

Todd's paralysis- secondary generalized seizure

III (A-d) fibers

Touch, pressure, fast pain, temperature (lightly myelinated)

II (A-b) fibers

Touch, pressure, vibration

"woman w/aids, acting weird, hyperreflexia, w/multiple ring enhancing lesions" Dx? tx?

Toxo give sulfadiezene & pyrimethamine

Presents with follicular conjunctivitis and pannus(neovascularization) or cornea?

Trachoma (from Chlamydia Trachomatis)

impaired fluency

Transcortical Motor Aphasia Frontal, supplemental motor area, basal ganglia

impaired comprehension

Transcortical Sensory Aphasia Inf Temporal Lobe Left Posterior Cerebral Artery

Type of herniation from posterior fossa mass

Transtentorial or transforaminal

Elbow Extension

Triceps Radial C7 Triceps Reflex

short sudden electric pain in face triggered by touch

Trigeminal Neuralgia MRI Tx Carbamazepine

Delirium Tremens

occurs 3-7d after pt's last drink autonomic instability, impaired mental function, agitation, sympathetic sx tx: benzos

Meds used for Parkinson's disease that can lead to anticholinergic excess.

Trihexyphenidyl, Benztropine - flushing, anhidrosis/dry mouth, hyperthermia, mydriasis/vision changes, delirium/confusion, urinary retention/constipation

Medication to decrease parkinsonism from neuroleptic use

Trihexyphenidyl, anticholinergic

T or F, Guillan Barre involves peripheral nerves, cranial nerves, and nerve roots.

True!!1

Key diff between Tuberous Sclerosis and Sturge Weber

Tub Sclerosis - get adenoma sebaceum & ash leaf spots as cutaneous abnormality Sturge weber - port wine stain, nevus flemmus.

Neurocutaneous D/O

Tuberous Sclerosis (AD) NF1, 2 (AD) Sturge Weber (Sporadic) VHL (AD) Ataxia Telengectasia (AR)

Weber test

Tuning fork on top of skull. Louder in one ear = conduction problem in that ear or sensorineural problem in normal ear

Acute agitation treatment in the elderly

Typical and atypical antipsychotics, a.k.a. haloperidol and quetiapine

Herpes Zoster Opthalmicus

Tzanck smear or viral culture/PCR of vesicular fluid slit lamp exam, corneal exam with flurosceine to look for herpetic lesions on cornea tx: oral antivirals

Lacunar Infarct aka POST limb of Int Capsule damage

U/L motor deficit (face and arms) *no sensory loss or visual changes. NOTE - Intracerebral haemmorhage is caused due to ,stress imposed on the vessels by HTN.branches of lenticulostriate arteries which supply the internalcapsule,develop macroaneurysms called charcot bouchard aneurysms,ruptur e of these aneurysms produce intracerebral haemorrhage

Initial workoup of delirium

UA and serum electrolytes before imaging

ALS

UMN & LMN But no sensory deficits!!!

Amyotrophic Lateral Sclerosis

UMN and LMN signs + progressive weakness with EMG confirmation of at least 3/4 levels of neuraxis with motor denervation 3/4-bulbar, cervical, thoracic, and lumbrosacral tx: riluzole

When a patient has pronator drift, this finding is relatively sensitive and specific for ___ disease. Romberg? Rapid alt movements?

UMN disease. pyramidal/CST - weakness in supination causing the pronator muscles to be dominant Romberg: proprioception Rapid Alt movements: Cerebellar

Facial N palsy

UMN lesion --> corticobulbar tract. C/L lower face paralysis LMN lesion --> facial nucleus. I/L upper and lower face paralysis + can't close eyelid, ant 2/3 taste, salivation, lacrimation, facial movt. "ALexander Bell w/ STD's" A - AIDS L - Lyme Dz Bell - Bell's Palsy S - Sarcoidosis T - Tumors D - Diabetes

can you used LMWH or UFH for stroke? What to do if history of afib?

Unfractionated hep or LMWH should be avoided in management of patients with acute stroke due to high risk of bleeding. However can use dabigatran or warfarin if history of afib!! (to anticoagulate)

Migraine usu lasts for how long?

Usu < 72 hours Rx - OTC, NSAIDs thennnn Triptans, Metoclopramide Prophylaxis - Gabapentin, Amitriptyline, Propanolol.

What vestibular structure has otoliths?

Utricle and saccule. They are in a gelatinous membrane that move hair cells that are oriented in many directions

MCC of neuropathy gnomonic

VITAMINS V-vitamin deficiency, vasculitis I-infection (TB, leprosy) T-toxic A-amyloid M-metabolic (EtOH, DM, porphyria, Hyperthyroid, liver, renal) I-idiopathic, inherited N-neoplasm S-systemic (SLE, PAN, multiple myeloma)

dorsal thalamic nuclei

VPL: spinothalamic-->somatosensory cortex (Remember: L transmits Limb info) VPM: trigeminal & gustatory--->somatosensory cortex (Remember: M for makeup, which goes on the face and MMMM for tastes good) VL: Basal ganglia, cerebellum --> motor cortex (Remember: L for moving your Limbs) LGN=light=CNII MGN=music=superior olive and inferior colliculus Anterior: mammillary bodies-->A-->cingulate Pulvinar: P, T, and O lobes to association cortex in P, T, and O lobes CM: cortex, SC, basal ganglia --> CM--> same stuff

Taste projects to what part of thalamus?

VPM

Stepwise decline Early executive dysfunction Cerebral infarction & or deep white matter changes on neuroimaging

Vascular dementia

Stepwise decline, early executive dysfunction, cerebral infarction and or deep white matter changes on neuroimaging

Vascular dementia

Syncope: Prolonged standing or emotional distress, painful stimuli. w/ prodromal symptoms of nausea, warmth, and diaphoresis

Vasovagal or neurally mediated syncope (Neurocardiogenic)

Vegetative state vs Coma

Vegetative state - eyes are open and might look awake; but don't meet criteria for brain death Coma - also unresponsive but completely unresponsive to external stimulus.

Satiety center in hypothalamus

Ventromedial nucleus (destruction = obesity, hyperphagia, savage behavior. Destruction of dorsomedial nucleus of thal also causes savage behavior)

Recommended prophylactic meds for cluster HA.

Verapamil Lithium Ergotamine

Alternate syndromes w/ contralateral hemiplegia and ipsilateral cranial nerve involvement, Possible ataxia

Vertebrobasilar system lesion (supplying the brain stem)

Usually due to viral syndrome Acute onset of single episode that can last days Severe vertigo but no hearing loss, patient falls down toward side of lesion Abnormal head thrust test.

Vestibular neuritis

Lateral medullary syndrome (PICA syndrome) affects:

Vestibular nuclei ICP NA CN IX roots Vagal nerve roots ALS DTT/N Descending sympathetic tract (Horner's)

Causes of chemotherapy-induced peripheral neuropathy (CIPN)?

Vinca alkaloid (vincristine) Platinum based meds (cisplatin) Texanes (paclitaxel) Weeks after tx, symmetrical paresthesias in stocking-glove pattern, loss ankle jerk reflex and loss P and T sensation

Inciting infections for GB

Viral - CMV, HIV, Hepatitis GI - Campy Influenza vaccination

Abnormal evoked response in MS

Visual evoked response

Why facial nerve lesion causes sensitivity to sound

Visual learner innervates the stapedius muscle of the middle ear, undamped transmission of acoustic signals across the bone produces hyperacusis

The best way to monitor respiratory function Guillian Barre syndrome

Vital capacity

Medication shown to delay progression of Alzheimer's disease

Vitamin E

Thrombotic vs Embolic vs Lacunar Stroke Presentation

Wake up from sleep w sx, can be acute or gradual --> Thrombotic Rapid, acute onset of sx. Max deficits initially --> Embolic

marchiafava-bignami syndrome

occurs due to effects of EtOH in CNS leading to frontal-type dementia, seizures, pyramidal signs, focal demyelination and necrosis of corpus callosum.

Manifestation of damage to the ulnar nerve at the cubital

Weakness in the interosseous and lumbrical muscles of the hand, causes claw deformity

Encephalopathy (confusion), Oculomotor dysfunction (bilateral abducens palsy, horizontal nystagmus, Gait ataxia (wide-based gait)

Wernicke encephalopathy Risk: Alcohol, Malnutrition, Hyperemesis gravidarum Tx: IV thiamine followed by glucose

impaired repetition and comprehension

Wernicke's Aphasia- language comprehension post part of Superior Temporal Gyrus MCA- inferior division w/ contra sup quadrantanopia

Foster-Kennedy syndrome is most commonly associated with:

olfactory groove meningioma

ipsilateral side gaze palsy, contralateral side INO

one and a half syndrome- damage to PPRF and MLF

rexed lamina 9

Where are somatic motor neurons located within the SC?

intracranial tuberculoma

one of more common brain lesions in developing world. presents w/ features expected for inflammatory mass lesion. can calcify and can lead to hydrocephalus. can look like tumor, abscess, or cystercercosis on imaging. could require neurosurgical resection.

hydroxyamphetamine

pharmacologic agent that distinguishes between preganglionic horner's and post-ganglionic. post will NOT dilate but pre will when these eyedrops are applied.

Amantadine

pharmacologic therapy for PD that is used early to treated bradykinesia, rigidity and gait disturbance.

geste antagoniste

phenomenon that dystonias can be diminished by sensory tricks like touch in affected body part. they can also be suppressed by relaxation and sleep and are exacerbated by stress and fatigue.

kids with midface hypoplasia, cleft lip/palate, digital hypoplasia, hirsuitism, developmental delay, hypoplasia of distal phalanges of toes and hands

phenytoin use in pregnancy

paraneoplastic cerebellar degeneration

acute or subacute pancerebelar syndrome w/ truncal, gait and limb ataxia and dysarthria and occular dysmetria and nystagumus. usually ass. w/ gyn or SCLC and can manifest before tumor is discovered. MRI normal, CSF may have increased protein or lymphocytic pleocytosis but usually normal. anti-Yo and anti-HU antibodies.

Central Pontine Myelinolysis "Locked in Syndrome"

acute quadriplegia in setting of brisk sodium correction tx: slowly correct sodium

vestibular neuritis

acute unilateral peripheral vestibulopathy. no real infammation. sudden spontenous onset of vertigo, nausea, vomiting. peaks in 24 hours and resolves over a few days-weeks. will have unilateral nystagmus.

West syndrome, generalized seizure disorder characterized by recurrent spasm treatment

adrenocorticotropic hormone

Adrenal dysfunction with progressive degeneration of white matter

adrenoleukodystrophy

MCC bug in brain abscesses

aerobic and anaerobic strep

hemiparetic gait

affected leg is stiff and circumducted during swing phase. increased adductor tone leads to scissoring of feet during walking.

jaw jerk reflex

afferrent: V3 sensory; efferent: V3 motor

Febrile seizure

age 6mo-6yro during acute febrile illness tx: tyelenol

Presbycussis

age related hearing loss, audiometry with clinical hx of gradually progressive bilateral hearing loss tx: hearing aid

Levitiracetam

agitation, neuropsychiatric distrubance

Wallenberg Syndrome

aka Lateral Medullary aka PICA vertigo nystagmus (horiz & vertical) I/L pain & temp in face C/L pain & temp in UE and LE I/L Horner's CN 9,10 involvement (Medulla): dysphagia, hoarseness (I/L vocal cord paralysis), diminished gag reflex.

Wernicke's encephalopathy

alcoholic + classic triad of confusion, ataxia, and oculomotor involvment (nystagmus, gaze palsy) tx: IV thiamine before glucose

apraclonidine

alpha 1 and 2 agonists that directly dilates affected eye in horners b/c works directly on dilator muscle.

Complex partial seizure

alteration in consciousness, automatisms (finger rubbing, lip smacking, chewing, or swallowing) and postictal confusion is present, longer duration of seizure activity tx: AED if multiple unprovoked seizures

50 year old female with cognitive and personality changes, neurodegeneration of fronto-temporal region

pick's disease

Medial Medullary Syndrome

alternating hypoglossal hemiplegia - occlusion of Ant Spinal A. C/L motor loss of arm and leg Tongue deviated TOWARDS lesion

MCC of lobar hemorrhage

amyloid angiopathy (no systemic amyloidosis) just the protein like in AD. beta amyloid. just one lobe partial or complete (example parietal lobe)

Refsum disease

an inherited condition that causes *vision loss, absence of the sense of smell (anosmia)*, and a variety of other signs and symptoms. The vision loss associated with this is caused by an eye disorder called retinitis pigmentosa. This disorder affects the retina, the light-sensitive layer at the back of the eye. Vision loss occurs as the light-sensing cells of the retina gradually deteriorate. The first sign of retinitis pigmentosa is usually a loss of night vision, which often becomes apparent in childhood. Over a period of years, the disease disrupts side (peripheral) vision and may eventually lead to blindness. Vision loss and anosmia are seen in almost everyone with this disease, but other signs and symptoms vary. About *one-third of affected individuals are born with bone abnormalities of the hands and feet*. Features that appear later in life can include *progressive muscle weakness and wasting; poor balance and coordination (ataxia); hearing loss; and dry, scaly skin (ichthyosis)*. Additionally, some people with this disease develop an abnormal heart rhythm (arrhythmia) and related heart problems that can be life-threatening.

epistaxis, a localized mass, and a bony erosion point towards what in an adolescent?

angiofibroma in nose.

contraindications to bupropion

anorexia/bulemia epilepsy - increased risk of seizure

bilarteral loss of pain and temperature and bialteral weakness - preservation of light touch and vibration

anterior cord syndrome usually 2/2 trauma or anterior spinal artery insult (off the vertebral artery)

antibodies found in myesthenia gravis

anti-nAChR, anti MUSK, anti LRP-4

anti mag antibodies (myelin associated glycoprotein)

antibodies that damage schwann cells that lead to muscle weakness, sensory problems, and other motor deficits usually starting in the form of a tremor of the hands or trouble walking. can be associated w/ amyloidosis.

trihexylphenidyl benztropine

anticholinergics used in PD to treat tremor predominant disease. can be used to treat wearing-off of L-dopa. SE: anticholinergic

treatment of acute migraine

antiemetics prochlorperazine, chlorpromazine, metoclopramide

oxcarbazepine

antiepileptic Na channel blocker used to treat partial seizures. SE: sedation, hyponatremia

zonisamide

antiepileptic that works through multiple mechanisms. used to treat partial and generalized seizures. SE: sedation, kidney stones, weight loss.

Basilar skull fracture

any hx of trauma + raccoon eyes, fluid dripping from nose (rhinorrhea), clear fluid dripping from ear (otorrhea) or ecchymosis behind the ear (battle's sign)

anterior ischemic optic neuropathy

anything that leads to ischemia of retina or optic disc. so included in this category is CRAO, TIA, temporal arteritis. associated w/ HTN, DM, hypotensive epidoseds, RAPD. will have unilateral segmental disc edema.

perisylvian aphasia

aphasia in which repetition is impaired.

wernicke's aphasia

aphasia that may present w/ contralateral homonomous superior quadrantinopia

transcortical aphasia

aphasias where repetition is spared.

delta waves

are EEG waves seen during deep sleep. < 4 hz

transverse myelitis

area of inflammatory demyelination in spinal cord. usually partial, just particular tracts at a given level. can be unilateral or bilateral, motor or sensory deficits. can present a similar clinical picture of transection of the spinal cord. develops over a longer period of time and eventually will have evidence of inflammation such as CSF pleocytosis

Guilian Barre Syndrome

areflexic ascending weakness/paralysis involving both distal and proximal muscles over course of hrs to days with antecedent respiratory or GI illness autoimmune acute inherited demyelinating polyneuropathy post exposure to infection/vaccination, molecular mimicry of self against myelin, c. jejuni associated pathogen LP: high protein, nml WBC NCS: acute demyelination +/- axonal damage tx: IVIG or plasmaphoresis

gardener w/ n/v ringing, rash 10 days, oval patches on neck, decreased sensation to pinprick, absent ankle reflexes, increased axillary pigmentation, nails-leukonychia striata"

arsenic poisoning

polymyositis

ass w/ Crohn's disease, vasculitis, myesthenia gravis. CD8 cells.

sxs of optic disc swelling

ass. w/ optic neuritis, anterior optic neuropathy, and increased ICP. most common is transient visual obscurations (dimming) lasting only a few seconds and usually precipitated by change in posture or valsalva. can also lead to pain and sudden vision loss.

carotid artery dissection

can lead to ischemic stroke. usually presents w/ severe retro-orbital headache, ipsilateral to lesion. usually involves anterior circulation territories and occur due to thrombosis of ICA or embolis that arises from dissection. can have ipsilateral horner's, perspiration. get vascular imaging if this is suspected. Tx: warfarin

nonketotic hyperglycemia

can lead to lethary and drowsiness and seizures of all kinds. can also have syndrome of dystonia and chorea w/ reversible T1 hyperintensity in basal ganglia. can also lead to cerebral edema.

lyme disease

can lead to meningitis, cranial nerve palsies, and polyradiculopathy. usually occurs several weeks after bite. starts w/ HA, stiff neck and myalgias and leads to frank signs of meningismus w/ cranial nerve palsies. in several months in can progress to polyradiculopathy (w/ pain), polyneuropathy, encephalopathy (white matter signal change) or combination. CSF might not be diagnostic until late. will have lymphocytic pleocytosis w/ normal glucose. PCR for spirochete. MRI can show patchy foci of signal change. EMG, NCS can be useful. might need IV abx in high doses, usually ceftriaxone.

broca's area/aphasia

can lead to subtle deficits in comprehension, especially w/ complex grammatical construction w/ preposition and passive voice (the lion was killed by the tiger). can be associated w/ facial weakness.

M gravis vs ALS

can look very similar b/c motor weakness shows up BUT MGravis has normal reflexes & no sensory loss. ALS - can get hyperreflexia or hypo (UMN & LMN).

Alcohol withdrawal seizures

can occur 12-48 hrs after alcohol cessation alcoholic, acute pancreatitis, tremulousness tx: benzos, CIWA protocol (determines dose of ativan PRN)

stiff-person syndrome

can occur in autoimmune paraneoplastic syndromes. stiffness of axial/trunk muscles and slow spread to proximal limbs. is fluctuating and progressive develop lumbar lordosis leading people to walk like tin man. will have painful muscles spasms and continuous motor unit activity > central problems. can have abs vs. GAD or amphiphysin. Tx: benzos and baclofen.

coagulopathic bleeding in heat stroke patients

can present w persistent epistaxis look for core temp > 40 C / 104 F

labrythine concussion

can result from head injury, irrespective of skull fracture. can lead to vertigo, hearing loss, tinnitus.

Pott disease

can spread from spine to epidural space leading to spinal cord compression. can spread through disk space to adjacent vertebral bodies (vs. metastatic cancer which will just be in bone).

horner syndrome in a patien with headache and recent head injury

carotid or vertebral artery dissection

low back pain radiating to right buttock and posterior thigh after lifting heavy weight, urinary incontinence, absent achilles reflex on right, decreased perianal sensation, straight leg raise pain,

cauda equina syndrome - lower motor neuron signs - assymetric

Herpes Zoster Ophthalmicus

caused by VZV!

splenium of corpus callosum stroke

causes alexia w/out apgraphia. due to PCA occlusion

"ophthalmoplegia w/partial vision loss s/p head infxn"

cavernous sinus thrombosis

achromatopsia

central color blindness, can be found in single hemisphere or quadrisphere.

bilateral loss of pain and temp in upper extremities as well as weakness, preservation of fine touch

central cord syndomre 2/2 trauma or intraaxial neoplasm or dilation of the central canal leg fibers run more laterally so they are spared by the STT decussates immediately anterior to the central canal

Acute painless mono-ocular vision loss

central retinal a. occlusion central retinal v. occlusion (acute or subacute)

gait instability, difficulty with rapid movements, nystagmus, intention tremor

cerebellar dysfunction- alcoholics

sudden onset ataxia, vomiting, depressed level of consciousness

cerebellar stroke

facial weakness ataxia nystagmus neck stiff

cerebellum

Torticollis

cervical dystonia tx: botox at SCM

metoclopramide side effect

cervical dystonia, tardive dyskinesia, Parkinsonism

pain that start in the neck and radiates to digits

cervical radiculopathy

Frontotemporal dementia

changes in personality (hyperorality or language deficits), memory and visuospatial skills retained until late MRI-frontotemporal atrophy tx: supportive psych meds PRN

waddling gait (Trendelenburg gait)

characteristic of hip girdle weakness. weakness of abductors leading to consequent failure to stabilize weight bearing hip and causes pelvis to tilt TOWARD opposite side during walking

wernicke encephalopathy

characterized by ataxia, opthalmoplegia, and confusion. tx w/ thiamin can lead to quick resolution of opthalmoplegia and confusion but ataxia can persist for longer.

CNS manifestation of SLE

characterized by psychosis, and affective disorders. chorea/transverse myelitis (ass w/ APL syndrome) HA/seizures distal symmetric polysensory neuropathy or mononeuropathy multiplex (usually due to associated vasculitis)

CI to LP

check for Papilledema - esp if neg CT but high probability. If no papilledema, can go ahead w LP. Otherwise wait and repeat CT.

ascending paralysis L>R absent DTR the whole bit. next step?

check for a tick. asymmetric ascending paralysis.=tick neurotoxin. tx remove tick.

how differentiate b/t median nerve compression at wrist vs elbow?

check thumb flexion- if intact-->wrist if not-->elbow also if lose sensation over thenar-->elbow

Drug induced neuropathy

chemo can cause nerve damage that are length dependent tx: stop drug

Childhood Absence Epilepsy

child age 5-10 with brief staring spells w/o postictal confusion EEG 3Hz spike and wave pattern tx: ethosuximide

benign rolandic epilepsy

childhood epilepsy syndrome that has nocturnal preponderance of either simple partial seizures involving face or mouth, or generalized tonic-clonic. EEG=centrotemporal spikes Tx: carbamazepine or don't have to treat

lennox-gastaut

childhood epilepsy syndrome that is associated w/ intellectual disability. characterized by tonic, atonic, myclonic, absence and generalized tonic-clonic seizures. EEG findings=slow (1-2 Hz) spike and wave Tx: valproate, lamotrigene, febamate, rufinamide

Arnold Chiari Malformation type I

children low lying cerebellar tonsils obstruct CSF flow-->hydrocephalus-->HA and possible syringomyelia of spinal cord dx: MRI tx: decompressive surgery for symptomatic pts

SSPE epi

children, rarely seen after 18, 6-8 years after an episode of measles

treatment of alzheimer's disease

cholinesterase inhibitor i.e. donezepil memantine blocks NMDA

post concussion syndrome

consequence of mild TBI. characterized by HA, dizziness, sleep disturbance, cognitive impairment, behavioral disorders, such as irritiability. usually imaging is normal. Tx sxs.

Brain metastasis

constitutional symptoms (wt loss, fatigue, fevers) spread hematogenously ct chest/ab/pelvis most likely primaries that metastasize to brain: lung, breast, melanoma, rcc

Medial medullary infarction

contralateral paralysis with contralateral loss of DC pathway, tongue deviation to the injured side

Hemiballismus

contralateral subthalamic nucleus lesion in MRI involuntary violent flailing of extremity

vestibulo-occular reflex

coordinates eye movements w/ head movements, elicited by slow passive head movements. semicircular canals and otoliths communicate rotation and acceleration to vestibular nuclei and then to abducens nucleus. abnormalities of this lead to nystagmus.

Wilson Dx

copper deposits in basal ganglia and causes neuro signs "parkinsonism" w/ slowed movements, tremor, ataxia, dysarthria, dysphagia, and dystonia confirm dx w liver biops with quantitative copper assay elevated free copper, low ceruloplasmin or elevated urine copper tx: copper chelation with penicillamine

symmetric lower extremity motor and sensory symptoms

cord compression

treatment for increased ICP

correct cause, elevate head of the bed (30 degrees), Hyperventilation, Mannitol or hypertonic saline (osmotic diuretic), carniotomy

anton's syndrome

cortical blindness due to bilateral lesions of visual cortex. will have visual loss on contfrontation but have anosognosia and are unaware of deficit.

unilateral coarse tremor, rigidity, increased reflexes, limb apraxia/dystonia or alien limb syndrome

corticobasal ganglionic degeneration only one that as similar assymetric parkinson tremor

child with large supracellar mass containing specs of calcium

craniopharyngioma

Migraine

criteria: 1. lasts 4-72 hrs 2. has two of the following: nausea, photophobia, or phonophobia tx: nsaids prn or acetaminophen/asa + caffeine, triptans (5HT1A agonists) if severely disabling (missing work etc) or occur more than 4x a month-use daily prophylactic meds: propranolol, TCAs, depakote, topamax

Why L-Dopa

crosses the blood-brain barrier

Multiple Endocrine Neoplasia type 1

pituitary adenoma, parathyroid hyperplasia, and pancreatic neuroendocrine tumors 2/3 P's and + fam hx HA, amenorrhea and milky breast discharge elevated PRL and Ca

post delivery-sudden headache w/n/v often associated with a rapidly worsening visual field defect or double vision, bitemporal hemianopia

pituitary apoplexy

treatment of guillan barre

plasmaphoresis or IVIG

aching shoulders and thighs, difficulty climbing stairs, proximal muscle weakness and tenderness - diagnosis

polymyositis - symmetric proximal muscle weakness

floppy baby with feeding difficulties and heart failure

pompe's disease acid maltase deficiecny

coma pinpoint pupils

pons

Sturge Weber Syndrome

port-wine stain v1 and v2 distribution +/- leptomeningeal angiomas dx: CT with calcifications kids can have intractable to med seizures, risk of developing glaucoma tx: seizure control +/- glaucoma therapy

reserpine and tetrabenzine

possible tx for tardive dyskinesia, but usually is unresponsive.

spinocerebellar tract

post/dorsal: to ipsilateral cerebellum from muscle spindles. ant/ventral: to bilateral cerebellum from GTOs. BOTH carry proprioception info.

pupillary dilation circuit

posteriolateral hypothalamus-->IML column-->superior cervical ganglion-->pupillary dilator (vial carotid artery into cavernous sinus, then on abducens)

treatment for restless leg syndrome.

pramipexole or ropinirole (dopmaine agonist) - also check iron b/c low iron can cause restless leg syndrome

meningioma

predilection for cerebral convexities, falx cerebri, sphenoid wing. ass w/ NF2, progesterone receptors. Isointense in T1, T2 w/ intense contrast enhancement. calcifications on CT (psamoma bodies) can be recurrent Tx: radiation <4 cm or resection if possible

Tourette's Syndrome

presence of multiple motor tics 1. vocal (grunting, echolalia) or coprolalia-obscene words present for at least a yr tx: clonidine or guanfacine

Brain tumors

present 1. progressive focal deficit 2. seizure 3. sx of ICP or combo of any of the previous primary: not systemic sx (wt loss, fevers, fatigue), from support cells of cns (astrocytes, oligodendrocytes, schwann cells, ependymal cells, lymphocytes, meninges or primitive neuroectodermal cells) biopsy malignant: systemic sx

CSF Lymphoma related to AIDS

present as single or multiple lesions dx: CSF with EBV tx: HAART, steroids, radiation

Tuberous sclerosis

cutaneous lesions, seizures, and mental retardation ash-leaf shaped hypopigmented macules, facial adenoma , lumbrosacral skin thickening and yellowing MRI-cortical tubers and or subependymal nodules echo-cardiac rhabdomyoma, renal US-renal agniomyolipoma tx: vigabatran or ACTH

Craniopharyngioma

cystic calcified suprasellar lesion on MRI sits inferior to optic chiasm - big and presses up for eye deficits

TB (meningitis)

present in much of the world. can present in CNS in several waves. arises from hematogenous dissemination of pulmonary focus. has a predilection for basal meninges leading to cranial nerve palsy, and other features of meningitis. more likely to lead to hydrocephalus. usually subacute on chronic, insidious presentation, and prolonged prodrome of malaise, and nonspecific constitutional symptoms precede the neck pain and stiffness. will have lymphocytic leukocytosis in CSF, and VERY low glucose, Acid fast bacilli that can take weeks to grow. Use PCR

spinal abscess

present w/ neck or back pain and focal neurologic deficits consistent w/ spinal cord compression. can be acute or insidious. +/- fever. due to instrumentation, anterior infection in disk or vertebrae. Dx: spine imaging w/ contrast. NO LP!! because it can lead to seeding. need to have image guided LP. Blood cx can sometimes reveal organism. Tx: long term IV abx. sometimes drainage is necessary, especially if there is compression.

Acute angle closure glaucoma

presents acutely, intense unilateral pain + redness, mid dilated and fixed pupil anticholinergics or sympathetics can precipitate attack dx: ocular tonometry (elevated pressures) tx: acetazolamide + beta blocker to rapidly reduce IOP, later give pilocarpine to constrict pupil

Acute Disseminated Encephalomyelitis (ADEM)

presents after vaccination or viral illness with diffuse demyelination of the braina dn spinal cord-->HA and encephalopathy dx: MRI and LP tx: IV steroids and consider plasmaphoresis/IVIG

Dementia with Lewy Bodies

presents initially w dementia characterized by fluctuating levels of alertness, visual hallucinations, falls and REM sleep behavior disorders tx: anticholinesterase inhibitors donepezil and Ldopa for parkinsonisms

brain abscess

presents like focal process. can have headache, seizures and increased ICP, +/- fever. invades from neighboring sites of infection or due to instrumentation. can result from hematogenous dissemination from IE or IC states (usually will be multiple). usually will be mixed flora. blood Cx can sometimes identify. SPECT to differentiate neoplastic vs. infection (both are ring enhancing). start w/ CT,MRI w/ contrast. Tx: IV abx for a long time.

cerebellar infarction/hemorrhage

presents w/ abrupt vertigo, vomiting, ataxia. can have compression of brainstem leading to decreased consciousness. can also lead to hydrocephalus. could be a surgical emergency.

Medulloblastoma

presents with hydrocephalus + sx of ICP (papilledema) and cerebellar findings biopsy: hyperchromatic nuclei in a pseudorosette pattern tx: surgery, radiation, chemo based on staging and pathology, ventricular shunt

alexia w/out agraphia (pure alexia)

preserved ability to write but impaired reading. lesion situated in dominant occipital lobe and involves splenium of corpus callosum. fibers connect visual cortex to wernicke's. will usually have accompanying contralateral homonomous hemianopia.

Suspect ___ in an HIV-infected patient with an AMS, EBV DNA in CSF, and solitary, weakly ring-enhancing periventricular mass on MRI.

primary CNS lymphoma

Idiopathic torsional dystonia

primary dystonia that may be familial. is ATP bindingprotein torsinA mutation. manifest as torticollis, writers cramp, blepherospasm. Tx: botulinum toxin

inability to look down sqaure wave jerks on EOM

progressive supranuclear palsy upright posture, not usually flexed, no tremor

Acoustic neuroma (vestibular schwannoma)

progressive unilateral sensorineural hearing loss tumor on CNVIII at the cerebellopontine angle-->hearing loss gradual hearing loss due to nerve compression, tinnitus and HA air conduction > bone tx: surgical removal vs. sterotactic radiotherapy vs. observation

Neurologic side effect of isoretinoin

psuedotumor cerebri

Lyme disease

pts from the NE with outdoor/tick exposure followed by first erythema migrans rash, HA, arthralgias, myalgias neuro sx: meningitis, radiulopathy, neuropathy, encephalopathy, bell's palsy dx: serum lyme abs tx: IV ceftriazone, or oral doxy if only bell's palsy

advanced sleep phase disorder

pts sleep or awaken earlier than they desire

multifocal motor neuropathy (MMN)

pure motor neuropathy that affects multiple nerves. probably immune mediated. will be slowly progressive, asymetric, predominately distal limb weakness, usually beginning in arms. will be in distribution of specific nerves. can have mild sensory stuff but not a lot/not objective. Dx: EMG/NCS that show nerve conduction block. CSF protein is usually normal. IgM vs GM1 in 60-80%. Tx: IVIg, rituximab, cyclophosphamide and other immunosuppresants.

most common site of hypertensive hemorrhage

putamen

left hemiparesis hemisensory loss gaze palsy to the right

putamen (basal ganglia) bleed

Tx for Myesthenia gravis

pyridostigmine and immune modulator like azathioprine or cyclosporine and steroids. IVIg and plasmapheresis.

treatment for toxoplasmosis

pyrimethamine

intense pain, vesicles in ear, facial paralysis

ramsay hunt syndrome

MC MS presentation

random parasthesias, motor loss, or optic neuritis - on and off.

saccades

rapid eye movements that redirect eyes to new fixation object. orginate in frontal eye fields and superior colliculus CONTRALATERAL to DIRECTION OF GAZE. project to contralateral PPRF. will overshoot or undershoot if pathologic (hyper or hypometric). can also cause unwanted movements such as square wave jerks, occular flutter and opsoclonus (intrusions).

Paroxysmal dyskinesia

rare group of movement disorders--recurrent attacks of hyperkinesis w/ preserved consciousness. can be kinesogenic where hyperkinesis is triggered by episodes of sudden movements and are brief; or nonkinesogenic, that are triggered by EtOH, fatigue, and stress and last longer. Tx: carbemazepine

Vestibular migraine

recurrent unilateral vestibular dysfunction without associated hearing loss like meniere's + nml hearing tx: migraine prevention

Meniere's dx

recurrent vertigo, hearing loss, tinnitus, fullness, nystagmus, unilateral audiogram-low tone hearing loss tx: low salt diet, diuretics

diabetic amyotrophy/lumbosacral radiculoplexus neuropathies

related to DM. is a proximal neuropathy. can be immune mediated, possibly associated w/ vasculitis. leads to foot drop, weakness/pain in hip and thigh, and decreased patellar reflex

Optic Neuritis

relative afferent pupillary defect, difficulty discrminating the color red suspect MS tx: high dose IV steroids

erectile dysfunction Tx

remove drug; sildenafil, vardenafil (cGMP phosphodiesterase inhibitor)

diagnosis of CIDP

cytoalbuminologic dissociation - protein increased in CSF without increase in cell count (also seen in AIDP) EMG - slow conduction velocity, prolonged F wave latencies, conduction block with dispersion of the compound muscle action potentials segmental demyelinatoin of nerve axons on biopsy

lewy body dementia

shows extraordinary sensitivity to neuroleptics (marked worsening to these drugs). hard to treat both psychiatric and parkinsonian aspects of this disorder.

Romberg sign

sign of tabes dorasalis

Right parietal cortex damage

dominant hemisphere - get Hemineglect on C/L side

medications that can cause parkinsonism

domperidone (Motilium) or ondansetron, metaclopramide, prochlorperazine, neuroleptics

tx of restless leg syndrome

dopamine agonists (pramipexole) or alpha-2-delta calcium channel ligands (i.e. gabapentin)

VPL

dorsal column and spinothalamic (aka ALA) tract synapse here and then goes to sensory cortex

Ant Spinal A. occlusion

dorsal columns (vibration) only spared

B12 def

dorsal columns damaged

Uncal herniation

downwards herniation to brainstem Can be seen w epidural hematoma (can't cross, so just goes down) - blown pupil (I/L fixed, dilated CN 3)

cocaine eye drops

drops that fail to lead to dilation in any form of horner's because this only inhibits reuptake of NE. so if there is no nerve firing, then NE in cleft, so no change in dilation w/ blocking uptake).

recent onset gait imbalance and vertigo w/ antibiotics

drug induced ototoxicity- Aminoglycoside (gentamicin)

adrenoleukodystrophy

due to mutation in very long chain fatty acid oxidation. is X linked. leads to white matter hyperintensity of MRI. may present as neuropathy or myelopathy in adults.

Krabbe disease

due to mutation of galactosylceramide P galactosidease on chromosome 14. will have globoid cells with PAS-positive granules.

Hunter's syndrome

due to mutation of iduronate sulfatase on X chromosome. leads similar phenotype as hurler's but without corneal clouding.

bilateral medial tegmental stroke

due to occlusion of basilar artery, usually leading to a coma.

necrotizing myopathy

due to statins, cyclosporine, propofol, EtOH. CK will be increased can have acute or insidious proximal muscle weakness.

critical illness myopathy

due to steroids and NM blocking agents in pts w/ sepsis. will be generalized weakness. CK can be normal or increased.

HIV associated neuropathies

due to virus. are usually distal and sensory neuropathy. can be due to treatments or virus itself. mononeuropathy and mutilple mononeuropathies are usually late occurences and are ass. w/ HSV, CMV, HepC, and syphilis.

neuronal (axonal degeneration)

dying of the axon and loss of myelin due to damage to cell body.

akasthesia

dysphoric state w/ subjective sense to be in constant motion--inability to sit or stand still. Tx: benzos or b-blockers.

Juvenile Myoclonic Epilepsy (JME)

early adolescence + myoclonic jerks preceding development of generalized seizures a couple yrs later tx: levetiracetam, topiramate, lamotrigine

Chronic demyelinating polyneuropathy

similar to GBS, but different in that it 1. presents subacutely 2. may take either a chronic progressive course or take a relapsing-remitting course 8 wks dx: lp with albuminocytologic dissociation , NCS/EMG will show demyelination tx: IVIG or plasmapheresis

gaze evoked nystagmus

similar to end point nystagmus but increased amplitude and occurs w/ less eccentric position. usually due to drug intoxication but also cerebelar/brainstem pathology.

Herpes Zoster Oticus

similar to labyrinthitis: vertigo, retroauricular pain, constant vertigo, decreased hearing + vesicular lesions tx: antivirals + supportive for pain

delayed sleep phase disorder

sleep onset delayed until early morning w/ consequent awakening later than desired. tx=bright light and melatonin to reset circadian clock

MG EMG findings

slow repetitive nerve stim leads to decremental response. single fiber recordings are most sensitive and will show increased jitter.

bradyphrenia

slowness of thought. characteristic of parkinson's disease.

prosody

subtle elements of effective communication like changing tone of voice that reside in non-dom hemiphere. can sound monotone w/out this. also lose ability to sense tone of voice in others.

Myoclonus

sudden brief muscle contraction (hiccups, hypnic jerks) Wilson's Dz metabolic encephalopathy Rx - Clonazepam, Valproate

Vision loss in Temporal Arteritis

sudden, PAINLESS complete loss of vision. Can be intermittent - give STEROIDS asap to avoid permanent vision loss!!

alternating hemiplegias

superior: midbrain-PCA middle: pons-basilar inferior: medulla-Ant.Sp.A Lesions of corticospinal tract (UMN) combined with cranial nerve signs (LMN).

anterior choroidal artery

supplies basal ganglia structures like the putamen and GP

posterior choroidal artery

supplies the most inferior temporal lobe aspects

PCA deep branches

supplies the posterior thalamus

DA agonists (ropinerol)

supposedly are drugs that if given early in Parkinson's, then they can reduce the dopa-induced dyskinesias later on.

Hypertensive encephalopathy

suspect in a pt with elevated BP, papilledema and w/o signs of intracerebral hemorrhage, stroke, or meningitis tx: acute reduction in bp by 25% and diastolic to 100-110 via labetalol and nicardipine

Dystonia

sustained muscle contraction (torticollis) Huntington's, Wilsons Drug Induced (EPS). Rx = Anticholin. (Benztropine) or Antihistam (Diphenhydramine)

Acute rheumatic fever

sydenham's chorea, erythema marginatum, murmur-carditis, immigrant from rural area therefore no abx for past Group A strep infection bc of lack of access

arching spasms of the back and neck

tardive dyskinesia

chewing, lip smacking, tongue rolling

tardive dyskinesia Dopamine Blockers Tx- removal, reserpine, tetrabenzine

Lobes commonly involved in traumatic brain injury

temporal and inferior frontal lobes

77 yo with 3 weeks of fever, fatigue, severe headaches, sensitivity to bright light, pain in the hips and shoulders, weight loss, pain to palpation in muscles of shoulders and hips, anemia, ESR 85 - next step in *diagnosis*

temporal arteritis and polylmyalgia rheumatica - temporal artery biopsy

Malignant hyperthermia

tensing up, tachy, febrile tx: dantrolene can have combined respiratory and metabolic acidosis, increased serum CPK, potassium, and urine myoglobin

Axillary nerve damage

test shoulder abduction and sensation of lateral shoulder + sulcus sign

anomia

tested by naming test. is a good test (sensitive) to detect aphasia.

fluency

tests for language fluency. done by listening to patient's spontaneous speech. if they can string at least 7 words together into phrase. "tip of the tongue" phenomenon.

contralateral hemiparesis and sensory loss nonreactive small pupils upgaze palsy eyes deviate toward hemiparesis

thalamic hemorrhage

hemisensory loss with severe dysesthesia

thalamic stroke

c/l hemiparesis/hemianasthesia upwards gaze palsy, miosis, eyes toward hemiparesis

thalamus

Essential Tremor

typical progressive action tremor and head nodding, temporarily responds to alcohol, can have a family history tx: beta blockers or primidone, deep brain stimulation for refractory cases

Tension headache

typically mild-moderate, band of pressure around the head OTC nsaids

anisocoria

unequal pupil size

Glioma/astrocytoma

usually occur in 4th decade of life can present w/ seizures usually bright on T2 and FLAIR, usually no enhancement. tx: surg for grade 1 and possible 2. 7 year median survival.

Early Alzheimer's Dx

early: anterograde memory formation + visuospatial skills, forgetting recent events and get lost workup: depression, TSH, B12, RPR, MRI (multi-infarct, hydrocephalus, or inflammatory lesions) dementia: 2 cognitive domains (memory and learning new info + visuospatial function, language, executive function, or personality) tx: acetylcholinesterase inhibitors (donepezil), memantadine (NMDA antagonist)

Uncal herniation + compressive 3rd nerve palsy

edema from a large CVA maximizes 3-5 d after insult significant mass effect with concern for herniation tx: mannitol, intubation, and hyperventilation, craniotomy to relieve pressure

Compression of 3rd nerve

emergency, dilated nonreactive pupil, likely 2/2 aneurysm in PCA tx: angiography + neurosurgical clipping or coiling of aneurysm

HSV encephalitis

encephalitis involving medial temporal lobe nml glu, high lymphocyte count

cat-scratch encephalitis

encephalitis, increased signal intensity in the pulvinar

NMJ disorders

usually proximal muscles affected predominantly. some can involve ptosis, extraoccular, bulbar and neck muscles. EMG/NCS will have pathogonomnic findings for some disorders. some have serum markers.

Vasovagal syncope

vagal (parasympathetic) reaction-->cardiac inhibition or vasodilation and subsequent CNS hypoperfusion-->LOC eg. blood, fear, emotional, response, sight of blood, post-tussive or straining dx: history tx: avoid triggers

<15 ml/kg

value of FVC in GBS where one should transfer pt to ICU and intubate. why you need to get PFTs as soon as you suspect that a patient has GBS.

imagign in schizophrenia

enlargement of cerebral ventricles

Meniere's disease

excess endolymphatic fluid in cochlea, causes hearing loss, vertigo, dizziness, nausea, vomiting, tinnitus.

syringobulbia

expansion of the syrinx into brainstem leading to brainstem neurologic signs.

Jerk nystagmus

eye drifts away from fixation in a pursuit-like movement and then returns w/ a fast saccade. direction is defined as FAST saccade direction.

Ischemic 3rd nerve palsy

eye is inferiorlateral occulomotor nerve, pupils reactive to light can be 2/2 DM self resolving

GCS

eye opening (4) verbal (5) motor (6) 13-15 mild 8-12 moderate 3-7 severe

INOP (intranuclear opthalmoplegia)

eyes adduct during accommodation but DO NOT adduct on attempted looking at object to one or both sides. May be due to MLF damage.

peripheral neuropathy of hands and feet, angiokeratomas, CV and renal disease

fabry disease alpha-galactosidase A deficiency ceramide trihexoside accumulates Xlinked recessive

MC neurologic injury in sarcoid

facial paralysis

Viral Meningitis

viral (aseptic) meningitis nml glu, high lymphocyte count dx: PCR for HSV and enterovirus tx: acyclovir

solitary nucleus

visceral sensory info (like taste) for CN VII, IX, X

Craniophyryngioma

failure to thrive (grow) in kiddo despite normal appetite chronic HA, decreased peripheral vision, low IGF-1 and GH that do not increase w/ insulin administration benign suprasellar mass: visual field defects, hypopituitarism or HA bimodal age of presentation 5-10yo or 60-70 yo dx: MRI with cystic suprasellar mass tx: transcranial or transphenoidal surgical resection pluse post op radiation

Myasthenia Gravis

fatiguable weakness that improves with rest (ptosis and diplopia) autoantibodies to ach receptors on postsynaptic nerve terminal EMG with repetitive nerve stimulation CT screen for thymoma tx: acetylcholinesterase inhibitors pyridostigmine

Serotonin Syndrome

febrile, rigid, tremulous extremities, clonus tx: cyproheptadine

Rett Syndrome

female child + regression of developmental milestones

Bacterial Meningitis

fever, HA, neck stiffness ecchymotic rash-N. meningitidis dx: LP (low glu, high WBC-neutrophils) gram stain: gram neg dipplo-n. meningitidis, gram pos lancet shaped dipplo-s. pneumo tx: empiric IV abx-ceftriaxone, vanco, amp (elderly + immunocompromised)

Bacterial meningitis

fever, HA, stiff neck n. meningitidis head ct-->lp (glu, protein, wbc, rbc, gram stain, cultures) + blood cx ct before lp to avoid herniation csf: low glu, high protein and wbc (neutrophil predominant) ceftriaxone + vanc (+/- amp)

Fingers involved in carpal tunnel syndrome

first 3 and 1/2 digits and occasionally thenar eminence

CT without contrast

first line to r/o hemorrhage White = hemorrhage Black = ischemia

frontal gait

flexed posture w/ feet slightly apart. magnetic gait b/c difficulty lifting feet of ground. will have small shuffling hesitent steps. typical of hydrocephalus, tumor, stroke, neurodegenerative disease.

parts of language test

fluency repetition comprehension naming reading writing

complex partial seizure

focal onset and impairment of awareness. commonly arise from temporal lobe or frontal lobe. can include automatisms, speech arrest, or continuation of activities they were doing when seizure started. if frontal can have bizarre bilateral movements like running in circles.

oligodendrocytes

form myelin in CNS

Psychogenic Nonepileptic spells

form of conversion disorder though to be an unconscious rxtn to psychological stress stress at home, long seizures, little post ictal confusion, alternating movements, shaking head side to side, closed eyes, intact consciousness while shaking bilaterally, or pelvic thrusting dx: long term EEG monitoring

Diabetes Sensorimotor Polyneuropathy (DSPN)

gradually progressive sensory loss in a stocking glove distribution, impaired glucose metabolism tx: tight glucose control, but cannot reverse the damage

trigeminal autonomic cephalgias

group of disorders characterized by unilateral trigeminal distribution of pain, accompained by prominent ipsilateral autonomic sxs. Tx: triptans, steroids, avoid EtOH. verapamil or Li for PPX.

Post concussive syndrome

induced by mild head injury characteristics: non-specific neuro findings (HA, irritability, mild cognitive difficulties, dizziness, depression, anxiety) tx: brain rest aka avoid activities that require concentration

West Syndrome

infantile spasms 1. spasms 2. hyparrythmia 3. mental retardation tx: ACTH or prednisolone (suppress CRH)

Polymyositis

inflammatory myopathy subacute progressive symmetric proximal muscle weakness dx: muscle biopsy after EMG tx: glucocorticosteroids, immunosuppressants (azathioprine or methotrexate)

Dermatomyositis

inflammatory myopathy with symmetric weakness that occurs more often in females and has a pathognomonic heliotrope rash and gottron's papules dx: CK high and EMG shows myopathy, muscle biopsy shows inflammation and perifasicular atrophy tx: tx: glucocorticosteroids, immunosuppressants (azathioprine or methotrexate)

infant with dropping eye lids, constipation, drooling, no suck or gag reflex, flaccid paralysis

ingestion of botulism spores tx: IgG

Progressive multifocal leukoencephalopathy

leads to dementia, focal cortical dysfunction and cerebellar abnormalities. occurs in immunocompromised pts. leads to demyelination by infection of oligodentdrocytes by JC virus. will have multiple focal white matter abnormalities, typically in posterior brain. CSF will be normal.

CN VI palsy

leads to esotropia. can be a nonlocalizing lesion such as a sign of increased ICP.

The Right frontal eye field controls eye movement to the ___.

left

Cauda equina syndrome

lumbar spinal stenosis back pain, leg weaknesss, numbness in a saddle like distribution between thighs, urinary incontinence, decreased reflexes tx: neurosurg, IV steroids if neoplastic

Analgesic rebound headache

made clinically based on chronic, frequent use (3+ d/wk) of abortant or analgesic pain meds tx: stop frequent use of analgesic meds and change meds w/o rebound

spinocerebellar lesion

main symptom is ipsilateral ataxia

chronic progressive distal symmetric diabetic polyneuropathy

mixed sensory-autonomic-motor neuropathy. have variants like small fiber (painful), large fiber (ataxic), and autonomic.

tx of narcolepsy

modafinil and armodafonil

tx of GBS

monitor for arrythmias and FVC <15 then intubate IVIG, plasmaphoresis

ADEM (acute disseminated encephalomyelitis)

monophasic leading to areas of demyelination. usually follows viral infection or vaccination. will have multiple lesions, are patchy, bilateral and confulent. usually go to posterior white matter. can lead to behavioral, cognitive abnormalities. lesions are acute and enhance w/ Gad. CSF will show lymphocytic pleocytosis, increased protein but usually NOT oligoclonal bands. usually recovery is complete. IV steroids can shorten duration.

Mild Cognitive Impairment

more significant impairment in a cognitive domain than normal aging, but not significantly affecting activities of daily living 5-16% annual risk of progressing to dementia per yr compared w general elderly pop. risk of 1-3%

progressive supranuclear palsy

most characteristic feature is failure of vertical gaze, dysarthria, dysphagia, extrapyramidal symptoms (rigidity), gait ataxia, dementia, falls and gait issues are prominent early. atrophy of dorsal midbrain, GP, subthalamic nucleus

Melanoma

most common brain met w/ hemorrhagic component.

Charcot Marie Tooth (Type 1)

most common heritable neuropathy distal sensorimotor loss wasting occurs as weakness progresses and hammer toes/high arches present CMT type 1 is a demyelinating neuropathy and type 2 is axonal neuropathy

Duchenne muscular dystrophy

most common inherited muscular dystrophy, affects males with typical onset 3-5 yro difficulty walking around age 12 and difficulty breathing in the 2nd or 3rd decade cardiomyopathy complication mutation in dystrophin gene on X chromosome dystrophin deficiency on muscle biopsy or mutation analysis of periopheral leukocytes tx: glucocorticoids

Toxoplasmosis

most common intracranial pathology in AIDS pts, CD4+ <100 presentation: ICP, ring enhancing leisons on MRI/CT with contrast dx: CSF PCR for toxo tx: pyrimethamine-sulfadiazine for 6wks2

channelopathies

most common is periodic paralysis. is autosomal dominant. has episodic muscle weakness. ass w/ changes in [K]. has hypo and hyper types. hypoK-mutation in poreforming subunit of skeletal muscle VGCC leading to secondary dysfunction of Na/K ATPase. HyperK-mutation in VGNa channels.

Obstructive Sleep Apnea

most common medical cause of excessive daytime sleepiness middle aged, obese male who snores with polycythemia, crowded airway (large tonsils), or nocturia dx: polysomnography (sleep study) tx: CPAP to keep airway open

24-h urine copper

most sensitive test for Wilson's disease

nucleus aMbiguus

motor innervation for swallowing (CN IX, X, XI)

foot drop, excessive knee and hip flexion while walking, slapping quality, falls, distal sensory loss

motor neuropathy - usually L5 radiculopathy "steppage gait"

Mononeuritis Multiplex

multifocal monneuropathy due to an inflammatory cause such as vasculitis tx: high dose steroids for immunosuppresiion +/- cyclophosphamide

PML MRI

multiple demyelinating, non-enhancing lesions with no mass effects

Neuroleptic Malignant Syndrome

muscle rigidity and tremor, CPK and urine myoglobin, fever tx: cooling measures + IV hydration stop haloperidol bromocriptine or dantrolene

Myotactic reflex =

muscle stretch reflex

Hypothyroid myopathy

muscle weakness in proximal, aches, difficulty combing hair and climbing stairs, wt gain, feeling cold and constipated

Statin induced myopathy

myalgias + proximal muscle weakness + rhabdo (CK and urine)

Transverse myelitis

myelopathy + MRI with confirmatory inflammation (myelitis) transverse myelitis-postinfectious, acute infectious or related to systemic disorders or demyelinating disorders dx: MRI tx: IV methylprednisolone

grip myotonia, facial weakness, foot drop, dysphagia, testicular atrophy, baldness, cataracts

myotonic musclar dystrophy autosomal dominant resp complications

naproxen vs ibuprofen

naproxen is longer acting used in patients who require frequent dosing

Lesion of pulvinar can cause

neglect syndromes and attention deficit

high T2 signal in cerebellar cortex

neoplastic cerebellar degeneration - > SCA

part of brain affected in HD

neostriatum, atrophy of head of caudate nucelus and putamen

glioblastoma multiforme

neovascular proliferation and necrosis commonly in deep white matter, basal ganglia, thalamus. will enhance w/ contrast and have surrounding edema. Tx: surgery and temozolomide, carmustine/lomustine, bevacizumab, gamma knife. but poor prognosis.

Optic nerve glioma

neurofibromatosis type 1 cafe-au-lait patches, neurofibromas, axillary/inguinal freckling, iris hamartomas, sphenoid dysplasia (long bone abnormalities), high risk for optic nerve glioma presents w gradually progressive vision loss, proptosis, and strabismus dx: MRI-fusiform enlargement of the optic nerve

Meningioma

neurofibromatosis type 2 risk for vestibular schwannomas dx: MRI with dural based lesion tx: surgery

treatment of tourette's

neuroleptics i.e. haloperidol and pimozide however Risperidone has better side effect profile

Trigeminal neuralgia

neuropathic pain from abnormal vessel loop contacting trigeminal nerve tx: carbamazepine, can do trigeminal ganglion nerve block if refractory

Oligodendroglioma

new onset seizures + papillodema no focal neuro deficits MRI: calicified mass + fried eggs tx: surgery + chemo ~7yrs

delayed sleep syndrome

night owl delayed onset of sleep. fxn at night can't was up in the am

rx to tx htn in ICH

nitroprusside SE methb

Partial seizure

no change in alteration in consciousness and sx can be localized smell-temporal lobe visual-occipital lobe motor-frontal lobe dx: EEG and MRI

Left parietal cortex damage

non-dom hemisphere --> Aphasia

Cerebral Palsy

nonhereditary, nonprogressive (fixed) movement and postural disorder perinatal neuro insult + abnml movements (spastic paresis) periventricular leukomalacia

tiredness, occasional forgetfulness or word finding difficulty, trouble falling sleep

normal aging

trauma pain, recovering opioid addict meds?

normal managemnet in acute give morphine, the only different is they need close monitoring

ICP

normal value is <15 mmHg need CPP of 60-75 mmHg monitor w/ IV presser monitor if GCS<9 or abnormal CT.

Ulnar nerve entrapment at the elbow

numbness and tingling in hand, worse after leaning on elbows, no neck pain, weakness spreading fingers and flexing 5th digit, sensory loss in 5th digit and medial aspect of 4th digit dx: NCS tx: conservative treatment w/ elbow pads

peripheral nystagmus

nystagmus that is usually unidirectional w/ fast phase away from lesion. will usually have rotary component but never vertical. is inhibited by visual fixation and ass. w/ tinnitus and deafness. romberg sign will be toward slow phase. will be short in duration but recurrent.

Cerebellar Pathology

nystagmus, intention tremor, dysdiadochokinesis and cerebellar gait same side problems (ipsilateral) MRI w/ contrast

Occipital lobe damage results in...

visual disturbances.

spinocerebellar ataxia, eye sx, absent reflexes, decreased immune response, myopathy

vitamin E deficiency

FEF controls

voluntary movement of eyes (not reflexive)

fat cheeks, thin extremities, short stature, enlarged kidneys and abdomen, hypoglycemia, lactic acidosis, hyperuricemia, hyperlipidemia,

von gierke disease glucose-6-phosphatase deficiency

Cerebellar hemangioblastoma

von hippel lindau disease CNS hemangiomas (retinal, cerebellar, spinal) and renal/liver cysts increased risk for pheo and rcc dx: renal imaging tx: surg excision

gaze away from side of lesion (wrong way eyes, eyes toward side of deficits)

will be gaze preference in seizure (increased activity in FEF leads to contralateral movement of eyes) can be due to thalamic hemorrage can also be due to lesion in pontine basis and tegmentum that lead to contralateral hemipariesis (CST has NOT decussated yet) but involvement of PPRF and abducens causes ipsilateral gaze weakness.

thyrotoxic myopathy

will commonly affect proximal muscles. will have normal to brisk reflexes. If due to graves then can also be ass w/ myesthenia CK is usually normal.

brain causes of weakness

will have UMN pattern. pattern of involvement helps to localize. etiologies=stroke, demyelination, traumatic injury, brain tumor, infection.

classic migraine

will have aura, usually scintilating scotoma, marches across visual field in 15-20 minutes.

episodic ataxia (inherited)

will have brief epiosdes of ataxia, vertigo nausea, vomiting EA-1 cuased by K channel mutation--have inter attack myokymia. EA-2 due to alpha1 subunit (pore) of P/Q type VGCC. attacks last longer w/ inherited nystagumus.

LE syndrome

will have decremental response on slow repetitive nerve stimulation. will have INCREMENTAL response on fast repetitive nerve stimulation. will also be accompanied with autonomic dysfunction. Tx: 3,4 diaminopyridine +/- pyridostigmine.

neuroleptic malignant syndrome

will have fever, decreased arousal, rigidity, autonomic dysfunction. can be due to antipsychotics or due to L-dopa withdrawal and with TCAs. tx: benzos, dantrolene, bromocriptine, amatadine

plexus disorders

will have multiple muscle weakness but do not conform to pattern of single nerve or nerve root. will have sensory findings and dropped reflexes. etiologies=inflammation, radiation, mets, hemorrhage, trauma, diabetes.

ependymoma

will have perivascular pseudorosettes. intraventricular location can lead to hydrocephalus. if in spinal cord can lead to conus medullaris or cauda equina syndrome.

schwannoma

will have verocay bodies w/ uniform spindle cells. get MRI w/ contrast. can tx w/ gamma knife if <3 cm. surg can lead to facial nerve defects.

early onset MG

will present in 20-30s, is found in women>men, and will be associated w/ thymic hyperplasia

late onset MG

will present in 60-70s, is seen in men>women, and will be associated more w/ thymoma.

CIDP

will present w/ slowly evolving leg weakness that spreads, widespread areflexia, loss of vibratory sense (large fibers), weakness of neck flexors. can have painful paresthesias and other sensory loss. Get NCS. can be associated w/ HIV, monoclonal gammopathy, hodgkin lymphoma. can get improvement with steroids. otherwise IVIg periodically for years often is needed.

neurocysticercosis

will present with new onset focal seizure. HA and cystic lesions w/ calcification that are ring enhancing. Tx: albendazole and steroids to control inflammation.

PML

will show patchy, nonenhancing, foci of T2 hyperintensity of subcortical white matter

tPA time frame

within 3 hours of onset. no ASA *if after 3 hrs --> just give ASA. - (backup - Clopidogrel or Ticlopidine) (verus MI - door to balloon time is 90 min)

paraphasia

word substitution errors. can be substituted based on sound (spool for school). seen in broca's aphasia.

agnosia

word that means normal perception but is stripped of meaning.

sundowning

worsening of Delirium when the sun goes down (at night)

child younger than 9 do you image spine agfter fall?

yes. especially with distracting injury. children younger than 9 who experience blunt trauma or falls shoudl have their spine imaged

Myotonic Dystrophy

young 30s male + weakness in neck, hands, feet, muscle stiffness, frontal balding, temporal wasting, and cataracts dx: EMG myotonia

Ependymoma

young children + hydrocephalus sx bc ependymal cells line the ventricles so overgrowth can lead to obstruction of flow 1. mass confined to ventricles 2. calcifications on CT 3. blepharoplasts on biopsy/path

Pseudotumor cerebri (idiopathic intracranial HTN)

young, obese females who complain of chronic headaches and may have associated sx of transient visual disturbances, pulsatile tinnitus, or gradual visual field loss isotretinoin use can be a cause dx: head CT, LP with elevated opening pressure (>20-25), visual field eval tx: LP to normalize pressure, acetazolamide decreases CSF production, lasix, wt loss

Locked in syndrome

~ coma but full paralysis EXCEPT for muscles required for vertical eye movt, blinking, and respiration. Infarct of the ventral pons! (pons = pinpoint pupils)

Arnold chiari assoc w...

Syringomyelia

Cerebral Perfusion Pressure

= Mean Arterial Pressure - Intracranial Pressure

3Hz spike and wave pattern

Absence Seizure- assoc with hyperventilation

SSPE CSF studies

increased gamma globulin fraction and presence of oligoclonal bands

Developmental Regression

actually meet your milestones, THEN there's a delay! Bad prognosis.

headache with severe , tearing, nausea, vomitting, eye pain, fixed, dilated pupil

acute angle closure glaucoma

b-blocker

tx for neurogenic syncope. suppress overactive cardiac mechanoreceptors.

Brain death characteristic findings.

Absent cortical and brain stem functions Spinal cord may still function, *DTR may be present*

Argyll Robertson pupil

Accomodates but does not react (to light) - syphilis

Schwannoma

Acoustic neuroma arising form Schwann cells investing vestibular portion of CN VIII. Found in cerebellopontine angle

Leigh disease

due to mutation in mitochondrial genes. can be autosomal recessive or X linked. leads to bilateral putaminal hyperintensity on MRI.

Neuromyelitis Optica

MS variant characterized by bilateral optic neuritis and myelitis without brain involvement dx: aquaporin 4 ab (NMO ab) tx: IV steroids

MRI "hot cross bun" signal in brainstem

MSA

MRI high signal lateral to the striatum

MSA

___ is commonly associated with burst fracture of the vertebra and is characterized by total loss of motor function below the level of lesion with loss of pain and temperature on both sides below the lesion.

*Anterior cord syndrome* - MRI - intact proprioception

MS Presentation

"DdddOoN'T SiiiN" D - Demyelinating, Diplopia, Dizziness, Dawson's fingers O - optic neuritis, oligoclonal IgG bands N - neck, L'hermitte's sign T - T cell mediated S - scanning speech I - INO, intention tremor, incontinence N - nystagmus Charcot's Triad = scanning speech, INO, nystagmus * stay near equator (baseline) ** sx can worsen transiently w hot showers!

SAH causes...

"MAKE a SAH" M - Marfans A - coarctation of Aorta K - ADPKD E - Ehler danos S - Sickle cell A - Atherosclerosis H - family Hx

CI to tPA therapy

"SAMPLE STAGES" S - stroke or head trauma in last 3 mo A - anticoag w INR > 1.7 or increased PTT M - recent MI P - prior ICH L - low plat < 100,000 E - elevated bp (>185/110) S - surgery in last 14 days T - TIA A - Age <18 G - GI or GU bleed in last 21 days E - elevated or low glucose (<50 or >400) S - seizures presenting w stroke

Hint for Sensorineural hearing loss w Rinne & Weber

"SuRinA" - Sensorineural - Rinne's Test: Air cond >> Bone (normal) Weber goes to unaffected ear (I should learn to be unaffected).

Parkinsons

"TRAP 'eM" T - pill-rolling Tremor (at rest). R - cogwheel or lead pipe Rigidity (clench 1 hand, try to extend other forearm) A - Akinesia, bradykinesia - difficulty initiating movt P - posture (stooped over, shuffling narrow gait) 'M - Micrographia (small writing) & Masked facies

First line treatment for idiopathic intracranial HTN.

*Acetazolamide* (inhibits choroid plexus carbonic anhydrase) +/- furosemide - optic nerve sheath decompression or LP shunting is recommended for pts refractory to medical tx

When a patient with parkinsonism prsents with autonomic symptoms (hypotension, impotence, incontinence) suspect what?

MSA

vascular parkinson's

"lower half PD", w/ rigidity in legs and gait impairment. must have other evidence of diffuse vascular changes.

Subarachnoid hemorrhage

"thunderclap HA", secondary to aneurysmal bleed, hyperacute rapid onset dx w/ CT non-con (bleed is white) Angio to locate aneurysm + neurosurg consult nimodipine prophylatically to prevent vasospasm 4-14 D later if CT neg. LP-zanthrochromia (yellow) or RBC tubes 1-4

Edinger Westphal neurons intervate:

(Preganglionic neurons going to ciliary ganglion). Postganglionic neurons from ciliary ganglion innervate the ciliary muscles (for accomodation) and the sphincter pupillae muscles (for pupil constriction/pupillary light reflex)

spinothalamic tract

(aka anterolateral path) lateral: pain, temp medial/ant: crude touch, P

anterior corticospinal tract

(descending) voluntary motor info

Most likely source of brain bets in decreasing frequency? Which ones usually present with multiple mets? **

**Lung > Breast > **Melanoma > Colon

AMD

1. Wet AMD: - abnormal vessel formation (neovasc) causes serous fluid & blood leakage. - hemorrhage & subretinal fluid Rx: anti-VEGF inhib. 2. Dry AMD: - gradual decrease in vision - drusen / pigment changes from atrophy of central retina. Rx: OTC vitamins *CENTRAL (macular) vision loss. PAINLESS.

Optic Neuritis

- PAINFUL (vs AMD - painless) - CENTRAL vision loss - U/L - loss of COLOR optic disk EDEMA (afferent pupillary n. demyelination) - MS

Complications post subarachnoid hemorrhage.

- Rebleeding (first 24 hours) - Vasospasm (after 3 days - CT angiography detects vasospasm and prevented with Nimodipine) - Hydrocephalus/INC ICP - Seizures - Hyponatremia (usually from SIADH)

opioid prescription for pain

1. acute pain use even for addict monitor for pain control 2. severe piano from bony mets: short acting

gardener w/ n/v ringing, rash 10 days, oval patches on neck, decreased sensation to pinprick, absent ankle reflexes, increased axillary pigmentation, nails-leukonychia striata (white marks on nails)

- arsenic poisoning

Chorea what 3 dz

1. HD 2. wilson's dz cu in Basal ganglia 3. sydenham's chorea in RHD

2 episodes of light-headedness, nausea, dimming of vision and LOC while shaving. Lost control of bladder function also. Last month Echo and ECG normal - diagnosis

- carotid sinus hypersensitivity - had prodrome like vasovagal

acute cerebellar ataxia

- child with acute onset unsteady gate following chickenpox

concussion 2 years ago, spinning sensation and NV for 1 hour on airplanes and elevators and when sneezing - diagnosis

- endolymphatic fistula

CSF with lymphocytes and elevated RBC

- herpes encephalitis - get CSF PCR for herpes

carpal tunnel pain refractory to ibuprofen and splinting - next step

- injection of triamcinolone (steroid) into the carpal tunnel [also surgery]

motor neuropathy, microcytic anemia, hyperuricemia

- lead poisoning

immigrant, coarse features, widespread patches of loss of light touch and pinprick sensation, especially on tip of nose and ears, thick peroneal nerves - diagnosis

- leprosy

Acute stroke, what do we usu NOT give?

1. Hep or Enox (high risk of bleeding - just avoid it!) 2. Warfarin (unless from cardiac source/Afib) 3. Anti-hypertensive meds (unless BP > 220/120 or serious heart pathology. Give IV labetalol or Nicardipine) *slight hypertension and hypoxemia is okay - need to maintain perfusion!

x-ray shows osteoblastic lesions on spine - next step

- measure PSA - osteoblastic lesions are mets from prostate [osteoLytic lesions come from Lung]

homemade liquor, headache, abdominal pain, dizziness, *vision loss*, pallor of optic discs, abdominal tenderness, weakness in extremities - toxin

- methanol

drug to decrease frequency of weekly severe, throbbing generalized headache preceded by period of seeing bright spots

- migraine with aura - *beta blockers* are used for migraine prophylaxis

muscle pain and weakness after exercise, and myoglobin in the urine (dark urine) - diagnosis

- muscle phosphorylase deficiency (McArdle disease)

left facial weakness and ear pain - location of abnormality

- nerve - can get ear pain in Bell's Palsy

headache, confused, BP 240/150, funduscopic exam shows hemorrhages and exudates - treatment

- nitroprusside

bell's palsy - next step in diagnosis

- no further testing [can treat with steroids]

M Gravis

- prox m. weakness (usually UE > LE worse at end of day) - ocular changes (ex- double vision) - bulbar m. of oropharynx/jaw (cramps w chewing or dysarthria, hoarseness w talking, dysphagia w solids & liquids) - no sensory involv (motor only - NMJ - nicotinic receptors) - ALWAYS get CT of chest to r/o thymoma!! Easy fix.

weakness of right arm and leg, sensation to pinprick and temperature decreased over left upper and lower extremities, sensation to vibration decreased over right foot , reflexes increased on right - location of lesion

- spinal cord - brown sequard (right hemisection)?

Painful eyelid..

1. Hordeolum (Stye) 2. Chalazion 3. Blepharitis

GB Treatment

1. IVIG and Plasmapheresis 2. NO CORTICOSTEROIDS (think post infxn - don't want to drop immune response)

glaucoma

-Open Angle: common in elderly -Closed Angle: aqueous humor builds up behind iris--> inc IOP. DM risk factor.

Treatment for Alzheimer's

-selective loss of cholinergic neurons. -Cholinesterase inhibitors- DONEPEZIL, GALANTAMINE, RIVASTIGMINE. Mod-severe= Memantine- N-methyl-D-aspartate receptor antagonist.

patient with symmetric resting tremor, orthostasis, impotence

MSA - P vs MSA-C which has cerebellar sx

Features of Medial Medullary Syndrome? Infarct of?

1) Contralateral Weakness (Uncrossed Over CSpinal Tract) 2) Contralateral Decrease in Position and Vibration (Medial Lemniscus) 3) Ispilateral Tongue WEakness (Hypoglossal nerve + Nuclei) ASA, Vertebral ARtery

Contraindications to triptan use

1) Familial hemiplegic migraine 2) Uncontrolled HTn 3) CAD 4) Prinzmetal angina 5) Pregnancy 6) Ischemic stroke 7) Basilar migraine

What would you give to treat MG crisis?

1) IVIG + Plasmapheresis 2) Corticosteroids

Features of Lateral Medullary Syndrome?

1) Loss of Pain/Temp (Cross over spinal tract and nucleus of 5th nerveF -> Ipsilateral Face -> Contralaterlal Body 2)Bulbar Muscle Weakness aka dysphagia and dysarthria (Nucleus Ambigus) 3)Vertigo (Vestibular Nuclei) 4)Ipsilateral Ataxia (Inf Cerebellar Peduncle) 5)Ascending Symp Fibers (Hoerners)

Pelizaeus-Merzbacher disease

due to mutation in proteolipid protein. leads to pendular nystagmus and dysmyelination of CNS. is a peroxisomal disorder.

when to get a head CT after head injury?

1. persistent headache 2. emesis 3. age older than 60 or <16. 4. drug or etoh intoxication 5. persistent anterograde amnesia 6. soft tissue or bony injury above the clavicles 7. seizure

Conductive vs Sensorineural. How to tell the diff.

1. Low freq goes first --> Conductive. High freq goes first --> Sensorineural. 2. Abnormal Rinne test (bone >> air); Weber - sound lateralizes to affected side = Conductive. Normal Rinne test (air >> bone); Weber - sound lateralizes to the unaffected side = Sensorineural.

Central vision loss

1. AMD (hence, macular but painless). 2. Optic neuritis (painful - nerve damage in MS)

Tx MG

1. Acetylcholinesterase inhibitors (anticholinesterases) - Pyridostigmine or neostigmine - SE: abd cramps, fasciculations, muscular weakness 2. Immunosuppressive agents - prednisone, azathioprine, cyclosporine 3. Thymectomy

MCC of vision issues in Developed Countries

1. Age < 65 --> DM 2. Age > 65 --> AMD 3. Cataracts 4. Galucoma

MCL SAH

1. Ant Communicat w/ Ant Cerebral 2. Post Communic w/ Internal Carotid 3. MCA bifurc)

Types of Peripheral Vertigo

1. BPPV & Meniere's (not assoc w infxn) 2. Vestibular neuritis & Labrynthitis (assoc w infxn - ex: dizziness and N/V). 3. Ottotoxic drugs (Aminoglycosides, Diuretics) 4. Acoustic Neuroma (Schwannoma - CN 8) *BPPV and vestibular neuritis - don't see hearing loss and tinnitus *Labrynthitis and vest neuritis - see hearing LOSS, diplopia, nystagmus, unsteady gait, etc too

SAH & Temporal Arteritis... workup

1. CT w/out contrast. 2. Then.... - SAH or infxn suspected, need to f/u with LP if ICP isn't elevated. - Temporal Arteritis suspected → need ESR.

Cherry red spot

1. Central Retinal A. occlusion (need intra-arterial thromolysis of Opthalmic A. within 8 hrs!!) 2. Tay Sach's (no hepatosplenomegaly) 3. Neimann Picks (with hepatospenomegaly)

Pt w foreign object in eye..

1. Check w penlight - if not visible, but pt in acute distress 2. Slit lamp exam w/ Fluorescein

Emergency Eye Changes

1. Closed angle glaucoma - Timolol, Pilocarpine, IV acetazolamide/mannitol 2. Central Retinal A. occlusion - intra-arterial thrombolysis within 8 hrs!! 3. Retinal Detachment - surgery asap

Hearing Loss, Two Types

1. Conductive (lesions in ext/middle ear) 2. Sensorineural (lesions in cochlea or CN 8)

Types of Tremor

1. Essential - action tremor. better w ETOH 2. Physiologic - low amplitude, usu due to increased symp activity (b/c of caffeine, anxiety, hyperthyroidism, etc) 3. Cerebellar - tremor worsens as you get closer to target + dysmetria, ataxia + gait issues 4. Parkinsons - tremor at rest; disappears w voluntary movt (guy can't get on bike, but can ride it flawlessly)

Cotton wool spots

1. Retinal V occlusion 2. Diabetic retinopathy, non proliferative

bloody LP tap 2 dz

1. SAH 2. HSV encephalitis

Stroke or TIA & you're out of the 3-4 hr range.. what do you do?

1. Start ASA! *giving it in 24 hrs reduced recurrence of ischemic stroke. 2. If they are already on ASA in general, add Dipyramidole or Clopidogrel. 3. Anticoag (Warfarin or Dabigatran) if Afib. NO HEP or tPA!!

Types of Stroke

1. TIA 2. Ischemic (Embolic, thrombotic, lacunar) 3. Hemorrhagic (SAH, ICH)

Watch out for in pt's with HA

1. The person that is on chronic analgesics = rebound analgesic HA! Rx don't work. Wean from analgesic - dont give narcotics 2. The person that claims nothing works and has recurrent migraine HA - they might be faking it...

Uveitis vs Acute Glaucoma

1. Uveitis - sensitive to light still, red, MODERATE pain, blurry - decreased visual acuity. 2. Acute Glauc - fixed, dilated pupil, non-reactive, VERY painful, N/V - peripheral vision loss.

TIA's - Vertebrobasilar vs Carotid Presentation

1. Vertebrobasilar - hits IC - dizziness, double vision, vertigo, I/L face & C/L limbs, hoarseness, dysphagia, HA. = MESSY 2. Carotid - Amaurosis fugax - C/L extremity - speech is altered

Causes of Conjunctivitis & Treatment

1. Viral - cold compress; hand washing 2. Gonorrhea - (Hyperacute onset of copious, purulent discharge - bad!!) - Ceftriaxone 3. Chlamydia - Doxy or Erythro 4. Allergic - remove allergen; cold compress; anti-histamines

Trisomy 21

1. mental retardation 2. atlantoaxial instability-->odontoid compression of spinal cord 3. early onset alzheimers in 50s

Lennox-Gastaut Syndrome

1. multiple types of seizures 2. cognitive dysfunction 3. <3Hz spike and wave discharges

GABA

Made from glutamate in the NUCLEUS ACCUMBENS. Is lacking in Huntington disease.

Restless leg syndrome

1. urge to move limbs 2. uncomfortable feeling relieved w/ mvmt 3. symptoms worse at night 4. symptoms worse w rest tx: DA agonist-ropinirole, pramipexole, levodopa

EEG changes w Tonic-Clonic seizure

10 Hz activity in tonic phase, then slow waves in clonic phase. *look out for incont, tongue biting, apnea.

Abortive treatment for cluster headache

100% oxygen

acute tx of cluster headache

100% oxygen may also add NSAIDs and ergotamine

Protein that is highly sensitive and specific to prion disease

14-3-3 proteinase inhibitor

oliogodendroglioma

1p19q deletion. 35-45 years old frontal lobs/basal ganglia/thalamus. can have calcifications, fried egg appearance. presents w/ seizures in 70%. T1 low intensity, T2 high intensity, if enhances then will have negative prognosis. Tx: resection if possible, + procarbazine, lomustine, vincristine and temozolamide and radiation.

kid with bilateral acoustic neuromas and cataracts

NF2

MMSE score of less than ___ is suggestive of dementia (total maximum is 30).

24 points - Neuro imaging shows atrophy which is more prominent in the temporal and parietal lobes

5 mins of generalized tonic clonic seizures in someone w/ refractory epilepsy

2mg IV ativan if that doesn't work try another 2mg IV ativan + 20mg/kg fosphenytoin, then another dose ativan + consider intubation and propofol or phenobarbital/pentobarbitol

Biting tongue, loss of bowel/bladder control is characteristic of?

2ndary generalized seizures!! ... these typically have tonic clonic manifestations remember initially can have auras like burning smell.

myclonus

4 categories: physyiologic essential epileptic symptomatic essential occurs in isolation w/out other neurologic sxs--improves w/ EtOH. Tx: clonazepam or valproic acid Symptomatic can be due to uremia, hypercapnia, liver failure, wilson's, creutzfeld-jakob, hypoxic brain injury.

Narcolepsy/Cataplexy

4 components: 1) excessive daytime drowsiness w/ narcolepsy 2) cataplexy 3) sleep paralysis 4) hypnagogic hallucinations/hypnopompic hallucinations due to loss of orexin/hypocretin secreting neurons. hypocretin will be <100 pg/mL in CSF (diagnostic). or Dx w/ PSG w/ mutiple sleep level test w/ several short naps. monitor latency to sleep onset and REM sleep. REM latency <8 min w/ >2 episodes is diagnostic. Tx: modafinil cataplexy tx: TCA (clomipramine) or SNRI/SSRI or GABA metabolite *sodium oxybate*.

Temporal Arteritis, complications

50% can get PMR, but pt is usually already on steroid (which is the treatment for PMR - rare to get) Look out for Gluc ind complications: Osteoperosis, DM, Myopathy (prox m. weakness but no TTP)

isocortex (neocortex)

6 layers, most of cortex -afferents to 3 and 4 -efferents to cortex: 3, to thalamus: 6, to BG/SC/BS: 5

Large cerebellar hemorrhage manifestations other than cerebellar signs

6th nerve paralysis, conjugate deviation and blepharospasm

normal Post Void Residual

< 50 mL

Migraine

? AD inheritance (incomplete penetrance) Mc presents WITHOUT aura, ~1-2x/month, females. If aura - bright, flashing lights (10-20min). Can correlate to menses, 2days before or the last day (ma have to do w estrogen W/D) May be assoc w low serotonin - look for relation to cheese, wine, chocolate. U/L HA - 4-72 hrs per episode. rx - 1st line: NSAIDs 2nd line - Sumatriptans or DHE (dihydroergotamine 5HT1 agonist. CI in CAD, TIAs, PVD, and preggos) Prophylaxis if weekly episdoes: Amitriptyline and Propanolol (MUST BE TAKEN DAILY). 2nd line - Verapamil.

Neuro 19 A 53-year-old woman is evaluated in the office for a 1-week history of paresthesias that began symmetrically in the feet and progressed to involve the distal legs and, more recently, the hands. She says she is unsteady when walking, has lower limb weakness, and has difficulty going upstairs. The patient has no history of pain or bowel or bladder impairment. Personal and family medical history is noncontributory, and she takes no medications. On physical examination, vital signs are normal. Weakness of distal lower extremity muscles is noted, with stocking-glove sensory loss and areflexia. Deep tendon reflexes are absent. Plantar responses are flexor, and gait is unsteady. No sensory level is present across the thorax. Mental status, language, and cranial nerve function are normal. Complete blood count results, erythrocyte sedimentation rate, serum creatinine and creatine kinase levels, and liver chemistry test results are normal. A chest radiograph shows no abnormalities. Which of the following is the most appropriate next diagnostic test? A Electromyography B MRI of the spinal cord C Serologic testing for West Nile virus D Sural nerve biopsy

A Guillain-Barré syndrome is a disorder associated with rapidly progressive extremity weakness, paresthesias, and areflexia. This patient should undergo electromyography (EMG). She has a rapidly progressive disorder affecting the peripheral nervous system, most compatible with a clinical diagnosis of Guillain-Barré syndrome. Patients with Guillain-Barré syndrome typically develop paresthesias distally in the lower extremities that are followed by limb weakness and gait unsteadiness. In addition to sensory loss and limb weakness, deep tendon reflexes are characteristically absent or markedly reduced. The diagnosis is confirmed by EMG, which usually shows a demyelinating polyradiculoneuropathy. Cerebrospinal fluid (CSF) analysis characteristically shows albuminocytologic dissociation, whereby the spinal fluid cell count is normal but the spinal fluid protein level is elevated. CSF analysis may also yield normal results early in the course of the disease. However, a normal CSF cell count is useful in excluding other infectious conditions, such as polyradiculoneuropathies associated with HIV and cytomegalovirus infection, infection due to West Nile virus, and carcinomatous or lymphomatous nerve root infiltration. By definition, symptoms in patients with Guillain-Barré syndrome peak within 4 weeks of onset. Poor prognostic features include rapid symptom progression, respiratory failure, EMG evidence of axonal degeneration, and advanced age. Intravenous immune globulin and plasma exchange are equally efficacious in the treatment of Guillain-Barré syndrome. MRI of the spinal cord would be inappropriate as the next diagnostic test. The patient's presentation of distal extremity sensory loss with areflexia suggests a disorder of the peripheral nervous system. A spinal cord lesion (myelopathy) would be an unlikely cause of the symptoms noted on clinical examination. The absence of bowel or bladder impairment, the lack of a sensory level across the thorax, and the upper and lower limb areflexia argue against a central nervous system disorder affecting the spinal cord. West Nile virus infection should be considered in every patient with symptoms of extremity weakness that begin acutely and progress over days to weeks. However, the presence of paresthesias and sensory loss on examination are not typical of West Nile virus infection. This enteroviral illness affects the anterior horn cells, causing limb weakness in the absence of sensory loss. Most cases of West Nile virus infection cause only minor symptoms that are indistinguishable from those of other viral illnesses. Biopsy of the sural nerve is considered in the diagnostic evaluation of patients with a marked peripheral neuropathy of unknown cause. The sural nerve is a sensory nerve that supplies sensation to the lateral distal leg and lateral aspect of the foot. Sural nerve biopsy is appropriate in patients with suspected vasculitis or amyloidosis and occasionally in patients with chronic inflammatory demyelinating polyradiculoneuropathy; it is also used to exclude neuropathic conditions resulting from neoplastic infiltration or other inflammatory conditions, such as sarcoidosis. Symmetric signs and symptoms and diffuse areflexia are not typical of vasculitis. The acute onset and rapid symptom progression would argue against amyloidosis, chronic inflammatory demyelinating polyradiculoneuropathy, or other infiltrating peripheral nerve disorders. Sural nerve biopsy is not necessary in patients with suspected Guillain-Barré syndrome.

Neuro 9 An 88-year-old woman is evaluated in the emergency department 1 hour after the acute onset of language disturbance and right-sided weakness. The family members who witnessed the onset say that the symptoms progressed over a few minutes and that there were accompanying symptoms of nausea and vomiting; they describe the patient holding her head as if in pain. She has a 20-year history of hypertension but no other medical problems; her only medication is lisinopril. During the examination, the patient becomes increasingly difficult to arouse and vomits repeatedly. She is afebrile, blood pressure is 220/110 mm Hg, pulse rate is 110/min, and respiration rate is 20/min. There is no nuchal rigidity. Carotid upstrokes are normal; there are no bruits and no jugular venous distention. Other than tachycardia, the cardiopulmonary examination is unremarkable. Global aphasia and right hemiplegia are noted. On the basis of her preliminary clinical evaluation, which of the following is the most likely diagnosis? A Intracerebral hemorrhage B Ischemic stroke C Meningitis D Transient ischemic attack

A The classic presentation of intracerebral hemorrhage is the sudden onset of a focal neurologic deficit with subsequent symptomatic progression over minutes to hours. This patient most likely has had an intracerebral hemorrhage. The classic presentation of an intracerebral hemorrhage is the sudden onset of a focal neurologic deficit with subsequent symptomatic progression over minutes to hours. Headache, vomiting, hypertension, and an impaired level of consciousness are its most common clinical accompaniments and help distinguish an intracerebral hemorrhage from an ischemic stroke. Intracranial hemorrhage, which accounts for 11% of stroke deaths, has symptoms similar to those of ischemic stroke at presentation; therefore, it cannot always be reliably distinguished from ischemic stroke by clinical criteria alone. The definitive diagnostic study is CT of the head or MRI of the brain. Imaging provides diagnostic and prognostic information. The volume of intracranial hemorrhage and level of consciousness are the two most powerful predictors of outcome in this setting. A severe hemispheric ischemic stroke can cause severe focal neurologic dysfunction but generally does not progress or worsen as rapidly as occurred with this patient. Meningitis, although sometimes fulminant, is rarely this acute in presentation. As with subarachnoid hemorrhage, it may be associated with nuchal rigidity. Encephalitis, but not meningitis, can cause cerebral dysfunction, such as aphasia. A transient ischemic attack is a brief episode of focal neurologic dysfunction, without progressive worsening, that usually lasts less than 30 minutes and so is unlikely to be the correct diagnosis in this patient.

Neuro 68 A 24-year-old woman is evaluated in the office for an 8-week history of severe daily headache. The headache is generalized and throbbing in quality and is associated with intermittent nausea and vomiting, episodes of visual blurring provoked by coughing and laughing, and the perception that she can hear her heartbeat. The headache has been unresponsive to oral administration of two different triptans and to a 1-month course of amitriptyline. The patient has a history of migraine without aura since age 13 years. Her only current medication is ibuprofen, as needed. On physical examination, her temperature is 36.4 °C (97.5 °F), blood pressure is 128/80 mm Hg, pulse rate is 88/min, respiration rate is 20/min, and BMI is 34. She has impaired lateral gaze with the left eye, indistinct disc margins, and no retinal venous pulsations. There is no impairment of visual acuity or visual fields to confrontation. Results of a complete blood count, liver chemistry studies, and measurements of serum creatinine, electrolyte, and thyroid-stimulating hormone levels are normal. An MRI of the brain and magnetic resonance venography of the brain show no abnormalities. Lumbar puncture reveals an opening pressure of 380 mm H2O. Results of cerebrospinal fluid analysis are normal. Which of the following is the most appropriate treatment at this time? A Acetazolamide B Nortriptyline C Propranolol D Sumatriptan, subcutaneously E Valproic acid

A Acetazolamide is the option of first choice for the medical treatment of idiopathic intracranial hypertension. This patient should be treated with acetazolamide. She has idiopathic intracranial hypertension, a disease that primarily affects young obese women. She has a progressive daily headache associated with pulsatile tinnitus and transient visual obscurations, the most common symptoms of this disease. She also has papilledema and impaired lateral gaze to the left on presentation. These signs and symptoms suggest the possibility of a left sixth cranial nerve palsy. Abducens nerve palsies are sometimes seen in patients with idiopathic intracranial hypertension because of compression of the sixth cranial nerve as a result of elevated intracranial pressure. Definitive diagnosis is established by an elevated cerebrospinal fluid (CSF) pressure and normal results on CSF analysis. Because there is not yet evidence of visual field or visual acuity impairment, urgent surgical intervention is not necessary and medical therapy is appropriate. However, all patients with suspected idiopathic intracranial hypertension must undergo a thorough ophthalmologic evaluation, including formal visual perimetry testing, to detect enlargement of the blind spots or visual field defects that are not detected by confrontation visual field testing at the bedside. Acetazolamide is the medical option of first choice for the treatment of idiopathic intracranial hypertension. Although its exact mechanism of action is unclear, acetazolamide is a carbonic anhydrase inhibitor that decreases the production of CSF and relieves intracranial hypertension. No evidence suggests that nortriptyline, propranolol, and subcutaneously administered sumatriptan are appropriate treatments of idiopathic intracranial hypertension. Nortriptyline also has the potential adverse effect of weight gain, which could worsen the clinical course in this patient. Valproic acid is approved by the U.S. Food and Drug Administration for the preventive treatment of migraine. Although this patient has a history of migraine and her current headache is associated with features often seen during acute migraine attacks (nausea, emesis, severe headache, throbbing quality), the headache does not otherwise resemble migraine because it is persistent, progressive, unresponsive to triptan medications, and associated with some abnormal findings on examination. Valproic acid is therefore not appropriate in this circumstance, especially with its potential for weight gain as an adverse effect.

Neuro 31 A 31-year-old woman is evaluated for an 8-month history of diurnal fatigue. She has a 3-year history of relapsing-remitting multiple sclerosis (MS) treated with interferon. The fatigue, which is worse in the early afternoon, has been present since her last MS attack, from which she has otherwise recovered completely. The patient, who has two young children, reports no current problems with sleep or mood and no interferon-related side effects. She exercises regularly. She has a history of depression and hypothyroidism. Current medications are interferon beta-1a and levothyroxine. On physical examination, temperature is 36.9 °C (98.4 °F), blood pressure is 95/70 mm Hg, pulse rate is 76/min, and BMI is 23. Results of general physical and neurologic examinations are normal. Results of laboratory studies are also normal, including a complete blood count, erythrocyte sedimentation rate, liver chemistry studies, and serum folate, thyroid-stimulating hormone, and vitamin B12 levels. Which of the following is the most appropriate next step in treatment? A Amantadine administration B Corticosteroid therapy C Discontinuation of the interferon beta-1a D Vitamin B12

A Adequate rest and regular physical exercise can reduce multiple sclerosis-related fatigue, but many patients require treatment with stimulants, such as amantadine. This patient should receive a course of amantadine. Fatigue is the most common symptom of multiple sclerosis (MS) but is also highly prevalent in the general population. The fact that the patient's fatigue began with a relapse strengthens its association with her MS, but evaluation for other causes is still required. Her review of systems, physical examination, and laboratory studies do not reveal another likely cause, and she is already sleeping and exercising regularly. Therefore, pharmacologic therapy can reasonably be offered. Amantadine has been shown in several small placebo-controlled studies to reduce MS-related fatigue. The mechanism is not clearly understood but is likely related to the drug's stimulant properties. Although a brief course of corticosteroids may provide a temporary boost of energy, this strategy is not a practical solution because the beneficial effects will by definition be brief. Longer-term use of corticosteroids is also not advised because of the high risk of adverse effects. Discontinuation of interferon beta-1a is unnecessary because the patient does not perceive any adverse effects from the drug. Flu-like symptoms and fatigue related to interferon beta-1a are usually temporally related to individual injections. Although fatigue can also be related to vitamin B12 deficiency, injection of the vitamin is not indicated for this patient because the patient's physical examination and normal laboratory values show that she is not vitamin B12 deficient.

Neuro 79 A 70-year-old man is seen in the office for routine follow-up of partial seizures that began 2 years ago after a stroke. At that time, he was started on phenytoin, 300 mg/d, and has had no subsequent seizures; he tolerates the medication well, with no reported adverse effects. His current medications are phenytoin, an angiotensin-converting enzyme inhibitor, a statin, and aspirin. Results of physical examination, including a neurologic examination, are normal. Laboratory studies show a total serum phenytoin level of 9 mg/L (35.6 µmol/L) (therapeutic range, 10-20 mg /L [39.6-79.2 µmol/L]). Results of a complete blood count and liver chemistry tests are normal. Which of the following is the most appropriate next step in management? A Continue the phenytoin at the current dosage B Increase the phenytoin dosage C Measure the free serum phenytoin level D Substitute gabapentin for the phenytoin

A Adjustments to the dosage or type of antiepileptic drug used by a patient with epilepsy should be based on clinical seizure control and drug side effects rather than strict serum drug levels. The most important considerations that should guide decisions about adjusting epilepsy medication dosage or changing the type of drug used are the current clinical seizure control and the presence of adverse side effects. This patient reports a prolonged period of freedom from seizures and the absence of adverse effects while on a stable dosage of phenytoin. Although the patient's total serum phenytoin level is mildly subtherapeutic, this in itself is not a sufficient indication to make a change in the type or dosage of medication. Therefore, the patient should be kept on phenytoin at his present dosage of 300 mg/d as long as a seizure or adverse effect of medication does not occur. Monitoring of serum antiepileptic drug levels can be useful when it is necessary to confirm that a level is therapeutic in a patient with uncontrolled seizures, when toxicity is suspected, or when medication adherence needs to be confirmed; none of these scenarios applies to this patient. Serum drug level monitoring can be particularly helpful with phenytoin because of its narrow therapeutic window and its nonlinear pharmacokinetics, which can result in unexpectedly large changes in serum concentration from small adjustments in oral dosage. For phenytoin and other highly protein-bound drugs, checking the free (as well as the total) serum drug levels may be necessary if there is concern about therapeutic effectiveness or toxicity in the presence of a low serum protein level or polypharmacy with other highly protein-bound drugs; again, these indications do not apply to this patient.

Neuro 62 A 78-year-old man is re-evaluated after a 5-mm left middle cerebral artery aneurysm is discovered incidentally on an MRI of the brain obtained because of a previous symptom of extreme dizziness, which has since resolved. The patient has no other relevant personal or family medical history and takes no medications. On physical examination, blood pressure is 138/82 mm Hg, pulse rate is 80/min, and respiration rate is 18/min. Results of physical examination, including neurologic examination, are normal. A magnetic resonance angiogram (MRA) shows a solitary unruptured aneurysm but no additional intracranial aneurysms. No hemorrhage, infarction, mass, or mass effect is evident. Which of the following is the most appropriate next step in the management of this patient's aneurysm? A Annual MRA B Endovascular coiling of the aneurysm C Nimodipine administration D Surgical clipping of the aneurysm

A Annual magnetic resonance or CT angiography to monitor aneurysmal growth is appropriate as management of a low-risk unruptured intracranial aneurysm. This patient should have an annual magnetic resonance angiogram (MRA) or CT angiogram to monitor aneurysmal growth. For patients without a prior subarachnoid hemorrhage, the lowest-risk aneurysms are those in the anterior circulation and less than 7 mm in diameter. The annual risk of rupture for an aneurysm of the size of this patient's is 0.05% annually. The risk of neurologic disability associated with intervention exceeds the potential benefit. After 3 successive years of annual monitoring, an MRA or CT angiogram obtained once every 3 years is sufficient. The second report from the International Study of Unruptured Intracranial Aneurysms was a prospective observational study of patients who either underwent open or endovascular repair of asymptomatic intracranial aneurysms. Open surgery was associated with surgery-related death or poor neurologic outcome of nearly 13% at 1 year, compared with approximately 10% for endovascular repair. Complication rates increased with increasing age (30% at age 70 years or older), aneurysm size, and location of the aneurysm in the posterior circulation. Because the complication rate of intervention is likely to exceed the complication rate of observation alone, neither clipping nor coiling is indicated. Nimodipine is administered routinely in patients with subarachnoid hemorrhage in order to prevent vasospasm. This drug is not appropriate for a patient with an unruptured intracranial aneurysm. It is generally recommended that patients should refrain from smoking, heavy alcohol consumption, amphetamines, cocaine, and excessive straining and the Valsalva maneuver when taking this drug.

Neuro 54 A 56-year-old man is evaluated in the office for a 1-month history of intermittent weakness of the left foot, a 6-month history of progressive right arm weakness, and 1-year history of muscle cramps. He says he feels lately as if he is "catching" his foot on things when ambulating. He has noticed no shortness of breath, dysphagia, or other bulbar symptoms and reports no other pain, sensory loss, or bowel or bladder impairment. The patient is otherwise healthy, has no history of disease, and is unaware of any family history of neurologic disorders. He takes no medications. Results of a general medical examination are normal. Neurologic examination reveals normal speech, language, and mental status. His tongue appears atrophic with fasciculations. He has diffuse weakness and atrophy of the proximal muscles in the right arm; fasciculations are noted. Left arm strength and muscle bulk are normal. Moderate weakness of the distal muscles in the left leg is noted, with fasciculations present in both lower extremities. Deep tendon reflexes are brisk in the upper and lower limbs, and the plantar response is extensor bilaterally. Sensory examination reveals no abnormalities, and there is no appendicular ataxia. Laboratory studies show a serum creatine kinase level of 602 U/L. Results of a complete blood count; measurement of serum creatinine, electrolyte, and vitamin B12 levels; and liver chemistry studies are normal. A radiograph of the chest shows no abnormalities. Which of the following is the most likely diagnosis? A Amyotrophic lateral sclerosis B Cervical myelopathy C Chronic inflammatory demyelinating polyradiculoneuropathy D Primary lateral sclerosis

A Both upper and lower motoneuron findings are typically present in amyotrophic lateral sclerosis, which helps distinguish this disorder from its mimickers, such as multifocal motor neuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, and primary lateral sclerosis. Amyotrophic lateral sclerosis (ALS) is the most likely diagnosis in this patient. Patients with ALS typically have progressive, asymmetric, painless extremity or bulbar weakness on presentation. The absence of sensory loss and the lack of bowel or bladder impairment are also suggestive of ALS. The combination of upper motoneuron findings (hyperreflexia, extensor plantar response) and lower motoneuron signs (atrophy, fasciculations) seen on neurologic examination strongly suggests ALS, which is a fatal, neurodegenerative motoneuron disease that affects both the upper and lower motoneurons. The term motoneuron disease is used to describe the heterogeneous group of disorders affecting the upper motoneuron, the lower motoneuron, or both; for example, progressive muscular atrophy is a motoneuron disease that affects the lower motoneuron, and primary lateral sclerosis is a motoneuron disease that affects the upper motoneuron. It is unclear whether different motoneuron disorders are distinct disorders or reflect different manifestations of a single disease. What is established, however, is that ALS has the worst prognosis of them all, with a mean survival of 3 to 5 years. Cervical myelopathy should be considered in patients presenting with arm and leg weakness but is an unlikely cause of this patient's symptoms. The presence of tongue atrophy and fasciculations, absence of sensory loss, and lack of bowel or bladder impairment would not be typical of a cervical myelopathy. Nonetheless, MRI studies are indicated in patients with suspected ALS to exclude myelopathy. Chronic inflammatory demyelinating polyradiculoneuropathy is a treatable neurologic condition that causes weakness, sensory loss, and depressed deep tendon reflexes. The absence of sensory loss and the hyperreflexia seen in this patient are not typical of this disorder. Chronic inflammatory demyelinating polyradiculoneuropathy is an immune-mediated, inflammatory disorder that causes demyelination of peripheral nerves and nerve roots. An elevated cerebrospinal fluid protein level, characteristic findings on an electromyogram showing demyelination and conduction block, or a sural nerve biopsy can establish the diagnosis. Chronic inflammatory demyelinating polyradiculoneuropathy is a neurologic disorder that is expected to respond to immune-modulating therapy. Corticosteroids, intravenous immune globulin, plasma exchange, mycophenolate mofetil, and azathioprine have been the primary therapies used in its treatment. Primary lateral sclerosis is not the diagnosis in this patient, given the lower motoneuron findings of atrophy and fasciculations on examination. A motoneuron disorder affecting the upper motoneuron, primary lateral sclerosis causes slowly progressive weakness and spasticity. In affected patients, spasticity predominates over weakness and is typically symmetric and lower limb predominant. Later in the disease course, most patients develop pseudobulbar features, with dysarthria and emotional lability. Primary lateral sclerosis is a clinical diagnosis that can be established only by excluding other diseases. Spinal cord MRIs are particularly useful in this regard. Primary lateral sclerosis is a slowly progressive neurodegenerative disorder with a much better prognosis than ALS; long-term survival is expected. There are no medications approved by the U.S. Food and Drug Administration for the treatment of primary lateral sclerosis.

Neuro 50 A 68-year-old man is admitted to the hospital for evaluation of a transient 15-minute episode of left facial droop, slurred speech, and left arm weakness. Three years ago, the patient had a radical neck dissection as treatment of head and neck cancer and subsequently had radiation therapy. He also has hypertension and hyperlipidemia and has a 40-pack-year smoking history. Family history is noncontributory. Current medications are lisinopril, atorvastatin, and aspirin. On physical examination, temperature is normal, blood pressure is 156/88 mm Hg, pulse rate is 88/min, and respiration rate is 14/min. A right carotid bruit is heard on auscultation. No abnormal findings are noted on neurologic examination. Results of laboratory studies are normal. An MRI of the brain reveals a small wedge-shaped, cortical diffusion-weighted positive region of signal change occupying the right hemisphere. A magnetic resonance angiogram of the neck shows 80% stenosis of the right internal carotid artery. In addition to aspirin therapy, which of the following is the most appropriate next step in treatment? A Carotid angioplasty and stenting B Carotid endarterectomy C External carotid to internal carotid artery bypass surgery D Intravenous administration of heparin

A Carotid angioplasty and stenting should be used in patients with symptomatic severe (>70%) internal carotid artery stenosis who are not eligible for surgical treatment with carotid endarterectomy. This patient, who has had an ischemic stroke and has symptomatic severe internal carotid artery stenosis (>70% stenosis), should undergo carotid angioplasty and stenting. Although carotid endarterectomy is still considered the gold standard of surgical therapies for patients with such stenosis, it cannot be performed in those who have stenosis that is difficult to access surgically (above the C2 level), medical conditions that greatly increase the risk of surgery, or other specific conditions, such as radiation-induced stenosis or restenosis after carotid endarterectomy. For such patients, the less invasive combination of carotid angioplasty and stenting is preferable. The U.S. Food and Drug Administration has approved carotid angioplasty and stenting for patients with symptomatic severe carotid artery stenosis who are classified as high surgical risk or who have unfavorable anatomy that precludes a surgical approach. Other candidates for nonsurgical treatment of severe stenosis include patients with a history of radical neck surgery, spinal immobility, dissection, an ostial lesion below the clavicle, the presence of a tracheostomy stoma, and contralateral laryngeal nerve paralysis. External carotid to internal carotid artery bypass surgery was shown not to be effective for the surgical treatment of carotid artery stenosis, but this surgery may be considered for symptomatic carotid artery occlusion (that is, 100% occlusion). Studies of unfractionated and low-molecular-weight heparin have not shown any benefit for the vast majority of patients with acute ischemic stroke. Overall, the small reduction in recurrent ischemic stroke associated with anticoagulants is counterbalanced by an increase in hemorrhagic strokes. The American Heart Association and the American Academy of Neurology recommend that patients with ischemic stroke should not be treated with anticoagulation. Therefore, heparin administration is not appropriate for this patient.

Neuro 86 A 24-year-old woman is evaluated for a 2-month history of abnormal movements affecting both arms. She describes these movements as intermittent and irregular writhing movements and says she cannot suppress them. The patient had an unprovoked deep venous thrombosis in her right leg 2 years ago and a miscarriage 8 months ago but has had no other medical problems. She has no known family history of neurologic disease or thrombophilia and takes no medications. General physical examination findings are normal. Neurologic examination reveals random, irregular movements of the hands and arms but is otherwise unremarkable. Which of the following is the most likely diagnosis? A Anti-phospholipid antibody syndrome B Huntington disease C Parkinson disease D Wilson disease

A Chorea can occur in patients with anti-phospholipid antibody syndrome or systemic lupus erythematosus. This patient most likely has anti-phospholipid antibody syndrome. She has movements consistent with chorea, with no other identifiable neurologic signs or symptoms. Chorea is characterized by random, nonstereotyped movements that can affect virtually any body part. Anti-phospholipid antibody syndrome is characterized by a history of a thrombotic event (including recurrent fetal loss) in association with a persistent lupus anticoagulant or persistently elevated levels of anticardiolipin or B2-glycoprotein I antibodies. Lupus anticoagulants or elevated levels of anti-phospholipid antibodies are often present in patients with systemic lupus erythematosus; they also occur in patients with cancer or infections and in association with the use of certain drugs (for example, hydralazine, procainamide, phenothiazines). A multitude of neurologic disorders can occur in patients with anti-phospholipid antibody syndrome, including chorea. Chorea has also been associated with systemic lupus erythematosus and may occur early in the course of either disorder, even before other signs develop. Levels of anticardiolipin antibodies, antinuclear antibodies, and anti-double-stranded DNA antibodies should be measured in the diagnostic evaluation of chorea. Huntington disease is not likely in this patient. A progressive, autosomal dominant disorder, Huntington disease has a mean age of onset of approximately 40 years. A history of psychiatric disturbance may precede the motor manifestations of the disease, and cognitive abnormalities are present in nearly all affected patients, appearing early in the course of the disease. Chorea is also present in nearly all patients, and other extrapyramidal findings, such as dystonia and parkinsonian signs, are commonly seen. Chorea commonly occurs in patients with Parkinson disease who are on dopamine therapy and is thought to reflect high levels of dopamine in the basal ganglia. Typically, these choreiform movements, also known as dyskinesias, occur at the peak of the dopamine medication effect and, if mild, may not be bothersome to the patient. Given that this patient has no symptoms of Parkinson disease, such as a resting tremor, and is not on medication, this diagnosis is very unlikely. Wilson disease is an autosomal recessive disorder that most commonly results in psychiatric symptoms and parkinsonian signs, neither of which are present in this patient. Chorea occurs in approximately 15% of patients with Wilson disease.

Neuro 23 A 60-year-old man is admitted to the hospital for generalized tonic-clonic seizures that began 30 minutes ago. Clinical seizure activity continues for another 60 minutes, during which time the patient is intubated, placed on a ventilator, and given lorazepam and fosphenytoin, both intravenously. After his seizures stop, he is transferred to the intensive care unit, where he remains comatose. The patient has been receiving chemotherapy and whole-brain radiation therapy for recently diagnosed small cell lung cancer. He indicated at the time of his diagnosis that he wants everything possible done to prolong his life ("full code"). On examination, the patient is comatose, with no response to deep pain stimulation. Cranial nerve examination is significant for reactive pupils. Results of laboratory studies are noncontributory. Other than sinus tachycardia, the electrocardiogram is unremarkable. Which of the following is the most appropriate next step in management? A Continuous electroencephalographic monitoring B Discussion of withdrawal of care with the family C Intravenous phenobarbital D MRI of the brain

A Continuous electroencephalographic monitoring is indicated for patients who remain unresponsive after resolution of clinical status epilepticus to distinguish nonconvulsive status epilepticus from a postictal state. This patient should undergo continuous electroencephalographic monitoring. Generalized convulsive status epilepticus, which this patient initially exhibited, is a neurologic emergency with significant morbidity and mortality. Complications include hemodynamic instability, acidosis, rhabdomyolysis, pulmonary edema, respiratory failure, and irreversible brain injury. His early treatment with lorazepam was appropriate as first-line therapy. Over time, the clinical manifestations of status epilepticus can become increasingly subtle, but the electrical seizures can continue unabated, a state referred to as subtle or nonconvulsive status epilepticus. Motor manifestations may be limited to rhythmic muscle twitching in the face or eyes or to low-amplitude multifocal myoclonus in an obtunded or comatose patient. The unwary or inexperienced observer may mistake subtle generalized convulsive status epilepticus for postictal obtundation. Failure to diagnose and treat ongoing status epilepticus increases the risk of an adverse outcome, even with ventilatory and critical care support. Ongoing electrical seizure activity will lead to increasing physiologic derangement and neuronal injury. In this patient and others who remain unresponsive or have persistent altered mentation after treatment for clinical status epilepticus, it may be impossible to distinguish on clinical grounds alone the effect of ongoing electrical seizure activity from postictal lethargy or the side effects of medication. For this reason, continuous electroencephalographic monitoring is strongly advocated for optimal management. If ongoing status epilepticus is demonstrated, additional intravenous antiepileptic drugs should be administered at doses sufficient to terminate the electroencephalographic seizures. Withdrawal of care at this point is premature because the patient has not yet had an adequate evaluation. Adding phenobarbital or other medications to this patient's drug regimen carries the risk of complications, such as hypotension, and thus would not be the best choice, unless the patient were still experiencing seizures. Obtaining an MRI of the brain should never delay other necessary diagnostic tests or therapies required to treat the status epilepticus. MRI is also unlikely to provide useful additional information in this patient whose cerebral metastasis constitutes a known underlying cause of his seizure activity.

Neuro 57 A 45-year-old woman is admitted to the hospital with mild left hemiplegia, left hemineglect, and dysarthria. A CT scan of the head reveals a large right hemispheric infarction due to an occluded right middle cerebral artery. She has a history of anti-phospholipid antibody syndrome diagnosed 3 years ago after an episode of iliofemoral venous thrombosis. She was treated for 18 months with warfarin but elected to discontinue treatment. There is no relevant family history. Her only medication is aspirin. Forty-eight hours later, a progressive deterioration of her mental status is noted. The patient had previously stated that she wants everything possible done to prolong her life ("full code"). Examination now shows that she has severe left hemiplegia and that her head and eyes are deviated to the right. A repeat CT scan of the head reveals a large hypodense region occupying the entire right middle cerebral artery territory, a local mass effect, and a 7-mm midline shift from right to left. Which of the following is the most appropriate therapy at this time? A Decompressive hemicraniectomy B Intra-arterial thrombolysis C Intravenous heparin D Use of an endovascular mechanical clot-retrieval device

A Decompressive surgery can be a life-saving intervention in a patient who develops malignant brain edema after a hemispheric stroke. This patient has malignant brain edema as a result of her ischemic stroke and should be treated with decompressive hemicraniectomy. Young patients with major infarctions affecting the cerebral hemisphere or cerebellum have a heightened risk of brain edema and increased intracranial pressure. Reducing any edema and close monitoring for signs of neurologic worsening, particularly during the first 3 to 5 days after the stroke when the edema maximizes, are recommended. Meta-analyses of randomized, controlled trials have shown that decompressive hemicraniectomy for malignant stroke reduces morbidity and mortality. Intra-arterial thrombolysis is an option for the treatment of selected patients who have had a major stroke within the past 6 hours due to occlusion of a major intracranial artery. Although this patient's stroke was caused by an occluded right middle cerebral artery, she is long past the time window for this acute stroke therapy. Delay is associated with an increased risk of hemorrhagic conversion and reduced symptomatic benefit. Long-term anticoagulation may play a role in the prevention of recurrent ischemic stroke in a patient with anti-phospholipid antibody syndrome. However, acute intravenous administration of heparin in this patient who has a large acute cerebral infarction is more likely to accelerate the possibility of hemorrhagic conversion of the infarction and thus to worsen her current clinical state. The endovascular mechanical clot-retrieval device has been used to extract thrombi from occluded intracranial arteries. However, guidelines recommend that such a device must be used within 8 hours of a stroke. This patient is now beyond that 8-hour time window.

Neuro 90 A 69-year-old woman is evaluated in the emergency department for the sudden onset of headache, nausea, vomiting, and imbalance. The patient has hypertension for which she takes lisinopril. She has no other relevant personal or family medical history. On physical examination, blood pressure is 160/90 mm Hg, pulse rate is 80/min, and respiration rate is 18/min. Her Glasgow Coma Scale score is 12 (of a possible 15), which indicates moderate brain injury. Right finger-to-nose and heel-to-shin testing reveals dysmetria. Results of laboratory studies, including a complete blood count, a metabolic profile, and coagulation tests, are normal. A CT scan of the head shows an acute right cerebellar hemorrhage (>4 cm in diameter), perihematoma edema, compression of the right pons, and effacement but no compression of the fourth ventricle. Which of the following is the most appropriate next step in management? A Hematoma evacuation B Labetalol, intravenously C Recombinant factor VII, intravenously D Ventriculostomy

A Emergent surgical evacuation of the hematoma is indicated for patients with a cerebellar hemorrhage greater than 3 cm in diameter who are deteriorating neurologically or who have brain stem compression and/or hydrocephalus from ventricular obstruction. This patient has signs of neurologic deterioration, given her score on the Glasgow Coma Scale. She has an acute cerebellar hemispheric hemorrhage that is greater than 3 cm in diameter and thus should be considered for neurosurgical intervention. The priority in such cases is to decompress the posterior fossa by surgically evacuating the hematoma. Patients with posterior fossa hematomas are at risk for life-threatening complications, including herniation and hydrocephalus, without such intervention. Blood pressure management in intracranial hemorrhage remains controversial. No trial has demonstrated that blood pressure control in this setting affects outcome, and there is concern about reducing cerebral perfusion pressure in patients with elevated intracranial pressure. The American Heart Association guidelines recommend that mean arterial blood pressure be kept between 70 mm Hg and 130 mm Hg. This patient's mean arterial blood pressure is 113 mm Hg, and thus intravenous administration of labetalol is not indicated. More importantly, urgent evacuation of the hematoma is likely to be life-saving and thus is the treatment of choice. Recombinant factor VII was promising as an experimental therapy for acute intracerebral hemorrhages, but the definitive phase 3 trial did not yield efficacious results. It is not approved by the U.S. Food and Drug Administration for treatment of such hemorrhages. A ventriculostomy may be indicated in a patient with a cerebellar hemorrhage who develops hydrocephalus. Because this patient's CT scan does not yet demonstrate that complication, ventriculostomy is inappropriate as the next step in management.

Neuro 11 An 84-year-old man is evaluated for the gradual onset of progressive memory loss over the past 2 years. In the past 4 months, he has twice been unable to find his way home after going to the local supermarket; his wife now goes with him whenever he leaves the house. His wife also has assumed responsibility for the household finances after the patient overdrew their checking account for the third time because of subtraction errors in their checkbook. He has hypertension treated with hydrochlorothiazide and hypothyroidism treated with levothyroxine. His mother had onset of Alzheimer dementia at age 79 years and died at age 86 years. His only other medication is a daily multivitamin. On physical examination, temperature is 36.9 °C (98.4 °F), blood pressure is 130/80 mm Hg, pulse rate is 72/min, respiration rate is 14/min, and BMI is 25. His level of alertness, speech, and gait are normal. His score on the Folstein Mini-Mental State Examination is 24/30, including 0/3 on the recall portion. Results of laboratory studies, including a complete blood count, serum vitamin B12 measurement, thyroid function tests, and a basic metabolic panel, are normal. An unenhanced MRI of the brain shows no abnormalities. Which of the following is the most appropriate treatment at this time? A Donepezil B Memantine C Quetiapine D Sertraline E Discontinuation of all current medications

A First-line pharmacotherapy for mild Alzheimer dementia is an acetylcholinesterase inhibitor. This patient should receive donepezil. The Folstein Mini-Mental State Examination (MMSE) discriminates well between the major stages of dementia used for prognosis and management purposes. The MMSE score range of 21 to 25 corresponds to mild dementia, 11 to 20 to moderate dementia, and 0 to 10 to severe dementia. This patient has Alzheimer dementia and is at a mild to moderate stage of impairment. The most appropriate medication with which to begin treatment is an acetylcholinesterase inhibitor of which there are currently three: donepezil, rivastigmine, and galantamine. Multiple large, prospective, randomized, double-blind, placebo-controlled studies have shown in patients with mild, moderate, or severe Alzheimer dementia the efficacy of donepezil (and its superiority to placebo) in the preservation of instrumental and functional activities of daily living and in the reduction of caregiver stress. Other studies have found that patients treated with donepezil have improved cognitive function compared with those treated with placebo. Donepezil was safe and well tolerated in this patient group. Memantine is also used to treat Alzheimer dementia, but only in patients with moderate to severe impairment. There is no evidence that memantine has any effect in earlier stages of Alzheimer dementia or that it alters the course of the disease. With a score of 24/30 on the MMSE, this patient has mild dementia, which makes memantine an inappropriate treatment. In patients with severe dementia, memantine can be used alone or added to an acetylcholinesterase inhibitor. Quetiapine is an antipsychotic drug, and sertraline is an antidepressant agent. Although both can be used in patients with Alzheimer dementia, their use is limited to treatment of behavioral symptoms of psychosis and depression, respectively, neither of which this patient has exhibited. However, if these medications are to be used in such patients, the risks must first be carefully weighed against the benefits. The U.S. Food and Drug Administration has reported that the use of second-generation antipsychotic medications (aripiprazole, olanzapine, quetiapine, and risperidone) in elderly patients with dementia is associated with increased mortality. Although it is important to consider the potential cognitive side effects of prescription (and nonprescription) medications, those taken by this patient are not associated with such effects. Therefore, there is no need to risk potential harm to this patient by discontinuing his blood pressure and thyroid medications.

Neuro 45 A 50-year-old man is admitted to the hospital after having two generalized tonic-clonic seizures in a 24-hour period. He has had seizures in the past, which were always attributed to alcohol withdrawal. Ten years ago, he was in a major motor vehicle collision that was related to alcohol intoxication. He has end-stage liver disease secondary to alcoholic cirrhosis but has been sober for 2 years and is awaiting a liver transplant. His kidney function is normal. His current medications include nadolol, spironolactone, and furosemide. On physical examination, the patient is awake and alert, is afebrile, and has a normal blood pressure and pulse rate. Neurologic examination findings are normal. The general physical examination reveals changes consistent with chronic liver disease, including jaundice and ascites. Laboratory studies show a serum creatinine level of 0.6 mg/dL (45.8 µmol/L) and no blood ethanol. Serum electrolyte levels are normal. An MRI of the brain shows an area of chronic encephalomalacia in the left frontotemporal head region, consistent with old trauma. An electroencephalogram shows left temporal sharp waves. Which of the following is the best treatment for this patient? A Levetiracetam B Oxcarbazepine C Phenytoin D Valproic acid

A For patients who should avoid hepatically metabolized antiepileptic drugs, either because of drug interaction or underlying liver disease, levetiracetam, gabapentin, and pregabalin can be used. Given his clinical history, MRI findings, and electroencephalographic findings, this patient is likely to have epilepsy. Treatment of epilepsy in a patient with an underlying hepatic disease requires careful selection and management of the antiepileptic medication. Levetiracetam, gabapentin, and pregabalin are the preferred choices in patients with significant liver disease because they do not undergo significant hepatic metabolism and have low protein binding. Therefore, levetiracetam is most appropriate for this patient. For antiepileptic drugs that are hepatically metabolized or highly protein bound, alterations of hepatic enzymatic pathways and hypoalbuminemia can result in unexpected drug toxicity. For these reasons, oxcarbazepine, phenytoin, and valproic acid would be less favored options for this patient. Additionally, some antiepileptic drugs should be avoided because of their potential hepatoxicity, particularly valproic acid and felbamate. In patients who have undergone or are expected to undergo organ transplantation, it is particularly important to consider potential drug interactions that might alter the effectiveness of their immunosuppression regimen. Cytochrome P450 enzyme inducers, including phenytoin and oxcarbazepine, and inhibitors, including valproic acid, can be problematic in this population. Highly protein-bound drugs, such as phenytoin, are also best avoided when possible. Levetiracetam, gabapentin, and pregabalin are preferred for these patients because of the lack of significant drug interactions. Because these three medications are renally excreted, the dosage may need to be lowered in the presence of renal insufficiency. Dosing should be based on clinical response rather than the serum drug level because the therapeutic range for these agents is quite broad.

Neuro 76 An 88-year-old man is brought to the emergency department within 45 minutes of the witnessed onset of dysarthria and right face, arm, and leg weakness. Recombinant tissue plasminogen activator is administered intravenously 105 minutes after symptom onset. The patient is then admitted to the intensive care unit, and 4 hours after thrombolysis, his neurologic symptoms and signs are rapidly improving. On physical examination, vital signs are normal, except for a blood pressure of 190/105 mm Hg. There is right pronator drift, right facial droop, and mild residual dysarthria. Which of the following is the most appropriate treatment of this patient's elevated blood pressure? A Intravenous labetalol B Intravenous nitroprusside C Oral nifedipine D Withholding of antihypertensive medications

A In a patient with ischemic stroke treated with recombinant tissue plasminogen activator (rtPA), systolic blood pressure should be kept below 180 mm Hg and diastolic below 105 mm Hg for 24 hours after rtPA treatment; intravenous labetalol or nicardipine can best achieve this goal. In patients who have received recombinant tissue plasminogen activator as treatment of stroke, systolic blood pressure should be maintained below 180 mm Hg and diastolic blood pressure below 105 mm Hg for at least the first 24 hours after thrombolysis treatment. According to current clinical guidelines, intravenous administration of labetalol or nicardipine can best achieve this goal (class II recommendation). Therefore, of the options listed, intravenous administration of labetalol is most appropriate for this patient whose systolic blood pressure is 190 mm Hg and whose diastolic blood pressure is 105 mm Hg. Intravenous administration of nitroprusside should be instituted only if either labetalol or nicardipine proves unsuccessful in controlling this patient's blood pressure. Intravenous nitroprusside is considered second-line therapy because it may be associated with increased intracranial pressure. Oral administration of nifedipine is inappropriate because of its rapid absorption, which can result in a secondary precipitous decline in blood pressure. Withholding antihypertensive medications is inappropriate in this patient. Excessively high blood pressure is associated with an increased risk of symptomatic hemorrhagic transformation after thrombolytic therapy and may be prevented with careful adjustment of the blood pressure to target levels recommended by treatment guidelines.

Neuro 41 A 79-year-old woman is to be transferred from the emergency department to a hospital ward for ongoing care. She awoke at home 5 hours ago with slurred speech, difficulty swallowing food and drink, and left hemiparesis. A right hemispheric ischemic stroke was diagnosed in the emergency department after a CT scan of the head confirmed a right hemispheric infarction. Because the time of stroke onset could not be determined, no recombinant tissue plasminogen activator was administered. The patient has no other medical problems and takes no medications. On physical examination, blood pressure is 168/86 mm Hg, pulse rate is 80/min, and respiration rate is 18/min. Neurologic assessment reveals dysarthria, dysphagia, left facial droop, and left hemiparesis. Laboratory studies show a plasma LDL cholesterol level of 158 mg/dL (4.09 mmol/L) but no other abnormalities. Which of the following is the most appropriate first step in management after transfer is completed? A Bedside screening for dysphagia B Oral administration of an angiotensin-converting enzyme inhibitor C Oral administration of a statin D Physical therapy and rehabilitation consultation

A In a patient with stroke, dysphagia screening should be performed before food, oral medication, or liquids are administered. On admission to a hospital ward, a patient with stroke should be given nothing by mouth (kept NPO) until a swallowing assessment is conducted. Dysphagia screening is especially appropriate for this patient, who had difficulty swallowing when she first awoke with stroke symptoms. Dysphagia occurs in 45% of all hospitalized patients with stroke and can lead to poor outcomes, including aspiration pneumonia and death. Bedside screening of swallowing ability should thus be completed before oral intake of any medication or food; if the screening results are abnormal, a complete examination of swallowing ability is recommended. The American Heart Association/American Stroke Association recommends a water swallow test performed at the bedside by a trained observer as the best bedside predictor of aspiration. A prospective study of the water swallow test demonstrated a significantly decreased risk of aspiration pneumonia of 2.4% versus 5.4% in patients who were not screened. Angiotensin-converting enzyme inhibitors, statins, and aspirin are appropriate treatments for secondary stroke prevention in some patients, but they should not be orally administered before ruling out the risk of aspiration. Like most patients with stroke, this patient will undoubtedly require physical therapy and rehabilitation during her recovery. However, consulting with the department(s) responsible for such care is not an immediate concern and should not be the first step taken when the patient arrives in the hospital ward.

Neuro 55 A 32-year-old man is evaluated in the office for a 6-month history of excruciating headaches, which occur up to 12 times per day and last approximately 10 minutes each. He is pain free between attacks. The pain is centered around and behind the left eye, and each attack is associated with conjunctival injection, lacrimation, and rhinorrhea. The patient has an 8-pack-year smoking history. He takes a combination of acetaminophen, caffeine, and aspirin, usually taking a total of four to six tablets daily. Results of a physical examination, including a neurologic examination, are normal. Which of the following is the most appropriate treatment for this patient's condition? A Indomethacin B Lamotrigine C Prednisone D Verapamil

A Indomethacin is the treatment of choice for paroxysmal hemicrania. This patient should receive indomethacin. He most likely has paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias, which are characterized by pain referred to the first division of the trigeminal nerve and by accompanying cranial autonomic symptoms, including lacrimation and rhinorrhea. An attack of paroxysmal hemicrania has an intermediate duration (mean, 15 minutes) and an intermediate episodic frequency (mean, 11 per day). Treatment with indomethacin can immediately and completely resolve the headache. Usually, the response occurs within the first 48 hours after treatment is initiated. Indomethacin is not effective for treating any of the other trigeminal autonomic cephalalgias. Therefore, a positive response to the drug helps distinguish between paroxysmal hemicrania and the other trigeminal autonomic cephalalgias. Lamotrigine is the most effective drug for the treatment of the syndrome of Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT syndrome), another trigeminal autonomic cephalalgia. There is no evidence supporting its usefulness in the treatment of paroxysmal hemicrania. Prednisone and verapamil are commonly used treatments for cluster headache, yet another trigeminal autonomic cephalalgia. As with lamotrigine, there is no evidence supporting their usefulness in the treatment of paroxysmal hemicrania.

Neuro 26 A 22-year-old man is evaluated in the emergency department for a 12-hour history of mild headache, nausea, and vomiting. His roommate had similar symptoms the previous day. He is given intravenous fluids and prochlorperazine and begins to feel better until his head suddenly becomes stiff and turns to the right; he cannot move it to the midline or to the left, and he reports cramping and aching in the right neck muscles. Neurologic examination shows the head to be turned to the right with sustained contraction of the left sternocleidomastoid muscle but is otherwise unremarkable. Which of the following is the best treatment for this patient? A Benztropine B Botulinum toxin C Phenytoin D Recombinant tissue plasminogen activator E Tetanus immune globulin

A Medications that block dopamine receptors can cause acute dystonic reactions, which can be readily treated with benztropine. This patient is experiencing a prochlorperazine-induced dystonic reaction, which is best treated with benztropine. Acute dystonic reactions are characterized as torticollic (twisted or a turned head, neck, or face), oculogyric (deviated or rolling gaze), buccolingual (pulling sensation of the tongue), or opisthotonic (trunk or entire-body spasm). Medications, such as prochlorperazine, that block dopaminergic receptors in the extrapyramidal system can result in acute dystonic reactions. Intravenous anticholinergic agents, such as benztropine or diphenhydramine, are the treatment of choice in the acute treatment of dystonic reactions. Benzodiazepine medications can also be helpful in the acute setting. Oral anticholinergic medications are continued for 48 to 72 hours to prevent relapse. Cervical dystonia, or spasmodic torticollis, is a chronic condition associated with abnormal head posturing and tremor. Botulinum toxin is often beneficial for this condition. Acute dystonic reactions can be misdiagnosed as seizures, tetanus, stroke, and other conditions. Phenytoin is an antiseizure medication, tissue plasminogen activator is appropriate treatment for strokes under certain conditions, and tetanus immune globulin is administered for tetanus prophylaxis. None of these drugs is indicated for acute dystonic reactions.

Neuro 36 An 81-year-old woman is evaluated in the office for increasing difficulty with activities of daily living, including dressing and feeding herself, over the past 6 months. She has had gradually progressive cognitive decline for the past 5 years and now needs 24-hour help from a caregiver; Alzheimer dementia was previously diagnosed. The patient also has hypertension and hypothyroidism. Her mother had onset of Alzheimer dementia at age 80 years and died at age 87 years. Current medications are donepezil, 10 mg/d; lisinopril, 5 mg/d; levothyroxine, 0.1 mg/d; and a daily multivitamin. On physical examination, temperature is 36.4 °C (97.5 °F), blood pressure is 120/78 mm Hg, pulse rate is 68/min, respiration rate is 14/min, and BMI is 24. Her level of alertness, speech, and gait are normal. The patient scores only 12/30 on the Folstein Mini-Mental State Examination. Results of a complete blood count, a basic metabolic panel, a serum vitamin B12 measurement, and thyroid function tests are normal. A CT scan of the head without contrast shows no evidence of tumor, hemorrhage, or infarction. Which of the following is the most appropriate next step in treatment? A Add memantine B Add quetiapine C Add sertraline D Increase the dosage of donepezil E Stop all medications

A Memantine is a first-line agent for treatment of moderate to advanced Alzheimer dementia. This patient has Alzheimer dementia that is moderately advanced and now has difficulties with basic activities of daily living. The N-methyl-D-aspartate receptor antagonist memantine is the only drug approved by the U.S. Food and Drug Administration as first-line treatment of moderate to advanced Alzheimer dementia. Although evidence is limited, there is some suggestion that the stepped approach of adding memantine to a regimen that includes a cholinesterase inhibitor (such as donepezil) results in a modest additional benefit over substituting memantine for the cholinesterase inhibitor. Quetiapine is an antipsychotic medication and sertraline is an antidepressant agent. Although both drugs can be used in patients with Alzheimer dementia, their use is limited to the treatment of the behavioral symptoms of psychosis and depression, respectively, neither of which this patient has at this time. There is no approved indication for using donepezil beyond its recommended maximum dosage of 10 mg/d. In fact, data suggest that doing so is associated with increased adverse effects and not with increased efficacy. Discontinuing the donepezil taken by this patient without substituting another drug to manage her functional decline would not help slow or otherwise improve the course of her disease. Although the potential cognitive side effects of the other prescription (and nonprescription) medications taken by this patient must also be considered, lisinopril, levothyroxine, and daily multivitamins have no association with such effects.

Neuro 13 A 62-year-old woman is evaluated in the emergency department for a 2-week history of progressive shortness of breath, which has culminated over the past 3 days in a change in the sound of her speech and occasional swallowing difficulties. She says that approximately 8 weeks ago, she began to have blurred vision late in the day, particularly when driving home from work, and occasional weakness in the upper and lower limbs. Three weeks ago, she had a urinary tract infection treated with ciprofloxacin. The patient takes no other medications On physical examination, blood pressure is 118/56 mm Hg, pulse rate is 68/min, respiration rate is 22/min, and arterial oxygen saturation on ambient air is 98%. Her lungs are clear to auscultation. She has moderate bilateral proximal weakness in the upper and lower limbs, with mild weakness distally. Deep tendon reflexes are normal, and there is no appendicular or truncal ataxia. She has fluctuating ptosis and diminished facial strength. Speech is nasal and slurred; her language is normal. Which of the following is the best treatment option for this patient? A Plasma exchange B Prednisone C Pyridostigmine D Repeat ciprofloxacin therapy

A Myasthenic crisis, a potentially life-threatening neurologic emergency characterized by muscle weakness severe enough to necessitate intubation, typically requires plasma exchange therapy. This patient has a rapidly progressive neurologic disorder with weakness of limb, bulbar, and respiratory muscles and should undergo plasma exchange. Given the absence of sensory loss and normal deep tendon reflexes, myasthenia gravis is the most likely diagnosis. Because of the rapid progression of the disease and the involvement of respiratory and bulbar muscles, the patient should be admitted to the hospital for management of a myasthenic crisis. Myasthenic crisis is a potentially life-threatening neurologic emergency characterized by weakness that is severe enough to necessitate intubation. Electromyographic studies, including repetitive stimulation of motor nerves, are indicated to establish a diagnosis of myasthenia gravis. Plasma exchange is the treatment of choice in patients with myasthenic crisis. Myasthenia gravis is an autoimmune disorder that results from antibody-mediated attacks on the postsynaptic neuromuscular junction. This process may impair neuromuscular transmission by functional blockade of the acetylcholine receptor, by accelerating the degradation of acetylcholine receptors, and by causing damage to the postsynaptic membrane of the neuromuscular junction. Plasma exchange presumably removes these circulating antibodies. Prednisone is an effective therapy in the treatment of patients with myasthenia gravis. However, it is not appropriate as the initial, sole therapy in patients in the midst of a myasthenic crisis. A worsening of muscular weakness is seen in some patients within the first 3 weeks of the initiation of this medication. Even if this complication does not occur, the beneficial effects from prednisone typically do not occur for 3 to 4 weeks after drug initiation. In a patient with myasthenic crisis, prednisone is often initiated after the patient has been stabilized and is improving with plasma exchange. Used in this fashion, prednisone should reach maximum efficacy by the time the beneficial effects of plasma exchange have waned (6-8 weeks). Pyridostigmine is an inhibitor of aceylcholinesterase, which increases the amount of acetylcholine in the neuromuscular junction. This medication can be given orally or intravenously and may offer symptomatic benefit in patients with myasthenia gravis. Patients with mild symptoms due to myasthenia gravis can occasionally be treated with pyridostigmine alone. Although this medication may be of some benefit in patients with myasthenic crisis and should be considered, definitive immune-modulating therapy is indicated as the most appropriate treatment. Ciprofloxacin and other fluoroquinolone medications are contraindicated in patients with myasthenia gravis; the ciprofloxacin the patient received 3 weeks ago to treat a urinary tract infection may have precipitated her current myasthenic crisis. Other medications that should be avoided in patients with myasthenia gravis include lithium, aminoglycosides, magnesium, and macrolide antibiotics.

Neuro 2 An 18-year-old male college student is evaluated for a single generalized tonic-clonic seizure that began when he was asleep in his dormitory and resolved uneventfully. He has no history of head trauma, meningitis, or prior seizure and no family history of epilepsy. He takes no medications. Results of physical examination, including a neurologic examination, are normal. Results of laboratory studies, including a complete blood count, a serum electrolyte panel, and a urine toxicology screen, are also normal. An MRI of the brain and an electroencephalogram show no abnormalities. Which of the following is the most appropriate management of this patient's seizure? A Initiate no drug therapy at this time B Initiate therapy with carbamazepine C Initiate therapy with lamotrigine D Initiate therapy with valproic acid E Refer for epilepsy surgery evaluation

A Unless special circumstances exist, drug therapy is generally not started in patients with a single unprovoked seizure. Drug therapy should not be initiated in this patient at this time. After a single unprovoked seizure, the risk of recurrence in the subsequent 2 years has been reported to be 30% to 40%. The risk of recurrence is greatest in patients with status epilepticus on presentation, with an identifiable underlying neurologic cause, or with abnormal results on an electroencephalogram (EEG). Patients with a partial seizure who are age 65 years or older or who have a family history of epilepsy may also be in a higher-risk category. The appropriate recommendation for this young patient, who has experienced a single idiopathic seizure but has no personal or family history of epilepsy, no identified neurologic cause of his seizure, and normal results on an EEG, is that no medication be started. As with all medical treatment recommendations, patient preference must be taken into account, and some patients in the low-risk group may elect to start therapy after a single seizure, particularly if they have a high-risk occupation. If a second seizure occurs in the future, the recurrence risk is greater than 60%, and antiepileptic medical therapy should be recommended at that time. Of note, driver's license privileges are restricted in every state in the United States for persons who have experienced a seizure. Specific restrictions vary by state, with typical requirements of a seizure-free period of 3 to 12 months in order to again operate a motor vehicle; a few states make exceptions for a single seizure. Reinstatement of driving privileges depends on demonstrating freedom from seizures for the specified period and there being a reasonable expectation of future seizure control. Initiation of antiepileptic medication is not required by law. Epilepsy surgery is reserved for patients who have disabling seizures that cannot be controlled with medication. Such surgery is not indicated for this patient, who had a single seizure that resolved uneventfully.

Neuro 70 A 33-year-old woman is evaluated in the emergency department for paresthesia that began in the left face and spread over 30 minutes to the left arm and leg, clumsiness of the left hand that began 30 minutes ago, and an emerging right-sided throbbing headache. She is otherwise healthy but has a family history of migraine. Her only medication is a daily oral contraceptive pill. On physical examination, temperature is normal, blood pressure is 140/82 mm Hg, pulse rate is 110/min, and respiration rate is 20/min. All other examination findings are normal. Results of laboratory studies and a CT scan of the head are also normal. Which of the following is the most likely diagnosis? A Migraine with aura B Multiple sclerosis C Sensory seizure D Transient ischemic attack

A When assessing a patient with the acute onset of focal neurologic deficits, the examiner should include stroke mimickers, such as migraine with aura, in the differential diagnosis. This patient with stroke symptoms is most likely experiencing a migraine with aura and not a stroke. Migraine with aura is a stroke mimicker; stroke mimickers account for nearly one third of all stroke-alert calls in an emergency department. The clinical clues supporting a diagnosis of migraine are the patient's young age, the absence of vascular risk factors, the family history of migraine, and the presence and spread of the sensory symptoms. An MRI with diffusion-weighted imaging can rule out an acute ischemic stroke and thus help confirm the diagnosis of migraine with aura. Although multiple sclerosis (MS) should be in the differential diagnosis, this patient is less likely to have MS than a migraine or stroke because her presentation was more acute than would be typical in MS. Finding evidence of central nervous system demyelination on an MRI is the usual way MS is diagnosed; when such evidence is lacking, demyelination can sometimes be suggested by abnormal findings in the cerebrospinal fluid. Symptoms of stroke and transient ischemic attack (TIA) are described as negative or are said to involve loss of function. For example, there may be hemiparesis (a motor deficit affecting half the body) or bland sensory loss (numbness, loss of sensation, diminished sense of touch) in half the body. In contrast, partial seizures account for positive motor symptoms—such as involuntary unilateral muscle movement, twitching, and jerking—or positive sensory symptoms—such as paresthesia, tingling, or a feeling of "pins and needles." Sensory seizure symptoms generally reflect the anatomic organization of the sensory homunculus on the contralateral primary sensory cortex, whereas migraine symptoms may not. The sensory aura of a migraine generally spreads slowly over half the body. Rapidity of onset is another helpful clue in distinguishing migraine from TIA and seizure. A TIA comes on very rapidly (seconds), and seizures generally manifest in less than 1 minute. Migraine with aura, on the other hand, presents more slowly (over minutes, as with this patient), and symptoms spread slowly from region to region.

NF1

AD (Chr 17 - for 17 letters in name) Cafe au lait spots (tan colored) Neurofibromas - growths along nerves - compression/pain/neuropathy/PAINFUL etc. - Lisch nodules (iris hamartomas) - freckling of the axilla/inguinal area. - macropcephaly - short height

NF2

AD (Chr 22) - B/L acoustic neuromas here (or U/L). - Cataracts. - macropcephaly - short height

VHL

AD - VHL tumor suppressor gene mutation Hemangioblastoma - cerebellum & retina Pheochromocytoma Renal Cell CA

rapid severe pain and vision loss, halos around lights, injected eye, mid-dilated pupil, tearing, nausea, vomiting

Acute Close Angle Glaucoma caused by: Anti-Ach, Sympathomimetic Dx: Tonometry

spinal muscular atrophy (Werdnig-Hoffmann disease)

A hereditary degenerative disease of LMN. "Floppy baby" with age of death ~7mo :(

glutamate and aspartate

AA that serve as excitatory NTs. Glutamate is the main NT of the CSTr.

Level of hypothalamospinal tract

Above T1. Damage = ipsilateral Horner's syndrome

Ring enhancing lesion

Abscess (distant infection- strep, bacteroides), Toxoplasmosis

Syncope with exertion or during exercise

AOrtic stenosis, HCM, anomalous coronary arteries

Ataxia Telengectasia

AR Sx in childhood Looks very similar to Friedrich's ataxia but ALSO have telengectasias. High risk of cancer also. *(part of Neurocutaneous D/O too - only AR one)

Ataxia Telangiectasia

AR - ATM gene mutation A - ataxia (b/c cerebellar degeneration) T - (low T cells) IMMUNOCOMPROMISED (URI infxns) M - Multiple telengiectasias (usu in sclera)

Friedrich's Ataxia

AR d/o Sx in young adults. Triad: 1. Neurologic (spinocerebellar ataxia) 2. Cardiac (concentric hypertrophic CM) 3. Skeletal (scoliosis, hammer toes) +/- DM **death ~ age 20 (from CM or respiratory issues)

fredric ataxia

AR disorder of progressive ataxia. arms>legs plus dysarthria. loss of reflesxes, spasticity and extensor plantar response, impaired vibration and position sense. due to mutation in frataxin. GAA repeat expansion. can have hearing loss, DM, hypertrophic cardiomyopathy, arrhythmias.

carotid artery stenosis <70%

ASA if greater and sx--> CEA

abrupt onset bilateral flaccid paralysis and loss of pain and temp leads to what?

ASA lesion.

Superior oblique muscle (CN IV) ____ the eye.

Abducts, depresses, INTORTS eye (inferior oblique extorts eye). Injury = vertical diplopia when looking down *SOI-IOE* (Sup oblique intorts, inf oblique extorts)

Why Bell's palsy recovery can lead to involuntary twitching

Aberrant regeneration of the facial nerve

Migraine Abortives and Preventives

Abortives- Triptans, Nsaids, Acetaminophen, Antiemetics (Metoclopramide, Prochlorperazine,), Ergots (Dihydroerogtamine) Preventives- Topiramate, Divalproex sodium, TCA, Beta Blockers

dilated with segmental contraction and near light dissociation

Adie's Pupil- parasympathetic interruption

Rx Seizures

Adults --> If lasting more than 2 minutes → IV benzo and Phenytoin Children → Phenobarbital is first line anticonvulsant (seizures) Absence → Ethosuximide then Valproic Acid

MC causative organisms in brain abscess

Aerobic and anaerobic strep and bacteriodes

Risk factors for Alzheimers dementia.

Age Female gender Positive family history Head trauma Down's syndrome

Dandy-Walker syndrome

Agenesis of cerebellar vermis. 4th ventricle becomes an enlarged cyst. (Remember: Cerebellar prob means you would NOT be a dandy walker.)

inability to recognize objects

Agnosia Sensory Association area lesion

EEG Relaxed Eyes Closed

Alpha 8-13Hz

Alpha axons

Alpha motor neurons (innervate extrafusal fibers)

Wave seen on EEG with relaxed person

Alpha wave, 8-13 hz

Early, insidious short term memory loss, language deficits, spatial disorientation, later personality changes

Alzheimer

"accumulation of cerebral cortical plaques and tangles"

Alzheimer's

paranoid, memory loss, ct generalized cortical atrophy + hippocampus atrophy

Alzheimer's! late. frontotemporal is personality weird stuff early + no generalized cerebral atrophy.

Amyloid B protein w dementia

Alzheimers (sits Extracellular)

Painless, rapid, transient <10min, monocular vision loss

Amaurosis fugax -curtain descending over visual field. -Retinal ischemia due to atherosclerotic emboli from ipsilateral carotid artery. Chekc w/ DUPLEX ULTRASOUND OF NECK

Common antibiotics that cause vestibular toxicity

Aminoglycosides

What is one of the most common side effects of ECT?

Amnesia!

Most common cause of lobar hemorrhage in elderly patients without hypertension

Amyloid

Huntington's disease can be caused by

Glutamate excitotoxicity = neuron death. Glutamate is normally removed by astrocytes but in HD it remains bound to receptor in striatal neurons. Caudate nucleus atrophies (and lateral ventricles enlarge as a result)

Semicircular ducts sense

Angular acceleration/deceleration of head (rotational movement). Hair cells in the crista ampullaris respond to endolymph flow

Amantadine side effects

Ankle edema and livedo reticularis

Opposite side somatosensory and motor defitic in lower extremities, Abulia (lack of will or initiative), dyspraxia, emotional disturbance, urinary incontinence

Anterior Cerebral artery occlusion

Bitemporal lower quadrantanopia can be caused by damage to what artery?

Anterior communicating artery (most common anuerysm of Circle of Willis)

Commonly associated with burst fracture of vertebra? Presentation

Anterior cord syndrome. 1) Bilateral loss of pain & temp + UMN symptoms below lesion *Intact vibratory and proprioception

Wednig Hoffman

Anterior horn cells - floppy newborn baby!

Mammillothalamic tract enters what nucleus?

Anterior nucleus of thalamus

Antibodies present in paraneoplastic cerebellar degeneration

Anti-Purkinje cell antibodies also called anti-yo that are found in women with breast cancer and other gynecologic malignancies

Tremor in Parkinson's

Anti-cholinergics (but not for elderly pts)

First step in suspected meningitis

Antibiotics, then MRI, then LP; ceftriaxone + amp good choice

Aortic Arch & Carotid Sinus, responses to BP changes

Aortic Arch (CN 10) responds to HIGH BP only. Carotid Sinus/Body (CN 9) responds to high and low BP.

Aphasia vs Apraxia

Aphasia - language difficulty Apraxia - motor difficulty w speech

inability to carry out learned motor task

Apraxia Frontal or parietal lesions in dominant hemisphere

Site most likely to produce a noncommunicating hydrocephalus

Aqueduct of Sylvius, connects the third and fourth ventricle

small, poorly reactive pupil with preserved near response

Argyll Robertson Pupil- syphilis

Niemann-Pick dx

Ashkenazi Jewish, developmental delay, cherry red macula, hepatosplenomegaly

Tay Sachs dx

Ashkenazi Jewish, developmental delay, cherry red macula, no hepatosplenomegaly

MCC fungal malignant otitis externa and osteomyelitis of the base of the skull

Aspergillus

rubrospinal tract

Assists in limb flexion. Note crossing over.

Most common primary brain tumor in adults

Astrocytoma

Indication for prophylactic migraine treatment

At least three attacks per month- use topiramate, divalproex, gabapentin

Cerebellar hemorrhage presentation

Ataxia, vomiting, occipital headache, gaze palsy, and facial weakness

overflow incontinence

Atonic Bladder- LMN conus, cauda equina, sacral plexus increased capacity and compliance

Sleep Stages

Awake- Beta REM- Alpha 8-13Hz Stage 1- Theta Stage 2- Sleep Spindles, K Complexes Stage 3- Delta Stage 4- Beta

Anterior dislocation of shoulder will cause what nerve injury?

Axillary Nerve.

Vision change in B12 deficiency

Blind spot enlarges and extends into central vision

Riluzole

Glutamate inhibitor currently approved for patients with ALS

Neuro 28 A 73-year-old retired woman is evaluated in the emergency department 6 hours after experiencing the sudden, explosive onset of a severe headache. The patient has hypertension controlled by diet and exercise. There is no relevant family history. She has no allergies and takes no over-the-counter medications. On physical examination, she is in obvious distress from the headache. Temperature is normal, blood pressure is 179/108 mm Hg, pulse rate is 119/min, and respiration rate is 14/min. There is no meningismus. No subhyaloid retinal hemorrhages are noted. Neurologic examination shows a normal level of consciousness and no focal abnormalities. Results of laboratory studies and a CT scan of the head without contrast are normal. Which of the following is the most appropriate next diagnostic test? A CT angiogram of the head B Lumbar puncture C Magnetic resonance angiogram of the brain D Magnetic resonance venogram of the brain E MRI of the brain

B A lumbar puncture with subsequent cerebrospinal fluid analysis is necessary in any patient with thunderclap headache and normal findings on a CT scan to fully evaluate a possible subarachnoid hemorrhage. This patient should have a lumbar puncture. A thunderclap headache is a severe and explosive headache that is maximal in intensity at or within 60 seconds of onset. CT scanning is the first test to be conducted in a patient with thunderclap headache in whom a subarachnoid hemorrhage is suspected; a ruptured intracranial aneurysm is the most serious cause of such headaches. The ability to detect subarachnoid hemorrhage is dependent on the amount of subarachnoid blood, the interval after symptom onset, the resolution of the scanner, and the skills of the radiologist. On the day of the hemorrhage, extravasated blood will be present in more than 95% of patients, but in the following days, this proportion falls sharply. If an initial CT scan of the head reveals nothing, a lumbar puncture should be performed next in patients with this presentation. In an important minority (<5%) of patients with thunderclap headache who have no abnormalities on a CT scan and no blood in the cerebrospinal fluid (CSF), the CSF contains metabolites of hemoglobin that may be detected by spectrophotometry. This finding is diagnostic for subarachnoid hemorrhage. Subsequent angiography can confirm the presence of a ruptured aneurysm. It should be noted that spectrophotometry is not universally available in all hospitals. CT angiography and magnetic resonance angiography have similar test characteristics. Their sensitivity for detecting a ruptured aneurysm, with the more invasive conventional angiography as the gold standard, is currently 95%. CT angiography can be performed faster than can magnetic resonance angiography, but the latter uses no radiation and no contrast injection. Both modalities can play a role in the work-up of a patient with undiagnosed thunderclap headache when lumbar puncture and standard CT are nondiagnostic. Conditions other than aneurysm in the differential diagnosis of thunderclap headache can also be detected by CT angiography or magnetic resonance angiography, including arterial dissection and reversible cerebral vasoconstriction syndrome. Magnetic resonance venography may be necessary in a patient with thunderclap headache, but only when cerebral venous sinus thrombosis is part of the differential diagnosis. Cerebral venous sinus thrombosis is more common in young adults. Major risk factors for such thrombosis in adults include conditions that predispose to spontaneous thromboses, including inherited or acquired thrombophilia, pregnancy, oral contraceptive use, malignancy, sepsis, and head trauma. An MRI is as sensitive as a CT scan in the acute phase of subarachnoid hemorrhage but is not superior. Any subarachnoid blood not evident on a CT scan is unlikely to be seen on an MRI. A few days after the hemorrhage, an MRI is better for detecting blood, with fluid-attenuated inversion recovery (FLAIR) and T2-star images being the most sensitive. An MRI can also help in the detection of other conditions in the differential diagnosis, such as an acute ischemic stroke, pituitary apoplexy, a third-ventricle colloid cyst, and an acute hypertensive crisis.

Neuro 75 A 25-year-old woman is evaluated in the office for a 1-week history of numbness of the left leg. She also had a 2-week history of diplopia and vertigo 1 year ago; an MRI obtained at that time is shown . There is no family history of neurologic problems. She takes no medications. On physical examination, vital signs are normal. Plantar response is extensor on the left. There is mild loss of vibratory sense in both feet with a patchy, reduced pain sensation throughout the left lower extremity. Laboratory studies are noncontributory. A repeat MRI of the brain shows only the same periventricular white matter lesion. MRIs of the cervical spine and thoracic spine show no abnormalities. Which of the following is the most appropriate next step in diagnosis? A Electronystagmography B Lumbar puncture C Magnetic resonance angiography D Magnetic resonance spectroscopy

B Cerebrospinal fluid analysis is useful when the clinical setting is suspicious for multiple sclerosis but neuroimaging is inconclusive. This patient should next undergo lumbar puncture. There is already a high clinical suspicion of multiple sclerosis (MS) because of the patient's age, her prior transient neurologic symptoms, the abnormal findings on neurologic examination, and the periventricular white matter lesion seen on two MRIs of the brain. However, these imaging and examination results are insufficient to confirm that multiple regions of the central nervous system are affected at different times (dissemination in time and space), which is one of the diagnostic criteria of MS. Up to 85% of patients with MS have an abnormal finding on cerebrospinal fluid analysis, such as the presence of oligoclonal bands or elevation of the IgG index. Therefore, lumbar puncture is the most appropriate next step in diagnosis. Confirmation of the diagnosis of MS at this stage would allow intervention with immunomodulatory therapy and result in a lower risk of both future relapses and accumulation of neurologic impairment. In the assessment of vestibular function, electronystagmography (ENG) uses electrodes to record eye movements to help discriminate between central and peripheral causes of vertigo. However, this patient's current leg numbness, history of diplopia and vertigo, and findings of an extensor plantar response, a loss of vibratory sense, and reduced pain sensation of the leg point to a process not confined to vestibular function but associated with manifestations that are separate in both time (two neurologic events over 2 years) and space (different parts of the central nervous system). This is most compatible with MS. Furthermore, in a patient without symptoms of vertigo, the ENG is likely to be normal. For similar reasons, magnetic resonance angiography, a noninvasive imaging technique used to detect vascular lesions, is unlikely to be helpful in this patient with probable MS. Magnetic resonance spectroscopy is at present a research technique that is not useful for MS diagnosis but may in the future play a role in monitoring disease progression and determining whether therapeutic interventions have neuroprotective effects.

Neuro 89 A 68-year-old man is evaluated in the office for a 4-week history of continuous left leg pain and weakness. The pain is severe, burning, and in the distribution of the anterior thigh. He has had a 4.5-kg (10-lb) weight loss in the last 3 weeks. The patient had prostate cancer 1 year ago and underwent radiation therapy to the pelvis. He also has hypertension treated with lisinopril. General physical examination findings are normal, except for a BMI of 31. Neurologic examination shows normal speech, language, and cranial nerve function. Strength and deep tendon reflexes are normal in the upper extremities and in the right leg. There is diffuse weakness, greatest in the quadriceps, hip adductor, and iliopsoas muscles, and absent knee and ankle jerks in the left leg; plantar response is flexor. Sensory loss is evident in the distribution of the saphenous sensory nerve on the left. Laboratory studies show an elevated fasting plasma glucose level of 132 mg/dL (7.3 mmol/L). All other laboratory results, including erythrocyte sedimentation rate; serum electrolyte, creatinine, and thyroid-stimulating hormone levels; and liver chemistries, are normal. Which of the following is the most likely diagnosis? A Cauda equina syndrome B Diabetic lumbosacral radiculoplexus neuropathy C Myelopathy D Radiation-induced lumbosacral plexopathy

B Diabetic lumbosacral radiculoplexus neuropathy (diabetic amyotrophy) is characterized by severe, initially unilateral lower limb pain and weakness. Diabetic lumbosacral radiculoplexus neuropathy, or diabetic amyotrophy, is the most likely diagnosis in this patient. This neuropathy is a subacute, progressive disorder that causes asymmetric leg pain, sensory loss, and weakness. Weight loss of 4.5 kg (10 lb) or more occurs in most affected patients. Many patients with this disorder are unaware that they have diabetes mellitus before the development of diabetic lumbosacral radiculoplexus neuropathy, and in most patients, glycemic control is not severely compromised. This disorder usually begins with unilateral leg pain followed by weakness and sensory loss, which spread to involve the contralateral leg nearly all the time. Weakness is often greatest proximally initially, but over time, diffuse weakness involving proximal and distal muscles ensues. Electromyographic studies characteristically show dysfunction at the level of multiple peripheral nerves, the lumbosacral plexus, and multiple nerve roots. MRI studies of the lumbosacral plexus are typically normal in this disorder but are most helpful in excluding an infiltrative neoplastic process, which can present similarly. Cauda equina syndrome due to a compressive, infiltrating, or inflammatory process affecting multiple lumbosacral nerve roots should be considered in this patient. However, the absence of abnormal signs or symptoms in the right leg would be unusual in this syndrome. Patients with cauda equina syndrome typically have bilateral (but sometimes asymmetric) leg pain, weakness, and areflexia. Bowel and bladder impairment can occur, and saddle anesthesia is expected in affected patients. Myelopathy due to demyelinating disease or other disorders should be considered in patients with lower limb weakness, sensory loss, and pain but is unlikely in this patient. Absent reflexes in the left leg suggest a lower motoneuron problem affecting either multiple nerve roots or the lumbosacral plexus in that limb. A myelopathy would be expected to cause hyperreflexia, spasticity, and an extensor plantar response in the lower limbs. When there is a history of pelvic irradiation, radiation-induced lumbosacral plexopathy should be considered in patients with progressive leg weakness. However, the rapid progression of symptoms and the presence of pain in this patient are not typical of radiation-induced lumbosacral plexopathy. Moreover, the mean time to symptom onset after radiation exposure is 3 to 6 years, although it has been reported to range from 1 month to 18 years after radiation exposure. Radiation-induced damage can affect the brachial plexus (typically, in breast cancer) or lumbosacral plexus. The frequency of brachial plexus injury after radiation therapy has been reported to range from 1.8% to 9.0%. Typical symptoms include gradually progressive weakness and sensory loss.

Neuro 78 A 46-year-old man is evaluated in the office for a 6-month history of a resting right-arm tremor. He says that his writing has gotten smaller during this time and that he has had difficulty buttoning his dress shirts. The patient reports no prior medical problems and is not aware of any neurologic problems in his family. He takes no medications. Results of a general medical examination are normal. Neurologic examination shows a paucity of facial expression (hypomimia). Cranial nerve function is normal. Motor examination shows normal strength but mild left upper limb rigidity and a 5-Hz resting tremor of the right upper limb. Deep tendon reflexes are normal, as are results of sensory examination. There is no truncal or appendicular ataxia. Diminished arm swing is noted bilaterally, but it is worse on the right. A tremor in the right upper limb is noted during ambulation. Left upper limb alternating motion rates are diminished. Which of the following is the best treatment for this patient? A Amantadine B Pramipexole C Primidone D Propranolol

B Dopamine agonist medications are used as first-line treatment of Parkinson disease in patients younger than 65 years, whereas levodopa is used in patients age 65 years or older. This patient should be treated with pramipexole. He has classic signs of Parkinson disease, including tremor, rigidity, and bradykinesia. There are no effective neuroprotective agents to treat this disorder. Dopamine agonist medications, either pramipexole or ropinirole, are indicated for the initial treatment of the parkinsonian symptoms in this young patient with apparent Parkinson disease. Motor complications, such as dyskinesias (abnormal involuntary movements), an end-of-dose "wearing-off" effect, and fluctuations, may be less frequent and less severe with dopamine agonist medications than with levodopa. Levodopa, a precursor of dopamine, is the most efficacious medication used to treat the symptoms of Parkinson disease but is typically initiated only in patients older than 65 years. The associated development of motor fluctuations occurs at a rate of 10% annually in these patients but may develop more rapidly and be more severe in younger patients taking levodopa as an initial medication. Carbidopa is administered in conjunction with levodopa to prevent the peripheral conversion of levodopa to dopamine. Amantadine has been used in the treatment of Parkinson disease since the 1970s but is not currently a medication of choice for initial therapy. Its mechanism of action in the treatment of Parkinson disease is unknown, but amantadine may increase dopamine release via antagonism at the N-methyl-D-aspartic acid receptor. The drug also has anticholinergic properties. Amantadine may reduce dyskinesias, one of the motor complications of Parkinson disease. However, any initial improvement in their parkinsonian symptoms that patients taking amantadine experience may be modest and not sustained. Primidone is used in patients with essential tremor. Essential tremor is distinguished from Parkinson disease by its occurrence with limb movement and the lack of parkinsonian signs on examination. Likewise, propranolol would not be of any benefit in this patient with the typical resting tremor of Parkinson disease. Propranolol remains the drug of choice in the treatment of patients with essential tremor.

Neuro 35 A 53-year-old woman is evaluated in the office for a 4-month history of tremor. The tremor affects both upper extremities and is present "most of the time." She has a 15-year history of type 2 diabetes mellitus; she also has a history of hypertension, gastroparesis, and chronic kidney disease. Medications are insulin glargine, insulin lispro, lisinopril, hydrochlorothiazide, and metoclopramide. On examination, she has diminished pedal pulses. Speech, language, and mental status are normal. Cranial nerve function is normal, although a paucity of facial expression is noted. Movements are slow, and there is mild bilateral upper and lower extremity rigidity. Deep tendon reflexes are normal, as are results of manual muscle strength testing. Sensory examination reveals distal sensory loss. She had a mildly stooped posture but no postural instability. A 4-Hz resting tremor in both upper extremities is noted, as is a prominent postural tremor. Which of the following is the most likely diagnosis? A Dementia with Lewy bodies B Drug-induced parkinsonism C Multiple system atrophy D Parkinson disease

B Drug-induced parkinsonism is a potential complication of dopamine-blocking medications, including metoclopramide. Drug-induced parkinsonism is the most likely diagnosis in this patient. This disorder has classically been associated with neuroleptic medications but can occur with any dopamine-blocking medications, including metoclopramide. Although metoclopramide causes drug-induced parkinsonism in one third of all patients using it, the disorder is particularly underdiagnosed in such patients. Establishing a diagnosis of drug-induced parkinsonism is critical because stopping dopamine-blocking medications can reverse or improve parkinsonian features in these patients. Cognitive impairment in conjunction with parkinsonism occurs in patients with dementia with Lewy bodies, but the latter diagnosis is unlikely in this patient, given her apparently normal cognition. Multiple system atrophy is a heterogeneous, progressive, and ultimately fatal neurodegenerative disorder associated with parkinsonian features and with cerebellar and autonomic signs and symptoms of variable severity. Early multiple system atrophy would be a consideration in this patient if she were not on a medication known to induce signs and symptoms of parkinsonism. The parkinsonism in some patients with early multiple system atrophy cannot be distinguished from Parkinson disease and may even be responsive initially to levodopa. Most patients with multiple system atrophy, however, have bilateral parkinsonian signs and lack significant tremor, findings that are atypical of Parkinson disease. Parkinson disease should be part of the differential diagnosis but is an unlikely cause of the symptoms in this patient. Although there are parkinsonian signs and symptoms, there are several atypical features that should prompt consideration of an alternative diagnosis. The presence of symmetric signs and symptoms (tremor and rigidity) and the postural tremor in this patient suggest a condition other than Parkinson disease. Other features that suggest an alternative condition in patients with parkinsonian signs and symptoms include early falls, rapid progression, poor or waning levodopa response, dementia, early autonomic failure, and ataxia. Moreover, this patient is taking metoclopramide, a medication known to cause parkinsonism.

Neuro 3 A 36-year-old woman is evaluated in the office for a history of migraine, with and without aura, since age 16 years. She has an average of three attacks each month and consistently experiences an attack 2 days prior to menstruation; this headache is more difficult to treat than those not associated with menstruation. Although she typically obtains pain relief within 2 hours of taking sumatriptan, the headache recurs within 24 hours after each dose during the period of menstrual flow. Sumatriptan, orally as needed, is her only medication. Results of physical examination are unremarkable. Which of the following is the most appropriate perimenstrual treatment for this patient's headaches? A Estrogen-progestin contraceptive pill B Mefenamic acid C Sumatriptan plus naproxen, orally D Sumatriptan, subcutaneously E Topiramate

B Evidence supports the use of mefenamic acid for perimenstrual prophylaxis of menstrually related migraines, with treatment starting 2 days prior to the onset of flow or 1 day prior to the expected onset of the headache and continuing for the duration of menstruation. This patient should be treated with mefenamic acid. She has migraine with aura, migraine without aura, and menstrually related migraine. Her menstrually related headaches are less responsive to acute therapy than are the non-menstrually related attacks, and headache recurs daily throughout menses. The best management for this patient is, therefore, the perimenstrual use of a prophylactic agent. There is evidence that supports the use of mefenamic acid for perimenstrual prophylaxis, with treatment starting 2 days prior to the onset of flow or 1 day prior to the expected onset of the headache and continuing for the duration of menstruation. In this patient, that would mean beginning 3 days before the onset of menstrual flow and continuing throughout menstruation. The use of combined oral contraceptive therapy (estrogen plus progestin) is contraindicated in this woman because of her history of migraine with aura. Women with migraine with aura are at a two-fold increased risk of ischemic stroke, ischemic myocardial infarction, and venous thromboembolism. The risk of stroke is increased further, up to eight-fold, in women with migraine with aura who use combined oral contraceptive pills. No evidence supports the oral use of either sumatriptan plus naproxen sodium or topiramate for the perimenstrual prophylaxis of menstrually related migraine. Similarly, there is no evidence supporting the subcutaneous use of sumatriptan in this setting. In fact, the higher recurrence rate with the subcutaneous formulation may prove counterproductive.

superior cerebellar artery lesion

Blockage in which cerebellar artery(s) is most likely to cause vertigo, nausea and vomiting without brainstem signs?

Neuro 29 A 25-year-old woman comes to the office to ask about discontinuing her epilepsy medication because she is concerned about its potential long-term adverse effects. She has a 9-year history of generalized tonic-clonic seizures and rapid, shock-like body jerks consistent with myoclonic seizures on awakening. After her first seizure, she had an MRI with normal findings and an electroencephalogram showing generalized polyspike and wave abnormalities. She was started on lamotrigine and has done relatively well, having only rare seizures when she does not get adequate sleep or indulges in binge alcohol drinking. Her last generalized tonic-clonic seizure occurred 1 year ago. Results of physical examination, including neurologic examination, are normal. Which of the following is the best advice regarding discontinuation of the lamotrigine? A Begin a slow taper over the next 3 months B Continue life-long treatment C Discontinue after she is seizure free for 2 years D Discontinue now

B Juvenile myoclonic epilepsy requires lifelong antiepileptic drug therapy. This patient requires life-long treatment of her epilepsy. Appropriate recognition of specific epilepsy syndromes is essential because it will guide the clinician in selecting the appropriate therapy, making the correct prognosis, and, in some instances, providing genetic counseling. A history of myoclonic and generalized tonic-clonic seizures on awakening with onset in adolescence is highly suggestive of the syndrome of juvenile myoclonic epilepsy, which is one of the most commonly encountered forms of epilepsy, possibly affecting 5% to 10% of all patients with epilepsy. Seizures in juvenile myoclonic epilepsy are generally well controlled with medication, but relapses can be provoked by sleep deprivation, alcohol, or exposure to flickering lights. If medication is withdrawn, seizures will recur in 75% to 100% of patients. Therefore, in this patient with a history of juvenile myoclonic epilepsy, medication withdrawal is not recommended either now, 3 months from now, or in 2 years; juvenile myoclonic epilepsy requires lifelong therapy.

Neuro 88 A 30-year-old man is reevaluated for management of partial epilepsy. He first developed seizures 1 year ago after surgical treatment of a ruptured aneurysm of the right middle cerebral artery. He was started on phenytoin (400 mg/d) at that time but continues to have monthly episodes in which he experiences a rising epigastric sensation followed by loss of awareness lasting 1 to 2 minutes and then several hours of confusion and significant fatigue. He also reports gait imbalance and excessive fatigue related to the phenytoin. He takes no other medications. Physical and neurologic examination findings are noncontributory. Laboratory studies show a current serum phenytoin level of 18 mg/L (71.3 µmol/L) (therapeutic level, 10-20 mg/L [39.6-79.2 µmol/L]). Which of the following is the most appropriate next step in management? A Add another antiepileptic drug to his current regimen B Gradually transition from phenytoin to a new antiepileptic drug C Increase the dosage of phenytoin D Refer for epilepsy surgery evaluation E Refer for vagus nerve stimulation

B Monotherapy is preferred over polytherapy drug regimens as treatment of epilepsy. This patient should gradually transition to a new antiepileptic drug. He continues to have disabling seizures, despite taking phenytoin for 1 year and despite having a high therapeutic serum drug level. Only approximately half of the patients prescribed a first antiepileptic drug for seizure control will continue on that drug. For the others, initial therapy will be unsuccessful, either from lack of efficacy or from unacceptable side effects. When patients are unsuccessfully treated with a first antiepileptic drug, the recommendation is to try a second antiepileptic drug as monotherapy. It is generally recommended to gradually transition between drugs, titrating the new drug to therapeutic range before tapering off the initial agent. There is no compelling evidence that polytherapy improves seizure control over monotherapy. However, the primary rationale for continued monotherapy in a patient unsuccessfully treated with a first antiepileptic drug is the increased rate of adverse effects and drug interactions when antiepileptic drugs are used in combination. Concerns about adherence and increased expense also play a role. Therefore, adding another antiepileptic drug to this patient's drug regimen is not appropriate as the next step in management. Although an increase in phenytoin dosage might be considered in some cases, this patient has already reported clinically significant adverse effects from the drug. These would likely limit his ability to tolerate a higher dosage. Epilepsy surgery and vagus nerve stimulation are reserved for patients with medically refractory seizure disorders and are generally not considered until at least two antiepileptic drugs have been shown to be ineffective in controlling seizures. This patient has thus far tried only phenytoin therapy. A referral for either of these treatments is premature at this point.

Neuro 22 A 65-year-old man is evaluated for worsening gait unsteadiness and falls. He first noticed the unsteadiness 1 year ago while walking and has started to fall recently, falling four times in the past 2 weeks. Approximately 3 years ago, he developed erectile dysfunction and has had increasing constipation ever since that time. On physical examination, vital signs are normal except for the supine blood pressure, which is 190/105 mm Hg; blood pressure decreases to 76/50 mm Hg when he stands without a compensatory increase in the pulse rate. Results of mental status testing are normal. He has mildly slurred speech. Testing of cranial nerve function, including testing of extraocular movements, reveals no abnormalities. Manual muscle strength in the upper and lower extremities is normal, but he has mild rigidity of the extremities and mild appendicular ataxia. His gait is slow with a reduced stride length and arm swing, and he has marked postural instability. Which of the following is the most likely diagnosis? A Dementia with Lewy bodies B Multiple system atrophy C Parkinson disease D Progressive supranuclear palsy

B Multiple system atrophy is a sporadic, heterogeneous, neurodegenerative disorder that causes impairment of multiple neurologic systems, including the autonomic nervous system, the extrapyramidal system, and the cerebellum. Multiple system atrophy is the most likely diagnosis in this patient. He has a progressive neurologic disorder characterized by signs and symptoms that suggest impairment of multiple neurologic systems; these include the autonomic nervous system (orthostatic hypotension, erectile dysfunction, constipation), the extrapyramidal system (rigidity, impaired gait), and the cerebellum (limb ataxia). Multiple system atrophy is a progressive, ultimately fatal neurodegenerative disorder that typically causes dysautonomia, parkinsonism, and ataxia, in some combination, in affected patients. Multiple system atrophy is a clinical diagnosis that is suggested by the presence of these various features in the same patient. Dementia with Lewy bodies is also typically associated with parkinsonian features and should be considered in the differential diagnosis of this patient. Dementia with Lewy bodies is associated with cognitive impairment, parkinsonian signs and symptoms, and possible evidence of dysautonomia. However, gait or limb ataxia is not expected, and the degree of dysautonomia typically is not as severe as that seen in multiple system atrophy. The prominent dysautonomia, early falls, absence of a resting tremor, and presence of appendicular ataxia in this patient argue against Parkinson disease as the diagnosis. Early multiple system atrophy can, however, be difficult to distinguish from Parkinson disease, especially because some affected patients may respond initially to carbidopa-levodopa, a medication used to treat parkinsonian symptoms in Parkinson disease. Progressive supranuclear palsy should also be part of the differential diagnosis in this patient. A rare neurodegenerative disorder, progressive supranuclear palsy is associated with parkinsonian signs and early falls due to marked postural instability. However, significant dysautonomia and ataxia are not expected. Marked impairment in vertical gaze is a hallmark of progressive supranuclear palsy.

Neuro 18 A 70-year-old woman is evaluated for recent loss of consciousness. She says she had a "strange feeling" before losing consciousness. Her husband, who accompanied her on this visit and witnessed the event, reports that she fell to the ground and exhibited generalized stiffening and then shaking. He further states that her eyes rolled back in her head and that she was unresponsive for 1 minute, had urinary incontinence, and was profoundly confused, speaking garbled, nonsensical sentences, for 2 to 3 minutes after regaining consciousness. She had a similar episode 2 months ago; evaluation at that time, which included electrocardiography, stress electrocardiography, and a continuous-loop event electrocardiographic recorder, revealed no abnormal findings. The patient takes no medications. Results of a physical examination, including a neurologic examination, are normal. Results of MRI of the brain and electroencephalography during waking and sleeping are also normal. Which of the following is the most likely diagnosis? A Cardiac arrhythmia B Partial seizure with secondary generalization C Transient ischemic attack D Vasovagal syncope

B Normal findings on an electroencephalogram and MRI do not rule out a diagnosis of seizure. The history and provided eyewitness account are most consistent with the diagnosis of a partial seizure with secondary generalization. This type of seizure is characterized by sensory, motor, autonomic, or psychic (déjà vu, depersonalization) phenomena with or without altered awareness at onset followed by generalized tonic-clonic activity. Accurate classification of a seizure relies predominantly on the clinical history, with diagnostic testing used to confirm or clarify the suspected cause. When a patient has loss of consciousness with urinary incontinence and stiffening and shaking lasting 1 to 2 minutes, seizure should always be very strongly considered as the cause. When there is a high clinical suspicion of epilepsy, as in this patient, normal findings on an MRI of the brain and electroencephalogram (EEG) do not rule out that diagnosis. An MRI shows an epileptogenic lesion in only 10% to 20% of patients with epilepsy, and a routine EEG shows abnormalities in only 25% to 50% of patients with confirmed epilepsy. Approximately one third of syncopal episodes are reflex mediated or neurocardiogenic. Cardiac arrhythmias are responsible for approximately 15% of syncopal episodes and are most common in patients with cardiac risk factors or evidence of structural heart disease. Onset is usually sudden, may or may not be associated with palpitations, and can occur in any position. However, this patient's previous normal cardiac evaluation decreases the likelihood of an arrhythmia, and incontinence rarely results from causes of transient loss of consciousness other than seizure. The extended postictal period of confusion and speech impairment seen in this patient is also atypical of cardiac arrhythmia. Patients recovering from loss of consciousness due to arrhythmia are more typically alert or at most mildly confused after the loss of consciousness. Although the premonitory aura this patient experienced is most suggestive of seizure, such an aura has also been reported in cases of cerebral hyperperfusion from cardiac arrhythmia or neurocardiogenic syncope. A transient ischemic attack is not associated with this patient's findings and is not associated with loss of consciousness. Vasovagal syncope occurs as the result of sudden vasodilatation and bradycardia resulting in hypotension and cerebral hypoperfusion and is the most common cause of neurocardiogenic syncope. Syncope with pain, emotional stress, cough, micturition, or defecation supports a vasovagal cause, which is often preceded by warning symptoms of lightheadedness, nausea, and diaphoresis. Generalized stiffening and shaking lasting seconds can occur with vasovagal syncope due to reduced cerebral blood flow, but this type of syncope does not explain the aura, prolonged loss of consciousness, postictal confusion, and incontinence experienced by the patient. Thus, this diagnosis is unlikely.

Neuro 81 An 82-year-old man is evaluated in the office for an episode of hesitancy in speech, word-finding difficulty, right facial droop, and weakness and awkwardness of the right hand and arm. The episode occurred early yesterday, lasted 20 minutes, and was witnessed by his wife. The patient has a history of coronary artery disease, hypertension, and hyperlipidemia. Current medications are metoprolol, aspirin, hydrochlorothiazide, and lovastatin. On physical examination, temperature is normal, blood pressure is 148/88 mm Hg, pulse rate is 70/min, and respiration rate is 12/min. Neurologic examination reveals no abnormalities. Which of the following is the most appropriate next step in management? A Add clopidogrel B Admit to the hospital C Order outpatient diagnostic studies D Schedule a follow-up visit in 1 week

B Patients with a diagnosis of a recent transient ischemic attack are at an appreciably high short-term risk of stroke and should be evaluated in a hospital in an expedited and emergent fashion. This patient should be admitted to the hospital. Given his clinical history, he most likely has had a recent transient ischemic attack (TIA). His ABCD2 score (based on Age, Blood pressure, Clinical features, the Duration of symptoms, and the presence of Diabetes) is 5: one point is for his age (>60 years), one point for his hypertension, one point for a symptom duration of greater than 10 minutes, and two points for the focal weakness he described. This score is moderately high and carries an estimated stroke risk of 5% over the next 2 days, 7% over the next week, 10% over the next 30 days, and 12% over the next 3 months. Therefore, the most appropriate response is for this patient to undergo urgent evaluation within the next 24 hours at an emergency department, at a hospital during a brief admission, or at an organized urgent TIA clinic. It is reasonable to review the stroke prevention regimen of a patient with risk factors and make adjustments to any antiplatelet medications in the context of a new stroke or TIA. However, the priority is an expedited evaluation to determine the cause and mechanism of the stroke or TIA, such as symptomatic extracranial carotid artery stenosis amenable to endarterectomy, intracranial stenosis amenable to angioplasty and stenting, or cardioembolism with requirements for long-term anticoagulation. Therefore, adding clopidogrel to this patient's drug regimen is not the most appropriate next step in management. Outpatient diagnostic studies may play a role in the assessment of this patient, but only if they occur and results are back within 24 hours. Because this scenario is unlikely, such studies are clearly not the most appropriate next step in management. Given the high probability of an acute ischemic stroke event and the high short-term risk of stroke in this patient, scheduling a follow-up appointment in 1 week in the absence of diagnostic testing or evaluation could be life threatening.

Neuro 59 A 58-year-old man is evaluated in the emergency department after having a generalized tonic-clonic seizure 1 hour ago while sleeping; the seizure was witnessed by his wife. The patient has postictal lethargy and confusion. Stage IV non-small cell lung cancer characterized by a large, surgically nonresectable lesion in the right lung and by liver and pancreatic metastases was diagnosed 6 months earlier. He has been receiving chemotherapy for the past 5 months. On examination, the patient is afebrile; other vital signs are also normal. He is lethargic but arousable and is oriented to self but not to time or place. Mild weakness and incoordination of the right upper extremity are noted. Results of laboratory studies are normal. An MRI of the brain with contrast shows nine ring-enhancing lesions at the gray-white junction that involve both cerebral hemispheres and are consistent with metastases. The largest of these is in the left precentral gyrus and is associated with edema in the surrounding white matter. Which of the following is the best management option? A Brain biopsy B Palliative whole-brain radiation therapy and corticosteroid administration C Stereotactic radiosurgery of the left precentral gyrus D Surgical resection of accessible cerebral metastases

B Patients with multiple cerebral metastases and/or advanced systemic disease are not candidates for neurosurgical resection and should instead receive palliative treatment. The recommended treatment for this patient who has multiple cerebral metastases is palliative whole-brain radiation therapy and corticosteroid administration. Brain metastases are a common complication of systemic cancer and are associated with a poor prognosis. Treatment decisions are based on the location and number of cerebral lesions, the severity of neurologic symptoms, and the extent and prognosis of the systemic cancer. The main goal of therapy is to improve neurologic deficits by reducing the volume of the space-occupying metastases and the surrounding edema and to prevent symptom progression. Treatment response is directly related to the time from diagnosis to radiation therapy. In the presence of a known primary tumor with a likelihood of metastasizing to the central nervous system, such as lung cancer, brain biopsy to confirm the metastatic nature of the lesions is not needed. Stereotactic radiosurgery and surgical resection are generally restricted to patients with a single metastatic lesion and with reasonably controlled systemic disease. Neurosurgery will occasionally be considered for palliation in patients with multiple metastases, but only if there is a single lesion causing immediately life-threatening or severely disabling symptoms. In this patient, the presenting symptoms of seizure and mild weakness would not justify such aggressive intervention; these symptoms would be better treated with whole-brain radiation therapy and corticosteroids.

Neuro 6 A 45-year-old woman is evaluated in the emergency department for a 2-day history of increased leg weakness, ataxia, fatigue, and urinary incontinence. She has no other systemic symptoms. The patient has a 20-year history of multiple sclerosis, which has followed a progressive course over the past 5 years. Her baseline neurologic status, recorded 1 month ago, includes moderate bilateral spastic leg weakness, bilateral extensor plantar responses, mild sensory loss, and ambulation with a cane. Her only medication is interferon beta-1a; her dosage has been stable for the past 3 years. On physical examination, temperature is 38.1 °C (100.6 °F), blood pressure is 110/70 mm Hg, pulse rate is 90/min, and respiration rate is 16/min. The patient is unable to ambulate because of severe leg weakness. There is no costovertebral angle tenderness. Results of general physical and neurologic examinations are otherwise normal, including tests of mental status, cranial nerve function, and upper extremity neurologic status. Results of a complete blood count show a leukocyte count of 11,000/µL (11 × 109/L). Her urine is cloudy and is positive for nitrites and leukocyte esterase on urinalysis. Results of serum electrolyte measurement, liver chemistry studies, and renal function tests are all normal. A radiograph of the chest reveals no abnormalities. Which of the following is the best drug therapy for this patient? A Baclofen B Ciprofloxacin C Methylprednisolone D Prednisone

B Pseudorelapses in multiple sclerosis are the worsening of neurologic symptoms because of another cause, such as a systemic infection requiring antibiotic treatment or supportive care, and should be differentiated from true relapses, which may require corticosteroid treatment. Ciprofloxacin is the best drug therapy for this patient. In light of her new urinary incontinence, fever, and abnormal results on urinalysis, she most likely has a urinary tract infection, which is manifesting clinically as a worsening of her baseline neurologic deficits (leg weakness and ambulatory impairment). She is experiencing a pseudorelapse of her multiple sclerosis (MS)—a neurologic deterioration caused by a change in general health status or by factors in the external environment (such as heat). Pseudorelapses are usually caused by systemic infections or medications. Treatment of the underlying cause (in this patient, with the antibiotic agent ciprofloxacin), obtaining a urine culture to ensure the appropriate antibiotic choice, and supportive care with an antipyretic agent constitute the appropriate treatment. Pseudorelapses need to be differentiated from true MS relapses, which are not associated with systemic symptoms, such as fever. Patients with chronic, progressive forms of MS and moderate or severe baseline neurologic impairment are at high risk for pseudorelapses. Occult urinary tract infection is a common culprit. Symptomatic therapy with antipyretic and antispasticity drugs only is not appropriate for this patient because her primary infection would be left untreated. Additionally, antispasticity drugs, such as baclofen, are likely to worsen her leg weakness. Pseudorelapses are typically self-limited, and spontaneous neurologic improvement usually occurs during or shortly after successful treatment of the underlying infection. Therefore, inclusion of a corticosteroid at treatment onset is unnecessary and could interfere with the treatment of the infection. Because corticosteroids do not treat and may aggravate the primary infection, high-dose methylprednisolone or prednisone should never be given as the only therapy to patients with MS and a suspected urinary tract infection. Occasionally, a systemic infection triggers a new MS relapse; in patients with such a relapse, corticosteroids could be used to treat the neurologic impairment after cure of the infection and exclusion of other causes of deterioration have been documented.

Neuro 84 A 73-year-old woman is evaluated in the emergency department for the onset of a severe, "explosive" headache 8 hours ago. She initially rested in a dark bedroom after headache onset, but when the pain did not abate, her husband drove her to the hospital. The patient has hypertension controlled with lisinopril. Family medical history is noncontributory. As assessment in the emergency department begins, she becomes nauseated, vomits, and then becomes rapidly and progressively more obtunded. Intubation and mechanical ventilation are required. On physical examination, temperature is normal, blood pressure is 188/102 mm Hg, pulse rate is 120/min, and respiration rate is 20/min. The patient exhibits a flaccid quadriplegia, and meningismus is present. Both pupils are 4 mm in diameter and nonreactive; the oculocephalic reflex is absent, and the corneal reflex is absent bilaterally. She has a depressed level of consciousness, with a Glasgow Coma Scale score of 3. Subhyaloid hemorrhages are noted on funduscopy. Results of a complete blood count (with differential) are normal, as are blood urea nitrogen, serum creatinine, and serum electrolyte levels. A CT scan of the head shows an extensive acute subarachnoid hemorrhage and mild prominence of the temporal tips of the lateral ventricles. Which of the following neurologic complications is most likely to have caused this patient's rapid deterioration? A Hydrocephalus B Rebleeding C Syndrome of inappropriate antidiuretic hormone secretion D Vasospasm

B Rebleeding is the most imminent danger after a subarachnoid hemorrhage. The most likely complication to have caused this patient's rapid deterioration is rebleeding. In the first few hours after an initial hemorrhage, up to 15% of affected patients have a sudden deterioration of consciousness, which strongly suggests rebleeding. In patients who survive the first day, the rebleeding risk is evenly distributed during the next 4 weeks, with a cumulative risk of 40% without surgical or endovascular interventions. Occlusion of the responsible aneurysm is thus the first aim in the management of a subarachnoid hemorrhage and is usually performed by coiling or clipping. Typically, patients who develop hydrocephalus after having a subarachnoid hemorrhage are initially alert but then experience a gradual reduction in consciousness over the next 24 hours. Downward deviation of the eyes and small, unreactive pupils indicate dilatation of the proximal part of the cerebral aqueduct with dysfunction of the pretectal area. Although this patient may have secondarily developed hydrocephalus, her symptoms and examination findings suggest that the principal reason for her precipitous decline is early rebleeding. On average, one in five patients who sustain subarachnoid hemorrhages will have mildly enlarged ventricles on the initial CT scan but not frank hydrocephalus. The syndrome of inappropriate antidiuretic hormone secretion is recognized as a potential complication in patients with subarachnoid hemorrhages and other critical care neurologic conditions. Its symptoms and signs are muscle cramps, weakness, altered sensorium, coma, and seizures. This syndrome causes the electrolyte disturbance of hyponatremia. This patient's serum electrolytes were determined to be in the normal range at the time of her evaluation in the emergency department. Vasospasm-induced cerebral ischemia after a subarachnoid hemorrhage has a more gradual onset than occurred in this patient. It often involves more than the territory of a single cerebral artery. The clinical manifestations evolve gradually over several hours and consist of hemispheric focal deficits, a reduction of consciousness, or both. The peak frequency of vasospasm is from 5 to 14 days after the subarachnoid hermorrhage.

Neuro 66 A 55-year-old man is evaluated in the postanesthesia care unit for an inability to move his right arm and leg and a marked delay in his ability to obey commands and instructions. Two hours ago, he underwent femoropopliteal bypass surgery because of severe lower extremity peripheral artery disease. The neurologic deficits were first noticed 15 minutes ago, immediately after extubation. In addition to peripheral artery disease, the patient has a history of hypertension, hyperlipidemia, coronary artery disease, and type 2 diabetes mellitus. Medications include aspirin, metoprolol, lisinopril, simvastatin, and metformin. On physical examination, blood pressure is 162/88 mm Hg, pulse rate is 80/min, respiration rate is 18/min, and BMI is 31. Pupils are 3 mm in diameter, symmetric, and reactive to light. He is aphasic, has right hemiparesis, and has a right extensor plantar response. An immediately obtained CT scan of the head shows a subtle hyperdensity of the left middle cerebral artery in the sylvian fissure but no other obvious abnormalities. Which of the following is the most appropriate next step in management? A Administration of naloxone B Intra-arterial clot extraction C Intravenous administration of recombinant tissue plasminogen activator D MRI of the brain

B Recombinant tissue plasminogen activator as treatment of acute stroke is contraindicated in patients who have had major surgery in the past 14 days. This patient should undergo intra-arterial clot extraction. His presentation is not merely that of someone just emerging from anesthesia or still exhibiting the effects of narcotic analgesia. Rather, the patient is behaving clinically as if he sustained an intraoperative or postoperative stroke. A CT scan of the head obtained so soon after symptom onset will not yet reveal ischemic changes, although it will exclude hemorrhage as a cause of his neurologic symptoms. His CT scan strongly supports the presence of an occluded left middle cerebral artery. The ideal acute treatment for a perioperative ischemic stroke in this patient, whose recent surgery makes him ineligible for recombinant tissue plasminogen activator (rtPA), is consultation with an endovascular neurosurgeon for intra-arterial clot extraction with a device approved by the U.S. Food and Drug Administration. As with the intra-arterial administration of thrombolytics, the use of these devices is limited to comprehensive stroke centers that have the expertise to perform these procedures safely. Although the clot extraction device is recognized by American Heart Association/American Stroke Association guidelines as a reasonable intervention for extraction of intra-arterial thrombi in carefully selected patients for the management of acute stroke, the expert panel also recognizes that the utility of the device in improving outcomes after stroke is unclear. Although the narcotic analgesia he received during surgery may be contributing to this patient's small pupils, it is not the probable explanation for his hemiparesis. Therefore, administration of naloxone or any other opioid antagonist is not appropriate management. The patient, who was last known to be neurologically intact 2 hours ago at the start of the operation, awoke from anesthesia with the neurologic deficit. Although he is within the traditional 3-hour time window for intravenous administration of recombinant tissue plasminogen activator, this therapy is contraindicated within 14 days of major surgery. A diffusion-weighted MRI of the brain would be more sensitive than the CT scan was in detecting acute ischemia but is unnecessary in this patient. The diagnosis of acute ischemic stroke can satisfactorily be established clinically, and the MRI does not add anything of value to the patient's management.

Neuro 1 An 84-year-old man is evaluated for a 5-year history of a gradually worsening gait and a 2-year history of cognitive impairment and urinary incontinence. Twelve years ago, he sustained a closed head injury that caused a mild traumatic subarachnoid hemorrhage and a 5-hour loss of consciousness. Medications include zolpidem (when needed as a sleep aid) and a daily multivitamin. On physical examination, temperature is 36.2 °C (97.2 °F), blood pressure is 128/78 mm Hg, pulse rate is 76/min, respiration rate is 14/min, and BMI is 27. The patient's gait is slow and unsteady and is marked by small, shuffling steps. His level of alertness, speech, posture, arm swing, and muscle tone are all normal, and he has no tremor. He scores 24/30 on the Folstein Mini-Mental State Examination, losing one point in the orientation portion for incorrectly stating today's date, three points in the serial calculation portion, and two points in the recall portion. Results of a complete blood count, a basic metabolic panel, serum vitamin B12 measurement, thyroid function tests, and a urinalysis are normal. An MRI of the brain is shown . Which of the following is the most likely diagnosis? A Alzheimer dementia B Normal pressure hydrocephalus C Parkinson disease D Vascular dementia

B The triad of gait apraxia, dementia, and urinary incontinence, especially when accompanied by enlarged ventricles, is suggestive of normal pressure hydrocephalus. This patient exhibits the classic triad of gait impairment (specifically, gait apraxia), dementia, and urinary incontinence that typifies the potentially reversible syndrome of normal pressure hydrocephalus (NPH). This triad of symptoms eventually occurs in most patients with dementia, and the diagnosis of NPH is often considered but much less often proved to be the correct diagnosis. In this patient, however, strong evidence supports a diagnosis of NPH, including the MRI evidence of ventriculomegaly. Although Alzheimer dementia (AD) is also associated with cognitive impairment and impaired gait, gait does not improve after removal of cerebrospinal fluid in AD as it does in NPH. AD is so common in elderly patients with cognitive impairment that excluding it as a cause can delay the diagnosis of NPH; this delay may help explain some of the eventual shunt failures that occur even in patients with well-diagnosed NPH. Therefore, recognizing reversible dementia syndromes as soon as possible is imperative because of the therapeutic opportunity these syndromes represent. The only symptom this patient has that is shared by patients with Parkinson disease is a shuffling gait. Otherwise, his presentation—normal posture, arm swing, and muscle tone and the absence of a tremor—is quite different. Likewise, this patient has no history of or symptoms suggesting stroke or vascular disease, such as sudden onset of neurologic signs or symptoms, which makes vascular dementia unlikely. Although coincident vascular, Alzheimer-type, and Parkinson-type pathology is a common finding in autopsy studies, even in neurologically unimpaired healthy elderly adults, this fact should not constitute the basis for a diagnosis in the setting of a classic reversible dementia syndrome.

Neuro 47 A 44-year-old woman is evaluated for a 4-month history of worsening gait and bilateral leg numbness. She underwent gastric bypass surgery 8 years ago but has no other relevant personal or family medical history. Her only medications are a daily multivitamin and vitamin B12. On physical examination, temperature is normal, blood pressure is 130/80 mm Hg, pulse rate is 90/min, respiration rate is 14/min, and BMI is 29. The patient has moderate gait ataxia, lower extremity spasticity, hyperreflexia, and bilateral extensor plantar responses. Strength is normal, but vibration and proprioceptive sensation is impaired in both feet. Results of laboratory studies show a mild normocytic anemia. Serum vitamin B12, vitamin E, methylmalonic acid, and homocysteine levels are all normal. Nerve conduction studies and an electromyogram reveal a moderate axonal peripheral neuropathy. MRIs of the cervical spinal cord and the thoracic spinal cord show no abnormalities. Which of the following is the most appropriate next diagnostic study? A Antinuclear antibody testing B Measurement of serum copper and zinc levels C MRI of the brain D Serum neuromyelitis optica (NMO)-IgG autoantibody test

B Vitamin B12 and copper deficiencies are associated with malabsorption syndromes related to gastric bypass surgical procedures and can cause anemia and a syndrome of progressive myeloneuropathy. Serum copper and zinc levels should be measured in this patient. She has a progressive myeloneuropathy syndrome and mild anemia, both caused by copper deficiency. Gastric bypass surgery is a risk factor for this clinical syndrome, which can be caused by vitamin B12 deficiency, copper deficiency, or both. Given that her serum vitamin B12 level is normal, copper deficiency is the most likely cause. Overingestion of zinc can also impair copper absorption. Therefore, both serum copper and zinc levels should be evaluated. In patients with copper deficiency, the spinal cord MRI can be normal or demonstrate signal changes involving the dorsal columns. Antinuclear antibody testing is useful for detection of an inflammatory or a rheumatologic abnormality, but such a disorder is unlikely in the absence of any clinical symptoms or signs suggesting a systemic inflammatory disease. An MRI of the brain may be appropriate in some patients with myelopathy to investigate for multifocal diseases, such as multiple sclerosis (MS). This patient, however, has no cerebral symptoms or signs, and the clinical syndrome exhibited and electromyography results suggest a myeloneuropathy. Neuromyelitis optica (NMO) can be distinguished from MS by the NMO-IgG autoantibody test. Long considered an MS variant, NMO is now recognized as a distinct demyelinating disease with a predilection for the optic nerves and spinal cord. An MRI of the spinal cord typically shows extensive lesions in patients with NMO. This patient's normal results on spine MRI make the diagnosis of NMO unlikely and the NMO-IgG autoantibody test unnecessary.

How does neuroblastoma differ from pheochromocytoma?

Both have elevated HMA AND VMA but pheochromocytoma presents with signs of increases autonomic tone: fainting spells, sweating, palpitations, and hypertension.

Neuro 16 A 75-year-old woman is seen for a follow-up evaluation. Two weeks ago, she was brought to the emergency department after slipping in the bathroom and hitting her head on the tub. A CT scan of the head obtained at that time showed a small calcified lesion over the right sphenoid wing. A subsequent outpatient contrast-enhanced MRI of the brain showed a 2-cm dura-based lesion with homogeneous enhancement, consistent with a meningioma; no mass effect or edema was detected. She is otherwise healthy, has no general or neurologic symptoms, and takes no medications. Vital signs and physical examination findings are normal. Which of the following is the best management option for this patient? A Chemotherapy B Serial MRIs of the brain C Stereotactic radiosurgery D Surgical resection

B Small, asymptomatic meningiomas in older adults should be monitored with serial imaging. This patient should have serial MRIs of the brain. Meningiomas are benign, slow-growing tumors that are often discovered as an incidental finding when head imaging is obtained for evaluation of unrelated symptoms. Their appearance on CT scans and MRIs is diagnostic. Appropriate management depends on the age of the patient and the size and location of the tumor. For this patient, who is elderly and has a small, asymptomatic meningioma, the most appropriate management strategy is to follow up with serial MRIs of the brain. In patients with incidentally diagnosed meningiomas, most clinicians perform imaging studies every 3 to 6 months during the first year. If the tumor remains stable, yearly imaging may be performed for the next 5 to 10 years. Densely calcified meningiomas are particularly indolent. Chemotherapy is not warranted in this patient because meningiomas are usually benign in histology and behavior. In patients with large or symptomatic meningiomas, surgical resection is the treatment of choice, whereas observation usually is appropriate for small, asymptomatic meningiomas until evidence of progressive enlargement or symptoms develop. Stereotactic radiosurgery, which selectively irradiates a sharply defined target, is used for symptomatic meningiomas that are not amenable to surgical resection. Surgical resection is generally recommended only for patients who are symptomatic or for young patients in whom it is likely that symptoms will develop over time with a slow-growing tumor.

Central cord syndrome

B/L UE sensory loss but LE are okay!! aka Syringomyelia. (Look for elderly pt w acute hyperextension of the neck)

Syringomyelia

B/L loss of P/T in a capelike distribution

Coma, pupils

B/l Fixed dilated pupils - severe Anoxia U/L dilated pupil - CN 3 damage Pinpoint poils - Pons damage, narcotics, or ICH Abnormal pupillary light reflex - structural lesion (hemorrhage or mass), drugs that affect pupils (Morphine, Atropine), anoxic encephalopathy, recent eye drops.

Why give B1 before glucose in hypoglycemia?

B1 is a substrate in the TCA glycolysis pathway. Will deplete B1 otherwise.

atrophic gastritis, peripheral neuropathy, unsteady gait, LE hyporreflexia, babinskis sign, vibration sensation decreased, can't recall 3 objects on MSE - diagnosis

B12 deficiency

Recurrent, brief episodes brought on by predictable head movts or position change No neuro or auditory symptoms Dix-Hallpike maneuver causes nystagmus.

BPPV - crystalline deposits (canaliths) in the semicircular canals that disrupt the flow of fluid in the vestibular system - canalith repositioning maneuver (Epley maneuver)

vertigo w/ changes in position with possible n/v

BPPV- moving calcium carbonate crystals Downbeating and torsional nystagmus with Dix-Hallpike Tx- Eply maneuver

Tension HA

Band-like pain/ache around teh head - generalized or more intense around the neck or back of head. Look for tender muscles. Stress, depression or anxiety. Rx - NSAIDs, if severe can give Sumatriptan.

Pseudo tumor cerebri

Benign intracranial htn, patient is female, obese, high ICP, headaches with visual disturbances and menstrual irregularities isolated CNVI palsy worry about blindness! tx: acetazolamide

What medication can cause falls in elderly as well as paradoxical agitation?

Benzodiazepines!!

What is an anticholinergic used in Parkinsonism that is good at treating tremors but not with the bradykinesia?

Benztropine.

Treatment of acute dystonia?

Benztropine. Diphenhydramine.

Treatment of akathisia?

Beta blocker Benzo

MS treatment to reduce frequency of exacerbations

Beta interferon or glatriamer acetate

Location of dentothalamic fibers and function?

Between ML and ALS in midbrain, lesion causes cerebellar dystaxia with intention tremor

Elbow Flexion

Biceps Musculocutaneous C5, C6 Biceps, Brachioradialis Reflex

Musculocutaneous nerve innervation

Biceps brachii, brachialis, coracobrachialis muscles; sensory information from lateral cutaneous nerve of the forearm

Kluver-Bucy syndrome

Bilateral damage to the amygdala results in disinhibited behavior. Associated with HSV-1.

Triad of 1) Focal Neurological Deficits 2) Headaches 3) Fever should get you thinking of?

Brain Abscess

Isolated, round, smooth-bordered, ring enhancing lesion on CT in immunocompetent person

Brain abscess

impaired fluency and repetition

Broca's Aphasia- language production post part of Inferior Frontal Gyrus (dominant- usually left) MCA- superior division w/ contra arm, face weakness

Parkinson Treatment

Bromocriptine, Pramipexole - good first line, DA agonists. For hesitancy/immobility - spec Pramipexole

Loss of pain and temp contralaterally, usually 1-2 levels below cord injury, Ipsilateral hemiparesis and diminished propioception, vibratory sensation, and light touch at the level of the spinal cord injury and below.

Brown-Sequard syndrome.

Inclusion body myositis

Bx will show endomesial inflammation, basophillic rimmed vacuoles.

Neuro 73 A 36-year-old woman is evaluated in the emergency department for a 3-day history of confusion and falls. Her husband, who accompanied her, says that her symptoms seem to be getting worse. She has a 20-year history of Crohn disease that necessitated a partial small bowel resection because of stricture formation 5 years ago; she had a partial colectomy for fistulae 2 years ago and has had recent weight loss due to diarrhea. Current medications include prednisone and azathioprine. On physical examination, temperature is 35.6 °C (96.0 °F), blood pressure is 142/76 mm Hg, pulse rate is 90/min, respiration rate is 14/min, and BMI is 17. Temporal muscle wasting, sunken supraclavicular fossae, and absent adipose stores are noted. Abdominal examination reveals surgical scars and a few low-pitched bowel sounds, but results are otherwise normal. On neurologic examination, the patient is confused; she is unable to state the date and does not know the name of the hospital. Marked nystagmus is noted. There is no nuchal rigidity or obvious motor weakness. Deep tendon reflexes are reduced, and plantar responses are flexor. The patient has a markedly ataxic gait. Which of the following is the best initial management? A Electroencephalography B Haloperidol C Thiamine D Vancomycin, ampicillin, and ceftriaxone

C Wernicke encephalopathy is due to thiamine deficiency; may result in mental status changes, ophthalmoplegia, nystagmus, and unsteady gait; and is best treated with thiamine. This patient should receive thiamine. She has Wernicke encephalopathy, a syndrome that results from deficiency of vitamin B1, an important coenzyme in several biochemical pathways of the brain. Typical clinical manifestations of the disorder include mental status changes, nystagmus, ophthalmoplegia, and unsteady gait, all varying in intensity from minor to severe. The typical clinical triad of ataxia, areflexia, and ophthalmoplegia is seen in only 19% of affected patients. Conditions associated with Wernicke encephalopathy include AIDS, alcohol abuse, cancer, hyperemesis gravidarum, prolonged total parenteral nutrition, postsurgical status (particularly gastric bypass), and glucose loading (in a predisposed patient). The disorder develops in patients who are malnourished as a result of malabsorption, a poor diet, increased metabolic requirements during illness, or thiamine deficiency. Recognition of the disorder and treatment with intravenous administration of thiamine are essential. Establishing a diagnosis of Wernicke encephalopathy can be difficult, but treatment with thiamine should not be withheld while considering other disorders. The diagnosis remains a clinical one and should be considered in any patient with poor nutrition or a disorder that can result in impaired absorption of food who exhibits one or more of the classic features of mental status change, gait impairment, and ocular signs. Measurements of serum thiamine level and erythrocyte transketolase activity lack specificity as diagnostic tests and may not be readily available. An MRI of the brain can help rule out other conditions and may show some characteristic changes of Wernicke encephalopathy, such as paraventricular signal changes in the thalamus, mamillary bodies, periaqueductal region, and cerebellum. Although these MRI changes seem to be quite specific for this disorder, their sensitivity is only 53%. Because Wernicke encephalopathy remains a clinical diagnosis, other neurologic disorders should be considered in this patient after thiamine has been administered. Electroencephalography can help exclude a seizure disorder, such as nonconvulsive status epilepticus. Infections, including encephalitis and meningitis, for which intravenous administration of broad-spectrum antibiotic drugs (such as vancomycin, ampicillin, and ceftriaxone) may be appropriate also should be part of the differential diagnosis and can be excluded with cerebrospinal fluid analysis. Haloperidol is not indicated in this patient, who is confused but has no apparent history of psychosis or agitation. Some patients with Wernicke encephalopathy do have agitation, hallucinations, and behavioral disturbances that can mimic an acute psychosis.

Neuro 4 A 52-year-old woman is evaluated for a 2-year history of burning feet. Symptoms are constant and are worse at night. The patient is overweight and has a history of hypertension treated with lisinopril. There is no known family history of peripheral neuropathy. On physical examination, the patient is afebrile; blood pressure is 134/88 mm Hg, pulse rate is 66/min, respiration rate is 12/min, and BMI is 28. Neurologic examination shows diminished pinprick and temperature sensation on the dorsal and plantar surfaces of both feet. Cranial nerve examination and testing of manual muscle strength, deep tendon reflexes, proprioception, and coordination reveal no abnormalities. Laboratory studies show a fasting plasma glucose level of 102 mg/dL (5.7 mmol/L). Results of a complete blood count, vitamin B12 measurement, and serum protein electrophoresis are all normal. Electromyographic testing shows a mild reduction in the sensory nerve action potential in the legs, compatible with a mild peripheral neuropathy. An MRI of the lumbar spine is normal. Which of the following is the most appropriate next diagnostic test? A Cerebrospinal fluid examination B Genetic testing for Charcot-Marie-Tooth disease C Glucose tolerance test D Skin biopsy

C A history of burning or lancinating distal extremity pain and examination findings showing only sensory loss suggest a small-fiber peripheral neuropathy, which is most frequently associated with diabetes mellitus and impaired glucose tolerance. A 2-hour glucose tolerance test to detect diabetes mellitus or impaired glucose tolerance is the most appropriate next diagnostic study for this patient. The patient's history of burning feet, in conjunction with neurologic examination findings showing only distal sensory loss (with normal reflexes and muscle strength), suggests a small-fiber peripheral neuropathy. The most common identifiable cause of small-fiber peripheral neuropathy is diabetes or impaired glucose tolerance. This patient's fasting plasma glucose level, which is just over the upper limit of normal, should prompt a 2-hour glucose tolerance test. This test is more sensitive and can detect patients with diabetes and a normal fasting plasma glucose level. Approximately 30% of patients with normal fasting plasma glucose levels will have an oral glucose tolerance test diagnostic for diabetes. A result of 140 mg/dL to 199 mg/dL (7.8 mmol/L to 11.0 mmol/L) from this test establishes a diagnosis of impaired glucose tolerance, and a result of 200 mg/dL (11.1 mg/dL) or greater is diagnostic of diabetes. An examination of cerebrospinal fluid (CSF) obtained on lumbar puncture is not indicated in this patient. CSF studies should be considered in patients with acute or rapidly progressive neuropathy or polyradiculoneuropathy and in patients with severe weakness, sensory loss, or absent deep tendon reflexes. In patients with Guillain-Barré syndrome or chronic inflammatory polyradiculoneuropathy, CSF examination typically shows a normal cell count with elevated CSF protein level (albuminocytologic dissociation). CSF testing in such patients also helps to exclude infectious disorders, such as West Nile virus, HIV, and polyradiculoneuropathy caused by cytomegalovirus, which can present similarly. Given the symptom of burning feet and the absence of high arches, hammertoes, or abnormalities on strength and reflex testing, Charcot-Marie-Tooth disease is not likely. Although Charcot-Marie-Tooth disease is a common cause of peripheral neuropathy, patients with this disorder typically do not have neuropathic pain, and many patients also do not have sensory symptoms, despite having sensory loss on examination. Many patients in whom Charcot-Marie-Tooth disease is ultimately diagnosed are unaware of a family history of peripheral neuropathy, so the lack of family history in this patient does not necessarily exclude the diagnosis. Genetic testing is commercially available for many of the different forms of this disease, but genetic testing should be considered only in those patients with a known family history or in those with long-standing neuropathic symptoms and high arches and hammertoes on examination. Other inherited causes of small-fiber peripheral neuropathy include the hereditary burning feet syndrome, hereditary amyloidosis, and the hereditary sensory autonomic neuropathies. A skin biopsy is not indicated in this patient, given that the electromyographic (EMG) study was abnormal and showed objective evidence of peripheral neuropathy. Abnormalities on EMG testing in patients with suspected small-fiber peripheral neuropathy are seen in only 25% to 30% of patients. When EMG studies are normal, skin biopsy or autonomic nervous system testing should be considered to establish the diagnosis of a small-fiber peripheral neuropathy. Skin testing for small-fiber peripheral neuropathy is commercially available and can show reduced numbers of nerve fibers in the epidermis. Because small-fiber nerves also make up the autonomic nervous system, autonomic nervous system testing can also establish the presence of a small-fiber peripheral neuropathy.

Neuro 69 A 54-year-old man with a 1-year history of Parkinson disease is brought to the office by his wife, who is concerned about her husband's recent excessive gambling. She says that in the past 6 months, he has been spending increasing amounts of time at a casino, where he rarely enjoyed going before the diagnosis of Parkinson disease. His behavior is otherwise unchanged. The patient has been taking ropinirole since the diagnosis and has had a marked diminution in tremor as a result; he has had no difficulties with or change in mood, cognition, or sleep. General physical examination findings are normal. Neurologic examination shows normal speech, language, mood, and mental status. There is mild left upper limb rigidity and a minimal resting tremor, but no other abnormalities are detected. Which of the following is the most likely cause of this patient's gambling problem? A Bipolar disorder B Frontotemporal dementia C Medication-related compulsive behavior D Parkinson-related dementia

C A potential adverse effect of dopamine agonist therapy is the development of compulsive behaviors, such as pathologic gambling, shopping, and hypersexuality. This patient has developed an excessive gambling behavior after receiving treatment with the dopamine agonist ropinirole for Parkinson disease. Patients who are initiated and maintained on dopamine agonist medications to control Parkinson disease should be warned about the potential for developing abnormal, compulsive behaviors, such as excessive gambling, excessive shopping, and hypersexuality. These adverse effects, which can also develop in patients taking such medications for restless legs syndrome, are likely due to effects on the dopaminergic reward centers in the brain. Factors that can increase the risk of these behaviors include a young age at diagnosis in men and a history of mood disorders, alcohol abuse, or obsessive-compulsive behaviors. Other potential adverse effects of dopamine agonist therapy include orthostatic hypotension, nausea, vomiting, hallucinations, and sleep attacks. These potential adverse effects of dopamine agonist medications should be discussed with patients. Sleep attacks, or the sudden irresistible urge to sleep, have garnered considerable medicolegal and social attention. There is controversy about whether such attacks are distinct from excessive daytime somnolence, an established potential side effect of dopamine agonist medications. However, there have been rare reports of sleep attacks occurring while driving and resulting in motor vehicle accidents. The current recommendation is to warn patients taking these medications about the risk of sleep attacks while driving. Bipolar disorder is an illness characterized by periods of mood elevation and one or more episodes of depression. The period of mood elevation is characterized by a distinct period of abnormal and persistently elevated, expansive, or irritable mood that lasts at least 1 week. This patient's mood is described as normal and he lacks a history of depression, both of which make bipolar disorder unlikely. Patients with frontotemporal dementia may develop compulsive behaviors but would be expected to also exhibit other signs of personality or behavioral change. This patient's only sign of such a change is his compulsive gambling. The patient has no history or examination findings suggesting that Parkinson-related dementia has developed, which makes it an unlikely cause of his excessive gambling. Parkinson-related dementia is not associated with compulsive gambling behavior.

Neuro 27 A 41-year-old man is evaluated in the emergency department for a 3-hour history of a severe headache. The headache began abruptly while he was at work and involved the left frontal temporal region and the left ear and jaw. The pain is pulsatile and severe. There are no associated symptoms. He has a history of migraine without aura. On physical examination, temperature is 36.2 °C (97.2 °F), blood pressure is 146/88 mm Hg, pulse rate is 68/min, respiration rate is 18/min, and BMI is 26. The patient has mild left ptosis and mild anisocoria (3 mm pupil on the left and 4 mm on the right). There is no meningismus. The remainder of the neurologic examination and funduscopic examination findings are normal. An electrocardiogram shows normal findings. Laboratory studies show a normal complete blood count, erythrocyte sedimentation rate, and serum chemistry levels. Results of a cerebrospinal fluid analysis are normal. An unenhanced CT scan of the head and neck shows no abnormalities. A magnetic resonance angiogram of the neck (left) and an MRI of the brain (right) are shown . Which of the following is the most appropriate next step in treatment? A Intra-arterial nimodipine B Intravenous dihydroergotamine C Intravenous heparin D Intravenous nitroglycerin E Stent-assisted aneurysm coiling

C Acute unilateral headache with associated Horner syndrome represents acute carotid dissection until proved otherwise. This patient has a carotid dissection, for which he should receive heparin. Acute headache with oculosympathetic paresis (Horner syndrome) is a common presentation of carotid dissection and must be assumed to be carotid dissection until proved otherwise. Such a headache may precede the onset of cerebral ischemia (transient ischemic attack or stroke) by days to weeks. Additional manifestations of this condition may include ipsilateral throbbing neck or orbital pain. The pain from carotid dissection may occur suddenly or develop gradually. Neurologic abnormalities due to ischemia in the ipsilateral hemisphere (causing contralateral numbness or weakness) or retina (causing ipsilateral monocular visual symptoms) also may occur. Magnetic resonance angiography of the carotid arteries and carotid duplex ultrasonography are indicated in the clinical evaluation of possible carotid dissection. Although the management of carotid dissection is controversial, most experts agree on the intravenous use of heparin in the acute setting to prevent distal embolism and stroke. Acute severe headache always requires imaging of the neck and cerebral blood vessels to rule out a vascular cause. This patient's magnetic resonance angiogram of the neck shows a long, tapered stenosis of the left internal carotid artery in the neck, which indicates dissection. Although this patient's CT scan of the head and neck had normal results and MRI of the brain reveals no abnormalities of the brain parenchyma, the MRI does show dissection of the internal carotid artery. There is no evidence to support the use of intra-arterial nimodipine or intravenous nitroglycerin in this setting. The carotid stenosis in this patient is due to the mural hematoma in the wall of a blood vessel, not to an arterial spasm. In addition, these drugs may decrease systemic arterial pressure and cerebral perfusion pressure distal to the severe carotid stenosis. Despite his migraine history and his severe unilateral and pulsatile headache, this patient's current headache does not fulfill the diagnostic criteria for migraine; there are no associated features of nausea, vomiting, photophobia, or phonophobia. Because of its potent α-adrenergic stimulation, dihydroergotamine, a migraine therapy, is contraindicated in patients with conditions that predispose them to vasospastic reactions. This medication is thus not appropriate in the presence of a carotid dissection. Stent-assisted aneurysm coiling is not indicated in this patient because he does not have an intracerebral aneurysm, according to the results of his imaging studies.

Neuro 77 A 34-year-old woman is evaluated in the office for right-sided facial paralysis that she noticed on awakening 1 hour ago. She has a 10-pack-year smoking history. Personal and family medical history is noncontributory. Her only medication is a daily oral contraceptive. On physical examination, temperature is 36.5 °C (97.7 °F), blood pressure is 110/70 mm Hg, pulse rate is 82/min, respiration rate is 14/min, and BMI is 26. Limb strength, reflexes, and tone are normal bilaterally. Findings from a sensory examination, which included her face, are also normal. When asked to raise her eyebrows, the patient does not elevate the right side. When asked to shut her eyes, she cannot close the right one, but the globe rotates upward, partially covering the iris. When asked to smile, the patient does not move the right side of her face. Which of the following is the most likely diagnosis? A Graves ophthalmopathy B Left cerebral infarction C Right facial nerve (Bell) palsy D Right trigeminal neuralgia

C Any cause of a complete facial neuropathy will impair the entire hemiface, including the forehead muscles. This patient's physical examination findings most strongly suggest right facial nerve (Bell) palsy. The precise cause of Bell palsy is not known, and it is still considered an idiopathic disorder. Research strongly suggests it may be the result of herpes simplex virus infection of the facial nerve. Bell palsy is not considered contagious. The seventh cranial nerve innervates all muscles of facial expression (the mimetic muscles). Any cause of a complete facial neuropathy will therefore impair the entire hemiface, including the forehead corrugators typically spared by cerebral lesions. Bell phenomenon describes the reflexive rolling upwards of the globe during eye closure. When a patient is asked to close the eyes, forced eyelid opening will reveal this phenomenon, as will the selective paralysis of the orbicularis oculi due to a facial neuropathy. Facial neuropathies will otherwise spare the extraocular muscles that govern globe movement. Because Bell palsy is a diagnosis of exclusion, clinicians need to make every effort to exclude other identifiable causes of facial paralysis, such as Lyme disease, acute and chronic otitis media, cholesteatoma, and multiple sclerosis. Other common causes of acute peripheral facial paralysis will often have findings on history or physical examination that suggest the correct diagnosis. Graves ophthalmopathy can cause proptosis or extraocular muscle edema with consequent eye movement abnormalities but is not associated with the facial hemiparalysis typical of facial nerve (Bell) palsy. Cerebral infarction, brain hemorrhage, or any structural brain lesion can cause weakness of the lower face but not of the forehead because the bilateral cortical representation of the midline forehead spares the forehead corrugators. Some limb or sensory abnormality is also often, but not universally, observed in the setting of cerebral infarction; no such abnormality was observed in this patient. Therefore, despite her cerebrovascular risk factors of oral contraception and cigarette smoking, this patient is unlikely to have had a cerebral infarction. The trigeminal nerve provides sensation, not movement, to the muscles of facial expression, so trigeminal neuralgia is not a likely diagnosis in this patient with normal sensation.

Neuro 65 A 40-year-old man is evaluated in the emergency department for ongoing seizure activity. His wife says that he was having a generalized tonic-clonic seizure when she awoke 45 minutes ago and that she called the paramedics when the seizure continued for more than 5 minutes; she also reports that he had three seizures yesterday, each consisting of staring and unresponsiveness with repetitive lip smacking and each lasting 2 minutes. The patient has a known history of partial epilepsy secondary to a cavernous malformation in the left temporal lobe and has an average of four seizures each year. He is on phenytoin therapy. On examination, he is comatose with continuous rhythmic twitching of the right face and arm. Blood pressure is 120/70 mm Hg, pulse rate is 80/min, and arterial oxygen saturation rate by pulse oximetry is 85% (on ambient air). The patient is intubated. Which of the following is the most appropriate next step in management? A CT of the head B Electroencephalography C Intravenous administration of lorazepam D Measurement of serum phenytoin level

C Benzodiazepines are the first-line treatment for status epilepticus. This patient should receive lorazepam. He most likely has status epilepticus, a neurologic emergency defined as seizures that persist or recur without interval recovery for a period of 30 minutes or longer. Approximately 50% of patients with status epilepticus have an established diagnosis of epilepsy, and among these patients, nonadherence to medication is a common precipitating event. Convulsive status epilepticus has a mortality rate of approximately 20%. When generalized convulsive status epilepticus continues, clinical signs of ongoing electrical seizure activity will often become increasingly subtle and may only involve subtle twitching of the eyes, face, or limbs, as seen in this patient. However minimal, this continuing electrical seizure activity must be corrected to avoid potential morbidity and mortality. Persistent seizure activity results in acute systemic complications, including fever, hemodynamic instability, acidosis, rhabdomyolysis, and pulmonary edema, all of which must be carefully managed. Airway, breathing, and circulatory status should be assessed at presentation and monitored continuously. Early diagnosis and therapeutic intervention are critical because an increase in the duration of status epilepticus is directly correlated with increased mortality. Treatment should not be delayed for diagnostic testing. Intravenous administration of a benzodiazepine (lorazepam or diazepam) is accepted as first-line therapy and may be administered by emergency medical personnel in the field. A CT of the head may ultimately be necessary to evaluate for bleeding of the cavernous malformation. Although emergent head imaging is often useful in the absence of a known underlying cause of status epilepticus, it should never delay treatment with a benzodiazepine. Because this patient's symptoms are quite consistent with status epilepticus, confirming the diagnosis with an electroencephalogram (EEG) and thus delaying treatment is unnecessary. In patients who are unresponsive or somnolent after status epilepticus, continuous EEG monitoring is strongly advocated to distinguish between ongoing nonconvulsive status and postictal states. Intravenous administration of phenytoin or phenobarbital is used as second-line therapy for status epilepticus not responsive to benzodiazepines. If administration of these agents is planned, it should not be delayed while awaiting results of serum drug levels.

Neuro 39 A 40-year-old woman is evaluated in the emergency department 30 minutes after having a 1-minute episode of involuntary jerking of the right hand that spread up the right arm and a subsequent 2-minute episode of loss of consciousness and witnessed generalized tonic-clonic seizure activity. She has a 1-month history of increasing confusion and low-grade headache. Two years ago, she was treated for cutaneous melanoma. On physical examination, the patient is awake and alert. Vital signs are normal. There are no signs of meningismus and no papilledema. She has mild right-sided facial droop and only antigravity strength in the right arm; strength is normal elsewhere. Laboratory studies, including a complete blood count, measurement of serum electrolyte and plasma glucose levels, and a urine toxicology screen, show no abnormalities. Which of the following is the most appropriate next diagnostic test for this patient? A CT of the head B Electroencephalography C Gadolinium-enhanced MRI of the brain D Lumbar puncture E Positron emission tomography

C Contrast-enhanced MRI is the diagnostic modality of choice when brain metastasis is suspected. This patient should have a gadolinium-enhanced MRI. Her melanoma has most likely metastasized to her brain. Brain metastases are 10 times more common than primary brain tumors, and metastatic disease of the central nervous system (CNS) is estimated to occur in 20% to 40% of adults with cancer. The tumors most likely to metastasize to the CNS in adults are lung and breast carcinoma and melanoma. Presenting signs and symptoms vary according to the size and location of the metastatic lesion(s) and occur as a result of local mass effect or increased intracranial pressure; common symptoms include seizure, headache, behavioral changes, and subacute progressive focal neurologic deficits. A high level of clinical suspicion for CNS metastasis is therefore justified when new neurologic deficits develop in patients such as this one with a known primary tumor. Contrast-enhanced MRI is the diagnostic modality of choice when brain metastasis is suspected. MRI has a higher sensitivity and specificity than CT scanning and is also safer because MRI contrast does not cause nephrotoxicity, and allergies are extremely rare. Furthermore, normal findings on a CT scan do not exclude the presence of a brain tumor, so a follow-up MRI would be required. Despite her witnessed tonic-clonic seizure, electroencephalography is not appropriate as the next diagnostic step because brain imaging is more likely to reveal a potential underlying cause for the seizure. MRI is the study of choice to detect tumors, gliosis associated with encephalitis, head trauma, and stroke that may be causing seizures. Lumbar puncture is also inappropriate in this patient because seizures are almost never present as the sole manifestation of a CNS infection or subarachnoid hemorrhage; lumbar puncture is indicated only if there are other indicators of these conditions. CNS infection is indicated by the presence of fever or altered mental status. Patients with subarachnoid hemorrhage may also have seizures, but this type of hemorrhage is associated with severe headache, altered mental status, syncope, and neurologic deficits and usually will be identified by CT or MRI imaging. Positron emission tomography (PET) can be used to identify malignant tumors with high metabolic rates and may be helpful in planning the next diagnostic step (which lesion to biopsy) and in prognosis. However, the use of PET before the establishment of a brain tumor with MRI is premature and not indicated.

Neuro 51 A 69-year-old woman is evaluated in the office for visual hallucinations that have occurred several times over the past 3 months. During the past year, she and others have noticed that she is walking more slowly, has a mildly stooped posture, and occasionally has saliva accumulating at the corner of her mouth. Over the past 3 years, she has had increasing difficulty organizing financial records, has forgotten appointments, and twice became lost in crowded but familiar parts of her town. According to her husband, who accompanied her to this appointment, her sleep over the past 10 years has been marked by semipurposeful and flailing limb movements and occasional clear speech, once taking the form of a speech to employees. Her mother had onset of Alzheimer dementia at age 77 years and died at age 82 years. Her only medication is a daily multivitamin. On physical examination, temperature is 36.6 °C (97.9 °F), blood pressure is 142/76 mm Hg supine and 110/68 mm Hg standing, pulse rate is 80/min supine and does not change with standing, respiration rate is 14/min, and BMI is 22. There is trace cogwheeling of the right upper limb. When the patient walks, her posture is slightly stooped, right arm swing is slightly diminished, and gait is slow without frank shuffling. Rapid alternating movements are mildly reduced in speed and amplitude in the right hand. She has mildly reduced facial expression and mildly reduced inflection of speech. She scores 27/30 on the Folstein Mini-Mental State Examination, losing one point for misstating the date and two points on the memory portion of the test. Her level of alertness is normal, and she has no tremor. Results of a complete blood count, basic metabolic panel, serum vitamin B12 measurement, and thyroid function tests are normal. An MRI of the brain without contrast shows no abnormalities. Which of the following is the most likely diagnosis? A Alzheimer dementia B Creutzfeldt-Jakob disease C Dementia with Lewy bodies D Frontotemporal dementia E Vascular dementia

C Dementia with Lewy bodies is characterized by dream-enactment behavior, cognitive decline, parkinsonism, and visual hallucinations. This patient has symptoms of dream-enactment behavior, cognitive decline, parkinsonism, and visual hallucinations, which together are the hallmark features of dementia with Lewy bodies. Also characteristic of this type of dementia are the physical findings of orthostatic hypotension and features of mild parkinsonism, such as a reduced degree of facial expression (hypomimia), reduced arm swing, stooped posture, and mild cogwheeling. Alzheimer dementia is a common comorbidity in patients with dementia with Lewy bodies. However, this diagnosis clearly does not in itself sufficiently account for the additional features of parkinsonism, dream enactment behavior, visual hallucinations, and dysautonomia. Creutzfeldt-Jakob disease is a rapidly progressive dementia that is associated with early age at onset and prominent myoclonus and typically results in death within a year of onset. This patient's 10-year history of symptoms and absence of myoclonus rule out that diagnosis. Because the hallmark features of frontotemporal dementia, such as apathy, perseveration, hoarding, disinhibition, and other personality changes, are lacking in this patient, that diagnosis is unlikely. This patient has no cerebrovascular risk factors for or history of stroke. Additionally, her MRI has no features to suggest a vascular etiology of her symptoms. Therefore, vascular dementia is very unlikely.

Neuro 15 A previously healthy 50-year-old woman is admitted to the hospital after three recent, transient episodes of nonfluent aphasia and right-hand numbness and weakness. One week before onset of these focal neurologic symptoms, she developed aching left jaw pain, which has persisted. She has no other medical problems and takes no medications. On physical examination, vital signs are normal. A left carotid bruit is heard on auscultation. Left miosis and left ptosis are noted. Results of laboratory studies and a CT scan of the head are normal. Which of the following is the most likely diagnosis? A Cluster headache B Giant cell arteritis C Spontaneous left internal carotid artery dissection D Spontaneous left vertebral artery occlusion

C Dissection of the cervical arteries, although an infrequent occurrence, is a leading cause of stroke in young and otherwise healthy persons. The most likely clinical diagnosis to explain this patient's focal neurologic symptoms is a left internal carotid artery dissection with resultant transient ischemic attacks (TIAs). Carotid dissection characteristically develops after head or neck trauma but may occur spontaneously. Manifestations of this condition include ipsilateral throbbing neck, head, or orbital pain with possible Horner syndrome. The pain from carotid dissection may occur suddenly or develop gradually. Such a dissection can cause a TIA or ischemic stroke by one of two mechanisms: either the mural hematoma expands to the point of occluding the lumen, or a thrombus forms and embolizes to cause distal arterial branch occlusion. Dissection of the cervical arteries, although an infrequent occurrence, is a leading cause of stroke in young and otherwise healthy persons. Magnetic resonance angiography of the carotid arteries and carotid duplex ultrasonography are indicated in the clinical evaluation of possible carotid dissection. A cluster headache is an excruciating unilateral headache of extreme intensity, with a typical duration of 15 minutes to 3 hours. Its pain is lancinating or boring in quality and is located behind the eye (periorbital) or in the temple, sometimes radiating to the neck or shoulder. The cardinal symptoms of a cluster headache attack are ptosis (drooping eyelid), conjunctival injection (redness of the conjunctiva), lacrimation (tearing), rhinorrhea (a runny nose), and, less commonly, facial blushing, swelling, or sweating. These features are known as the autonomic symptoms. The neck is often stiff or tender in the aftermath of a headache, with jaw or tooth pain sometimes present. Although this patient had ptosis and jaw pain, there were no other features to support a diagnosis of cluster headache. Additionally, one would not expect a patient with a cluster headache to have focal cortical dysfunction (such as aphasia). Giant cell arteritis (or temporal arteritis) is a granulomatous vasculitis that predominantly affects the extracranial branches of the carotid artery. This condition occurs almost exclusively in patients older than 50 years of age and has a female predominance. Clinical manifestations of giant cell arteritis include headache, jaw or tongue claudication, scalp tenderness, systemic symptoms, and fever. Involvement of the primary branches of the aorta also may cause limb claudication. Giant cell arteritis is not associated with pain along the carotid artery or cerebral hemispheric symptoms and would be an unusual diagnosis in this relatively young patient. The patient's clinical symptoms are aphasia, right hemiparesis, and right hemisensory loss; all are referable to the middle cerebral artery territory of the dominant left hemisphere and thus are consistent with an anterior circulation stroke event. There are no posterior circulation symptoms (in the vertebrobasilar artery territory) to suggest that a vertebral artery occlusion has occurred. Although an ipsilateral Horner syndrome can accompany a lateral medullary infarction secondary to a vertebral artery occlusion, a patient with this condition would also be expected to have dysphagia, hoarseness, a reduced gag reflex, vertigo, nystagmus, vomiting, ipsilateral cerebellar findings, ipsilateral loss of pain and temperature sensations in the face, and contralateral loss of pain and temperature sensations in the body, none of which this patient has.

Causes of pseudotumor cerebra. complication causes tx

Glucocorticoids Vitamin A OCPs causes by impaired absorption of CSF by the arachnoid villi tx: wt reduction and acetazolamide, shunting or optic n. sheath fenestration may be performed to prevent blindness MC complication: BLINDNESS***

Neuro 8 A 70-year-old woman is evaluated in the emergency department for a 6-week history of back pain that has become increasingly severe over the past 6 days and a 2-day history of progressive bilateral leg weakness. Two years ago, the patient was treated for breast cancer with lumpectomy and local radiation therapy. She has no other pertinent personal or family medical history and takes no medications. On examination, temperature is 37.1 °C (98.8 °F), blood pressure is 140/85 mm Hg, and pulse rate is 88/min. She had moderate spastic paraparesis and bilateral extensor plantar responses = Babinski. A T8 sensory level, impaired bilateral lower extremity proprioception, and moderate gait ataxia are also noted. Results of a complete blood count, erythrocyte sedimentation rate determination, and coagulation studies are all normal. An MRI of the spinal cord reveals an epidural mass causing destruction of the T6 vertebral body with spinal instability and moderately severe compression of the adjacent spinal cord. Which of the following is the most appropriate immediate treatment of this patient's spinal cord compression? A Decompressive surgery B Intravenous dexamethasone followed by decompressive surgery and chemotherapy C Intravenous dexamethasone followed by decompressive surgery and radiation therapy D Intravenous dexamethasone followed by radiation therapy and chemotherapy

C Epidural spinal cord compression usually requires immediate therapy with intravenous corticosteroids followed by decompressive surgery and radiation therapy. This patient has a typical presentation of epidural spinal cord compression, most likely from recurrence of the patient's previously treated breast cancer. The best treatment is intravenous dexamethasone followed by decompressive surgery and radiation therapy. The corticosteroid infusion likely reduces the tumor-related mass effect and edema, surgery provides immediate physical decompression of the spinal cord and the opportunity to stabilize the spine (if necessary), and radiotherapy targets the residual macroscopic and microscopic tumor burden. Observational studies support the use of intravenous corticosteroids in all patients with epidural spinal cord compression. Surgery is also warranted because of the concern about spinal instability. Furthermore, a randomized, multicenter, controlled clinical trial of therapy for epidural spinal cord compression, which included patients with metastatic breast cancer, showed that the combination of decompressive surgery and radiotherapy was associated with better ambulatory outcome than either treatment alone. In some scenarios, such as early spinal cord compression by a very radiosensitive tumor (including lymphoma and myeloma), intravenous dexamethasone plus radiotherapy may be appropriate. However, patients with such tumors were not included in the previously mentioned trial. There is no role for chemotherapy in the immediate treatment of epidural spinal cord compression. Chemotherapy may be indicated as part of a comprehensive program to control metastatic disease but will not provide the urgent relief needed for this cancer emergency.

Neuro 44 A 62-year-old woman is evaluated for a 1-year history of tremor that affects both upper extremities. She says that her handwriting has become sloppier since she first noticed the tremor and that she occasionally spills her morning coffee because of it. Although she feels otherwise healthy, she is concerned that she may have Parkinson disease. The patient has a history of hyperlipidemia controlled by diet and exercise but is otherwise healthy. Her mother, who died at age 79 years, had a similar tremor. Her only medication is a daily multivitamin. On examination, she has a mild tremor in the upper extremities that is present with the arms extended and during finger-to-nose testing. No resting tremor is apparent. Muscle tone and gait and limb coordination are normal. Administration of which of the following drugs is the most appropriate treatment of this patient? A Carbidopa-levodopa B Clonazepam C Propranolol D Ropinirole

C First-line medications used to treat essential tremor include propranolol, primidone, gabapentin, and topiramate. This patient should be treated with propranolol. She has a history and examination findings consistent with the presence of essential tremor. Essential tremor primarily occurs when a patient maintains a posture, such as when the hands are outstretched. Essential tremor also may be present during movement, particularly postural adjustments. Autosomal dominant transmission occurs in approximately half of patients with this condition. Essential tremor most commonly affects the upper extremities; however, the legs, head, trunk, face, and vocal cords may be involved. Up to 15% of patients with essential tremor have major disability associated with this condition. Progression of essential tremor is typically slow, with intermittent lengthy periods of stable symptoms. Features that may be predictive of a more severe essential tremor include a positive family history of tremor, longer tremor duration, voice tremor, and unilateral tremor onset. Alcoholic beverage consumption suppresses symptoms in most patients with this condition. Treatment options for essential tremor are often limited and frequently only partially effective. It has been estimated that 50% of patients with essential tremor have no response to medical treatment. First-line medications used to treat essential tremor include propranolol, primidone, gabapentin, and topiramate. Propranolol is typically the drug of choice in most patients with essential tremor because of its effectiveness, which has been established in multiple well-designed randomized clinical trials. Essential tremor is distinguished from Parkinson disease by its lack of parkinsonian features, such as rigidity, bradykinesia, postural instability, and resting tremor. Carbidopa-levodopa can be an appropriate choice to treat Parkinson disease but is not useful in the treatment of essential tremor. This tremor is typically postural and kinetic, with a frequency of 6 to 12 Hz. Clonazepam is considered a second-line medication in patients with refractory essential tremor and thus should not be used as the initial pharmacotherapy, given its questionable effectiveness in two randomized clinical studies and its frequent association with drowsiness. Ropinirole is a dopamine agonist medication used to treat Parkinson disease. Given the absence of any other signs of Parkinson disease, this medication would not be indicated in this patient.

Neuro 56 A 72-year-old man with a 3-year history of progressive dementia is evaluated in the emergency department for increased weakness, difficulty arising from a seated position, gross unsteadiness and slowness of pace while walking, and a 1-week history of urinary incontinence. His wife reports that after a fall 3 weeks ago in which he sustained no head trauma, he started to become weaker and more confused and now cannot ambulate without holding onto someone or using a walker. Although the patient has exhibited progressive mild gait change with shuffling and reduced left-sided arm swing over the past 2 years, he has not previously required a gait aid. At an evaluation 2 months ago, he scored 19/30 on the Folstein Mini-Mental State Examination. The patient also has chronic atrial fibrillation. There is no relevant family medical history. Current medications are donepezil, memantine, atenolol, and warfarin. On physical examination, temperature is 36.5 °C (97.8 °F), blood pressure is 96/60 mm Hg, pulse rate is 84/min and irregular, respiration rate is 16/min, and BMI is 23. The patient attends to the examiner but cannot rise from a seated position without assistance. His posture is stooped and he walks slowly with a shuffling gait, is very unsteady, and requires the assistance of the examiner to walk without falling. The patient fatigues rapidly after walking a few feet. There are no cranial bruises or other signs of trauma. He now scores 5/30 on the Folstein Mini-Mental State Examination. Laboratory studies show an INR of 2.3. Results of a complete blood count, a basic metabolic panel, measurement of serum vitamin B12 and thyroid-stimulating hormone levels, and a urinalysis are normal. A chest radiograph shows clear lung fields. An electrocardiogram shows atrial fibrillation, with a heart rate of 84/min. Which of the following is the most appropriate next diagnostic test? A Awake and asleep electroencephalography B Cerebrospinal fluid analysis C CT of the head D Echocardiography

C In elderly patients with chronic dementia who take warfarin, minor, even indirect head trauma can lead to bleeding and cause abrupt worsening of confusion. This patient should undergo CT of the head. His chronic course seems most compatible with a degeneratively based etiology, such as dementia with Lewy bodies. Although impaired cognition and gait are expected in this setting, the disease course tends to be slow and steady. The subacute deterioration affecting both cognitive and motor skills that this patient has exhibited most likely resulted from his fall. Although there was no witnessed direct head trauma, minor, even indirect head trauma is possible because he takes warfarin and thus is at increased risk for bleeding. The manner in which the patient has declined is itself nonspecific, and common medical causes of such decline, such as urinary tract infection, pneumonia, and some common metabolic disturbances, are unlikely. The possibility of greatest concern is some form of intracranial disturbance, specifically, a subdural hematoma. A CT scan of the head is thus the most reasonable test to perform because other tests have not revealed a more obvious cause for the patient's decline. The patient's course does not suggest a seizure, and so electroencephalography (EEG) is not appropriate as the next diagnostic test. If a subdural hematoma is diagnosed, the patient and family should be counseled that seizures may complicate his future clinical course and that EEG is now reasonable to evaluate any epileptiform symptoms that may arise. In the absence of concern about meningitis, cerebrospinal fluid analysis should not be considered until it is known whether there is increased intracranial pressure related to a subdural hematoma or other trauma-induced intracranial hemorrhage. There is an increased risk of brainstem herniation with lumbar puncture and of cardioembolic cerebral infarction from the reversal of anticoagulation needed before performing a lumbar puncture. Echocardiography would be reasonable if brain imaging disclosed a recent cerebral infarction as the cause of the decline. In the absence of such evidence, this test is inappropriate.

Neuro 71 A 25-year-old woman with epilepsy comes to the office seeking advice about pregnancy. She first developed seizures after sustaining a head injury in a motor vehicle collision at age 16 years. MRIs obtained since then have shown an area of encephalomalacia in the right temporal lobe. Her seizures were initially refractory to carbamazepine and valproic acid monotherapy. Carbamazepine was stopped, and lamotrigine was added to the valproic acid 1 year ago. She has not had any seizures since that time. Which of the following is the most appropriate management? A Advise the patient not to become pregnant B Continue the valproic acid and lamotrigine C Discontinue the valproic acid and continue the lamotrigine D Discontinue the valproic acid and lamotrigine E Substitute phenobarbital for her current medications

C Infants exposed to antiepileptic medication during the first trimester of pregnancy have a 4% to 6% chance of having a major congenital malformation; malformation rates are greatest in infants exposed to valproic acid and polytherapy antiepileptic regimens. This patient should discontinue taking the valproic acid but continue taking the lamotrigine. All current antiepileptic drugs are classified by the U.S. Food and Drug Administration (FDA) as pregnancy risk category C or D. Infants exposed to antiepileptic drugs during the first trimester of pregnancy have a risk of major congenital malformation that is twice that observed in the general population (4%-6% versus 2%-3%). This teratogenic risk is greatest in those whose mothers were on a polytherapy antiepileptic drug regimen or valproic acid monotherapy. Furthermore, there is early evidence indicating that maternal antiepileptic drug use during pregnancy can have an adverse effect on the long-term cognitive and behavioral development of the children born to these mothers. However, the potential for harm is not great enough to justify counseling every woman with epilepsy against becoming pregnant. In light of the potential risk to their offspring, women with epilepsy should discuss the risks and benefits of treatment with their care providers and modify any risk, if possible. Because there is no antiepileptic drug in FDA pregnancy risk category A or B, the potential of safely discontinuing antiepileptic drugs should be assessed when a woman with epilepsy desires pregnancy. This patient has a known underlying structural lesion, has a history of seizures that were initially difficult to control, and has been seizure free for only 1 year. All these factors indicate it is unlikely that she could be safely taken off antiepileptic drugs at this time. Most women with epilepsy will require continued drug therapy during pregnancy, with the goal of reducing the medications to the greatest extent that can still reasonably be expected to maintain seizure control. Low-dose monotherapy is optimal; whenever possible, valproic acid should be discontinued or replaced because of its particularly high teratogenic potential. This patient has been seizure free since lamotrigine was added to her regimen. The most appropriate next step in management, therefore, is to discontinue the valproic acid and attempt to control her epilepsy with lamotrigine monotherapy. There continues to be a common misconception that phenobarbital is safer than other antiepileptic drugs during pregnancy. However, current data from large pregnancy registry studies do not support this assumption but demonstrate instead a risk of major malformations and adverse effects on intelligence in children whose mothers took phenobarbital during pregnancy.

Neuro 61 A 17-year-old male high school student is evaluated in the emergency department 1 hour after having a generalized tonic-clonic seizure while eating breakfast with his family. He says he was out late the night before with classmates and drank six cans of beer over the course of the evening. He reports having sudden, involuntary jerks of his arms this morning before the convulsion and having had similar jerks on awakening over the past 2 months when sleep deprived. He reports no history of regular alcohol or illicit substance abuse. He takes no medications. Physical examination and neurologic examination findings are normal. Results of laboratory studies (including a complete blood count; measurements of serum electrolyte, plasma glucose, and serum ethanol levels; and a urine toxicology screen) are normal. A CT scan of the head shows no abnormalities. Which of the following is the most likely diagnosis? A Alcohol withdrawal seizure B Benign rolandic epilepsy C Juvenile myoclonic epilepsy D Temporal lobe epilepsy

C Juvenile myoclonic epilepsy is characterized by myoclonic and generalized tonic-clonic seizures on awakening that are often provoked by sleep deprivation or alcohol. This patient most likely has juvenile myoclonic epilepsy. Recognizing the specific epilepsy syndrome affecting a patient is crucial in selecting the appropriate therapy, making the correct prognosis, and, in some cases, providing genetic counseling. A history of myoclonic (rapid, unprovoked jerks) and generalized tonic-clonic seizures on awakening with onset in adolescence strongly suggests a diagnosis of juvenile myoclonic epilepsy. One of the most commonly encountered forms of epilepsy, juvenile myoclonic epilepsy may affect 5% to 10% of all patients with epilepsy. Seizures are often provoked by sleep deprivation, alcohol, or exposure to flickering lights. Alcohol withdrawal seizures develop in chronic users of alcohol and are generally seen in combination with other signs and symptoms of alcohol withdrawal, such as delirium, tremor, tachycardia, and diaphoresis. This patient's history does not suggest alcohol withdrawal as the likely cause of his recurrent seizures. Benign rolandic epilepsy is a syndrome seen in younger children and adolescents who have seizures, usually during sleep, that begin with focal sensory and/or motor symptoms involving the face, mouth, and throat that can then secondarily generalize. This benign syndrome is not associated with myoclonic seizures and so is not the diagnosis in this patient. Temporal lobe epilepsy is the most common of the localization-related epilepsies, a type of epilepsy resulting from a focal brain abnormality. Temporal lobe epilepsy can often be further categorized as the result of a focal abnormality of the mesial temporal lobe. The most common seizure classification associated with mesial temporal lobe epilepsy is complex partial seizure. Characteristically, patients with complex partial seizures are awake but exhibit altered awareness, such as unresponsiveness or staring. Patients also exhibit automatisms—such as lip smacking, swallowing, picking, or manipulating objects—or automatic (purposeless, repetitive) behaviors. Patients often describe a preceding aura and, most commonly, autonomic symptoms. About one third of complex partial seizures will generalize as tonic-clonic seizures. This patient's myoclonic jerking is not compatible with temporal lobe epilepsy.

Neuro 52 A 32-year-old woman is seen for a follow-up evaluation. She had a witnessed generalized tonic-clonic seizure 1 week ago and was evaluated in the emergency department, where results of physical examination, complete blood count, measurement of serum electrolyte levels, and urine toxicology screen were all normal. She is otherwise healthy, has no significant personal or family medical history, and takes no medications. Results of a repeat physical examination are also normal. In addition to electroencephalography, which of the following diagnostic tests should be performed next? A CT of the head B Lumbar puncture C MRI of the brain D Positron emission tomography

C MRI is superior to CT for detection of epileptogenic lesions. For patients whose clinical history includes new onset of a seizure for which no obvious provocative cause is identified, the standard evaluation consists of electroencephalography and MRI of the brain. These tests will help not only to confirm the diagnosis, but also to predict the risk of future recurrence and rule out any underlying condition (such as a brain tumor) that might require treatment in itself. MRI has been shown to be clearly superior to CT in detecting potentially epileptogenic lesions. For example, in one retrospective case series of 117 patients with refractory epilepsy, MRI detected 95% of histologically identifiable lesions, whereas CT only detected 32%. CT is the imaging modality of choice for new-onset seizures only when there is a suspicion of an acute cerebral hemorrhage or when a contraindication to MRI is present. Neither condition pertains to this patient. For adults with new-onset seizures, lumbar puncture is generally only indicated when the patient's history or physical examination findings lead to a significant clinical suspicion of an underlying infection or inflammatory cause. Infection of the central nervous system is indicated by the presence of fever or altered mental status. Patients with a subarachnoid hemorrhage, for whom lumbar puncture is sometimes appropriate, may also have seizures, but such a hemorrhage is associated with severe headache, altered mental status, syncope, and neurologic deficits and usually will be identified on a CT scan or MRI. Given this patient's uneventful prior history and normal results on physical examination, lumbar puncture is inappropriate. Positron emission tomography is sometimes used as part of a surgical evaluation for medically refractory epilepsy. It does not presently have any role in the evaluation of new-onset seizures.

Neuro 33 A 28-year-old woman in her first trimester of pregnancy is evaluated in the office for severe and frequent migraine attacks occurring up to four times per week. These attacks are associated with severe pain, nausea, and emesis and last up to 24 hours; acetaminophen and NSAIDs do not relieve her symptoms. The patient has a 10-year history of migraine. Results of physical examination, including a neurologic examination, are normal. Which of the following is the most appropriate treatment for this patient? A Amitriptyline at bedtime B Ergotamine tartrate as needed C Metoclopramide as needed D Valproic acid (extended release) twice daily E Verapamil three times daily

C Migraines during pregnancy that do not respond to simple analgesia can be safely and effectively treated with metoclopramide. Metoclopramide has been shown to have efficacy for both the pain and nausea associated with migraine. This drug has a U.S. Food and Drug Administration (FDA) rating of pregnancy category B. Acute medications with an FDA rating of pregnancy category A or B carry the lowest risk of harm to the fetus and should be used preferentially for the treatment of migraine during pregnancy. Amitriptyline and verapamil are used for migraine prophylaxis. Both have an FDA rating of pregnancy category C (risk to the fetus cannot be ruled out) and are not contraindicated for pregnant women. However, prophylactic migraine therapy should be avoided whenever possibly during pregnancy, especially in the first trimester. Often, migraine improves during the second and third trimesters of pregnancy, so acute therapy during the first trimester is preferred. If migraine continues to be disabling and occurs frequently during the second and third trimesters, prophylactic therapy could be considered, especially if the health of the mother and/or the fetus is compromised. Ergotamine tartrate and valproic acid have an FDA rating of pregnancy category X (studies have shown fetal abnormalities) and are contraindicated during pregnancy.

Meirp 48 A 68-year-old man is evaluated for fatigue. He says that for the past 2 weeks, he has been awakening at approximately 2 AM with a left-sided tremor and left-sided stiffness. Parkinson disease was diagnosed 3 years ago after he noted a left-hand tremor and a change in his handwriting; examination at that time showed mild parkinsonian signs, and he was started on carbidopa-levodopa. He currently takes immediate-release carbidopa-levodopa, which results in near-resolution of his parkinsonian symptoms; he notes, however, that the medication wears off if too much time passes between doses. During this recent period of early-morning awakening, he has occasionally taken extra carbidopa-levodopa, which has allowed him to fall asleep again. Results of a general medical examination are normal. Neurologic examination reveals slurred speech and a paucity of facial expression. Deep tendon reflexes are normal, as are results of manual muscle strength testing and sensory examination. He has mild upper extremity rigidity that is greatest in the left arm and a very mild resting tremor of the left upper limb. No appendicular or truncal ataxia is noted. Which of the following should be added to this patient's drug regimen to treat his fatigue? A Clonazepam, before bedtime B Donepezil C Extended-release carbidopa-levodopa, before bedtime D Fluoxetine

C Motor symptoms of Parkinson disease, such as tremor, rigidity and dystonia, can develop nocturnally and cause sleep disturbances; such symptoms may respond to extended-release carbidopa-levodopa therapy. This patient should take extended-release carbidopa-levodopa before bedtime. Patients with Parkinson disease develop both motor and nonmotor complications. One of the major nonmotor complications is fatigue, which occurs in roughly half of patients with Parkinson disease. The first step in evaluating fatigue in these patients is to ensure that their dosage of levodopa or a dopamine agonist is high enough to adequately treat their parkinsonian symptoms. Standing blood pressures should also be obtained in these patients, when symptomatic, to exclude orthostatic hypotension as the cause of fatigue. Sleep disorders are common in patients with Parkinson disease, and poor sleep is the likely cause of fatigue in this patient. The patient reports increasing stiffness and tremor at night, symptoms that reflect a "wearing-off" effect of levodopa. Taking extended-release carbidopa-levodopa immediately before bedtime should allow a more restful night's sleep and lessen his fatigue. The extended-release version of this medication has a longer duration of efficacy than the immediate-release version. Sleep disorders are also common in patients with Parkinson disease. Its motor manifestations (such as tremor, rigidity, and dystonia), as exhibited by this patient, can disrupt sleep. Restless legs syndrome, periodic limb movement disorder, rapid eye movement (REM) sleep behavior disorder, and obstructive sleep apnea are other sleep disorders that can all occur in patients with Parkinson disease and disrupt sleep. REM sleep behavior disorder is characterized by the lack of normal muscle atonia during REM sleep and may result in patients physically acting out their dreams, such that they may yell, punch, grab, shout, or even jump out of bed. REM sleep behavior disorder may precede other clinical signs of Parkinson disease. Although this disorder and periodic limb movements of sleep may respond to clonazepam, this drug is not indicated in this patient, who lacks a history of such movements. Donepezil is indicated in the treatment of patients with dementia and is therefore not appropriate for this patient who has no evidence of dementia. Psychiatric disorders can develop in up to 60% of patients with Parkinson disease. After dopaminergic therapy is optimized, most clinicians use selective serotonin reuptake inhibitors, such as fluoxetine, to treat depression in patients with Parkinson disease. These drugs and serotonin-norepinephrine reuptake inhibitors are first-line agents for those requiring drug treatment. Given the absence of any other symptoms of depression, fluoxetine is not indicated in this patient. Moreover, although depression is often associated with fatigue, this patient's fatigue is more likely the result of an inadequate dosage of medication.

Neuro 25 A 30-year-old man has a recent diagnosis of multiple sclerosis (MS). He experienced two transient neurologic episodes in the past 6 months, one involving optic neuritis and the other minor partial myelitis; he recovered completely from both events and is currently asymptomatic. MS was diagnosed after an MRI of the brain showed white matter lesions typical of the disease. He has no other pertinent personal or family medical history. Which of the following MS subtypes best describes the course of his disease? A Benign B Primary progressive C Relapsing-remitting D Secondary progressive

C Multiple sclerosis begins as a relapsing-remitting disorder in 85% of patients and a primary progressive disorder in 15% of patients; those with the relapsing-remitting type have a greater than 50% risk of developing a secondary progressive disease course. This patient experienced an episode of transient neurologic dysfunction (an attack) at disease onset and thus has relapsing-remitting multiple sclerosis (MS). Eighty-five percent of patients with MS have this type of disease onset. In relapsing-remitting MS, relapse frequency declines over time, and relapses do not become more severe with increasing disease duration; however, recovery from individual events may be slower and less complete. Primary progressive MS, which the other 15% of patients with MS have at disease onset, is defined as gradually worsening neurologic function over more than 1 year without recovery. This patient has recovered completely from two neurologic episodes and is currently asymptomatic. Therefore, he cannot be classified as having primary progressive MS. Secondary progressive MS is characterized by gradual, unremitting development of new symptoms over months to years in a patient who previously had a relapsing-remitting course. The lifetime risk of conversion from relapsing-remitting to secondary progressive disease is greater than 50%, but the onset and rate of the progressive phase are highly variable and not predictable for individual patients. The median time from MS onset until conversion to the secondary progressive phase typically ranges from 10 to 15 years. The establishment of a secondary progressive course is a risk factor for substantial disability, such as loss of independent ambulatory function; the median time from MS onset to the point at which unilateral gait assistance (such as a cane) is required is 15 to 25 years. This patient first had transient symptoms only 6 months ago and thus cannot be characterized as having secondary progressive disease. Benign MS is defined loosely as no or minimal neurologic impairment 15 or more years after MS onset. This category may encompass as many as 20% of all patients with MS. The definition of benign MS is controversial because continued follow-up of such patients often uncovers late progressive disease and disability accrual. However, a small minority of patients with MS live a long and essentially unrestricted life. The factors that predict a benign course early in the disease have not yet been identified. This patient's MS diagnosis is too recent to be categorized as benign at this point.

Neuro 60 A 33-year-old man is evaluated for a 3-day history of worsening weakness and numbness of the right arm and leg. He has a 5-year history of multiple sclerosis. His only current medication is glatiramer acetate. On physical examination, temperature is 36.5 °C (97.7 °F), blood pressure is 105/75 mm Hg, pulse rate is 68/min, and respiration rate is 14/min. Moderate right arm and leg weakness, hyperreflexia, an extensor plantar response, and vibratory sense impairment are noted. Which of the following should this patient receive to treat his acute relapse? A Empiric antibiotic therapy B Immune globulin, intravenously C Methylprednisolone, intravenously D Plasmapheresis E Prednisone, orally

C Multiple sclerosis relapses may resolve more rapidly with intravenous methylprednisolone therapy. Evidence from placebo-controlled trials involving multiple sclerosis (MS) and optic neuritis supports the intravenous use of methylprednisolone to speed recovery from acute MS relapses. The long-term outcome of an individual MS attack, however, is not affected by the therapy chosen. Therefore, in light of the potential adverse effects of such drugs, intravenous administration of corticosteroids should be offered only for relapses that result in substantial discomfort or reduced function. This patient's 3-day history of worsening weakness and numbness of the right arm and leg qualifies him for corticosteroid therapy. It is necessary to rule out infection as a cause of neurologic worsening in patients with MS because infections can cause a "pseudoexacerbation"; this patient, however, is asymptomatic except for his neurologic symptoms and is afebrile. Patients with moderate or severe MS-related disability are more susceptible to the effects of mild infections, especially urinary tract infections, and investigation for such infections is warranted, even in the absence of systemic symptoms. However, empiric antibiotic therapy to treat a possible occult infection is not warranted in the absence of evidence or a high clinical suspicion of infection. Intravenously administered immune globulin is an effective treatment for a number of immune system-mediated diseases, but evidence of its efficacy for MS is lacking. Clinical trials have found no benefit from immune globulin, either as add-on treatment to methylprednisolone for acute MS attacks or as monotherapy for acute optic neuritis. Plasma exchange therapy may be beneficial in the treatment of fulminant attacks of multiple sclerosis that are unresponsive to corticosteroids. Such therapy appears to be particularly useful in patients with severe attacks of neuromyelitis optica that do not improve with corticosteroids. However, plasmapheresis is not indicated for this patient at this time before a trial of methylprednisolone. Oral administration of prednisone was not as effective as intravenous administration of methylprednisolone in the Optic Neuritis Treatment Trial and so should not be the first choice to treat acute MS relapses.

Neuro 82 A 33-year-old woman is admitted to the hospital for evaluation and treatment of new-onset transverse myelitis that has resulted in severe paraparesis. The patient also has had two recent episodes of optic neuritis. She reports no systemic symptoms, such as fever, rash, arthralgias, or pulmonary problems. She has a history of hypothyroidism but no family history of any neurologic disorders. Her only medication is levothyroxine. Vital signs are normal on physical examination. Visual acuity is 20/200 in the right eye and 20/30 in the left. Bilateral optic disc pallor and severe spastic paraparesis with loss of all sensory modalities below T10 are noted. The patient requires bilateral assistance to ambulate 5 meters. Results of laboratory studies are normal, including a complete blood count, liver chemistry and renal function tests, and measurement of erythrocyte sedimentation rate and C-reactive protein level. The antinuclear antibody is positive, but anti-double-stranded DNA and anti-SSA/SSB antibodies are negative. Analysis of the cerebrospinal fluid shows a normal IgG index and no abnormalities in oligoclonal banding. An MRI of the spinal cord reveals an increased signal extending over five vertebral segments with patchy gadolinium enhancement. An MRI of the brain shows no abnormalities. Which of the following is the most appropriate next diagnostic test? A Electromyography B Serum antineutrophil cytoplasmic antibody test C Serum neuromyelitis optica (NMO)-IgG autoantibody test D Testing of visual evoked potentials

C Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system that is distinct from multiple sclerosis and associated with the autoantibody marker NMO-IgG (anti-aquaporin-4). This patient very likely has neuromyelitis optica (NMO), a severe demyelinating disease of the central nervous system that is distinct from multiple sclerosis (MS). She should be tested for the autoantibody marker NMO-IgG (anti-aquaporin-4). NMO occurs more commonly in nonwhite persons, is often associated with serum autoantibodies or other autoimmune diseases, and has a predilection for the optic nerves and spinal cord with relative sparing of the brain. This patient's spinal cord lesion is also characteristic of NMO because it extends over more than three vertebral segments; cord lesions in typical MS are usually less than two segments in length. The finding of the NMO-IgG autoantibody marker is approximately 75% sensitive and more than 90% specific for NMO. Differentiating between NMO and MS as early in the disease course as possible is important because the prognosis and treatment of the two diseases are different. NMO is a more severe disease treated with immunosuppressive drugs, whereas MS is initially treated with immunomodulatory therapies, such as β-interferon and glatiramer acetate. There are no symptoms or signs of peripheral nerve or muscle involvement in this patient. Therefore, electromyography is not indicated. Cytoplasmic and perinuclear-staining antinuclear cytoplasmic antibodies (ANCAs) may be detected in patients with systemic vasculitis. Although vasculitis is a rare cause of transverse myelitis, it is very unlikely to explain multiple episodes of optic neuritis and transverse myelitis in the absence of systemic symptoms. Visual evoked potential testing will confirm the known involvement of the optic nerves in this patient but will not otherwile aid diagnosis and so is unnecessary.

Neuro 80 A 26-year-old woman is evaluated in the office for a change in migraine symptoms. She began having migraine attacks shortly after menarche at age 13 years, experiencing an attack approximately every 2 months. For the past 8 weeks, these attacks have been associated with visual aura. The neurologic symptoms evolve over a period of 10 minutes, last less than 60 minutes, and are followed within 30 minutes by a severe, unilateral throbbing headache associated with nausea. Sumatriptan relieves the headache within 30 minutes. The patient also has asthma. Her mother and sister have a history of migraine. Current medications are an oral contraceptive pill started 9 weeks ago, sumatriptan as needed, a daily inhaled corticosteroid, and an inhaled β-agonist as needed. Results of physical examination, including neurologic examination, are normal. Complete blood count results, erythrocyte sedimentation rate, serum chemistry study results, thyroid-stimulating hormone level, and anticardiolipin and antinuclear antibody levels are normal. An MRI of the brain shows no abnormalities. Which of the following is the most appropriate next step in management? A Add propranolol B Add verapamil C Discontinue the oral contraceptive pill D Discontinue the sumatriptan E Measure serum lactate and pyruvate levels

C Oral contraceptive pills are contraindicated in women with migraine with aura, especially if the aura involves more than just simple visual aura, if there are additional stroke risk factors, or if the aura begins after the initiation of oral contraception. The American Academy of Obstetrics and Gynecology considers migraine with aura an absolute contraindication to the use of oral contraception. The International Headache Society strongly advises against the use of oral contraceptive pills in women who have migraine with aura that involves more than just the visual system and recommends stopping the pill in women who develop an aura after the pill is started. Patients who have migraine with aura have up to an eight-fold increased risk of ischemic stroke; this risk is tripled by smoking and quadrupled by the use of oral contraception. This patient, therefore, should discontinue using oral contraception. There is little evidence to support the use of propranolol or verapamil for the prevention of migraine in this patient. In any case, the frequency of her migraines (approximately one every 2 months) does not justify the use of preventive medication. Triptans are not contraindicated in patients with migraine with aura. Therefore, the sumatriptan can continue to be used for acute migraine relief in this patient. Mitochondrial Encephalopathy with Lactic Acidosis and Stroke-like episodes (MELAS) is a maternally inherited mitochondrial disorder associated with lactic acidosis and a high prevalence of migraine with aura. The hallmark of MELAS is the occurrence of nonischemic stroke manifesting as hemiparesis, hemianopia, or cortical blindness. The course is progressive and leads to progressive neurologic impairment and dementia. Other features may include short stature, hearing loss, and muscle weakness. However, this patient has no clinical or imaging features of this disorder, and measuring her serum lactate and pyruvate levels would not be appropriate.

Neuro 74 A 65-year-old woman comes to the office for her annual examination. She reports that she had a tonic-clonic seizure at age 24 years after the birth of her daughter but has been seizure free on phenytoin since that time. The patient also has osteoporosis, diagnosed after a screening bone density scan. Current medications include phenytoin, alendronate, calcium, and vitamin D. Physical examination findings are normal. Which of the following is the most appropriate next step in management? A Check the serum phenytoin level B Continue the current dosage of phenytoin C Discontinue the phenytoin in a tapered fashion D Substitute lamotrigine for the phenytoin

C Patients on antiepileptic medication who have been seizure free for 2 years should be considered for medication withdrawal. Phenytoin therapy should be discontinued in this patient in a tapered fashion. Although lifelong antiepileptic drug therapy is required for some patients and for some types of epilepsy, this is by no means always the case. As a general rule, discontinuation of antiepileptic drugs should be considered for patients who have been seizure free for 2 or more years. Medications should not be withdrawn in patients with epilepsy syndromes known to be lifelong, with underlying structural brain lesions, with symptomatic neurologic disorders, or (in most cases) with a history of medically refractory seizures. The risk of recurrent seizure when the patient is no longer taking the medication must always be balanced against the risks associated with continued antiepileptic drug treatment. Unfortunately, too many patients are treated unnecessarily for years because of the common misconception that antiepileptic drug therapy can never be safely discontinued. This patient has been seizure free for more than 40 years; in fact, the decision to initiate therapy was questionable because she only had a single event. Now she has osteoporosis, a condition which can be worsened by continued exposure to phenytoin. Therefore, the most appropriate next step in management is to gradually withdraw the medication. Because the phenytoin will be withdrawn, there is no need to determine a blood level prior to tapering the medication; the results will not affect when or how the medication will be tapered. This patient has been seizure free for more than 2 years and thus meets the criteria for careful withdrawal of the antiepileptic medication. There is no indication to substitute lamotrigine for the phenytoin or to continue the phenytoin.

Neuro 83 A 30-year-old man with epilepsy is evaluated for disabling seizures that occur once a month despite adherence to his drug therapy. He also reports excess sedation and cognitive slowing related to his medication. The patient has had partial complex seizures since age 16 years and has been previously treated with appropriate dosages of phenytoin, carbamazepine, and valproic acid; he currently takes lamotrigine, 200 mg twice daily (dose range, 100-600 mg/d). He is otherwise in excellent health and has no other medical problems. Physical examination, including neurologic examination, reveals no abnormal findings. An MRI of the brain shows an increased T2 signal and atrophy of the right hippocampus, both consistent with mesial temporal sclerosis. An electroencephalogram shows right temporal sharp waves. Which of the following is the most appropriate next step in management? A Add another anticonvulsant to his drug regimen B Increase the dosage of lamotrigine C Refer him for epilepsy surgery evaluation D Refer him for implantation of a vagus nerve stimulator

C Patients with disabling partial seizures that have not responded to treatment with two appropriate anticonvulsant drugs should be considered for epilepsy surgery. For patients with medically refractory partial epilepsy whose seizures are adversely affecting their quality of life, referral for epilepsy surgery evaluation is the most appropriate next step in management. Seizures arising from mesial temporal sclerosis, as is suggested by this patient's MRI, are particularly amenable to surgical cure, with seizure-free rates reported to be as high as 80% after resection. The most common surgical procedure is surgical resection of mesial temporal lobe sclerotic lesions. Quality of life and social functioning improve after successful surgery, and surgical morbidity and mortality are low. In fact, surgery may improve long-term mortality. Approximately 30% of patients with epilepsy have seizures that cannot be adequately controlled with currently available medical therapies. If seizures have not responded to two medications that are appropriate for the seizure disorder and are used at an adequate dosage, the likelihood of achieving complete control with subsequent medication trials is less than 10%. The patient described has continued disabling seizures despite trials of four medications, all appropriate for treating partial complex seizures and all given at adequate dosages. Therefore, adding another drug to his current regimen is unlikely to improve his condition. His current dosage of lamotrigine, although not the theoretical maximal daily dosage, represents a reasonable trial of the drug. A further increase in the lamotrigine dosage is not the best choice for this patient who reports limiting adverse effects on the current regimen. The vagus nerve stimulator is an approved therapy for medically refractory partial epilepsy that is best used as a palliative option for patients who are not candidates for resective surgery. This patient has no contraindications to such surgery.

Neuro 14 A 33-year-old man is evaluated for a 3-year history of progressive gait dysfunction accumulating in a stepwise manner, with periods of rapid worsening followed by a plateau in symptoms until the next relapse. He has no personal history of other medical problems. On physical examination, vital signs are normal. Severe spastic paraparesis, moderate loss of all sensory modalities in the lower extremities, and a T10 sensory level are noted. An MRI of the thoracic spinal cord is shown . An MRI of the brain shows no abnormalities. Cerebrospinal fluid analysis detects no oligoclonal bands. Which of the following is the most appropriate next diagnostic test? A Repeat lumbar puncture B Repeat MRI of the brain C Spinal angiography D Spinal cord biopsy

C Spinal dural arteriovenous fistula should be suspected in any patient with a chronic or subacute progressive myelopathy with episodes of more rapid, stepwise clinical deterioration. The most appropriate next diagnostic test for this patient is spinal angiography for the specific purpose of confirming the presence of a spinal dura-based arteriovenous fistula. Dural arteriovenous fistula should be suspected in any patient with a chronic or subacute progressive myelopathy with episodes of more rapid, stepwise clinical deterioration. In this patient, the diagnosis is suggested by the clinical history of stepwise spinal cord dysfunction without significant recovery and the MRI finding of a swollen spinal cord with dorsal flow voids, which is indicative of tortuous blood vessels. The spinal cord lesion represents cord congestion due to an abnormal connection between the high-pressure arterial and low-pressure venous drainage systems serving the spinal cord circulation. Patients with such spinal fistulas are often mistakenly diagnosed as having multiple sclerosis (MS), which is the most common nontraumatic disabling disease of young adults. Although repeating the cerebrospinal fluid analysis and the MRI of the brain would be a reasonable way to reevaluate the validity of an MS diagnosis, the spinal cord MRI findings in this patient are not typical of MS and make these repeated studies unnecessary. Spinal cord biopsy would be useful if there were a strong clinical suspicion of an unusual inflammatory myelopathy or a cord tumor. Nothing in the patient's examination or laboratory and imaging findings suggests either possibility.

Neuro 10 A 61-year-old man is evaluated in the office for a 6-month history of progressive weakness of the lower extremities. He says he has difficulty rising from a seated position and walking up stairs and also has episodes of dry eyes, dry mouth, and erectile dysfunction. The patient reports no ptosis, diplopia, dysphagia, or dyspnea. He has a 15-year history of hypertension and a 42-pack year smoking history. Family history is unremarkable. His only medication is hydrochlorothiazide. On physical examination, vital signs are normal. Manual muscle strength testing shows weakness in the proximal upper and lower limb muscles. Deep tendon reflexes are absent diffusely. Plantar responses are flexor. A sensory examination shows no abnormalities, and cranial nerve function is normal. Laboratory studies show normal serum levels of sodium, potassium, calcium, creatinine, glucose, and creatine kinase. Results of liver chemistry studies are also normal. Which of the following is the best diagnostic test for this patient? A Measurement of acetylcholine receptor antibody level B Measurement of parathyroid hormone level C Measurement of voltage-gated P/Q-type calcium channel antibody level D Muscle biopsy

C The diagnosis of Lambert-Eaton myasthenic syndrome, a neuromuscular junction disorder that causes progressive proximal muscle weakness and areflexia, precedes the clinical recognition of cancer in up to 50% of patients. This patient most likely has Lambert-Eaton myasthenic syndrome, as suggested by his history of proximal upper and lower limb weakness, the presence of autonomic symptoms (dry eyes/mouth, erectile dysfunction), and the finding of absent deep tendon reflexes on examination. These are characteristic signs and symptoms of the syndrome. Lambert-Eaton myasthenic syndrome is a neuromuscular junction disorder caused by disordered calcium channel function on the presynaptic nerve terminal. In most patients with this disorder, antibodies to voltage-gated P/Q-type calcium channel receptors exist. Lambert-Eaton myasthenic syndrome is typically a paraneoplastic syndrome, caused by or associated with an underlying malignancy, particularly small cell lung cancer. The diagnosis of Lambert-Eaton myasthenic syndrome precedes the clinical diagnosis of cancer in up to 50% of affected patients; therefore, in patients with newly diagnosed Lambert-Eaton myasthenic syndrome, a thorough search for an underlying cancer should be performed. If no evidence of malignancy is found, these patients should be evaluated serially for occult malignancy. In addition to elevated levels of voltage-gated P/Q-type calcium channel antibodies, the diagnosis can be confirmed through electrodiagnostic studies, particularly repetitive nerve stimulation studies, which show an increase in the muscle action potential (increment) after brief exercise. Elevated levels of antibodies against acetylcholine receptors are present in 90% of patients with generalized myasthenia gravis. Myasthenia gravis is an autoimmune disorder that results in neuromuscular transmission failure, causing weakness of limb and cranial muscles. The diagnosis is confirmed through electrodiagnostic testing, including repetitive nerve stimulation studies and, in some patients, single-fiber electromyography. The presence of an elevated acetylcholine receptor antibody level may provide additional confirmatory evidence supporting the diagnosis of myasthenia gravis. In this patient, the absence of any bulbar signs or symptoms, such as ptosis, visual symptoms (blurred vision or diplopia), or dysphagia, in conjunction with absent deep tendon reflexes, would argue against myasthenia gravis. Hyperparathyroidism, either primary or secondary, can result in proximal limb weakness. The normal calcium level in this patient would argue against a significant parathyroid disorder. Additionally, absent deep tendon reflexes would not be expected in a patient with hyperparathyroidism. Measurement of the parathyroid hormone level is therefore not indicated. Muscle biopsy is not likely to offer any additional diagnostic information in this patient with normal serum creatine kinase levels. Muscle biopsy is indicated primarily in patients with suspected inflammatory myopathies, such as polymyositis, dermatomyositis, or inclusion body myositis, and in certain hereditary myopathic disorders. Symptom onset in the seventh decade argues against a hereditary myopathy, as does the normal creatine kinase level. Although serum creatine kinase levels can be normal in patients with inclusion body myositis, deep tendon reflexes are typically normal, and weakness is most prominent in quadriceps and deep finger flexor muscles.

Neuro 40 A 48-year-old woman is evaluated in the office for memory loss of gradual onset and progression over the past year. She says she has difficulty recalling names of familiar people, has misplaced her glasses on numerous occasions, and is slower to find her car in large or crowded parking lots. The patient now requires help from her daughter to manage her finances and prepare large meals. She has no other problems or personal medical history, but several members of her family developed dementia between age 46 and 54 years, including her mother, maternal uncle, and maternal grandfather. Her only medication is a daily multivitamin. On physical examination, temperature is 36.7 °C (98.1 °F), blood pressure is 116/74 mm Hg, pulse rate is 72/min, respiration rate is 14/min, and BMI is 24. Her level of alertness, speech, and gait are normal. She scores 24/30 on the Folstein Mini-Mental State Examination, losing two points on the orientation section for misstating the date and year, one point in the serial calculation portion, and all three points on the recall portion. Results of a complete blood count, basic metabolic panel, serum vitamin B12 measurement, and thyroid function tests are normal. Genetic testing is positive for the presenilin-1 mutation. An MRI of the brain without contrast shows no abnormalities. Which of the following is the most likely diagnosis? A Autosomal recessive Parkinson disease with dementia B Creutzfeldt-Jakob disease C Early-onset familial Alzheimer dementia D Frontotemporal dementia E Vascular dementia

C The presenilin-1 mutation is specific for early-onset familial Alzheimer dementia. This patient's personal and family medical history is striking for dementia at an unusually young age. Her maternal family history in particular is strongly suggestive of early-onset familial Alzheimer dementia, which typically has symptomatic onset in a person's forties or fifties. Although genetic testing is not routine in the evaluation of dementia, all known causes of early-onset familial Alzheimer dementia involve an autosomal dominant mutation. Therefore, genetic testing was appropriate for this patient and should be considered in all patients with early-onset dementia that seems to follow an autosomal dominant pattern of inheritance. The presenilin-1 mutation is specific for early-onset familial Alzheimer dementia. Although mutations of the amyloid precursor protein and presenilin-2 gene are other known autosomal dominant mutations that can cause the disease, the presenilin-1 mutation is by far the most common cause, and testing for this mutation is commercially available. Because the mutation has been identified in this patient, it is appropriate to offer genetic counseling to her daughter. Because the presenilin-1 mutation is specific for early-onset familial Alzheimer dementia and because no evidence of parkinsonism was found on examination, it is unlikely that this patient has autosomal recessive Parkinson disease with dementia. In addition to evidence of parkinsonism, mutations in the parkin, α-synuclein, LRRK2, and other genes are associated with this disorder. The autosomal dominant mutations seen in early-onset familial Alzheimer dementia are not. Besides the specificity of presenilin-1 for Alzheimer dementia, this patient is also unlikely to have Creutzfeldt-Jakob disease or vascular dementia because characteristic MRI findings in those disorders are absent. Specifically, there is no "cortical ribbon" sign typical of Creutzfeldt-Jakob disease and no sign of previous cerebral infarction, which is seen in most patients with vascular dementia. No details are provided that suggest a frontotemporal pattern of dementia, although patients with Alzheimer dementia may occasionally exhibit such a pattern. Frontotemporal dementia and Alzheimer dementia are thus sometimes difficult to distinguish on clinical grounds alone. Even in the presence of a frontotemporal pattern of cognitive deficits, however, a presenilin-1 mutation is specific for early-onset familial Alzheimer dementia.

Neurotransmitter of corticobulbars and corticospinals

Glutamate (excitatory)

Neuro 43 A 30-year-old man is evaluated in the office for an 8-month history of intensely painful headaches, which occur up to 10 times per day and last approximately 15 minutes each. The pain is most severe around the left eye, and he has no pain between attacks. Each attack is associated with rhinorrhea, lacrimation, and conjunctival injection. The patient has a 12-pack-year smoking history. He takes a combination of acetaminophen, caffeine, and aspirin, usually taking a total of five tablets daily. Results of a physical examination, including a neurologic examination, are normal. Which of the following is the most likely diagnosis? A Cluster headache B Medication overuse headache C Paroxysmal hemicrania D SUNCT syndrome (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing)

C The various trigeminal autonomic cephalalgias, characterized by pain referred to the first division of the trigeminal nerve and accompanying cranial autonomic symptoms, can be distinguished by the duration and frequency of each attack. This patient has paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias. These cephalalgias are characterized by pain referred to the first division of the trigeminal nerve and cranial autonomic symptoms, including lacrimation and rhinorrhea. They differ from one another in the duration and frequency of attacks. A paroxysmal hemicrania attack has an intermediate duration (mean, 15 minutes) and an intermediate episode frequency (mean, 11 per day). Cluster headache, another trigeminal autonomic cephalalgia, closely resembles paroxysmal hemicrania except in the duration and frequency of attacks. Cluster headache is associated with one to eight attacks per day, and each attack has a mean duration of 60 minutes. Because this patient's headaches only last approximately 15 minutes each, cluster headache is unlikely. Although this patient overuses over-the-counter analgesics, he does not have a background of diffuse low-grade headache associated with medication overuse headache. The syndrome of Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing, or SUNCT syndrome, is another trigeminal autonomic cephalalgia with a very similar phenotype to paroxysmal hemicrania. However, SUNCT syndrome is usually associated with more than 30 attacks per day and a duration of 30 to 120 seconds for each attack. These parameters do not match this patient's symptoms. It is important to distinguish between the trigeminal autonomic cephalalgias because they are treated differently. Verapamil, indomethacin, and lamotrigine are, respectively, the treatments of first choice for cluster headache, paroxysmal hemicrania, and SUNCT syndrome.

Neuro 38 A 68-year-old man is admitted to the intensive care unit because of an exacerbation of chronic obstructive pulmonary disease (COPD). His course becomes complicated over the next 2 days by pneumonia and acute kidney injury, and he requires noninvasive positive-pressure ventilation with bilevel positive airway pressure. After 10 days, his medical condition stabilizes, but profound weakness of the extremities is noted. Besides COPD, the patient has a history of hypertension, hypothyroidism, and hyperlipidemia. One week before admission, he was started as an outpatient on prednisone, 60 mg/d, for his COPD; other medications include piperacillin-tazobactam, metoprolol, levothyroxine, and simvastatin. On physical examination, blood pressure is 134/90 mm Hg, pulse rate is 90/min, and arterial oxygen saturation is 96% on nasal oxygen, 2 L/min. Neurologic examination shows profound symmetric weakness of bilateral upper and lower extremity muscles. Deep tendon reflexes and cranial nerve function are normal. Laboratory studies show an elevated serum creatinine level at 1.5 mg/dL (114.5 µmol/L). Serum levels of creatine kinase and electrolytes are normal, as are results of liver chemistry studies. Which of the following is the most appropriate therapy for this patient? A Increased dosage of prednisone B Intravenous administration of immune globulin C Physical and occupational therapy D Plasma exchange

C Therapy for critical illness myopathy, a frequent complication in sepsis or multiorgan failure that can cause failure to wean from mechanical ventilation and limb weakness, involves physical and occupational rehabilitative therapies and avoidance of corticosteroids. This patient should have physical and occupational therapy. His history is quite typical of critical illness myopathy. Critical illness refers to the syndrome of sepsis and multiple organ failure. Critical illness myopathy or neuropathy is usually recognized in the intensive care unit, either in patients who have difficulty weaning from mechanical ventilation (up to 30% of patients) or in patients who have developed severe limb weakness during or after recovery from critical illness. Electrodiagnostic testing with electromyography should be considered in patients who develop generalized weakness or fail to wean from ventilation in this setting. Such testing can help exclude other conditions, such as neuromuscular junction disorders or polyradiculoneuropathy (Guillain-Barré syndrome), and confirm the diagnosis of critical illness myopathy or neuropathy. Physical and occupational therapy is ideally initiated immediately on recognition of this syndrome. Respiratory therapy is important to minimize the risk of superimposed pulmonary infection. Stretching and passive range-of-motion exercises help maintain joint mobility and prevent contractures. Later, as the patient improves, strengthening of upper and lower extremity muscle groups and training in activities of daily living can be performed. Most patients will transition into the inpatient rehabilitation unit once medically stable. Corticosteroids, such as prednisone, should be avoided, if possible, in patients with critical illness who develop peripheral neuropathy or myopathy during the course of their illness. Corticosteroids may play a role in the pathogenesis of critical illness myopathy, but myopathy can develop in patients who are critically ill even with no history of corticosteroid exposure. Treatment of critical illness polyneuropathy with intravenous immune globulin has been attempted, but this therapy has not been shown to be of benefit in this disease or in critical illness myopathy. Some attempts to target and interrupt the cascade of humoral factors associated with sepsis have been promising, but treatment of critical illness myopathy is supportive at this time. The primary, initial approach is directed toward the prevention and management of sepsis, the systemic inflammatory response syndrome, and multiple organ dysfunction. Aggressive treatment of infection, hypoxemia, and hypotension is critical. There is no evidence of a role for plasma exchange in the treatment of critical illness myopathy or neuropathy at this time.

Neuro 17 A 36-year-old woman is evaluated in the office for a 6-year history of headache. The patient says her headaches occur approximately three times each month, are associated with occasional nausea and pain that can be moderately severe and disabling, have a squeezing quality, and begin in the neck, radiating forward to involve the frontal head region bilaterally. Her headaches are preceded by yawning and irritability, last up to 24 hours, and can be triggered by stress or changes in barometric pressure. She has a history of mild depression treated with fluoxetine. Her mother and sister have a history of sinus headaches. On physical examination, blood pressure is 124/86 mm Hg, pulse rate is 78/min, and BMI is 21. Results of general physical and neurologic examinations are normal. The patient's mood appears euthymic. Which of the following is the most likely diagnosis? A Chronic migraine B Medication overuse headache C Migraine without aura D Sinus headache E Tension-type headache

C This patient most likely has migraine without aura. Approximately 90% of patients in an ambulatory care setting with a chief symptom of recurrent, moderately severe headache have a form of migraine. Her 6-year history of episodic headache meets the International Classification of Headache Disorders criteria for migraine. There is no single criterion that is necessary or sufficient to make the diagnosis of migraine. In this particular example, this patient does not have unilateral or throbbing pain, and there is no associated photophobia, phonophobia, or vomiting, features that are often considered necessary for the diagnosis. Her headache is preceded by premonitory symptoms (yawning and irritability), which occur in approximately 75% of patients with migraine. In addition, her attacks are triggered by stress and changes in barometric pressure, both of which are frequent triggers for migraine. Depression is a comorbid disorder that often leads to a misdiagnosis of tension-type headache. Chronic migraine (previously referred to as "transformed" migraine) is a very common cause of chronic daily headaches and is defined as headaches occurring on more than 15 days per month that meet the diagnosis of migraine without aura or respond to ergots or triptans (migraine-specific drugs) on at least 8 of those days. This patient does not meet the diagnostic criteria for this type of headache. Another chronic daily headache often confused with chronic migraine is medication overuse headache. This type of headache, which typically occurs when (or soon after) a patient awakens, is defined as more than 15 headache days per month and more than 10 days of use of acute headache medication per month. Analgesic therapy may decrease headache intensity, but the headache usually will not completely remit. Although the symptoms of this condition may be identical to those of migraine or tension-type headache, the efficacy of migraine-specific therapy in patients with medication overuse headache is intermittent or poor. Patients with this condition respond only when the medication overuse is addressed. Again, the patient described in the vignette does not meet the diagnostic criteria for this type of headache. Headache triggered by changes in barometric pressure is often mistakenly diagnosed as sinus headache. Patients with acute rhinosinusitis and sinus headache have symptoms consisting of nasal congestion, purulent nasal secretions, sinus tenderness, and facial pain. These findings are not present in this patient. Tension-type headache is a dull, bilateral, or diffuse headache that is often described as a pressure or squeezing sensation of mild to moderate intensity. This type of headache has no accompanying migraine features (such as nausea), and its pain does not prohibit activity, characteristics that do not match this patient's symptoms. Headache triggered by stress is often mistaken as tension-type headache, even though stress is one of the most commonly reported migraine triggers. Up to 75% of patients with migraine report neck pain that precedes or occurs during the attack, and this scenario is frequently mistaken for tension-type headache.

Neuro 20 A previously healthy 42-year-old woman is evaluated in the emergency department for the sudden onset of a severe occipital headache during defecation 8 hours ago, followed by two episodes of vomiting. She reports no neck stiffness or neurologic symptoms. Her mother and two sisters have a history of migraine. On physical examination, temperature is 36.8 °C (98.2 °F), blood pressure is 148/88 mm Hg, pulse rate is 90/min, and respiration rate is 20/min. The patient is in obvious distress as a result of the pain. No evidence of meningismus, papilledema, or focal neurologic signs is found. Cerebrospinal fluid examination reveals a normal level of protein and glucose and no leukocytes or erythrocytes. A noncontrast CT scan of the head shows no abnormalities. Which of the following is the most appropriate next step in management? A Admission to the hospital for overnight observation B Administration of sumatriptan, subcutaneously C CT angiography of the head and neck D Repeat lumbar puncture

C Thunderclap headache is a potential neurologic emergency that requires urgent imaging of the cerebral vasculature with either magnetic resonance or CT angiography after a noncontrast CT scan of the head and a lumbar puncture have excluded subarachnoid hemorrhage. This patient should undergo CT angiography of the head and neck. She has experienced a thunderclap headache, which is a severe and explosive headache that is maximal in intensity at or within 60 seconds of onset. Every thunderclap headache must be immediately evaluated to detect potentially catastrophic conditions, especially subarachnoid hemorrhage. Most of the other causes of thunderclap headache, such as an unruptured cerebral aneurysm, a carotid or vertebral artery dissection, cerebral venous sinus thrombosis, and reversible cerebral vasoconstriction syndrome, can be excluded by noninvasive angiography. Therefore, CT angiography of the head and neck is the most appropriate next step in management. CT angiography can detect unruptured aneurysms as small as 3 mm in diameter and thus is adequate to exclude this diagnosis. Magnetic resonance angiography (MRA) would also be appropriate in this setting. Both CT angiography and MRA can be performed with a venous phase to exclude cerebral venous sinus thrombosis. Given that most causes of thunderclap headache can be excluded by such noninvasive angiography and that prior cerebrospinal fluid analysis has shown no evidence of a subarachnoid hemorrhage in this patient, conventional cerebral angiography, in which a catheter is inserted into a large artery and advanced through the carotid artery, is unnecessary. Because of the potential for neurologic morbidity associated with several of the causes of thunderclap headache, admission for observation without evaluation of the cerebral vasculature would not be the best management option. Similarly, treatment with a vasoconstrictive drug, such as sumatriptan, would not be appropriate until the other causes of thunderclap headache have been excluded. Drugs with the potential to constrict extracranial and intracranial cerebral vessels can precipitate or exacerbate the cerebral ischemia that may be associated with arterial dissection and reversible cerebral vasoconstriction syndromes. Although it may take up to 6 hours for subarachnoid blood to sediment into the lumbar thecal sac, this patient's first lumbar puncture occurred more than 8 hours after the onset of symptoms. Repeating the lumbar puncture would, therefore, be unnecessary.

Neuro 87 A 31-year-old woman with a history of migraine is evaluated in the office for several severe migraine attacks that are not well controlled with over-the-counter analgesics. The headaches are severe, are associated with severe nausea and vomiting, and began 2 weeks ago after she delivered a healthy baby boy. She is currently breast feeding. Results of physical examination, including neurologic examination, are normal. Which of the following is the most appropriate treatment for this patient? A Butalbital B Extra-strength acetaminophen C Frovatriptan D Metoclopramide E Prochlorperazine

C Using frovatriptan or sumatriptan for treatment of migraine is compatible with breast feeding, according to the American Academy of Pediatrics. This patient should be taking frovatriptan for her migraine attacks. Frovatriptan and sumatriptan are both considered compatible with breast feeding by the American Academy of Pediatrics. The patient has not responded to first-line therapy with simple analgesics and is reporting severe migraine attacks, for which a triptan is indicated. The American Academy of Pediatrics has given butalbital and acetaminophen a rating of caution and metoclopramide and prochlorperazine a rating of concern for mothers who are breast feeding. Therefore, these drugs should be avoided in such mothers. In addition, the patient has already reported a lack of response to over-the-counter analgesics, so extra-strength acetaminophen is not likely to result in marked improvement.

Pathophysiology of hyponatremia in subarachnoid hemorrhage

Inappropriate secretion of vasopressin and increased secretion of atrial/brain natriuretic peptide

Kidney f(x) and TSH should be monitored in patients taking what type of medication?

Lithium.

painless loss of vision with central visual field defect, patchy weakness

MS

Shoulder Abduction

Deltoid Axillary C5

what to suspect in patient with bilateral trigeminal neuralgia?

MS

Visual hallucinations, spontaneous parkinsonism, fluctuating cognition

Dementia w/ Lewy body

Fluctuating cognitive impairment and bizarre, visual hallucinations respiradone aggravates stiffness

Dementia with Lewy bodies (DLB). sensitive to neuroleptics

Repression vs Suppression vs Denial?

Denial -> Failure to accept external reality Represison - > Subconcious blocking of internal state (when doctor gives diagnosis of cancer you feel no emotion cuz those feels are subconciously blocked) Suppression-> Concious blocking, MATURE behavior... ex woman diagnosed with cancer conciously decides not to think about it to stay strong for her family

Central Vertigo

MS CVA (vertebrobasilar insuff - recall! rare cause of syncope) Vestibular migraine (no hearing loss/tinnitus)

Diagnostic test for pseudodementia

Dexamethasone suppression test, 50% positive

Chronic Open Angle Glaucoma

Peripheral vision loss. Loss of ganglionic cells --> atrophy of optic disc --> CUPPING. Rx - (all topical meds) 1. B blockers 2. a agonists 3. CA inhibitor

microglial cell

phagocytic scavenger cells of the CNS Origin: mesoderm Remember Ms: Mesoderm-Macrophage-Multinucleated Giant cells

Epilepsia partialis continua

Persistent focal motor seizure activity

Most common presentation of a colloid cyst

Persistent obstruction of the flow of CSF

Essential Tremor, Rx

Diagnosis of exclusion. Rx - B blockers or Primidone *Primodone [an antioconvulsant] --> can precipitate Acute Intermittent Porphyria. (abd pain, port wine, psych issues, neuro issues). Confirm w increase in urine porphobilinogen.

Prophylaxis for seizures in a head injury

Phenytoin

Rett syndrome presentation

Rapid regression in cognitive status and early childhood; loss of language skills an eye contact; Stereotypic eye movement

a brain that has seized for >5minutes (status epilepticus) is at increased risk of developing...

Cortical laminar necrosis due to excitatory cytotoxicity

Medial medullary syndrome (Anterior spinal artery syndrome) affects:

Corticospinals ML Hypoglossal nucleus

Acute MS treatment

Corticosteroids

Rx Labrynthitis or Vestibular neuritis

Corticosteroids within 72 hours after onset + sedatives (Meclizine)

headache, bitemporal vision loss, growth retardation, decreased libido, or amenorrhea

Craniopharingioma

Pituitary adenoma has similar symptoms to

Craniopharyngioma

Most common cause of hypopituitarism in children

Craniopharyngioma (most common supratentorial tumor in children), compresses optic chiasm and hypothalamus

Elevated 14-3-3 CSF proteins sharp wave complexes on EEG

Creutzfeldt-Jakob Disease

MC neurologic infection in the world

Cysteicercosis, Taenia solium

Neuro 12 A 30-year-old woman is evaluated in the office for a 6-week history of severe left facial pain. She says the pain occurs multiple times each day, is confined to the left cheek and jaw, and is stabbing in quality. Each pain episode lasts only 2 seconds, but she may experience multiple consecutive episodes. The pain can be triggered by drinking cold fluid, chewing on the left side of the mouth, or touching the left cheek. Three years ago, she had intermittent paresthesias of the hands and feet, which led to a diagnosis of anxiety. She has been taking ibuprofen for pain control since her current symptoms began, but it has been ineffective. On physical examination, vital signs are normal. Pain is triggered during the examination by touching the left cheek. She has no long-tract pyramidal signs or pathologic reflexes. Facial sensation and strength are normal, as are findings of funduscopic examination. Which of the following is the most appropriate next step in management? A Administration of baclofen B CT of the head C Lumbar puncture D MRI of the brain E Nerve conduction studies of the extremities

D Because trigeminal neuralgia usually presents after age 40 years, its diagnosis in a younger patient should prompt an evaluation for secondary causes, such as multiple sclerosis, posterior fossa tumors, and vascular or aneurysmal compression of the trigeminal nerve. This patient, whose history is most compatible with a diagnosis of trigeminal neuralgia, should have an MRI of the brain. In 90% of patients with trigeminal neuralgia, idiopathic disease presents after age 40 years. Trigeminal neuralgia in a patient this young should prompt evaluation for secondary causes, such as multiple sclerosis, posterior fossa tumors, and vascular or aneurysmal compression of the trigeminal nerve. In light of her history of transient paresthesias 3 years ago, multiple sclerosis is high among the diagnostic possibilities for this patient. An MRI can show the demyelination typical of multiple sclerosis. Baclofen, although sometimes used in this scenario, is not a first-line drug for treatment of trigeminal neuralgia and should not be initiated at this stage because there is insufficient evidence from randomized, controlled trials to show significant benefit from non-antiepileptic drugs in trigeminal neuralgia. CT of the head is not the imaging procedure of choice for this patient. The resultant scan will not show demyelinating lesions and may not detect posterior fossa or skull-based lesions involving the trigeminal nerve. Although the detection of abnormalities on cerebrospinal fluid examination can be useful in supporting a diagnosis of multiple sclerosis, brain-imaging procedures should precede lumbar puncture, especially as long as other structural intracranial lesions are still part of the differential diagnosis. Once such lesions are excluded by the results of imaging studies, lumbar puncture may be appropriate to perform. Nerve conduction studies of the extremities are inappropriate for this patient because the paresthesias have been transient, the neurologic examination discloses no evidence of a peripheral neuropathy, and the structural and demyelinating causes of trigeminal neuralgia are best excluded with an MRI of the brain.

light headedness, headache, blurring of vision, buckling of knees

Syncope- cerebral hypoperfusion Neurogenic- inappropriate activation of parasympathtics Autonomic failure- don't release NE upon standing

Neuro 5 A 41-year-old man is evaluated in the emergency department for a 3-day history of confusion and visual loss and a 10-day history of gradually increasing headache. Two weeks ago, he had a 3-day history of severe gastroenteritis with diarrhea, nausea, and vomiting. A review of medical records shows that he is a factor V Leiden heterozygote. On physical examination, vital signs are normal. The patient has papilledema, a very mild right pronator drift, right homonymous hemianopsia, and fluent aphasia. Results of laboratory studies and a CT scan of the head are normal. Which of the following is the most appropriate next diagnostic test in this patient? A Carotid ultrasonography B Electroencephalography C Lumbar puncture D Magnetic resonance venography

D Cerebral venous sinus thrombosis should be considered in the differential diagnosis of any patient with headache, features of elevated intracranial pressure, progressive reduction in the level of consciousness, and seizures, especially if there is a known risk of hypercoagulability. This patient should undergo magnetic resonance venography. The most likely diagnosis, given his known hypercoagulability, likely dehydration, symptoms of mounting intracranial pressure, and eventual focal deficits, is cerebral venous sinus thrombosis with possible venous ischemia. Cerebral venous sinus thrombosis may present with signs and symptoms of intracranial hypertension, such as headache, papilledema, and visual problems; focal neurologic findings or seizures; and mental status changes, stupor, and coma. Major risk factors for cerebral venous sinus thrombosis in adults include conditions that predispose to spontaneous thromboses, such as inherited or acquired thrombophilia, pregnancy, oral contraceptive use, malignancy, sepsis, and head trauma. Magnetic resonance venography can readily detect obstruction of the venous sinuses by a thrombus and the damage to the brain caused by the resultant increased pressure and so is the most appropriate next diagnostic test. Of all the possible imaging modalities, magnetic resonance venography is the most sensitive test for detecting the thrombus and the occluded dural sinus or vein. Carotid ultrasonography assesses the extracranial vasculature but does not assist in the assessment of the intracranial vasculature. The patient's clinical presentation suggests elevated intracranial pressure from venous stasis. The intracranial venous sinuses need assessment, not the extracranial carotid arteries. Seizures are a recognized complication of cerebral venous sinus thrombosis. However, electroencephalography does not assist with the establishment of a preliminary diagnosis and should not be performed next. A lumbar puncture is contraindicated in this patient with evidence of elevated intracranial pressure.

Neuro 24 A 71-year-old woman is admitted to the hospital after having a witnessed morning seizure. She has a 3-week history of increasing gait unsteadiness and daytime somnolence and a 3-month history of progressive confusion and headaches; she typically naps 3 hours daily. Six month ago, the patient had fungal pneumonia caused by Coccidioides immitis infection. She has a 20-year history of type 2 diabetes mellitus and no relevant family history. Current medications are insulin glargine, metformin, and intravenous fosphenytoin (started on hospital admission). On physical examination, temperature is 38.2 °C (100.8 °F), blood pressure is 116/70 mm Hg, pulse rate is 96/min, respiration rate is 18/min, and BMI is 32. The patient is somnolent but arouses to voice. She is disoriented to time and place and scores only 12/30 on the Folstein Mini-Mental State Examination. There is mild diffuse symmetric hyperreflexia, and plantar responses are extensor bilaterally; the patient moves all four limbs equally. There is no papilledema. Leukocyte count is 14,000/µL (14 × 109/L), and erythrocyte sedimentation rate is 64 mm/h. All other results of laboratory studies, including measurement of serum vitamin B12 level, a basic metabolic panel, thyroid function tests, and urinalysis, are normal. A noncontrast CT scan of the head shows mildly dilated ventricles. An electroencephalogram shows moderately severe diffuse slowing but no epileptiform activity. Which of the following is the most appropriate next diagnostic test for this patient? A Apolipoprotein E genotyping B Cerebral CT angiography C Cisternography D Lumbar puncture and cerebrospinal fluid analysis E Repeat electroencephalography

D Cerebrospinal fluid analysis should be considered in any patient with potentially reversible causes of impaired cognition, particularly if meningoencephalitis is suspected. This patient has fungal meningitis due to Coccidioides immitis infection and thus should undergo lumbar puncture and subsequent cerebrospinal fluid analysis to detect the potentially reversible cause of her cognitive and other impairments. A 71-year-old woman with declining cognition may seem an obvious candidate for Alzheimer dementia, but any pathologic process affecting the brain can impair cognition. The symptoms described—subacute onset, headache, fluctuating level of alertness, fever, peripheral blood markers of inflammation, and mildly dilated ventricles on the CT scan—should all provoke suspicion of an infectious, inflammatory, or otherwise reversible cause rather than a degenerative one. The scenario described is of a patient with encephalopathy whose clinical, laboratory, and radiologic findings suggest some type of meningitic process. Because of the time course, an atypical infectious agent, such as fungal meningitis, should be considered, especially in light of her history of fungal pneumonia in the past. Other causes of chronic or subacute meningoencephalitis, such as autoimmune encephalopathy and carcinomatous meningitis, need also be considered. Apolipoprotein E (APOE) genotyping will not provide useful information in this patient. Specifically, discovering if this patient carries the APOE e4 allele and thus has an elevated risk for Alzheimer dementia would not help explain or treat her current symptoms and signs but could delay finding the probable infectious, inflammatory, or related cause of her symptoms. Although cerebral vasculitis can produce a clinical picture similar to this one, cerebral CT angiography is not sufficiently sensitive to diagnose vasculitis of the central nervous system. Making that diagnosis usually requires either invasive intra-arterial angiography or brain and leptomeningeal biopsy. The more usual application of cerebral CT angiography is in the assessment of acute stroke, of which there is no evidence in this patient. The finding of mildly dilated ventricles on this patient's CT scan is not relevant to her signs and symptoms, so further confirmatory testing by cisternography is unlikely to reveal the underlying cause of her findings. It is highly unlikely that a repeat electroencephalogram (EEG) will give much further insight into this patient's condition, given that the EEG obtained during the patient's symptomatic period showed no epileptiform activity. Seizures are a potential complicating feature of any meningitic or encephalitic process, but the occurrence of a seizure or EEG finding of occasional interictal epileptiform activity will not provide any additional information about the underlying cause of this patient's symptoms.

Neuro 58 A 58-year-old man is evaluated for a 5-month history of gait impairment and falls and a 1-week history of difficulty swallowing. His wife, who accompanied him, says that she has noticed a change in his speech over the past few weeks. He has no significant medical history, and there is no known family history of neurologic problems. General physical examination findings are normal; no orthostatic decrease in blood pressure is noted. Neurologic examination shows normal orientation and memory. Speech is slow and mildly dysarthric, and he appears to have decreased facial expression. Cranial nerve examination shows an impairment in vertical gaze. There is evidence of moderate axial and mild bilateral appendicular rigidity. No tremor is noted. Muscle strength and deep tendon reflexes are normal, as are sensory examination findings. There is no appendicular ataxia. Gait is slow, and there is significant postural instability. Which of the following is the most likely diagnosis? A Amyotrophic lateral sclerosis B Corticobasal degeneration C Parkinson disease D Progressive supranuclear palsy

D Early falls, symmetric bradykinesia and rigidity, and lack of a resting tremor or levodopa responsiveness characterize progressive supranuclear palsy and help distinguish it from Parkinson disease. This patient's history and examination findings are most consistent with a diagnosis of progressive supranuclear palsy. A sporadic, neurodegenerative disorder, progressive supranuclear palsy typically manifests as gait impairment and falls, slurred speech, and impaired swallowing. The presence of reduced facial expression, axial rigidity, and impairment of vertical eye movements on examination further suggests the diagnosis. Amyotrophic lateral sclerosis is not associated with parkinsonian signs such as rigidity and postural instability. Patients with bulbar-onset amyotrophic lateral sclerosis present with slurred speech (dysarthria) and swallowing dysfunction, later followed by the development of diffuse extremity weakness, atrophy, and fasciculations. Corticobasal degeneration is also a rare, sporadic, neurodegenerative disorder that can manifest as gait impairment, slurred speech, dystonia, or myoclonus. Parkinsonian signs are evident on examination and are characteristically asymmetric. Alien limb phenomena can occur, whereby an extremity will move independently of voluntary, conscious control. The lack of a resting tremor, the symmetric bradykinesia and rigidity, and the early falls distinguish this patient's condition from Parkinson disease. Patients with progressive supranuclear palsy do not respond favorably to levodopa therapy, a mainstay of Parkinson disease management.

Neuro 34 A 65-year-old man is evaluated for possible epilepsy. He reports three similar "spells" over the past month of which he recalls a feeling of déjà vu followed by a loss of awareness. He says that family members who witnessed these episodes have described him as being unresponsive during them, recounting that he stares and repetitively smacks his lips for 1 minute, followed by a few minutes of confusion and garbled speech before he returns to normal awareness. He has no significant personal or family medical history and takes no medications. Physical examination, including neurologic examination, reveals no abnormalities. Which of the following is the most likely diagnosis? A Absence seizure B Generalized tonic-clonic seizure C Myoclonic seizure D Partial complex seizure E Simple partial seizure

D Epilepsy that presents in adulthood is usually partial in onset; partial seizures that involve altered awareness are classified as complex partial seizures. This patient's "spells" were most likely partial complex epileptic seizures. The incidence of epilepsy is greatest in infants and older adults, with half of new-onset seizures presenting in persons older than 65 years. In large part, the latter fact reflects the increased prevalence of underlying brain diseases—particularly cerebrovascular disease, neurodegenerative conditions (such as Alzheimer disease), and brain tumors— that increase seizure risk in this segment of the population. Epilepsy is classified as partial or generalized on the basis of the areas of the brain involved at onset. Seizures that present in adulthood are usually partial in onset; partial seizures in which the patient maintains full awareness are classified as simple partial, whereas those involving an alteration of consciousness, as in this patient, are classified as partial complex. The most common site of onset for partial seizures is the temporal lobe. As described by this patient, temporal lobe events often begin with an aura of déjà vu, a rising epigastric sensation, or autonomic disturbances. Although automatisms, such as lip smacking, are occasionally reported in absence seizures, these features are atypical for that type of seizure and are more suggestive of partial complex ones. In this patient, the age of onset, duration (several minutes) of the event, presence of oral automatisms, and reported postictal confusion and speech impairment all suggest a partial complex seizure. Absence, generalized tonic-clonic, and myoclonic seizures are all manifestations of generalized epilepsy and are correlated with generalized epileptiform discharges on an electroencephalogram. Absence seizures are characterized by brief periods of staring and unresponsiveness, typically lasting seconds, with an immediate return to normal awareness. Absence seizures usually present in childhood. The length of this patient's episodes, his confusion before returning to normal awareness, and his age argue against absence seizure being the diagnosis. Generalized tonic-clonic (grand mal) seizures are characterized by stiffening of the trunk and extremities followed by generalized symmetric jerking. Such features were not reported in this patient. Myoclonic seizures consist of brief, shock-like muscle jerks without loss of awareness. These features are not consistent with the history this patient provides.

Neuro 46 A 74-year-old woman is admitted to the hospital after sustaining a severe left hemispheric ischemic stroke while alone at home. Her son found her collapsed in the living room when he went to visit her. The patient has hypertension for which she takes enalapril but no history of ischemic heart disease or heart failure. On physical examination, blood pressure is 190/105 mm Hg, pulse rate is 80/min, and respiration rate is 16/min. The patient has right hemiparesis, right facial droop, aphasia, and dysarthria. The remainder of the physical examination, including the cardiovascular examination, is normal. Results of laboratory studies, including serum creatinine level, are normal. A CT scan shows frank ischemic changes that occupy most of the left middle cerebral artery territory. An electrocardiogram and chest radiograph show normal findings. Which of the following is the most appropriate treatment of her hypertension at this time? A Intravenous labetalol B Intravenous nicardipine C Oral nifedipine D Withholding (Pausieren) of all antihypertensive medications

D For uncomplicated ischemic strokes in patients without concurrent acute coronary artery disease or heart failure, antihypertensive medications should be withheld if the systolic blood pressure is less than 220 mm Hg or the diastolic blood pressure is less than 120 mm Hg, unless there are other manifestations of end-organ damage. For uncomplicated ischemic strokes in patients without concurrent acute coronary artery disease or heart failure, consensus exists that antihypertensive medications, such as intravenous labetalol or nicardipine, should be withheld if the systolic blood pressure is less than 220 mm Hg or the diastolic blood pressure is less than 120 mm Hg, unless there are other manifestations of end-organ damage. This patient's systolic and diastolic blood pressure levels are below these limits. Many such patients have spontaneous declines in blood pressure during the first 24 hours after stroke onset. Oral nifedipine is an inappropriate treatment for this patient not only because of its antihypertensive qualities, but also because of its route of administration. Given the severity of her stroke deficits, in particular the dysarthria, she should receive nothing by mouth until a swallowing evaluation is carried out because of the high risk of aspiration. Notably, the patient is not eligible for recombinant tissue plasminogen activator therapy because the time interval between now and her previous symptom-free state is unknown. Aspirin (160 to 325 mg/d) administered within 48 hours of stroke onset results in a small but significant reduction in the risk for recurrent stroke during the first 2 weeks after the stroke and improves outcome at 6 months. Therefore, aspirin is recommended as initial therapy for most patients with acute stroke. However, aspirin should not be administered for at least 24 hours after administration of thrombolytics.

Neuro 64 A 28-year-old man is evaluated in the office for a 3-day history of blurred vision in the left eye and pain with eye movement. He has a history of rare migraine headaches but no other disorders. There is no pertinent family history. The patient takes no medications. On physical examination, temperature is 36.8 °C (98.2 °F), blood pressure is 105/65 mm Hg, pulse rate is 78/min, respiration rate is 12/min, and BMI is 22. A left central scotoma is noted on visual field testing. Corrected visual acuity is 20/50 in the right eye and 20/20 in the left; red desaturation and a relative afferent pupillary defect are noted in the left eye. Other findings of physical and neurologic examinations, including funduscopy, are normal. Results of laboratory studies are normal. An orbital MRI reveals enhancement of the left optic nerve with intravenous administration of gadolinium. An MRI of the brain shows six periventricular white matter lesions, each measuring 3 to 5 mm in diameter and one enhancing with gadolinium administration. Ophthalmologic consultation confirms that the clinical features are compatible with optic neuritis. Which of the following best describes this patient's approximate risk of developing multiple sclerosis over the next 10 to 15 years? A 10% B 20% C 50% D 90%

D If the brain MRI of a patient with a first-ever event of symptomatic central nervous system inflammatory demyelination shows lesions consistent with demyelination, the risk of developing multiple sclerosis approaches 90% over the next 10 to 15 years. The results of a brain MRI in a patient with new-onset neurologic symptoms suggestive of demyelination provide the most powerful prognostic information about his or her risk of developing multiple sclerosis (MS). Symptomatic patients with as few as one MRI brain lesion compatible with demyelination on clinical presentation have a risk of up to 90% that they will experience a second clinical event over the next 10 to 15 years, thereby confirming MS. Conversely, if the brain MRI reveals no white matter lesions, their risk over the same time period is slightly less than 20%. The brain MRI typical of MS reveals ovoid lesions in the periventricular white matter that sometimes enhance with gadolinium. This patient has also experienced left optic neuritis. When this occurs as a first-ever event of symptomatic central nervous system inflammatory demyelination, it is referred to as a clinically isolated syndrome, a term that implies some risk of developing MS in the future. Other such clinically isolated syndromes include brain stem or spinal cord events, such as myelitis. Long-term follow-up studies of all patients presenting with optic neuritis show that only approximately 50% of them later develop MS. This risk, however, is increased over that of the general population. Coupled with the implications of the brain MRI in this patient, the finding of optic neuritis confirms his very high risk.

Antipsychotic drugs block which type of receptor?

D2 (dopamine)

Neuro 63 A 64-year-old woman is evaluated for a 1-year history of increasing difficulty finding the right word in conversation and completing sentences; she sometimes says the wrong word accidentally. Her family now has difficulty understanding her, and she no longer has any interest in speaking on the telephone. Her ability to drive, shop, pay bills, and cook seems unimpaired. She has no other relevant personal or family medical history. Her only medication is aspirin, 81 mg/d. On physical examination, temperature is 36.6 °C (97.9 °F), blood pressure is 122/78 mm Hg, pulse rate is 80/min, respiration rate is 14/min, and BMI is 23. The patient is right-handed. Her level of alertness is normal, and her comprehension appears to be intact, with her correctly executing the commands to show the right thumb and two fingers on the left hand. Spontaneous speech is effortful, and she talks in short, telegraphic sentences filled with many mispronunciations (such as "posital" for "hospital"). She makes similar errors when trying to write words rather than speak them, can repeat no more than two words or four numbers at a time, and can repeat essentially no sentences. Results of a complete blood count, a basic metabolic panel, a serum vitamin B12 measurement, and thyroid function tests are normal. An MRI of the brain without contrast shows mild atrophy but is otherwise unremarkable. This patient's impairment in speech and writing is most likely due to which of the following disorders? A Alzheimer dementia B Creutzfeldt-Jakob disease C Dementia with Lewy bodies D Frontotemporal lobar degeneration E Vascular dementia

D In frontotemporal lobar degeneration, which encompasses the syndromes of progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia, symptom onset is insidious and progression gradual over the course of several years. This patient has progressive nonfluent aphasia that is most likely due to frontotemporal lobar degeneration. Progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia comprise the three main syndromes of frontotemporal lobar degeneration. Symptom onset is insidious and progression is gradual over the course of several years. Her early decline in social interpersonal conduct is typical of this disorder, as is her aspontaneity and economy of speech. Approximately 10% of patients with frontotemporal lobar degeneration, especially frontotemporal dementia, have concurrent motoneuron disease. The most prominent early symptom of Alzheimer dementia is memory impairment. Alzheimer dementia can also produce aphasia, although usually not a purely nonfluent form and not typically as an initial symptom. Other diagnoses should also be considered when the prominent early finding is a symptom other than recent memory impairment, such as impaired social behavior, gait difficulty, or hallucinations and delusions. Clinical suspicion for additional diagnoses also should be raised when the disease course is not insidious and chronically progressive. Finally, Alzheimer dementia typically begins at a later age than 64 years, an age that is more typical of frontotemporal lobar degeneration. Creutzfeldt-Jakob disease is a rapidly progressive dementia producing death, typically within a year of onset. Although the specific symptoms of this disorder are highly variable, this patient's gradual disease course does not resemble the more rapid one of Creutzfeldt-Jakob disease. This patient does not have two of the core clinical features (parkinsonism, fluctuations in cognition or level of alertness, and visual hallucinations) required for a diagnosis of dementia with Lewy bodies, and she is considerably younger than most patients with this disorder. As with Alzheimer dementia, aphasia can occur within the context of this dementia. Progressive nonfluent aphasia is often mistaken for a stroke because of the obvious speech impairment produced. With stroke, however, the aphasia would be of sudden onset, and the patient would likely have MRI evidence of cerebral infarction, both of which are lacking in this instance. Additionally, the patient has exhibited no other signs or symptoms of stroke and has no history of vascular disease. Therefore, vascular dementia is unlikely.

Neuro 49 A 38-year-old woman is evaluated in the office for a 10-month history of increasingly frequent headache. The headache is often worse in the morning on awakening. She has recently started keeping a headache diary, which reveals episodes on approximately 25 days of each month. The headache varies from a near-daily bilateral frontal dull throbbing to a severe left hemicranial throbbing associated with nausea, photophobia, and phonophobia. The patient has a 20-year history of migraine without aura and a history of depression. Her mother also has a history of migraine and depression, and her sister has a history of migraine. The patient has been taking propranolol for 3 months; a mixed analgesic containing butalbital, caffeine, and acetaminophen for mild or moderate headache at least 3 days per week for 9 months; rizatriptan for severe headache at least 2 days per week for 4 months; and citalopram for 1 year. Rizatriptan has become increasingly ineffective over the past month. Physical examination findings, including neurologic examination findings, are normal. Which of the following is the most likely diagnosis for her current symptoms? A Chronic migraine B Chronic tension-type headache C Idiopathic intracranial hypertension D Medication overuse headache

D Medication overuse headache is generally defined as a headache for more than 15 days per month and the use of acute headache medication on more than 10 days per month. This patient has medication overuse headache. She has a 20-year history of migraine but a 10-month history of chronic daily headache on more than 15 days per month. She has been using an acute headache medication (butalbital, caffeine, and acetaminophen) more than 10 days per month and a combination of this medication and rizatriptan on some of these days. These features define a medication overuse headache. Although the patient does have chronic migraine, her current symptoms most likely result from her overuse of acute medications and not from her long history of migraine. Medication overuse headache typically presents when or soon after a patient awakens, and the efficacy of migraine-specific therapy in patients with medication overuse headache is intermittent or poor. Furthermore, some of this patient's headaches lack the classic features of migraine, including a pounding, unilateral headache of approximately 1-day's duration associated with nausea and disability (taking to bed). Despite the patient's depression, her headaches are not fully characteristic of chronic tension-type headache, which is typically mild to moderate in severity, lasts from 30 minutes to 7 days, and is often described as a "band-like" constriction around the head. Tension-type headaches are not associated with nausea and vomiting, photophobia, or phonophobia. Idiopathic intracranial hypertension is a disturbance of increased intracranial pressure without evidence of intracranial disease, such as mass lesion, hydrocephalus, or venous sinus thrombosis. This disorder occurs most commonly in obese women of childbearing age but also may be associated with tetracycline therapy, oral contraceptive use, and hypervitaminosis A. Affected patients typically develop new onset of daily nonthrobbing headaches that may worsen with coughing and sneezing or in the supine position. Other clinical symptoms may include diplopia, transient episodes of monocular or binocular visual loss, and pulsatile tinnitus. Characteristic findings in patients with this condition are papilledema, an enlarged blind spot or visual field abnormalities, and possible sixth cranial nerve palsy. This patient's findings are not consistent with idiopathic intracranial hypertension.

Neuro 53 A 50-year-old man is evaluated because of abnormal findings on a brain MRI. Two weeks ago, he was involved in a motor vehicle accident in which he sustained shoulder, upper back, and neck injuries and head trauma without loss of consciousness. Results of an MRI of the cervical spine were normal, but an MRI of the brain revealed white matter abnormalities. The patient has a 15-year history of hypertension but no personal or family history of neurologic disease. His only medication is quinapril. On physical examination, blood pressure is 155/90 mm Hg; all other vital signs are normal. Results of neurologic examination are also normal. Which of the following is the most appropriate next step in diagnosis? A Lumbar puncture B Repeat MRI in 3 months C Visual evoked potential studies D No further testing

D Misinterpretation of nonspecific white matter lesions discovered on brain MRIs of patients without specific symptoms is a leading cause of the misdiagnosis of multiple sclerosis. This patient requires no further testing. He has MRI evidence of white matter lesions but no history or examination findings that suggest a diagnosis of multiple sclerosis (MS). Misinterpretation of white matter abnormalities incidentally discovered on the MRIs of patients with nonspecific symptoms is a leading cause of MS misdiagnosis. The lesions noted on this patient's MRI are not typical of those seen in MS; given his age and long-standing and incompletely controlled hypertension, they are most likely related to small-vessel cerebrovascular disease. MS lesions are typically larger, ovoid, and periventricular in location, as in the MRI shown . They sometimes enhance with gadolinium. The patient can be reassured that MS is extremely unlikely and counseled that he needs to improve his blood pressure control and be aware of other modifiable vascular risk factors. Lumbar puncture and visual evoked potential studies might be useful if MS were a serious clinical consideration but are unnecessary for this patient. There is currently no indication to repeat the MRI in 3 months for diagnostic purposes; additionally, findings will not influence management of his hypertension or other modifiable risk factors.

Neuro 37 A 75-year-old man is evaluated in the emergency department for a 2-day history of impaired balance, left-leg weakness, and urinary urgency and a 3-day history of constant midthoracic pain with occasional shooting pain in the left thorax at approximately the T7 dermatome. There is no history of trauma. The patient has degenerative arthritis of the lumbosacral spine. He is a current smoker with a 50-pack-year smoking history. The patient takes occasional ibuprofen or acetaminophen for back pain. On physical examination, temperature is normal, blood pressure is 140/85 mm Hg, pulse rate is 80/min, and respiration rate is 14/min. Neurologic examination shows left lower extremity weakness, hyperreflexia, an extensor plantar response, bilateral sensory impairment below the T7 dermatome, and gait ataxia. Findings from mental status, cranial nerve, and upper extremity motor and sensory examinations are normal. Results of a complete blood count, coagulation panel, and erythrocyte sedimentation rate measurement are also normal. A radiograph of the chest shows a parenchymal lesion in the right lung apex. Which of the following is the most likely diagnosis? A Acute L5 disk herniation B Epidural abscess C Epidural hematoma D Epidural metastases

D New and progressive symptoms referable to the spinal cord represent a neurologic emergency that should prompt evaluation for a compressive lesion. The most likely diagnosis is epidural spinal cord compression from metastatic cancer of the spine. The clinical feature of weakness accompanied by upper motoneuron signs (such as hyperreflexia and an extensor plantar response) and the detection of a sensory level localize the process to the thoracic spinal cord. The shooting thoracic pain is a localizing symptom that indicates involvement of the left T7 spinal nerve root. The patient is a long-standing smoker and has a lung mass suspicious for malignancy. Lung cancer is among the most common primary malignancies that metastasize to the spine. New and progressive symptoms referable to the spinal cord represent a neurologic emergency that should prompt evaluation for a compressive lesion. Clinical outcome, including ambulatory ability and mortality, depends on neurologic status at diagnosis. A lumbar disk herniation at the L5 level would typically cause low back pain and radicular pain (sciatica) in one lower extremity because most herniations are unilateral or asymmetric. The thoracic location of the pain and sensory level and the presence of upper motoneuron signs in this patient exclude that diagnosis. Other compressive lesions, such as an abscess or a hematoma, could result in the clinical symptoms and signs exhibited by this patient. However, epidural abscesses are usually accompanied by fever and an elevated erythrocyte sedimentation rate, both of which were absent in this patient. Likewise, epidural hematoma development is strongly associated with anticoagulant use; this patient was not taking any such medications, and he had normal results on a coagulation panel.

Neuro 42 A 50-year-old man is evaluated for a 12-year history of slowly progressive left leg weakness and trouble ambulating. There is no history of transient neurologic symptoms. He has a history of hypertension, coronary artery disease, and chronic low back pain. Current medications are sublingual nitroglycerin, atenolol, aspirin, and occasional NSAIDs. On physical examination, vital signs are normal. The patient has moderately severe spastic paraparesis that is worse on the left, with prominent circumduction of the left leg during ambulation. He requires a cane to ambulate 100 meters. Cerebrospinal fluid analysis reveals the presence of oligoclonal bands. MRIs of the brain and spine show lesions consistent with chronic multiple sclerosis. Which of the following is the most appropriate treatment for this patient? A Glatiramer acetate B Interferon beta-1a C Natalizumab D Physical therapy

D No therapies have convincing effects on the neurodegenerative processes that underlie progressive forms of multiple sclerosis. This patient has primary progressive multiple sclerosis (MS); no treatments have been shown to affect the disease course. Therefore, physiatry consultation for evaluation and treatment of his spasticity and back pain are most appropriate at this time. His gradually worsening neurologic function is very likely due to a degenerative loss of axons in the central nervous system; active inflammation, the hallmark of clinical relapses, plays a much less significant role in primary progressive MS than in the relapsing-remitting type. This patient's function and pain could likely be improved substantially with a focus on symptomatic therapies, beginning with physiatry consultation for evaluation and treatment of his spasticity, impaired mobility, and musculoskeletal pain. Symptomatic therapy in patients with multiple sclerosis can have a marked beneficial effect on patient comfort, function, and quality of life, even when further disease progression cannot be effectively stopped. Beta interferons, glatiramer acetate, and natalizumab, the currently approved MS therapies, are all most effective in altering the immunologic mechanisms that underlie relapses and thus are only appropriate for relapsing-remitting disease.

DDx of GBS

DDx for this condition is: transverse myelitis, NJM issues, myopathy

Neuro 72 A 79-year-old woman was hospitalized 4 days ago after sustaining a right hip fracture in a fall. She underwent surgical repair with right hip replacement 3 days ago and did not awaken from general anesthesia until 12 hours after extubation. As her alertness has increased, she has become increasingly agitated, yelling at the nurses and flailing her arms; mechanical four-limb restraints were placed 2 days ago. The patient has a 4-year history of progressive cognitive decline diagnosed as Alzheimer dementia. She also has chronic atrial fibrillation treated with chronic warfarin therapy. She has no other pertinent personal or family medical history. Current medications are donepezil, memantine, atenolol, warfarin, and low-molecular-weight heparin. On physical examination today, temperature is 37.2 °C (99.0 °F), blood pressure is 100/68 mm Hg, pulse rate is 100/min and irregular, respiration rate is 18/min, and BMI is 21. The patient can move all four limbs with guarding of the right lower limb. She is inattentive and disoriented to time and place and exhibits combativeness alternating with hypersomnolence. The remainder of the neurologic examination is unremarkable, without evidence of focal findings or meningismus. Which of the following is the most likely diagnosis? A Acute cerebral infarction B Acute worsening of Alzheimer dementia C Meningitis D Postoperative delirium

D Patients with chronic dementia, such as Alzheimer dementia, are at greater risk for delirium after surgery with general anesthesia. Abrupt worsening of confusion in elderly patients with chronic dementia usually results from an acute medical problem. In addition, patients with chronic dementia from almost any cause are at greater risk for delirium after surgery with general anesthesia. This patient with a hip fracture who underwent right hip surgery with general anesthesia and did not recover from the anesthesia until 12 hours after extubation most likely has postoperative delirium. Such delirium is highly predictable and often easily managed by identification and correction of any underlying disorders and the removal or reduction of contributing factors. In a patient with chronic atrial fibrillation who is confused postoperatively, the possibility of acute stroke must be considered. However, this patient has no clinical evidence of such an event, making this diagnosis extremely unlikely. Surgery does not exacerbate the dementia of Alzheimer dementia (or of any other cause) but rather produces a superimposed delirium. This patient has had dementia for 4 years that has abruptly gotten worse after surgery. Although not impossible, meningitis is highly unlikely in this setting, especially given the absence of any supporting physical examination findings, including meningeal irritation.

Neuro 67 A 65-year-old man is hospitalized because of a 6-week history of progressive weakness and incoordination of the left arm and leg. He is otherwise healthy, with no relevant personal or family medical history. He takes no medications. Vital signs and other general physical examination findings are normal. Neurologic examination confirms mild weakness of the left arm and leg with decreased rapid alternating movements. A complete blood count and peripheral blood smear are normal. A contrast-enhanced MRI of the brain is shown . Lumbar puncture is ordered, and no abnormalities are found on subsequent analysis of the cerebrospinal fluid, including cytologic examination and flow cytometry. Which of the following is the most appropriate next step in management? A Chemotherapy with intrathecal methotrexate B Corticosteroid administration C Hospice referral D Stereotactic brain biopsy E Whole-brain radiation therapy

D Primary central nervous system lymphoma is diagnosed by detection of a clonal B-cell population on cerebrospinal fluid analysis or by brain biopsy. This patient should undergo a stereotactic brain biopsy. He most likely has a primary central nervous system (CNS) lymphoma, given the distinctive radiographic signs. In immunocompetent patients, a primary CNS lymphoma is visualized most often as a solitary round mass with minimal surrounding edema that is situated in the deep white matter. Tumors are isointense to hypointense on T2-weighted MRI images and enhance homogeneously after contrast administration. Parenchymal brain metastases are rare in patients with systemic lymphoma. More commonly, parenchymal brain involvement occurs as an isolated site of disease (primary CNS lymphoma). Among patients presenting with a primary CNS lymphoma, occult systemic disease is present in less than 5%. The symptoms of CNS lymphoma are dependent on the site of tumor involvement. Primary CNS lymphoma is usually a B-cell non-Hodgkin lymphoma and is rare, accounting for less than 5% of intracranial tumors. It presents most commonly in the sixth and seventh decades of life in immunocompetent patients and in the fourth decade in HIV-infected patients. Despite its characteristic appearance, a primary CNS lymphoma cannot be accurately diagnosed by clinical presentation or neuroimaging studies. Because other conditions, both malignant and nonmalignant, can mimic primary CNS lymphoma, a tissue diagnosis is required before treatment can be appropriately initiated. Diagnostic modalities include stereotactic brain biopsy or cerebrospinal fluid analysis to detect a clonal B-cell population. Standard treatment of primary CNS lymphoma in immunocompetent patients consists of chemotherapy (usually high-dose methotrexate) followed by whole-brain radiation therapy if the patient's tumor is refractory to chemotherapeutic agents. However, neither of these treatments is appropriate before a biopsy establishes a tissue diagnosis. When lymphoma is suspected but not confirmed, corticosteroids should be avoided unless mandated by high risk of immediate morbidity and mortality from mass effect because they may cause the lesion to dramatically shrink or even disappear. This corticosteroid response is temporary and can complicate diagnosis by reducing the yield of diagnostic biopsy and delay therapy. The median survival of patients with CNS lymphoma is 3 years with combination chemotherapy and radiation therapy. Therefore, hospice referral is not indicated, particularly before the establishment of a tissue diagnosis.

Neuro 85 A 32-year-old woman is evaluated for a gradual increase in migraine frequency and severity over the past 6 months. Migraine attacks, which formerly occurred two or three times each month, are now occurring approximately three times each week, with each attack lasting at least 12 hours. She has a 15-year history of asthma, a 10-year history of migraine without aura, and a 4-year history of type 2 diabetes mellitus controlled by diet. Her mother and sister also have a history of migraine. Current medications are almotriptan as needed for acute migraine, an inhaled corticosteroid, and an albuterol inhaler as needed for wheezing. On physical examination, blood pressure is 130/80 mm Hg, pulse rate is 88/min, and BMI is 34. Results of a physical examination, including a neurologic examination, are normal. An MRI of the brain shows no abnormalities. Which of the following is the most appropriate treatment for this patient? A Gabapentin B Nortriptyline C Propranolol D Topiramate E Valproic acid

D Prophylactic medication should be initiated in patients with two or more migraine attacks per week. Prophylactic treatment should generally be initiated in patients with two or more migraine attacks per week. There is level 1 evidence to support the use of topiramate for the prevention of migraine, and the U.S. Food and Drug Administration has approved the drug for this purpose. This patient is obese (BMI of 34) and has type 2 diabetes mellitus. Any medication with the potential for weight gain must, therefore, be used with caution, given the morbidity associated with obesity and the potential to worsen her underlying hyperglycemia. Topiramate is associated with weight loss. Gabapentin is a second-tier drug because of the lower level of evidence supporting its use. It is also not approved by the FDA for the preventive treatment of migraine. There is no evidence of nortriptyline's efficacy in the preventive treatment of migraine. Moreover, it is also associated with weight gain. Propranolol, a nonselective β-blocker, is contraindicated in patients with persistent asthma and has a relative contraindication in patients with diabetes mellitus. Valproic acid, although also supported by level 1 evidence and FDA approval for migraine prevention, is associated with weight gain and is not the best treatment for this patient. Additionally, in light of the potential teratogenicity associated with this drug, it is often avoided in women of childbearing potential.

Neuro 30 An 81-year-old man is evaluated for the gradual onset and progression of memory loss over the past year. He says he has difficulty recalling the names of familiar people, has misplaced his wallet on numerous occasions, and is slower to find his car in large, crowded parking lots. He continues to manage his finances, travel with his wife, and perform the activities of daily living without difficulty. He has borderline hyperlipidemia that is managed by diet alone. A paternal uncle developed Alzheimer dementia at age 74 years. His only medications are aspirin and a daily multivitamin. On physical examination, temperature is 36.7 °C (98.1 °F), blood pressure is 126/82 mm Hg, pulse rate is 68/min, respiration rate is 14/min, and BMI is 26. His level of alertness, speech, and gait are normal. He scores 26/30 on the Folstein Mini-Mental State Examination, losing all three points on the recall portion and one point on the orientation section for incorrectly stating today's date. Results of a complete blood count, serum vitamin B12 measurement, thyroid function tests, and a basic metabolic panel are normal. An MRI of the brain without contrast shows no abnormalities. Which of the following is the most likely diagnosis at this time? A Alzheimer dementia B Dementia with Lewy bodies C Frontotemporal dementia D Mild cognitive impairment E Vascular dementia

D This patient has mild cognitive impairment (MCI), which denotes abnormal cognitive decline that is not severe enough to produce disability. His self-reported memory loss, which is confirmed by his performance on the Folstein Mini-Mental State Examination, is his only symptom; there are no other signs of dementia. Memory loss is nonspecific and is part of many dementia syndromes. However, the lack of any functional impairment in this patient makes MCI the most likely diagnosis at this time. Although there are no universally accepted criteria for MCI, the disorder has been defined as a memory abnormality corroborated by objective memory impairment on standardized tests, without general cognitive impairment or an effect on functional independence. The rate of progression to dementia is approximately 10% to 15% per year. Alzheimer dementia is the most common cause of MCI involving memory loss. Because this patient has no functional disabilities and thus does not meet the criteria for frank dementia, Alzheimer dementia is an incorrect diagnosis at this point. He may eventually develop the disease, given that the conversion rate of MCI to dementia is roughly 10% to 15% per year and that, at autopsy, approximately 80% of patients originally diagnosed with MCI have Alzheimer dementia. Early-stage symptoms that are characteristic of frontotemporal dementia include changes in behavior and personality, such as increasing apathy, disinhibition, or perseverative (repetitive to an exceptional degree) fixations. This patient has exhibited no such changes. The onset of dementia with Lewy bodies could also be characterized by memory loss. Besides clearly not having dementia of any sort at this stage of his illness, this patient lacks any of the other symptoms of dementia with Lewy bodies, such as parkinsonism, visual hallucinations, psychomotor slowing, and dream enactment behavior. Typical manifestations of vascular dementia include psychomotor slowing, a stepwise progression, and a history of stroke, none of which pertains to this patient.

Neuro 32 A 35-year-old woman is evaluated in the office for a 5-month history of right-hand numbness and tingling. She says that these symptoms involve the entire hand, seem to be worse when she drives or holds a book or newspaper, and have been awakening her at night. She reports no history of neck pain or hand weakness. Personal and family medical history is noncontributory, and she takes no medication. General physical examination reveals no abnormalities. Neurologic examination shows normal strength but sensory loss in the distribution of the median nerve in the right hand. Percussion elicits paresthesias at the wrist (Tinel sign). An electromyogram shows a mild right median neuropathy at the wrist, with no evidence of cervical radiculopathy. Which of the following is the most appropriate initial step in treatment? A Corticosteroid injection into the carpal tunnel B Gabapentin C Surgical referral for carpal tunnel release D Wrist splints

D Wrist splinting is an effective, conservative treatment of carpal tunnel syndrome in patients with mild symptoms. This patient should be treated with wrist splints. She reports symptoms typical of carpal tunnel syndrome. The electromyogram (EMG) confirms the presence of a mild median neuropathy at the wrist. Given the mild symptoms and the presence of only mild changes on the EMG, a trial of conservative therapy is most appropriate. Conservative treatment approaches offer advantages over surgical treatment in terms of decreased short-term morbidity and surgical risk; also, the adverse effects of drug therapy may be avoided if nondrug modalities are used and prove to be beneficial. Conservative therapies include wrist splints, physical therapy, ergonomic adjustments, and corticosteroid injections. The role of occupational and recreational activities using the hands is highly variable, but ergonomic education and modification can be considered if thought to be relevant for individual patients. For this patient with mild signs and symptoms of carpal tunnel syndrome, a trial of conservative therapy using wrist splints is indicated. A recently reported randomized controlled trial, which identified workers through a carpal tunnel syndrome surveillance protocol, assigned them to either the control group, who received ergonomic education, or the treatment group, who participated in a 6-week nocturnal splinting trial. Participants in the splinting group showed greater improvement than the control group, and this benefit was still present at 1 year. The initial response rate to corticosteroid injections at or near the wrist is 70%. The duration of response is quite variable, however, and patients often require repeat injections or, ultimately, carpal tunnel surgery. Patients with mild to moderate symptoms who do not respond to wrist splinting should be considered for corticosteroid injection. Gabapentin and other medications used to treat neuropathic pain are typically not indicated in carpal tunnel syndrome. Carpal tunnel surgery should be considered in patients who have not responded to conservative therapy and in patients with moderate to severe signs or symptoms, unless there is a contraindication to surgery. A recent systematic review of the different surgical treatment options for carpal tunnel syndrome failed to reveal improved outcomes in open versus endoscopic carpal tunnel release.

Destruction of prefrontal cortex results in:

Deficits in concentration, orientation, judgement, abstracting ability, problem solving ability. Inappropriate behavior

OVerflow incontinence, Causes

DM --> Neurogenic bladder (LMN lesion) Men w BPH, obstruction - look for nocturnal wetting & large PVR > 100mL. Rx - Bethanachol (M agonists), alpha blockers (decrease sphincter resistance)

other areas of neuronal loss in Parkinson's

DM of X, olfactory (usually first), locus coreulus, temporal mesocortex.

Hypoglycemia induced generalized seizures

DM pt dx: finger stick blood glucose is low tx: IV dextrose

focal compression neuropathies

DM pts are more susceptible to these. such as median nerve, ulnar nerve, and peroneal nerve.

circuit controlling micturition

DMF lobe of M region of pontine micturition center-->decreased urethral pressure followed by increased contraction of detrussor Lateral region of PMC-->contraction of urethral sphincter leading to storage Thus damage to PMC leads to loss of inhbitor control over spinal reflexes and automatic micturition when bladder is distended. Spinal bulbar circuit= urinary bladder-->PMC-->PNS Spinal segmental reflex arc=detrussor-->onuf's nucleus in sarcal SC-->pudendal nerve to external sphincter (micturition reflex)

Pick disease

Degenerative disease of frontal and temporal cortex, spares parietal and occipital lobes. Aggregates of TAU protein in neurons of cortex; behavioral and language symptoms arise early and progress to dementia.

Progressive Supranuclear Palsy

Degenerative disorder affecting the brain & looks almost EXACTLY like Parkinsons, but lacks: 1. Tremor 2. Opthalmoplegia

Lumbar spine stenosis is most commonly caused by ___.

Degenerative joint disease (DJD) - pain relief flexion of the spine - spinal MRI

pergolide, pramipexole, ropinerole, rotigotine

Da agonists used in PD SE: nausea, orthostatic hypotension, psychosis, hallucinations, dyskinesia used for treating wearing off effect of L-dopa, or can be used earlier in disease treatment and used in dyskinesias from L-dopa.

Internuclear opthalmoplegia (aka MLF syndrome)

Damage to MLF interneurons between abducens and oculomotor nuclei. ADDUCTION of affected eye impaired, but CAN accomodate. Nystagmus in UNaffected eye TOWARDS AFFECTED EYE Often in multiple sclerosis patients

transcortical aphasia

Damage to areas providing input to language region. 3 types: 1) motor: non-fluent, good comprehension 2) sensory: fluent, poor comprehension 3) mixed: bad comprehension and nonfluent. Repetition okay.

How does glycogen debranching deficiency differ from Von Gierkes?

Debranching has normal lactic acid and uric acid levels, but elevated liver transaminases!!

decerebrate rigidity

Decorticate rigidity will change to this when lesion expands downward to include rubrospinal tract (in addition to midbrain and CSTr).

Language deficits in Alzheimer's

Decreased fluency, dysnomia, and transcortical sensory aphasia

Optic nerve and chiasm are derived from what part of the brain?

Diencephalon

Damage to FEF causes:

Difficulty making voluntary eye movements towards the side CONTRALATERAL to lesion (for a limited time. temporary problem) Can still have reflexive movement through Tectospinal Tract. "eyes stick to the lesion"

Parkinsonism + early dementia, visual hallucinations, sensitive to Anti-Dopamine

Diffuse Lewy Body Disease

Pupillary findings in ICH

Dilated pupils - Putamen Pinpoint pupils - Pons Poorly reactive pupils - Thalamus

Encephalitis lethargica

Disturbed eye movements, unremetting parkinsonism in the setting of fevers, lethargy and HA

Age < 50, + stroke. What do you look for?

Do workup!! It's odd... Vasculitis Hypercoagulable state (Factor V, Protein C, S, APL, VDRL/FTA-abs, etc) Thrombophelia Use of cocaine

Right-sided weakness and speech difficulty, unable to write and repeat. where is the lesion

Dominant Frontal Lobe

RLS treatment

Dopamine agonists: pamiprexole and ropinerole

Finger Abduction

Dorsal Interossei Ulnar T1

Location of lesion in Parinaud syndrome

Dorsal midbrain in the region of the superior colliculus

Parinaud syndrome

Dorsal midbrain lesion involving superior colliculus. Can't look up ("doll's eye") with Argylle-Robertson pupil (AR you have the AR: Accommodation Reflex present, pupillary reflex absent). (Remember: You'd look paranoid with Parinaud's)

Wernicke-Korsakoff syndrome is a result of the destruction of which thalamic nucleus?

Dorsomedial

Keratoconjunctivitis sicca

Dry eye. Look for AI causes or meds (anti-cholinergics, anti-histamines) Rx - artificial tear / lubricating ointments

Morbidity in Subarachnoid Hemorrhage

Due to Ruptured saccular aneurysms Rebleeding in first 24hrs Vasospasm after 3 days. (arterial narrowing at base of brain due to degradation of blood and its metabolites and can lead to cerebral infarction (Avoid this w/ NIMODIPINE)

Entacapone side effects

Dyskinesia, hallucinatsion, confusion, nausea, orthostatic hypotension

Neuro 21 A 74-year-old man is brought to the emergency department by ambulance 1 hour after he had an acute witnessed onset of aphasia and right hemiparesis. He has a history of hypertension and atrial fibrillation. His current medications are hydrochlorothiazide, metoprolol, and warfarin. On physical examination, blood pressure is 178/94 mm Hg and pulse rate is 80/min and irregular. Neurologic examination confirms nonfluent aphasia, a right pronator drift, a right leg drift, and an extensor plantar response on the right. Results of laboratory studies performed within 1 hour of his arrival at the emergency department show an INR of 1.5. An electrocardiogram obtained on the patient's arrival at the emergency department confirms atrial fibrillation. A CT scan of head obtained within 1 hour of his arrival reveals no early ischemic changes. Which of the following is the best treatment? A Aspirin B Continuous intravenous heparin C Intra-arterial recombinant tissue plasminogen activator (rtPA) D Intravenous labetalol E Intravenous rtPA

E A patient with acute ischemic stroke who is taking warfarin but who has a subtherapeutic INR (≤1.7) is still eligible to receive thrombolysis with intravenous recombinant tissue plasminogen activator within the recommended window of 3 hours from stroke onset. This patient should receive intravenous recombinant tissue plasminogen activator (rtPA). He has clinical symptoms and signs and radiologic evidence of an acute left hemispheric stroke. The probable mechanism of stroke is a cardioembolism, given his history of atrial fibrillation and his subtherapeutic INR. He was brought to the emergency department within 1 hour of the witnessed onset of stroke symptoms, and his evaluation is completed 1 hour later. He does not appear to have any clinical, radiologic, or laboratory contraindication to receiving the preferred treatment of intravenous rtPA, and he can receive it within the recommended window of 3 hours from stroke onset. The standard dose is 0.9 mg/kg. Although he is on warfarin, rtPA is not contraindicated because his INR is 1.5; an INR less than or equal to 1.7 is required for rtPA administration in a patient on anticoagulation. Aspirin is indicated for acute ischemic stroke in patients who are not eligible for rtPA, as this patient is. For patients with acute stroke who are eligible for thrombolysis, aspirin should be withheld in the emergency department and for 24 hours after rtPA administration. Although long-term anticoagulation is an effective treatment for prevention of cardioembolic stroke in patients with atrial fibrillation, acute anticoagulation with heparin has not been shown to be beneficial in patients with acute ischemic stroke. Intra-arterial administration of rtPA is indicated for selected patients with acute stroke who have major intracranial artery occlusion within 6 hours of symptom onset and who are not otherwise candidates for intravenous administration of rtPA. This patient was eligible for intravenous administration of rtPA within 3 hours of stroke onset. The latter treatment takes precedence. Elevated blood pressure is common at the time of initial stroke presentation, even among patients without chronic hypertension. Rapid lowering of blood pressure may further impair cerebral blood flow and worsen the ischemic injury. Elevated blood pressure often will resolve spontaneously or improve gradually during the first few days after a stroke. The threshold for acute blood pressure lowering in patients with acute stroke who are eligible for thrombolysis is 185/110 mm Hg. In such a setting, preferred agents include intravenous infusions of labetalol or nicardipine. Because this patient's blood pressure is already below that threshold, there is no indication for intravenous use of labetalol at this time.

Most consistent neuroimaging finding in schizophrenia? OCD? PTSD?

Enlargement of Lateral Cerebral Ventricles. Structural Anomalies in orbital front cortex Decreased hippocampal volumes.

Neuro 7 A 50-year-old woman is evaluated in the emergency department after having a witnessed generalized tonic-clonic seizure. Her husband reports hearing her fall and finding her on the kitchen floor convulsing. The event lasted 2 minutes and stopped spontaneously. The patient has never experienced a similar episode, has no personal history of head trauma or meningitis, and has no family history of a seizure disorder. She has a history of hypertension treated with hydrochlorothiazide. On examination, the patient is lethargic but cooperative. She is afebrile, with a blood pressure of 146/80 mm Hg and a pulse rate of 70/min. She has mild weakness of the left face and arm that resolves over the next 3 hours. Results of laboratory studies, including measurement of plasma glucose level, are normal. An MRI of the brain shows chronic small-vessel ischemic disease but no acute abnormalities. Which of the following findings predicts a greater risk of a future seizure in this patient? A Age B Hypertension C Presence of secondary generalization D Small-vessel ischemic changes on an MRI of the brain E Todd paralysis

E After a single unprovoked seizure, a greater risk of recurrence is predicted if the event was a partial seizure or if the patient has Todd paralysis, status epilepticus on presentation, an age greater than 65 years, or abnormal findings on neurologic examination. The presence of Todd paralysis predicts a greater risk of future seizures in this patient. After a single unprovoked seizure, recurrence risk in the subsequent 2 years has been reported to be 30% to 40%. Many historical or examination findings predict a higher risk of recurrence, including presentation in status epilepticus, age greater than 65 years, known underlying neurologic disorder(s) or structural lesion(s), and Todd paralysis. Partial-onset seizures are also more likely to recur, perhaps because of the increased likelihood of there being an underlying causative structural lesion. Todd paralysis is a transient unilateral or focal weakness often seen after partial seizures, with or without secondary generalization. Todd paralysis or other focal abnormalities on the neurologic examination predict a greater risk of a future seizure. Patients with an electroencephalogram or head imaging scan showing a potentially epileptogenic lesion are also in the higher-risk category. Elderly patients have been shown to have a higher risk of recurrence after a first seizure compared with younger adults. This 50-year-old woman would not be in a higher risk category on the basis of age alone. Cerebrovascular disease, including prior stroke and intracranial hemorrhage, is a commonly identified cause of epilepsy in older adults. However, the presence of hypertension and other cerebrovascular risk factors does not predict a greater risk of future seizures. The presence or absence of secondary generalization does not affect the rate of future events and so is not relevant for determining this patient's risk. The relatively nonspecific finding of small-vessel ischemic disease on an MRI of the brain is not significantly correlated with seizure risk. More concerning vascular lesions include acute or chronic stroke or hemorrhagic lesions involving cortical structures.

beta waves

EEG waves of low amplitude and high frequency seen in frontal regions in awake state. are 14-30 hz.

theta waves

EEG waves seen during drowsiness and sleep. are 4-7 hz

alpha waves

EEG waves seen in posterior region in relaxed awake state w/ eyes closed are 8-13 hz.

Juvenille myclonic epilepsy

EEG=4-6 Hz polyspike and wave. Tx: valprote, lamotrigene, Keppra. will have myoclonis, absence, generalized tonic-clonic w/ early morning seizures. can be brought on by going without sleep.

M Gravis - how to diagnosis

EMG & Ach receptor Ab + (Edrophonium Tensilon test is less specific than these 2) NExt step? CT - check for thymoma, esp if < age 60. Could be easy fix.

multiple muscle weakness in a limb w/ relfex loss

EMG/NCS

How S Mansoni invades the nervous system

Eggs in the valveless veins of Baston, drains the intestines and lumbro-sarcal cord, granulomas develop around the spinal cord

MC location of ulnar nerve entrapment

Elbow, where the ulnar nerve lies at the medial epicondylar grove

Entropion vs Ectropion

Entropion - eyelid bent inwards Ectropion - eyelid bent outwards

Lewy Bodies

Eosinohilic intracytoplasmic inclusions = alpha synuclein protein 1. Lewy Body Dementia 2. Parkinsons

NO midline shift / NO cross of suture lines

Epidural hematoma (but can cross falx above). Lens shaped.

How do you fix BPPV?

Eply manuever - canalith repositioning manuever (Diagnose it with Dix Hallpike manuever - turn head back and forth then lay them supine and vertigo/nystagmus is towards affected ear)

B/L action tremor of the hands, usually w/o leg involvement, possible isolated head tremor w/o dystonia, no neuro signs, relieved by alcohol in many case.

Essential tremor

Bilateral action tremor of hands, w/out legs, relieved w/ alcohol in some

Essential tremor Tx: Propranolol. Alt: Primidone and Topiramate

Treatment options for absence seizures? Treatment for myoclonus?

Ethosuximide or Valproic Acid. Sodium Valproic Acid and Clonazepam.

absence seizure treatment

Ethosuximide or valproic acid

Conductive Types of Hearing Loss

External canal - cerumen, Otitis Externa, bony outgrowths (exostoses) Tympanic membrane perforation - trauma (direct or indirect), middle ear infxn Middle ear - Otitis media, allergic rhinitis, Ostosclerosis (bony fusion of stapes to oval window)

Gamma axons

Gamma motor neurons (innervate intrafusal fibers)

Ankle Plantar Felxion

Gastrocnemius, Soleus Sciatic S1, S2 Ankle Reflex

Motor manifestation of neurosyphilis

General paresis

Infantile Spasms, presentation and rx

Generalized epilepsy ~ 0-6months. [Muscular jerks of head, trunk and extremities in clusters] Arrest in psychomotor devpt from the 1st seizure onwards. + Mental retardation. + EEG - Abnormal hypsarrhythmia btwn episodes + Fam Hx Rx - ACTH, prednisone, clonazepam, valproic acid. (Help w spasm but not LT prognosis)

Seizures, LOC and incontinence... think

Generalized tonic-clonic seizures likely.

agraphia, acalculia, right-left confusion, finger agnosia

Gerstmann's Syndrome Inferior Parietal Lobe (Anglular Gyrus) in dominant hemisphere

astrocytes

Glia (support cells): Provide support by creating a matrix that provides nourishment; makes up BBB; reactive gliosis after injury--GFAP is a marker for this. Origin: neuroectoderm

CT/MRI showing butterfly appearance w/ central necrosis, heterogenous, serpiginous contrast enhancement

Glioblastoma Multiforme

Other name for grade 4 astrocytoma

Glioblastoma multiforme

5 Most common brain tumors:

Glioblastoma multiforme Meningioma Schwannoma Ependyoma Medulloblastoma (can metastasize through CSF tracts)

Most common glioma?

Glioblastoma multiforme (55% cases, malignant, rapidly fatal astrocytic tumor. common on frontal and temporal lobes)

impaired fluency, repetition, and comprehension

Global Aphasia large dominant hemispheric lesions

Glucocorticoid Ind Myopathy vs Statin induced Myopathy

Gluc induced: - muscle weakness but NO PAIN/TTP - normal ESR and CK Statin induced: - pain & TTP but +/- weakness - high CK

Progressive proximal muscle weakness & atrophy w/o pain or tenderness. LE muscles are more involved. ESR and CK normal. Dx the myopathy.

Glucocorticoid induced myopathy

Painless proximal muscle weakness, more prominent in the lower extremities. no muscle inflammation or tenderness, CK and ESR are normal

Glucocorticoid-induced myopathy

Tumor from von Hippel-Lindau syndrome

Hemangioblastoma

Lesion of the subthalamic nucleus causes

Hemiballismus

Hemorrhages are seen as [hyper/hypo]dense areas on CT scan, while infarcts are [hyper/hypo]dense parenchymal areas on CT scan.

Hemorrhages = Hyperdense Infarctions = Hypodense

Tay-Sachs deficiency

Hexosaminidase A

PCA damage

Homonymous Hemianopsia Memory issues Dyslexia

Hordeolum vs Chalazion

Hordeolum (Stye) - acute, PAINFUL localized swelling of the eyelid (usu Staph Aureus). - Warm compress. I&D if no change in 48 hours. Chalazion - chronic, STERILE, PAINLESS inflamm from blocked Meiobomian Gland. - Rx - warm compress; but may need I & Curettage. Biopsy if recurrent. Hint: Hordeolum = oh HOLY crap (painful, acute) Chalazion = chronic

Stye also known as? Abscess of eyelid. PAINFUL! Treatment?

Hordeolum. Warm Compress

ipsilateral miosis, ptosis, anhydrosis

Horner's Syndrome- sympathetic interruption

"degeneration of striatial gaba neurons"

Huntington's dz

Thrombotic Stroke

Hx of Atherosclerosis. (medium/large arteries). Thrombus forms leading to ischemia. - bifurcation of carotid - MCA (cerebral cortex)

Hydrocephalus vs Hydranencephaly

Hydrocephalus = blockage of cerebral aqueduct, excess fluid in ventricles and subarachnoid space Hydranencephaly = Bilateral hemispheric infarction secondary to occlusion of carotid arteries. Hemispheres replaced by extremely large ventricles

Light causes photoreceptors to _____.

Hyperpolarize

Cushings reflex

Hypertension BRADYcardia Decreased Respirations *findings assoc w high ICP that indicate impending brainstem compression!!

AV nickening & tortuosity of arteries

Hypertensive Retinopathy

Hyperventilation affect on seizures

Hyperventilation - accents Absence seizure only (note, no post-ictal state, but maybe don't remember! LOC)

Muscle pain, cramps, weakness in the proximal muscles, delayed DTR and myoedema Occasional rhabdo ESR normal, CK INC Dx. the myopathy.

Hypothyroid myopathy

ischemic stroke

Hypoxia after 5min causes irreversible damage. Hippocampus most vulnerable. Glial scar forms weeks later. tPA must be given bw 3-4.5hr

Sx for emergent craniotomy

I.e. - setting of Epidural hematoma 1. GCS <8 2. sx of ICP 3. hemiparesis 4. cerebellar sx

Basilar A. damage (ex: Vertebrobasilar --> Brainstem (spec Midbrain))

I/L motor sensory face (& CN involv) C/L motor sensory UE, LE, or ataxia Coma, locked-in 4 D's - Diplopia, dizziness, dysphagia, dysarthria (a mess)

Inclusion body myositis

IBM-inflammatory myopathy that occurs in elderly and leads to progressive weakness proximally dx: muscle biopsy shows inflammation + rimmed vacuoles and amyloid staining no tx

focal neuropathies associated w/ DM

III, IV, VI cranial nerve neuropathies. focal compression neuropathies (carpal tunnel, ulnar nerve, meralgia paresthetica). radiculopathy, usually thoracic leading to non-radicular pain, truncal sensory loss, focal weakness of abdominal wall. CIDP can also result.

ipsilateral adduction gaze palsy, contralateral abduction nystagmus

INO- MLF disruption

___ DEC the risk of embolic events in pts with native valve infective endocarditis.

IV abx - Sx is considered in pts with significant valve dysfunction, persistent/difficult to treat infection, or recurrent embolism

What is the monotherapy for acute migraine attacks, patients with N/V?

IV antiemetics (chlorpromazine, prochlorperazine, metoclopramide)

carotid dissection rx acutely

IV heparin

treatment for vertebral artery dissection

IV heparin

holocranial headache, vision changes, pulsatile tinnitus and papilledema w/ left lateral rectus palsy.

Idiopathic Intracranial HTN, Check lumbar puncture w/ an opening pressure measurement.

Decerebrate vs Decorticate Injury

If above the red nucleus (in midbrain) --> Decerebrate Injury (i.e. cerebellum, thalamus, etc) If below the red nucleus --> Decorticate injury (i.e. pons)

Hip Flexion

Iliopsoas Femoral L1, L2

What is the hallmark pathophysiology of normal pressure hydrocephalus?

Impaired CSF absorption.

astereognosis

Inability to identify object correctly by touch. Occurs in sensory cortex lesions.

disdiadochokinesia

Inability to perform rapid alternating movements. Sign of cerebellar damage--probably to lateral zone since it affects distal limbs. Other signs: ipsilateral limb ataxia, intention tremor, falling to SIDE OF LESION. (Cerebellar problems are always SAME side.)

Long-standing liver disease encephalopathy neuron changes

Increase in Alzheimer's type II astrocytes; large cells

Distinctive feature Lambert Eaton syndrome

Increase in strength briefly after repeated muscle activation

People with Huntington's show increased ___ and decreased ____ levels.

Increased Somatostatin Decreased Substance P

Common trigger for symptoms of demyelinating disease

Increased temperature

Heatstroke treatment

Induction of evaporative cooling to reverse hyperthermia

Scleritis

Infalmm of the sclera assoc w systemic immunological dz (i.e. RA) SEVERE, deep eye pain, redness and pain on palpatoion of eye. Rx - topical or systemic corticosteroids

Auditory: Colliculi and geniculate nucleus?

Inferior colliculi and medial geniculate nucleus

Construction apraxia can be seen in what lesion?

Inferior parietal lobe of NON-dominant hemisphere

Lesion of what area of the brain results in left/right confusion?

Inferior region of parietal lobe in the dominant hemisphere (usually right). Left sided neglect is more common

Episcleritis

Inflamm. of vessels in the lining just beneath the conjunctiva. REdness, irritation (AI involv perhaps), w blotchy areas of redness. Dull ache. Rx - Self limited.

Blepharitis

Inflammation of the eyelid assoc w Staph infxn - crusting of eyelid that sticks to lashes Rx - lid scrubs & warm crompresses.

another name for Guillan Barre

Inflammatory Demyelinatory Polyneuropathy

Muscle pain, tenderness & proximal muscle weakness Skin rash and inflammatory arthritis may be present ESR and CK INC Dx the myopathy.

Inflammatory myopathy

CN V1

Innervates dura above tentorium cerebelli. (Dura below is innervated by cervical nerves, mostly C2.) Referred pain to face/forehead with headache.

Bilateral internuclear opthalmoplegia

MS INO 2/2 lesion in medial longitudinal fasciculus (MLF) responsible for carrying signal from the abducens nucleus to the abducting eye to the contralateral oculmotor nucleus in the adducting eye dx: MRI for suspected MS

Keratitis vs Conjunctivitis

Keratitis - inflamm of acutal cornea!! (viral - HSV or VZV - vesicles, opacification) Conjunctivitis - inflamm of white area surrounding cornea (MC - viral after URI sx --> pink eye)

Neuroblastoma

Kid w palpable flank mass (can be midline) - from neural crest cells - can appear in abdomen (MC) or ANYWHERE along sympathetic chain!! - high HVA and MVA (~Pheo) but no sx like Pheo. - 70% have spread at time of diagnosis (bones, skull, BM, etc) Imaging - Xray and CT - calcifications and hemorrhage

Poliomyelitis

LMN damage due to ANTERIOR horn destruction - asymmetric motor loss - flaccid paralysis - areflexia - sensation intact - bulbar involv (CN 9,10) --> can cause resp/cardio invovl. Avoid by vaccinating!!!

Polio & Werdnig Hoffman Dz

LMN lesion only --> flaccid paralysis anterior horn destruction

polio

LMN lesions of anterior horn--> flaccid paralysis. CSF shows elevated WBC and protein.

Ia and Ib axons

Largest diameter, mylenated axons for proprioception. Ia innervates muscle spindle and Ib GTOs.

PICA lesion

Lateral medullary syndrome. Possible ipsilateral sx: loss of temperature sensation on face, partial paralysis of the soft palate, larynx, and pharynx, and ataxia

Blood supply to internal capsule

Lateral striate arteries (MCA) and anterior choroidal artery

Arteries associated with stroke

Lateral striate arteries (off middle cerebral artery) - internal capsule, caudate, globus pallidus, putamen

AE of Parkinson Rx, Later in life

Ldopa/Carbidopa - get dyskinesia (involuntary, choreic movts) - irreversible. - Ldopa is very dose dependent: on/off phenomenon of sx (fluctuation throughout the day). *consider starting Ldopa, Carbidopa later.

Wallenberg syndrome

Lesion of the structures of the lateral medulla including: nucleus and descending tract of the 5th nerve, nucleus ambiguus, lateral spinothalamic tract, inferior cerebellar peduncle, descending sympathetic fibers, vagus, and glossopharyngeal nerves

Lewy vs Hirano bodies?

Lewy = Parkinson's Hirano = Alzheimer's (found in hippocampus)

___ is characterized by fluctuating cognitive impairment, recurrent visual hallucinations, and motor features of Parkinsonism.

Lewy body dementia

Utricle and saccule sense

Linear acceleration and gravity. Hair cells in otolithic membrane; movement TOWARDS kinocilium = depolarize

Side effect of amantadine? Side effect of COMT inhibitors? (ex encaptone)

Livido Reticularis (mottled vascular pattern of lower extremities) Choreiform dyskinesia

Subconj Hemorrhage - eye changes

Localized patch of extravasated blood under conjunctiva. Look for trauma or Valsalva (coughing, sneezing, vomitting) to increase pressure.

Location of Blind spots (normally)

Located at the optic nerve head aka optic disk.

What is apraxia?

Loss of ability to carry out planned/purposeful movements? (Person understands that the examiner says brush your head but can move his hand in a purposeful way as to brush his hair... the signal to muscle doesnt go through!)

Perinaud syndrome

Loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergence retraction nystagmus

The treatment of choice for agitation in the elderly is ___.

Low-dose haloperidol

paraneoplastic cerebellar degeneration cancer types

Lung, ovarian, and lymphoma

controls activation of micturition

M Region of the Pons

M Gravis vs Lamb. Eaton vs GB

M gravis - normal reflexes Lamb Eaton - hyorreflexia GB - areflexia ALS - Hyperreflexia

M Gravis vs Muscle Inflammation

M gravis is a nerve issue --> so CPK normal. Muscle inflamm --> CPK is elevated.

Lacunar Stroke

MC presentation: limited neurological deficits (MCC - HTN) Small vessel. Thickening of small vessels leads to ischemia (not thrombus) Small vessels from MCA --> lenticulostriate a. Subcortical structures: - basal ganglia, thalamus, IC, and brainstem.

Pilocytic astrocytoma

MC primary brain tumor in kids low grade astrocytoma occurs in cerebellum: progressive imbalance, HA, vomiting, difficulty walking, HA worse in AM and wake up from sleep biopsy: spindle shaped cells + rosenthal fibers tx: complete surgical resection

MCA damage

MCA can cause "CHANGes" C - C/L motor sensory loss in Face/UE H - Homonymous Hemianopsia A - Aphasia (if Dom hemisphere - Left) N - Neglect (if Non-dom hemisphere - Right) G - Gaze preference TOWARDS

Anterior Spinal Artery lesion

MEDIAL medullary syndrome. Lesion on R may show R tongue deviation, L loss of proprioception (ML), and L hemiparesis (corticospinal tract).

Metanephros Mesonephros Paramesonephros

METAnephros - forms renal parenchyma (ex - Wilms Tumor) MESOnephros - male internal structures PARAMESOnephros - female internal structures Neural crest cells - sympathetic chains & adrenal medulla (ex - Neuroblastoma)

MLF

MR problem - ADDUCTION issue on I/L side. w/ compensation by abducting eye (horizontal nystagmus)

workup of essential tremor and treatment

MRI of brain and spine - PET shows increased uptake in the thalamus in ET propanolol or primidone head tremor is very rare in PD but can be seen in ET)

Imaging of choice for CST

MRI w gadolinium

Suspect neurofibromatosis type II in a young patient with acoustic neuroma and multiple cafe-au-lait spots. Best dx method for acoustic neuroma.

MRI with gadolinium - acoustic neuroma (gradual developing tinnitus and hearing loss)

Any lesion/structural issue in the brain or SC

MRI.

One of the few conditions to cause BTL trigeminal neuralgia

MS

Suspect ___ in a young female with bilateral trigeminal neuralgia.

MS

Trihexyphenidyl

Medication for Parkinson's disease that has anticholinergic effects, like benztropine

Three most common brain tumors before puberty

Medulloblastoma, ependymoma, and cerebellar gliomas

Metastatic malignancy that causes bleeds in the brain

Melanoma and choriocarcinoma

Cluster headache

Men>women, pain in the eyes for the temporal area, autonomic phenomena

Recurrent episodes of vertigo Preceded by ear fullness/pain Unilateral hearing loss & tinnitus

Meniere's disease/syndrome

episodic vertigo with n/v, progressive hearing loss, tinnitus, ear fullness

Meniere's- increase in endolymphatic volume

Myelomeningocele

Meninges and SC protruding

Meningocele

Meninges protruding

Tumor that causes hyper ostosis of the school

Meningioma

Wilms tumor arises from?

Metanephros (embryologic precurosr of renal parenchyma)

HIV and CMV brain infection pathology

Microglial nodules

Brain anatomy

Midbrain Pons Medulla

Differentiating between Neurocutaneous D/O: NF Tuberous Sclerosis Sturge Weber

NF: cafe au lait + axillary freckling +/- schwannomas & cataracts Tuberous Sclerosis: ash leaf spots, sebaceous adenoma, & shagreen patch. Sturge Weber - port wine stain aka nevus flemmeus (cavernous hemangiomas)

Opposite side somatosensory and motor deficits (face, arm, leg), Conjugate eye deviation toward side of infarct, Homonymous hemianopia, Aphasia (Dominant hemisphere), Hemineglect (nondominant hemisphere).

Middle Cerebral artery occlusion

unilateral throbbing headache w/ nausea, photo/phonophobia

Migraine Headache Triptan- abortive, CI coronary disease

Single nerve muscle weakness w/ numbness, tingling, or pain

Mononeuropathy Entrapment EMG/NCS

dysfunction of multiple peripheral nerves in succession, w/ pain

Mononeuropathy Multiplex- systemic vasculitis, metabolic, rheumatologic EMG/NCS

glioblastoma multiforme

Most common adult brain tumor, an astrocytoma found in cerebral hemispheres. Malignant. GFAP marker.

astrocytoma

Most common primary brain tumor in children. Occurs in cerebellum. GFAP marker.

anterior communicating a.

Most common site of saccular (berry) aneurysm. Causes visual field defects.

Parkinsonism + autonomic dysfunction, myoclonus, vocal cord paralysis

Multiple System Atrophy

Von Hippel-Lindau presentation

Multiple angiomatosis of the retina and cysts of the kidney and pancreas

Causes of symptomatic trigeminal around

Multiple sclerosis, basilar artery aneurysms, acoustic schwannomas, posterior fossa meningiomas

proximal symmetric weakness, can affect neck

Muscle Disorder- CK, EMG Myopathy, Muscular Dystrophy

Nerve often damaged fractures of the humorous

Musculocutaneous nerve

Motor manifestations of hypothyroidism

Myopathic weakness, delay in the relaxation phase of reflexes, cerebellar ataxia

NASCET trial

N. American Sx Carotid Endarterectomy Trial *Carotid endarterectomy if > 60% stenosis in symptomatic or > 70% in asymptomatic.

cafe au lait spots, axillary freckling, macrocephaly, feeding problems, short stature, learn disability

NF1

Resting tremor (4-6 Hx) that DEC with voluntary movt, usually involved legs & hands, facial involvement less common.

Parkinson's disease

Resting tremor (4-6 Hz) that decreases with voluntary movement. Involves legs & hands. Facial involvement less common.

Parkinson's disease

Lewy bodies

Parkinson's--eosinophilic inclusions in damaged substantia nigra cells.

corticobasal ganglionic degeneration

Parkinson-like syndrome. will have apraxia, cortical sensory impairments, alien-limb phenomenon, severe rigidity adn stimulus sensitive myoclonus.

Multiple system atrophy

Parkinsonism, autonomic dysfunction, widespread neurologic signs

"degen of DA neurons of sub nigra"

Parkinsons

Arms in Parkinsons vs NPH

Parkinsons - NO arm swing = "festinating gait" vs NPH - also gait issues but arm swing is normal

Resting tremor that decreases w/ voluntary movement, involves legs and hands

Parkinsons Tremor

multiple sclerosis

Plaques occur in the brain and spinal cord causing tremor, weakness, incoordination, paresthesia, and disturbances in vision and speech. Myelin loss in CNS fibers (oligodendrocytes). More common in women ~30.

Muscle pain & stiffness in the shoulder and pelvic girdles. Tenderness with DEC ROM at shoulder, neck and hip. Responds rapidly to glucocorticoids. INC ESR, normal CK Dx the myopathy.

Polymyalgia rheumatica

diffuse nerve muscle weakness, longest nerves first, sensory depression and depressed or absent reflexes

Polyneuropathy Demyeliniating-Charcot-Marie-Tooth, Guillain-Barre Axonal EMG/NCS

Millard-Gubler syndrome

Pons lesion causing ipsilateral loss of VI and VII with contralateral loss of CST

"Locked-in" syndrome is a result of a lesion in the ____.

Pons. Bilateral CS/CB affected, but CN III and IV spared, so communication via eye movements is possible.

conductive aphasia

Poor repetition but fluent speech. Damage to arcuate fasciculus.

Marcus-Gunn pupil

Poorer direct response than consensual response in the affected eye. The afferent pupil defect is a sign of optic nerve disease.

Ascending muscle weakness & Areflexia

Post resp/GI --> think Guillan barre!! * motor > sensory (but can have distal neuropathy. symmetric or asymmetric!!) - MOST IMPORTANT STEP: Spirometry --> need to get FVC (gs test) for ventilation.

Post traumatic brain injury and headache, confusion, amnesia, difficulty concentrating, vertigo, mood alteration, sleep disturbance, anxiety.

Postconcussive Syndrome

A few hours or days after mild TBI pt has HA, confusion, amnesia, difficulty concentrating, vertigo, mood alteration, sleep disturbance, and anxiety. Dx.

Postconcussive syndrome - resolve weeks to up to 6 months or more

Lesion in pure sensory strokes

Postereoventral nucleus of the lateral thalamus

Main blood supply to midbrain

Posterior cerebral artery

Contralateral hemianopia can be caused by damage to this vessel

Posterior cerebral artery (occipital lobe - visual cortex)

Artery aneurysm that causes third nerve palsy

Posterior communicating artery

Third nerve palsy caused by lesion to what artery?

Posterior communicating artery

One sided motor impairment, no sensory or cortical deficits, no visual problems

Posterior limb of internal capsule (lacunar infarct)

Treatment for Restless Leg Syndrome

Pramipexole or Gabapentin (alpha-2-delta calcium channel ligands)

Pre-septal vs Post-septal Cellulitis

Pre-septal: Peri-orbital Post-septal: Orbital Orbital also has: 1. PROPTOSIS 2. Opthalmoplegia (weakness, paralysis of eye m.) 3. Pain w EOM movt 4. Decreased visual acuity

Presentation 1) Areflexic weakness at level of lesion 2) Loss of pain/temp sensation in cape like distribution *Intact vibratory and proprioception

Syrinogmyelia

Primidone side effects

Precipitates acute intermittent porphyria, manifests as abdominal pain, neruologic and psychiatric abnormalities

Are preganglionic and postganglionic nerve cells myelinated?

Preganglionic - yes Post ganglionic - no

HIV, Ring enhancing periventricular lesion, EBV

Primary CNS Lymphoma

Behavioral changes, Rapid progression, myoclonus and/or seizures

Prio disease

Vitamin deficiency optic nerve injury

Probably B1, B12 and riboflavin

Function of arcuate nucleus of hypothalamus?

Produce inhibiting factors to stimular OR inhibit actions of hypothalamus (ie, prolactin-inhibiting factor/dopamine)

Parkinsonism + limited vertical gaze, neck regidity

Progressive Supranuclear Palsy

Ib fibers

Proprioception, Golgi tendon organs

Ia fibers

Proprioception, muscle spindles

CSF in uremic encephalopathy

Protein greater than 60

CN V3

This division of a CN innervates the external ear and tympanic memb.

Young, obese female w/ headache, normal brain imaging, elevated CSF pressure and papilledema

Pseudotumor cerebri. -Complication is BLINDNESS Tx: Weight reduction and Acetazolamide.

How does Congenital MG differer from Botulinism?

Pupils normal in MG aka you don't have the autonomic symptoms (constipation, drooling) in MG!! REMMBER MG DOES NOT HAVE AUTONOMIC SYMPTOMS AND DTRS are usually intact!!

Four lacunar stroke syndromes

Pure motor, sensory, ataxic hemiparesis, dysarthria - clumsy hand syndrome (no sensory)

Most common locations for a hypertensive hemmorhage?

Putamen and Thalamus.

Negri bodies are found in

Pyramidal cells of hippocampus and Purkinje cells of cerebellum (characteristic of rabies infection)

Knee Extension

Quadraceps Femoral L3, L4 Knee Reflex

What visual problem often accompanies Wernicke's asphasia?

Quadrantanopia

Frontal cortex damage

R or L --> get C/L motor deficits

"woman w/crawling tightness in calves, worse with sitting or lying in bed. better w/walking" dx tx

RLS Pramipexole-DA agonist

muscle weakness in nerves from a single nerve root, w/ radiating pain, tingling, depressed or absent reflex

Radiculopathy herniated disc, shingles EMG/NCS

Why lorazepam is a good choice for status epilepticus

Rapid acting but more slowly cleared from the body

Left temporal lobe lesion results in ...

Receptive aphasia

NMDA glutamate receptors

Receptor blocked with Mg. Requires tetanus to move Mg and cause an excitatory Ca influx. Long term potentiation.

Transcortical sensory aphasia

Reduction in the ability to understand complex linguistic structures

enkephalin

Released by inhibitory interneurons to block substance P. Endogenous analgesia system.

Neurogenic Claudication is relieved how?

Relieved while walking uphill (flexing spine) and sitting (flexing spine) Worse with walking downhill (extending spine) and standing (extending spine)

What are the most commonly found tumors in the cerebellopontine angle cistern?

Remember the acronym SAMEE: Schwannoma Arachnoid cyst Meningioma Ependymoma and Epidermoid

Listeria meningitis epi

Renal transplant, chronic renal disease and immunosuppressed

spinal trigeminal tract

Replaces Lissaur's tract. Carries fibers for pain, temp, touch for face via CN V, VII, IX, and X.

Chemoreceptors

Responsible for CO2/O2 regulation via lungs/respiration. 1. Central - responds to changes in pH and PCO2. (Not directly to PO2). 2. Peripheral - low PO2 (<60), high PCO2, and low pH stimulate carotid and aortic bodies.

Baroreceptors

Responsible for short term BP regulation via blood/heart. (LT regulation = RAAS) 1. Hypotension --> less afferent barorecept firing --> causes an increase in efferent SYMPathetic firing (and less PS) --> vasoconstriction. = Increase in HR, BP, & contractility. 2. Carotid massage = increased pressure on carotid sinus --> increase in afferent firing --> increases AV node refractory period = thus decreases HR. 3. Increased ICP (Cushing's rxn) --> causes arterioles to vasoconstrict --> cerebral ischemia nad increase in SYMP (hypertension) --> reflex baroreceptor-ind. Brady.

acute onset dystonia after taking antipsychotic

acute dystonic reaction tardive dyskinesia takes months to develop after taking an antidopaminergic drug

Swollen optic disk

Retinal V occlusion

Central Retinal V. Occlusion [thrombosis of retinal v)

Retinal hemorrhages, optic swelling, & cotton wool spots... Risk factors - coagulopathy, hyperviscocity, chronic glaucoma, atherosclerosis Rx: - If no macular edema or neovasc: conserv. rx - If edema: VEGF injection in eye *Note - pt may recover vision in the first 3 mo.

Delirium

Reversible, waxing and waning AMS due to underlying infxn/drug/dehydration/post-op/etc. Hallucinations are common (usu visual)

Complications of heat stroke

Rhabdo, renal failure, ARDS, and bleeding

Neiseria ppx

Rifampin (or CTX or cipro)

Hemi-neglect syndrome by ignoring left side of a space involves which part of brain?

Right (non-dominant) parietal lobe

Head turns left, cupula moves ____ and hair cells _____.

Right; depolarize

___ is currently approved for use in pts with ALS.

Riluzole - glutamate inhibitor

Most likely atypical to cause EPS (although EPS is unlikely with atypical)

Risperidone.

Subdural Hematoma

Rupture of bridging veins (Durable Bridge), crescent shaped, CONCAVE, (elderly, alcoholics, shaken baby, whiplash), midline shift, CANNOT cross falx/tentorium.

Status Epilepticus, Rx

Rx - IV Benzo (Lorazepam or Diazepam) + Loading Dose of Fosphenytoin + B1, Glucose, Naloxone (presumptively to treat potential causes)

Vasospasm in SAH, rx

Rx - Nifedipine

Level of radiculopathy with loss of ankle jerk

S1

Cauda Equina vs Spinal Cord Compression

SCC - motor and sensory loss, rectal tone loss, urinary retention, hyperreflexia, UMN Babinski --> can look very similar to Cauda Equina b/c bowel, bladder but look @ level of sx.... Ex - sensation loss below umbilicus r/o Cauda Equina b/c that involves peripheral nerve roots.

headache and confusion for past 3 days, seizure, recent hemoptysis, hyponatremia - diagnosis

SIADH - hemoptysis = small cell lung cancer - not decreased solute intake because then urine osmoality would be low (at 50) as body tries to recover all solutes

Lamotrigine

SJS rash

Cerebral Palsy

STATIC d/o of primarily motor deficits. Doesn't change in severity throughout life, just there. Look out for seizures, devpt delay & mental retardation Most important complication - Prematurity!!!

DNA mutations

STOP the Nonsense! (Nonsense - early stop codon) Frameshift - dysfxnl protein usually (note, missense - AA changes but its similar in structure so its okay)

posterior communicating a.

Saccular (berry) aneurysm here would cause CN III palsy.

Rx Meniere's Dz

Salt restriction & Diuretics Meclizine *Note, hearing loss can become permanent here

Lennox Gustaut

Seizures - many per day, often nocturnal ~ Ages 2-6 Mental retardation, behavior disorders, psychomotor devpt delay Usually treatment resistant

Creutzfeldt-Jakob disease EEG characteristics

Sharp triphasic synchronous discharges

Hoffman reflex

Sharply flick nail of distal phalanx of middle or index finger. Abnormal response is clawing of fingers and thumb indicating UMN problem.

Syncope: Triggers are cough, micturition, defecation

Situational syncope

C fibers

Slow pain and temperature (unmyelinated)

EEG of Alzheimer's disease

Slowing normal alpha activity (8 to 12 Hz)

What main artery supplies the thalamus?

Small, penetrating arteries from PCA.

What neurotransmitter is significantly reduced in Alzheimer's disease?

Somatostatin. You also see a loss of cholinergic neurons

Side effects levodpoa/carbidopa

Somnolence, confusion, hallucinations, and dyskinesias (later)

Weber test

Sound lateralized to impaired ear in conductive loss but to good ear in sensorineural loss.

weakness with LMN at and UMN below the lesion level, sensory loss below, increased reflexes below

Spinal Cord Lesion

Triad of fever, back pain, neurologic deficits w/ a hx of IV drug use, spinal procedure or distant infection like cellulitis or joint/bone

Spinal Epidural abscess Dx: MRI of spine Tx: Vanc and Ceftriaxone Aspiration/surgical decompression

Gradual worsening severe low back pain Pain worse in the recumbent position/at night Early signs: symmetric LE weakness, hypoactive, absent DTR Late signs: B/L Babinski reflex, DEC rectal tone, paraparesis/paraplegia with INC DTR, sensory loss.

Spinal cord compression - injury (MVA) - malignancy (lung, breast, prostate cancer, myeloma) - infection (epidural abscess)

Reflex Incontinence

Spinal cord injury, MS, DM, etc Patient cannot SENSE the need to urinate.

Vitamin D deficiency neurologic presentation

Spinocerebellar degeneration, polyneuropathy, and pigmentery retinopathy

When to intubate GB patients

Spirometry - check for FVC. A decline in FVC (esp to <20) = need to intubate

Sturge Weber

Sporadic!! Seen w little kids **Capillary angiomatosis of the PIA matter. 1. U/L Cavernous Hemangioma [Port wine stain aka Nevus Flemmus - Trigeminal distribution] 2. Seizures 3. Mental retardation 4. I/L glaucoma, I/L leptomeningeal angioma (brain changes), hemianopia. Skull Xrays after Age 2: gyriform, tramline intracranial calcifications Rx - remove brain hemisphere!!!

Prominent muscle pain/tenderness w/ or w/o weakness Rare rhabdomyolysis Normal ESR, CK INC Dx. the myopathy.

Statin-induced myopathy

Subclavian Steal Syndrome

Stenosis of Subclavian A proximal to Vertebral A. Exercise of L arm cause blood flow to reverse in the Vertebral system to supply BF to arm Sx - similar to Vertebrobasilar TIA + low pulse and pressure in L arm

Most common cause of drug induced myopathy

Steroids

variable fluency and comprehension

Subcortical Aphasia Left basal ganglia, thalamus

+ Midline shift / + cross of suture lines

Subdural hematoma (but cannot cross the falx b/c its below it). Crescent shaped.

Common consequence of ventriculoperitoneal shunt for normal pressure hydrocephalus

Subdural hematoma as the brain pulls away from its covering on the meninges

Acute Closed Angle Glaucoma

Sudden onset of PAIN, blurred vision, N/V. Pupil is fixed, dilated, NON-REACTIVE to light. *look for hx of dilated pupils - i.e. movie RX - IV acetazolamide can low IOP (also used for Idiopathic Intracranial HTN). Laser peripheral iridotomy = permanent cure.

Where is the ulnar nerve run

Superficially at the elbow at the ulnar groove

All sympathetic autonomic innervation to the head is through

Superior cervical ganglion (superior tarsal muscle, submandibular/sublingual glands, lacrimal gland, dilator of pupil, parotid gland)

Cochlear nerve projects to:

Superior olivary nucleus and lateral lemniscus (bilateral)

Cingulate Herniation

Supratentorial mass above Falx Cerebri causes brain UNDER the falx cerebri to be pushed to C/L side. No specific signs or sx. Can be seen w subdural hematoma

Meinere's disease

Swelling of membranous labyrinth due to too much endolymph (maybe). Vertigo, nystagmus, tinnitus, hearing loss. Feeling of fullness in affected ear

Marcus Gunn pupil

Swinging flashlight test: Shine in unaffected eye = constriction of both pupils; Shine in affected eye (optic nerve) = constriction of both pupils but much less than normal

Type of peripheral neuropathy commonly developed with chronic renal failure

Symmetric, distal, mixed sensorimotor neuropathy

Indications for carotid endarterectomy

Symptomatic stenosis of the internal carotid by more than 70%

Neuromyelitis optica

Symptoms of bilateral optic neuritis in association with transverse myelitis

Most common site of hypertensive hemorrhage

The putamen, internal capsule is adjacent and is almost always involved

Hypothalamic stroke...

Will see increase in hypothalamic temperature set point --> neurogenic Fever!

Atrophy of the lenticular nucleus?

Wilson's Disease.

Back pain w/ mets vs back pain w/ degenerative joint disease?

Worsens vs Improves with recumbency

Rett syndrome today

X-linked genetic disorder that only affects girls, male version fatal

Inheritance pattern of Duchenne and Becker muscular dystrophy? Myotonic dystrophy?

X-linked recessive AD

Xanthochromia in CSF

Yellow CSF = RBC's breakdown - implies blood is in CSF for several hours and not due to trauma. Usu 6-24 hrs only GOLD STANDARD for diagnosis!!

spinal shock

acute loss of sympathetic innervation leads to decreased vascular resistance and hypotension, but with intact vagal tone can lead to a paradoxical bradycardia loss of reflexes and rectal tone also suggests presence of spinal shock

hereditary spastic parapariesis

a group of inherited diseases whose main feature is progressive stiffness and contraction (spasticity) in the lower limbs, as a result of damage to or dysfunction of the pyramidal tract. is not a form of cerebral palsy even though it physically may appear and behave much the same as, for example, spastic diplegia. The condition sometimes also affects the optic nerve and retina of the eye, causes cataracts, ataxia (lack of muscle coordination), epilepsy, cognitive impairment, peripheral neuropathy, and deafness. HSP is caused by defects in the mechanisms that transport proteins and other substances through the cell. can sometimes treat w/ antispastic meds like baclofen.

HTLV-1 (tropical spastic paraparesis)

a medical condition that causes weakness, muscle spasms, and sensory disturbance by a virus resulting in paraparesis, weakness of the legs. As the name suggests, it is most common in tropical regions, including the Caribbean. presents w/: Leg instability Urinary dysfunction. Bowel dysfunction Back pain Erectile problems Psoriasis Polymyositis Patients with TSP may also exhibit uveitis (inflammation of the uveal tract of the eye), arthritis (inflammation of one or more joints), pulmonary lymphocytic alveolitis (inflammation of the lung tissues), polymyositis (an inflammatory muscle disease), keratoconjunctivitis sicca (persistent dryness of the cornea and conjunctiva), and infectious dermatitis (inflammation of the skin). tx: steroids, but only helps a little while.

Complex motor tic

a rapid, recurrent stereotyped movement simple-one muscle group complex-coordinated involvement of multiple muscle groups

Acute viral labyrinthitis

acute onset, severe vertigo + hearing loss constant (first few days) then periodic and worsened with sudden mvmt audiogram-sensorineural hearing loss or electronystagmogram shows caloric hypofunctioning MRI to rule out central cause tx: supportive + antiemetics, benzos or meclizine

how to bring on absence seizure

hyperventilation see 3hz spike and wave unless atypical absence then see slow spike and wave <2.5 hz

aneurysm associated with PCKD

acomm

Acute vestibular toxicity 2/2 to gentamicin (aminoglycoside)

bilateral vestibular toxicity hearing loss, oscillopsia (bouncy and blurry vision) and difficulty walking eg. gentamicin tx: stop offending drug

Tics

abrupt coordinated movements or vocalizations; suppression cuases anxiety and tics relieve anxiety. Pt can voluntarily suppress it!! Assoc w OCD and ADHD Rx - Clonidine, Pimiozide, Haloperidol (spec for Tourette's)

Chorea

abrupt, irregular movements Hereditary (Huntingtons - AD, Wilsons - AR - both hit basal ganglia),

pandysautonomia

acquired disorder, usually immune, where SNS and PNS are affected. often associated w/ abs vs. ganglionic AChR if autoimmune. can be seen in DM, GBS, amyloidosis, chagas disease, PNP syndromes, toxic/drug induced, mutiple system atrophy, lambert eaton and others. Dx: HR, tilt-table, sudomotor responses, catecholamine level. Ts: treat symptoms

HIV associated lumbosacral polyradiculopathy

associated w/ CMV in HIV pts. is a rapidly progressive flaccid paraparesis w/ sphincter dysfunction, perianal sensory loss, and lower limb areflexia.

hyperthyroid and CNS disease

associated w/ periodic paralysis, proximal myopathy, seizures, chorea and dysthryoid eye disease. also can be ass w/ myesthenia gravis.

CJD

associated w/ protein 14-3-3

Glioblastoma Multiforme

astrocytoma, middle to late adulthood large tumor that crosses the corpus callosum, heterogeneous contrast enhancement "pseudopalisading" cells and "glomeruloid" appearance dx: MRI + biopsy tx: dexamethasone to decrease edema and improve symptoms, surgery, radiation, chemo ~1yr prognosis

miller-fisher syndrome

ataxia, areflexia, opthalmoplegia. ataxia due to proprioceptive loss vs. cerebellum. could be vriant of GBS, probably post-infectious IgG vs. GQ1b. self-limiting, good prognosis.

Partial seizure w/ secondary generalization

aura (smell) localizing to temporal lobe that secondarily generalized to involve entire cerebral cortex-->loss of consciousness and generalized tonic clonic seizure "Jacksonian March" shaking starts in the hand and gradully expands to involve the arm and then whole body dx: EEG and MRI to see precipitating cause tx: if only one seizure no AED, 33% chance of developing epilepsy after one isolated seizure

Asperger's syndrome

autism spectrum disorder with impaired social dev but normal language and cognitive abilities

Autonomic neuropathy 2/2 DM

autonomic dysfunction: orthostasis, impotence, urinary incontinence, abnormal sweating, resting tachy, gastroparesis, constipation, dry mouth and loss of sympathetic sx

Ataxia telangiectasia

autosomal recessive with cerebellar findings (ataxia, dysarthria), ocular telangiectasias, and early death from recurrent infections or cancer (hodgkin's) defect in DNA damage repair

Prolactinoma

bitemporal hemianopsia, pituitary adenoma, galactorrhea and hypogonadism due to hypersecretion of PRL MRI-sellar mass tx: transsphenoidal removal

hematomyelitis

bleeding into center of the cord

internal auditory artery (branch of AICA)

blood supply to inner ear. can become infarcted leading to sudden deafness and vertigo.

Optic neuritis

blurry vision, pain with eye movement and relative afferent pupillary defect tx: steroids IV

parkinson's gait (shiffling not an option

hypo kinetic short accelerating steps no arm swinging

contralteral hemiparesis, hemisensory loss, honoymous hemianopia, gaze palsy

basal ganglia hemorrhage

location of hypertensive Intracranial Hemorrhage

basal ganglia-internal capsule, caudate nucleus, thalamus, pons, cerebellum

Intraparenchymal hemorrhage

basal ganglia. clues to hemorrhage: progressive deficit after initial onset, decreasing consciousness and signs of increased ICP (eg. vomiting) head CT tx: BP control keep in 140-160s correct INR <1.5 mannitol if needed

ICH where? C/L hemiparesis/ hemianesthesia, homonymous hemianopsia, gaze palsy toward lesion

basal ganglia/ putamen

Nuchal rigidity + Photophobia...

be careful, could be SAH instead of Meningitis though!!

proposed pathophys of migraine

begins w/ cortical spread in depression (CSD) that is a wave of hypopolarization leading to a release of chemical substrages such as K, AA, H+, NO. these activate CN V afferents leading to orthodromic transmission leading to trigeminal nucleus and brainstem PNS efferent projection activation.

when to give steroids in spinal trauma?

beneficial for adults with incomplete acute spinal cord injury within 8 hours of injury not beneficial for kids or complete spinal cord lesiosn

Adie's syndrome

benign dysautonomia present in young healthy women 1. tonic pupils 2. weak/absent LE reflexes

Pterygium

benign growth of conjunctiva

pseudotumor cerebri

benign intracranial HTN resulting from increased resistance to CSF outflow at the arachnoid villi. It occurs in obese young women and is characterized by papilledema, elevated CSF pressure.

child with focal seizure at night on one side of face and mouth including sensory changes

benign rolandic epilepsy dx: eeg with numerous spikes over left central and right centroparietal region usually has family history can involve the perirolandic location within the brain whcih is the lateral aspect of the left hemisphere near the central sulcus *centrotemporal*

tx of tardive dyskinesia

benzodiazepine, baclofen, vit E

Lithium toxicity

bilateral coarse tremor + ataxic gait when added to diuretics tx: stop li, change to nondiuretic antihypertensive

spect findings suggestive of Alzheimer's disease

bilateral temporal HYPOmetabolism.

"bulbar palsy and descending paralysis, lack of fever, and clear senses and mental status" dx

botulism

39. 67yo M with 3-day histor of fever and headache. Five years ago, he underwent placement of a mechanical aortic valve for treatment of sequelae of rheumatic fever. T 40 C, BP 110/65, P 110/min. 3/6, systolic ejection murmur is heard. Neuro examination shows mild left hemiparesis. Babinski's sign is present on the left. Greatest risk for which complications?

brain abscess

Brain death secondary to hypoxia-ischemia

brain death: unresponsiveness (coma), brainstem death as evidenced by absent brain stem reflexes and absent spontaneous breathing

child with hypotonia and vomitting, and then self-mutiliation and gout

build of of uric acid due to HPRT def. lesch-nyhan syndrome

Botulism

bulbar sx: generalized weakness with diminished reflexes autonomic sx-dry mouth, pupillary dilation/fixed preformed toxin from C. botulinum is ingested and inhibits presynaptic ACh release canned food nerve conduction: presynaptic neuromuscular blockade and reduced compound muscle action potentials (CMAPS) tx: equine antitoxin

viral meningits

can have PML's in CSF initially along w/ lymphocytes w/ increased protein and normal glucose.

Steroid induced myopathy

can induce a proximal muscle weakness with normal CK, look for other signs like acne too tx: change steroid meds

Female w HA after drinking wine

can also be Migraine, not just Serotonin Syndrome interaction

Dermatomyositis

can also lead to cardiac issues like conduction defects, tachyarrythmias, myocarditis, CHF, interstitial lung disease w/ anti-Jo1 antibodies. can also lead to microangiopathic disorder due to abs and complement destroying BV to muscles leading to ischemia. CD4 cells can use steroids or IVIg

causes of chorea

can be accompanied w/ inability to maintain sustained muscle contraction. Hereditary: huntington, wilson, neuroacanthoscytosis Drugs: neuroleptics, anti PD meds Toxins: EtOH, Anoxia, CO metabolic: hyperthyroid, hyper and hypoglycemia, hepatocerebral degeneration. Immune: SLE, rheumatic Vascular: caudate infarct or hemorrhage

central nystagmus (brainstem)

can be bi or unidirectional and comonly has purely horizontal component (no rotational). can also have vertical nystagmus. visual fixation does NOT inhibit nystagmus. not ass. w/ tinnitus or deafness. vertiginous feeling will be mild. can be chronic conditon can be caused by vascular, demyelination, neoplastic/paraneoplastic disorder.

post traumatic seizures/epilepsy

can be categorized as early (w/in 1 week) or later (>1 week). acute severe TBI more likely to get early seizures (usually tonic-clonic) and of those 25% go on to get epilepsy. 50% develop w/in one year if they get seizures at all. Tx: antiepileptics but does not decrease chance of epilepsy overall.

restless leg syndrome

can be caused by Fe deficiency so always do ferretin test. Tx: ropinerole/pramipexole 1/2 hour before anticipated sxs. can also use gabapentin.

drug induced inflammatory myopathy

can be caused by many drugs: cimetidine procainaminde, L-dopa phenytoin lamotrigene D-penicilamine CK will be increased, will be proximal

spinal cord disorders

can be due to anterior horn or CST lesions so can give LMN or UMN signs. get MRI spine and possibly LP.

steroid myopathy

can be from endogenous or exogenous sources. more common w/ triamcinolone and dexamethasone or >30 mg of prednisone. can decrease risk of this w/ alternate day dosing. usually is proximal muscle CK is normal. usually have type II fiber atrophy.

immediate return to play

can be okay to do this if there was NO LOC, concussion sxs last <15 minutes. if 2nd event is the same day then need a week of rest

diffuse axonal injury

can be seen on CT as multiple areas of punctate hemorrhage in deep white matter and corpus callosum.

Carotid dissection

can get associated horner's syndrome and ischemic stroke MR or CT angio start anti platelet therapy to prevent thrombus formation in the dissection

treatment for hungington's disease

chorea - haloperidol (dopamine blocking, monitor for parkinsonism or tardive dyskinesia) or tetrebenzine depression - SSRI aspiration - PEG tube genetic counseling fall prevention

Huntington Dx

choreiform mvmts, MRI w/ caudate atrophy, CAG expansion on chromosome 4

Alcoholic cerebellar degeneration

chronic alcohol use-->preference for cerebellar vermis + chronic gait ataxia MRI cerebellar atrophy

relapsing remitting progressive symmetrical polyneuropathy, + sensory symptoms and areflexia for more than 2 months

chronic inflammatory demyelinating polyneuropathy (CIDP)

Brain abscess

classic triad: subacute fever, HA, focal neuro signs can have increased ICP CT/MRI-ring enhancing lesion risk factors: any causes of bacteremia, preceding infections adjacent to brain (sinusitis, otitis) or an immunocompromised state dx: aspiration or blood cultures to target therapy tx: IV abx, possible surgical intervention or CT guided aspiration

Complex Regional Pain Syndrome

clinical diagnosis, hypersensitive to pain from prior injury tx: sympathetic block

Venous Sinus Thrombosis

clot forming in the dural sinuses causes venous congestion and leads to ischemia in areas not following typical vascular territory (bilateral thalamus, corpus callosum etc) occurs in hypercoagulable pts (preggo) MR venogram (clot in sinuses) tx: IV heparin followed by coumadin first then if clot doesn't resolve thrombolytic injection into sinuses

unilateral retroorbital pain and horner's syndrome

cluster headache tx: ppx = verapimil, lithium, ergotamine

cochlear pathway

cochlea--> cochlear nuc.--> superior olivary nuc.--> nuc. of lateral lemniscus--> inferior colliculus--> MGN--> auditory cortex

How does the ice pack test make ptosis go away for a myasthenia gravis patient

cold temperature improves muscle strength by inhibiting breakdown of acetylcholine at neuromuscular junction.

Drug induced Parkinsonism

common drugs: DA agonists-metoclopramide or neuroleptics (haldol) tx: anticholinergic drug-cogenitin

hyperventilation

common trigger for absence siezures

hydrocephalus types

communicating: blockage of arachnoid granulations non-communicating: blockage of flow of CSF (i.e. through aqueduct or formena of Luschka and Megendie)

drugs that can cause DI-Parkinsons

compazine, metoclopramide (dopamine receptor blockers) dopamine depleting agents - reserpein, tetrabenazine some atypical antipsychotics

mydriasis, ptosis, opthalmoplegia

complete CN III palsy

complications of SAH

complex partial seizur with 2ndary generalization vasospasm hyponatremia (2/2 ANP, cerebral salt wasting, or SIADH) QT prolongation, Twave inversion and arrhyhmias

N-acetyl aspartate

compound used w/ MC spectroscopy that shows neuronal loss.

Carbamazepine

hyponatremia

reticular activating system

damage to this area in midbrain may cause coma.

Carbidopa function

decarboxylase inhibitor

prevention of delirium

decrease noise and number of interruptions at nigth

NT alterations in huntington's disease

decreased GABA decreased Ach increased dopamine therefore tx = tetrabenazine and reserpine to inhibit VMAT and limit dopamine vesicle packaging and release haloperidol - Dopamine receptor antagonist

sx of tumor in kids

decreased appetite weight loss decreased school performance dizziness ataxia neck pain bulbar weakness eye movement abnormalities opisthotonos due to tumors usually being infratentorial

imaging in PTSD

decreased hippocampal volume

Nutritional neuropathy

decreased oral intake (eg. preggo vomiting), B1 (thiamine) deficiency (dry beriberi) or B6 deficiency

red desaturation

decreased perception of color red. occurs often in optic neuritis of MS.

imaging in panic disorder

decreased volume of amygdala

MRA

definitive diagnosis of stenosis of cerebral or neck vessels.

Idiopathic intracranial HTN

degeneration of post-ganglionic SYMP. neurons. - autonomic nervous system damage only

Syncope from orthostatic hypotension

dehydration, heat, diuretics, sudden lightheadedness on standing dx: orthostatic BP measurement is positive if it SBP drops >20/10 DBP supine vs. erect, or tilt test tx: rehydration

cognitic impairment, impaired attention, fluctuating course

delirium

HIV associated Dementia

dementia in pts with AIDS 2/2 toxic immune response from HIV virus in CNS. Insidious and later in course of HIV illness dx: MRI to rule out focal pathology or opportunistic infection, see characteristic diffuse atrophy tx: HAART

hallucinations, parkinsonism, fluctuating cognition/alertness

dementia with lewey bodies

parkinsonism + cognitive dysfunction

dementia with lewey bodies

pathophysiology of concussion

disruption of the ascending reticular activating system (ARAS) at the junction between the thalamus and the midbrain particular during rapid deceleration

Multiple Sclerosis

dissemination in time >1 mo apart and space 2 different CNS sites and UMN dx: MRI for MS lesions periventricular ovoid white matter lesions affecting corpus callosum or infratentorial region LP-CSF analysis for oligoclonal bands tx: IV steroids for acute flare, maintenance therapy intereron beta or glatiramer acetate

Urge Incontinence

detrusor instability elderly, nursing home pts Parkinsons, dementia, strokes can't make it ot teh bathroom in time Rx - Oxybutynin, TCA's (help sympathetic - hold it in)

Parkinson's Disease Dementia

development of cognitive impairment greater than one year after the onset off motor symptoms

Angelman syndrome

developmental delay, ataxic gait, happy, easily excitability, frequent hand flapping loss of maternal contribution to chrom 15

Prader-Willi Syndrome

developmental delay, hypotonia at birth, uncoordinated, constantly eating, wt gain, loss of paternal chrom 15

in up to 50% of people with depression, what test will be abnormal?

dexamethasone suppression test.

peripheral nerve disorders

different patterns depending on nerves involved. if polyneuropathy, will have distal weakness early. can have sensory and motor symptoms including pain. polyneuropathy will lead to decreased reflexes. EMG/NCS will help identify nerves. can be due to entrapment, vasculitis, metabolic, rheumatic, demyelination, or other etiologies.

Coma

differentiated from brain death by presence of respiratory drive and presence of some cranial nerve reflexes coma transitions to recovering state vs. vegetative state within 2 wks

Persistent Vegetative State

differentiated from coma in pts have sleep-wake cycles, no observable, repeatable, meaningful responses to external stimuli. Must be no improvement for 3 mo to be considered "persistent"

parasaggital cerebral cortex lesion

difficulty walking, increased tone and reflexes in legs, urinary incontinence, [memory impairment] - kinda like normal pressure hydrocephalus?

Adie pupil

dilated pupil that reacts sluggishly to light but better to accommodation

Anterior spinal artery syndrome

diminished flow int he anterior spinal artery due to surgery/embolism/stenosis leads to extensive infarct of the spinal cord but spares dorsal column (vibration and proprioception)

Syringomyelia

diminished pain and temp sensation over shoulders and upper extremities MRI for cavitation of central cord

Intra-arterial thrombolysis timeframe

directly going in - should be done within 6 hrs

Benzodiazepines

drugs that suppress N3 sleep

EtOH and SSRI

drugs that suppress REM sleep

Chronic subdural hematoma

due to bleeding of bridging veins, common in elderly 2/2 brain atrophy and stress on brain tx: craniotomy

myesthenic crisis

due to decreased NMJ transmission due to high level of autoantibodies. will have decreased NIF, decreased FVC, leading to difficulty breathing. need to go to ICU for plex, IVIg, stop AChE inhbitiors.

urge incontinence

due to detrussor hyperreflexia/instability. commonin pts w/ strokes/frontal love dysfunction, suprasacral SC lesion MS. can be accompanied by detrussor-sphincter dyssynergia. can lead to urinary retention (DSD).

perinaud syndrome

due to dorsal midbrain pathology that leads to upgaze disturbance, conversion-retraction nystagmus on attempted upgaze and light-near dissociation. usually due to pineal tumor compressing dorsal midbrain. large irregular pupils, LND, eyelid abnormalities, impaired convergence or convergence-retraction nystagmus.

mitochondrial myopathies

due to dysfunctional/mutated mitochondrial DNA from mom. but some are nuclear encoded protein. MEw/RRF=myotonic epilepsy w/ ragged red fibers MELAS=myopathy, encephalopathy, lactic acidosis and stroke progressive external opthalmoplegia Kearnes-Sager syndrome usually will have myopathy and peripheral neuropathy. increased lactate and pyruvate.

neuronal ceroid lipofuscinosis

due to excess lipofuscin storage. leads to dementia, myoclonus, ataxia, retinitis pigmentosa. have a variety of forms w/ different ages of onsets and severities.

tethered cord syndrome

due to hypertrophy of filium terminale. is a congenital defect.

hepatic encephalopathy

due to increased NH3 leads to increased glutamine and less available glutamate. also leads to increased GABA activation.

idiopathic intracrania hypertension

due to increased pressure in SubA space. worse when recumbent w/ some relief when upright. can be worse in AM and accompanied by pusatile tinnitus and transient visual obscurations that are precipitated by valsalva or any movement that transiently increases ICP. increased CSF pressure in absence of mass lesion. venous sinus thrombosis can mimic this so need to exlude that. Tx: repeated LP, diuretics, optic nerve fenestration, LP shunts. can lead to visual loss if not treated.

Atonic (Adie's pupil)

due to interruption of PNS from ciliary ganglion. leads to anisocoria, photophobia, blurred near vision (loss of some accomodation). will have light--near dissociation. polocarpine leads to major constriction in affected pupil.

INO

due to lesion of MLF (from abducens to contralateral motor of III). leads to inability to adduct eye (due to decreased III activity) in contralateral gaze (to the abducens nucleus). i.e. no left adduction in right gaze. will also have nystagmus in abducting eye. convergence adduction is preserved b/c this does not require MLF. can get bilateral in wernicke's, botulism, myesthenia, brainstem strokes and demyelination (MS).

gertmann's syndrome

due to lesion of inferior parietal lobe *angular gyrus* on dominant hemisphere. constellation of problems including agraphia, acalculia, right-left confusion, and finger agnosia (can't recognize finger).

Hurler's syndrome

due to loss of a-L-iduronidase. will lead to clouding of cornea and characteristic faces and dwarfism.

sensory ataxia

due to loss of proprioception from feet. gait is slow and cautious, wide based. contact w/ ground made by heel and have slapping sound w/ forefoot strike. hard to walk in dark or uneven surfaces.

alexander disease

due to mutation in GFAP. leads to rosenthal fibers on biopsy, macrocephaly, and dysmyelination of CNS.

metachromatic leukodystrophy

due to mutation in arylsulfatase. leads to cherry red spot, demyelinating disorder that can present as schizophrenia in adults. will have positive urine sulfatides.

canavan disease

due to mutation in aspartoacylase. meads to macrocephaly and dysmyelination of CNS. will show developmental regression, mental retardation, and poor head control

emery-dreifuss muscular dystrophy

due to mutation in emerin gene on chromosome 4. more rarely have AD form w/ mutation in laminA on chromosome 1. all are nuclear membrane proteins. characterized by: early onset joint contractures (elbows, ankles, cspine), hemeroperoneal pattern of weakness, cardiomyopathy w/ conduction abnormalities. CK is mildly, myopathic EMG, DNA test is Dx. -pacemaking and stretching. no specific treatment.

Gaucher disease

due to mutation in glucocerebrosidase on chromosome 1. leads to cherry red spot, and typical cells found in bone marrow w/ lysosomal inclusions.

binswanger disease (small vessel disease/subcortical leukoencephalopathy)

form of small vessel vascular dementia caused by damage to the white brain matter. White matter atrophy can be caused by many circumstances including chronic hypertension as well as old age. This disease is characterized by loss of memory and intellectual function and by changes in mood. These changes encompass what are known as executive functions of the brain. It usually presents between 54 and 66 years of age, and the first symptoms are usually mental deterioration or stroke. The histologic findings are diffuse, irregular loss of axons and myelin accompanied by widespread gliosis, tissue death due to an infarction or loss of blood supply to the brain, and changes in the plasticity of the arteries. The pathologic mechanism may be damage caused by severe atherosclerosis. The vessels that supply the subcortical white matter come from the vessels that support basal ganglia, internal capsule, and thalamus. It is described as its own zone by and susceptible to injury. Chronic hypertension is known to cause because it changes the tension of the smooth walls vessels and causes changes in the vessel diameter. Arterioles can become permeable resulting in compromise of the blood brain barrier. It has been shown that the disease targets the vessels in this zone of the subcortex, but spares the microcirculation's vessels and capillaries which may be attributed to a difference between Alzheimer's and this disease. Symptoms include mental deterioration, language disorder, transient ischemic attack, muscle ataxia, and impaired movements including change of walk, slowness of movements, and change in posture. These symptoms usually coincide with multiple falls, epilepsy, fainting, and uncontrollable bladder. memory is normal, executive function is impaired. Because this disease affects flow processing speed and causes impaired concentration, the ability to do everyday tasks such as managing finances, preparing a meal and driving may become very difficult. It has been found in the Graphical Sequence Test that these patients have hyperkinetic perseveration errors which cause the patients to repeat motion even when not asked whereas Alzheimer patients have semantic preservation because when asked to write a word they will instead draw the object of the word.

diabetic amyotrophy (proximal motor neuropathy)

found in diabetics. will have severe thigh and back pain, followed w/ in weeks by mild to severe hip and thigh muscle weakness and atrophy.

beta-endorphinergic neurons

found solely in the hypothalamus

cholinergic crisis

found w/ pts who have increased cholinergic drive due to over medication w/ cholinesterase inhibitors. usually in MG pts. will have respiratory muscle weakness and increased secretions, diarrhea, n/v and diaphoresis.

kid with large head, long face, large testes, protruding ears, poor attention span and low IQ

fragile X syndrome trinucleotide repeats of CGG

kids with myocarditis, loss of sensationa nd proprioception of lower extremities, wide-based gait, atrophy of cervical spinal cord

freidrich's ataxia trinucleotide repeat disorder, autosomal recessive

Benign Paroxysmal Positional Vertigo (BPPV)

frequent attacks of vertigo, short period of time, positional, Dix-Hallpike will elicit vertigo tx: Epley for canalith respositioning

Pseudodementia

from depression can impact memory tx: SSRI and psychotherapy

Retinoblastoma

fundoscopic exam-white retinal mass dx: CT retinal based intraocular mass + calcifications tx: radiation or chemo, enucleation

asymptomatic kid with cataracts

galactokinase deficiency

infant with hypotonia, bilateral cataracts, jaundice, hypoglycemia, convulsions

galactosemia defect in uridyl-disphosphate galactose-4-epimerase

alpha-2 microglobulin

gene on chr. 12 that is associated w/ azheimer's disease. predisposes to early onset, sporadic AD, and then even more likely to get late onset AD.

toxic metabolic encephalopathy

general term to describe global dysfunction that accompanies broad rage of systemic problems like sepsis, metabolic disturbance, or drugs is a clinical state, not a dx. can be mild to severe. will have waxing and waning of arousal, aggitated (delirium) or sleep. get UA, CXR, CMP, ABG.

MS, imaging

get MRI! + periventricular white matter lesions

Cupping of optic disk

glaucoma

ganglioglioma

glia and neurons in cerebral hemispheres of kids. will increase T2 on MRI w/ swollen gyri. surgical removal leads to excellent pronosis.

A previously healthy 27-year-old man is brought to the emergency department because of a 3-day history of progressive weakness and shooting pains in the lower extremities and a 6- hour history of difficulty rising from a chair and climbing stairs. His temperature is 37 oc (98.60F)_ Muscle strength in the lower extremities is markedly decreased proximally and distally; there is weakness of the intrinsic hand muscles bilaterally. Deep tendon reflexes are absent throughout. Which of the following is the most likely diagnosis?

guillain barre syndrome - Acute onset ,Loss of DTR ,Neuropathic pain

Late stage Alzheimer's

hallucinations, delusions, sleep disorders, aggression, or paranoia apraxia, lose ability to use utensils for eating, gait difficulty and pt ends up bed bound w poor motor control, poor swallowing, and minimal speech production

tx of delirium

haloperidol

nonREM sleep

has 3 stages N1-N3 N1=transitional state btwn wakefulness and sleep N2=intermediate sleep w/ sleep spindles and K complexes N3=deep wave sleep w/ delta waves (0.5-2 Hz)

spastic bladder

has decreased capacity, decreased compliance, uninibited detrussor contraction; usually occurs due to UMN/supraspinal lesions.

atonic bladder

has increased capacity, increased compliance but low voiding pressure and flow rate. usually due to lesion at level of conus medullaris, cauda equina, sacral plexus or peripheral nerve dysfunction. leads to overflow incontinence. will have constant dribbling +/- urge stress incontinence.

Ischemic stroke

head CT to rule out hemorrhage check PT/PTT.INR, CBC, chemistry tpa if within time limit (4.5hrs) -->targets the unaffected area around the infarct the penumbra if after time limit-->clot retrieval

Trochlear nerve palsy

head tilt to contralateral side to compensate, covering eye relieves double vision (if yes then diplopia is due to lack of coordination from both eyes) tx: reassurance and wait for recover other causes of diplopia: myasthenia gravis, oculomotor palsy, trochlear or abducens nerve palsies

Benign rolandic epilepsy

hemifacial or secondarily genearlized seizures that are preceded by somatosenosry aura and occur during sleep EEG-centrotemporal spikes

A frontal cortex lesion results in....

hemiparesis w/ motor aphasia if dominant lobe is involved

ipsilateral weakness and loss of fine touch and vibration contralteral loss of pain and temperature

hemisection of the cord

CMT-2 (charcot marie tooth-2)

hereditary axonal neuropathy. is different than others in the same family because it is not demyelinating.

limb-girdle muscular dystrophy

hereditary condition that will have decreased strength in proximal muscle of arms and legs. spares face, extraoccular, pharyngeal. less likely to get cardiomyopathy. AR and AD versions. most common is mutation in calpain-3, dysterlin, fukutin related protein. Dx: CK increased, western blot and DNA analysis to classify. Bx is nonspecific dystrophic changes

pathogens related to GBS

herpes mycoplasma h flu campylobacter

tx sah

hhh hypertensive hypervolemic hemodilution nimodipine

Temporal arteritis

high dose steroids to prevent vascular occlusion of the temporal artery and permanent vision loss dx w elevated ESR and temporal artery biopsy

unilateral unresponsive pupil, impaired corneal sensation, decreased DTRs

holmes-addie differes from a third nerve palsy as EOM intact and can constrict on accomadtion dx: 0.1% topical pilocarpine - causes constriction in H-A but no affect on normal pupil

marfinoid habitus, fair hair and eyes, developmental delay, thrombosis, downward lens dislocation

homocystinuria tx: vit B6 and 12, folate

periodic alternating nystagmus

horizontal jerk nystagmus that changes direction every 2-3 minutes. can be acquired through *MS, bilateral blindness and toxicity from anticonvulsants*.

2nd order Horner's

horner's due to lesion in cervicothoracic cord, root trauma, cervical spondylosis, pancoast tumor.

1st order Horners

horner's due to spinal cord lesionsand above such as syringomyelia, brainstem ischemia, hypothalamus.

Pancoast tumor

horner's syndrome: ptosis, anhydrosis, miosis + lung mass dilating is worse in dark (anisocoria-unequal pupils) meaning the sympathetics are not working correctly dx: CT

3rd order horner's

horners due to superior cervical ganglion and above. can be due to carotid dissection/trauma/throbosis, base of skull trauma, cavernous sinus issues.

"seizure, fever, +rbc/wbc in CSF w/EEG: b/l, periodic epileptiform discharges usu over temporal regions" dx? test? tx? eeg?

hsv encephalitis hsv pcr on csf acyclovir inte intermittent high amp slow waves over temporal lobe + PLEDs

autosomal dominant gene on chromosome 4

huntington's disease

Rabies encephalitis

hydrophobia-painful contraction of laryngeal, pharyngeal, and diaphragmatic muscles in response to swallowing liquids + hypersalvation pain, pruritis at sit of inoculation prior to encephalitis (viral replication at dorsal root)

HTN, eyes deviated toward, contralateral hemiparesis

hypertensive bleed into basal ganglia-putamen (int capsule- hemiparesis)

Autonomic output is controlled by:

hypothalamus

Hypothyroidism

hypothyroid pts can have difficult with cognition including memory and concentration test TSH and free T4

Pt w Parkinsonian sx + autonomic dysfxn

i.e. postural hypotension, sweating, bowel/bladder control ruined, impotence, etc "Shy Drager Syndrome" - dejenerative dz.

Vertibular neuronitis

idiopathic neuropathy of vestibular nerve CN III. sudden , constant and severe but no associated hearing loss bc cochlea is not inflamed tx: benzos, anticholinergics, and antihistamines for vertigo, antiemetics for sx control

dilated pupil (mydriasis)

if due to lesion at midbrain, then usually will be accompanied by other issues. occurs in isolation due to compression of PNS fibers on outside of CNIII.

Rabies infected bite suspected

if pt was not vaccinated give post-exposure prophylaxis with rabies immunoglobulin if pt was previously vaccinated provide two booster doses of vaccine on days 0 and 3

tx of CIDP

if pure motor - IVIG others - steroids, physical therapy

lateral pons lesion presentation

impaired seonsry and motor function of CN V

Face deficits

in both MCA and ACA - face is involved

apraxia

inability to carry out learned motor task despite having primary functions needed to carry out task such as comprehension, motor ability, sensation, coordination. sometimes distributed into ideational, ideamotor, limb-kinetic, but better to just describe what pts cannot do. test by asking pts to 1) pretend they are preforming 2) mimic action w/ doctor 3) use actual objects can use actions such as brush teeth, salute, etc. due to parietal and frontal lesions on dominant side. frontal can recognize but not do. parietal cannot recognize task.

prosopagnosia

inability to recognize faces. can occur w/ right or bilateral lesions in visual association areas.

agnosia

inability to recognize objects through one or more sensory modalities, even though there is nothing wrong w/ primary sense. e.g. cannot recognize object in visual field, but vision is preserved. could recgonize if they touched the object. could maybe even describe visual features but can't recognize object as a whole. caused by lesions in sensory association cortices. e.g. occipital temporal region (recognition of objects).

atasia-abasia

inability to stand or walk upright in normal manner. distance between feet duirng gait is unpredictable, can be seen in conversion disorder, but gait will be inconsistent and pt will seem to sway and recover from falling at the last minute. An acquired total inability to stand and walk can be seen in true neurological diseases, including stroke, Parkinson's disease, damage to the cerebellum, Guillain-Barré syndrome, normal pressure hydrocephalus and many others. In normal pressure hydrocephalus, for example, when the condition remains untreated, the patient's gait becomes shortened, with frequent shuffling and falls; eventually standing, sitting, and even rolling over in bed become impossible. This advanced state is referred to as "hydrocephalic astasia-abasia".

M-protein related peripheral neuropathies

includes MM, amyloidosis, macroglobulinemia, cryoglobulinemia, lymphoma, leukemia. develops as symmetric, sensorimotor neuropathy that affect legs>arms and leads to large sensory fiber loss and sensory ataxia and weakness. will have NCS that show demyelination and axonal loss. tx: treat underlying cause, PLEX, if due to anti-MAG then use rituximab.

patient with headache worse at night, worse when leans forward, mental status changes, falls

increase ICP

neuroimaging in autism

increase brain volume

Tangier disease

inherited disorder characterized by significantly reduced levels of high-density lipoprotein (HDL) in the blood. HDL transports cholesterol and certain fats called phospholipids from the body's tissues to the liver, where they are removed from the blood. HDL is often referred to as "good cholesterol" because high levels of this substance reduce the chances of developing heart and blood vessel (cardiovascular) disease. Because people with Tangier disease have very low levels of HDL, they have a moderately increased risk of cardiovascular disease. Additional signs and symptoms of this include a slightly elevated amount of fat in the blood (mild hypertriglyceridemia); disturbances in nerve function (neuropathy); and enlarged, orange-colored tonsils. Affected individuals often develop atherosclerosis, which is an accumulation of fatty deposits and scar-like tissue in the lining of the arteries. Other features of this condition may include an enlarged spleen (splenomegaly), an enlarged liver (hepatomegaly), clouding of the clear covering of the eye (corneal clouding), and type 2 diabetes.

walking with legs far apart, lifts legs high, feet make slapping sound when hit the floor

injury to posterior column, ie tabes dorsalis form tertiary syphillis

Cluster HA

intense U/L PERIORBITAL pain acute onset, wakes you up from sleep can last for up to 2 hrs I/L eye tearing, stuffy nose, Horners MC presents daily for 2-3 mo, then disappears for years. Reappears. - Worse w alcohol/sleep Rx: Prophylaxis - Verapamil (best) Treat - 100% O2, Sumatriptan *Note, don't focus on the recurrence every night @ the same time - might be first episode!!

acute axonal diabetic polyneuropathy

intensely painful acute or subacute progressive, systemic, SENSORY axonal peripheral neuropathy. will have diabetic neuropathic cachexia ass. w/ weight loss; insulin neuritis.

somnambulism

interruption of sleep by variety of complex motor activities including walking, driving, or eating.

one and a half syndrome

involves PPRF or VI nerve nucleus and adjacent ipsilateral MLF. so get lateral gaze palsy in ipsilateral and INO in contralateral eye. only have abduction in contralateral eye (b/c CN VI on contralateral side is only nucleus not affected in conjugate lateral gaze).

medial pons lesion presentation

ipislateral limb ataxia, contralateral eye deviation, and paralaysis of face, arm and leg

facioscapulohumeral muscular dystrophy

is AD . due to loss of D4Z4 repeats at tip of chromosome 4. leads to upregulation of homeobox-4, and gain of function. starts at face, scapula and upper arm. then descends to involve legs. begins in teenage years w/ slow progression. heart and respiratory function usually spared. Dx: CK modestly elevated. genetic testing is definitive test.

Foster Kennedy syndrome

is a frontal lobe tumor that leads to papilledema in contralateral optic disc and ipsilateral disc atrophy due to compression of optic nerve.

Tabes Dorasalis

late stage neurosyphilis: progressive ataxic gait, decreased proprioception, pupils small irregular and react to accommodation by not to light dx: CSF VDRL tx: IV penicillin G

AICA lesion

lateral inferior pontine syndrome affecting facial nucleus (Remember: fACIAl has AICA in it)

central/transtentoral, downward herniation

leads to decreased alertness, small pupils (due to disruption of sympathetics bilaterally), decorticate posturing. if herniation continues then midbrain compression w/ fixed midposition pupils and decerebrate posturing.

Epidural hematoma

lucid period after initial loss of consciousness trauma to middle meningeal artery late finding: compressive 3rd cranial nerve lesion from herniation dx: head CT crescent biconcave lense shaped bleed tx: neurosurg + craniotomy

leprosy neuropathy

is an infectious cause of neuropathy. will have sensory and motor involvement. has a predilection for cooler areas. DTRs are usually preserved. will have nerve hypertrophy.

hereditary neuropathy with liability pressure points

is characterized by recurrent episodes of numbness, tingling, and loss of muscle function (palsy) in the region associated with the affected nerve, usually an arm, hand, leg, or foot. An episode can last from several minutes to several months, but recovery is usually complete. Repeated incidents, however, can cause permanent muscle weakness or loss of sensation. This disorder is also associated with pain in the limbs, especially the hands. are more sensitive to pressure on nerves than average person. most common problem sites involve nerves in the wrists, elbows, and knees. The fingers, shoulders, hands, feet, and scalp can also be affected. Many people with this disorder experience carpal tunnel syndrome, which occurs when a nerve in the wrist (the median nerve) is involved.

neuromyelitis optica

is essentially transverse myelitis and optic neuritis but more severe. pain more common. can have neutrophilc pleocytosis. will have anti-aquaporin 4 antibodies. tx: steroids, plasmapheresis and chemo prognosis is usually poor w/ likelihood of paralysis or blindness.

HSAN (hereditary sensory and autonomic neuropathies)

is hereditary and affects autonomic sensory or motor nerves.

optokinetic nystagmus

is physiologic nystagmus that is a normal response to a continuously moving object.

dix hallpike

is test for loose othliths. + ear is one towards the grown will lead to nystagmus w/ positive test.

wallerian degeneration

is the disintegration of the axon and myelin distal to a site of injury in the peripheral nervous system.

presentation of cerebellar tumor

ispi ataxia, patients fall towards the lesion titubation (forward and back movements of trunk) nystagmus intention tremor ipsi hypotonia difficulty with coordinatoin and RAM may also block CSF flow and cause increased ICP and headaches and vomitting

acute onset memory impairment in alcoholic

karsakoff syndrome caused by thiamine deficiency (vit B1)

tPA guidelines

keep BP < 185/110 (anti-hypertensive meds ok here) no ASA for 24 hrs

Topiramate

kidney stone, mild cognitive difficulties, word finding difficulties

Autism

language delay, avoids eye contact, no social smile, hand flapping

vacuolar myelopathy

late complication of HIV. occurs w/ severe immunosuppression. resembles subacute combined degeneration. can also have urinary and sexual dysfunction. Its course is invariably progressive and leads to severe paralysis of the lower limbs, with loss of the ability to walk and of sphincter control. The differential diagnosis is extensive and includes metabolic, infective, and neoplastic spinal cord diseases. The diagnosis is based on the clinical observation and the exclusion of other causes of myelopathy via serologic, radiographic, and cerebrospinal fluid studies.

Neurogenic claudication

leg pain + paresthesias radiating down from back bilaterally w/ walking, relieved by flexing at the waist dx: MRI spine

children with many types of seiure, impaired cognitive function, slow spike and wave on EEG

lennox gaustat syndrome

basal ganglia blood supply

lenticulostriate a. - supplies sriatum, internal capsule. Damage causes contralateral hemiparesis.

Becker Muscular Dystrophy

less severe form of DMD reduced quantity or size of the dystrophin gene pts can walk past 15yro and most live until 40/50 YO dx: muscle biopsy + DNA analysis tx: glucocorticoids

trientine

less toxic Cu chelator used in wilson's disease.

PRES (posterior reversible encephalopathy syndrome)

leukoencephalopathy that develops in context of rapidly developing HTN and eclampsia or due to immunosuppresants like calcineurin inhibitors or cyclosporine. presents w/ acute confusion and cortical vision loss. Tx: underlying causes. might not always be reversible.

pharm therapy for parkinsons

levodopa (crosses the BBB) + carbidopa to limit breakdown in the gut + COMT inhibitor to prevent breakdown peripherally (entacapone and tolcapone) bromocriptine, rpinirole, pramipexole (D2 agonist) Selegiline and rasagiline (MAO-B inhibitors) Amantidine - blocks NMDA receptors and has mild attenuation of cardinal symptoms of resting tremor and dystonia *as well as drug induced dyskinesia*

suprapubic mass means

likely distended bladder :P

"child w/3 mos daily ha, blurred vision, urinary frequency. bitemporal visual field deficits, papilledema, b/l wkns of eye abduction" dx?

likely some sort of suprasellar mass (craniopharyngioma if cystic w/ca)

riMLF (rostral interstitial nucleus of MLF)

located in pretectal midbrain area, near CN III nucleus. controls upgaze of contralateral eye via pathways of CNIII nucleus that control inferior oblique and superior rectus. crossover is via posterior commisure.

Cord compression

look for UMN sx (pt w hyperreflexia) = must be SC lesion if UMN. Hx of cancer Can look ~ epidural abscess, but look for fever and risk factors (i.e.IVDA) Immediately - steroids and get MRI!

Post lumbar puncture headache (intracranial hypotension headache)

loss of fluid from lp and continued dural leak leading to low pressure in CSF can lead to HA while sitting/standing that is relieved by lying flat MRI: diffuse meningeal enhancement +/- sagging of cerebellar tonsils on sagittal view tx: self limited resolve within 2-5 d, fluids and bedrest

REM behavior disorder

loss of normal skeletal muscle paralysis during REM sleep leading to acting out of dreams. leads to increased risk of developing synucleinopathy (parkinsons, lewy body, multi-system atrophy). Tx: clonazepam

pathology of parkinsons

loss of pigment in substantia nigra neurons with intracytoplasmic eosinophilic inclusions (lewey bodies)

lower extremity pain worse with waling, decreased reflexes in knees,

lumbar spinal stenosis - neurogenic claudication

vitamin E deficiency neuropathy

main symptoms are hemolytic anemia and neurologic deficits. Diagnosis is based on measuring the ratio of plasma alpha-tocopherol to total plasma lipids; a low ratio suggests deficiency of this vitamin. Treatment consists of oral vitamin, given in high doses if there are neurologic deficits or if deficiency results from malabsorption. deficiency causes fragility of RBCs and degeneration of neurons, particularly peripheral axons and posterior column neurons. neural symptoms include: Gross lack of coordination of muscle movements with loss of deep tendon reflexes. Truncal and limb ataxia. Loss of vibration and position senses. Paralysis of extra-ocular muscles responsible for eye movements. Muscle weakness.

Narcolepsy

mean sleep latency <8 mins + early onset REM + sx of cataplexy (paralysis with emotion), hypnogogic/hypnopompic hallucinations, sleep paralysis (upon awakening) tx: stimulants modafinil

Carbon monoxide poisoning

measure carboxyhemoglobin tx: 100% O2 to displace CO from Hgb

phenobarb

med that can be used w/ Increased ICP to decrease cerebral metabolism but cannot be used for long periods of time.

child with trunk dystaxia, horizontal nystagmus, unbalanced gait, papilledema

medulloblastoma most commonly occurs at cerebellar vermis - this is posterior vermis syndrome

Kernig's sign (knee) means

meningitis

repeated hypoglycemia episodes

metabolic condition that can lead to permanent cognitive deficits and injury to anterior horn cells of SC and cause and ALS-like syndrome.

uremic neuropathy

metabolic neuropathy that is symmetric, distal, sensorimotor that can lead to foot drop and leg weakness.

porphyric neuropathy

metabolic neuropathy that is usually associated with acute intermittant porphyria. can be acute or subacute sensorimotor axonal peripheral neuropathy that leads to paresthesias, dysthesias of extremities. can have rapidly evolving weakness or paralysis w/ areflexia and abdominal pain.

Fetal Alcohol Syndrome

microcephaly, smooth philtrum, thin upper lip, microopthalmia

Acute angle closure glaucoma differential

migrain, cluster HA, temporal arteritis and keratoconjunctivitis

common migriane

migraine w/out aura.

topical capsaicin

mild to mod neuropathic pain (post herpetic neuralgia)

microcephaly, poor feeding, epilepsy, spasticity in newborn

miller-dieker syndrome = lissencephaly severe form - deletion of chromosome 17 milder can be x-linked transmission or autosomal dominant

triad of areflexia, ataxia, opthalmoplegia, premonidant cranial nerve deficiecnies

miller-fisher variant of GBS. CN rather than extremity weakness anti-GQ1b ganglioside antibodies

comitant strabismus

misalignment is constant in all directions of gaze but each eye has full ROM. usually is opthalmologic issue.

phoria

misalignment of eyes when binocular vision is absent. cross cover test uncovers this deficit.

tropia

misalignment of eyes when both eyes are OPEN and binocular vision is possible. made better by cover test.

incomitant strabismus

misalignment varies w/ direction of gaze. usually signals neurological problem.

systemic disorders that include neurologic sxs and DM

mitochondrial diseases, myotonic dystrophy, friedrichs ataxia, stiff man syndrome

fingolamide

mixed agonist/antagonist of sphingosine-1P receptor that leads to sequestration of autoreactive T cells. is an oral medication. before prescribing, need EKG because can lead to bradycardia, heart block.

anti-RNP

mixed connective tissue dz

Spondylitic myelopathy

osteoarthritis of cervical spine leading to radicular arm symptoms = compression of cervical cord MRI of C spine neurosurg consult for decompression

19. 6mo girl born with alumbosacral myelomeningocele which was successfully repaired at 2 days of age. Anterior fontanelle is 6 x 8 cm and bulging and the posterior fontanelle is 3 x 4 cm and bulging. severe motor and sensory deficits. Increasing head circumference. Cause of increased intracranial pressure?

overproduction of CSF

Brown Sequard Syndrome (hemisection of spinal cord)

pain and temp lost contralateral to lesion and are spared for about 2 levels below bc tract of Lissauer that runs rostral before crossing , absent reflexes loss of vibration and proprioception same side as lesion

Parinaud's syndrome

paralysis of upward gaze with possible tonic deviation of eyes downward caused by: hydrocephalus and pineal tumors (young kid + bulging fontanelles and "setting sun" sign) MRI + surg

Hemiplegic migraine

paralysis on one side of body followed by severe headache lasting hours + nausea and photophobia MRI to rule out stroke tx: treat like normal migraine, but avoid triptans bc increased risk of stroke

Corticobasal Degeneration (CBD)

parkinsons plus syndrome + "alien limb" that moves involuntary and purposeful movements

Multiple System Atrophy (MSA)

parkinsons plus syndromes + autonomic failure or cerebellar dysfunction (orthostatic hypotension, impotence and autonomic storms-->diaphoresis and flushing)

Progressive Supranuclear Palsy (PSP)

parkinsons plus syndromes + downward gaze palsy (vertical gaze palsy) early falls, dysarthria, frontal-lobe cognitive problems and rapid progression

key difference between AD and pseudodementia

patients with pseudodementia are distressed by their impairments - in AD they are often ignorant to it

dawson's fingers

pattern of MS lesions

EEG activity in herpes encephalitis

periodic epileptiform discharges

Bell's Palsy

peripheral 7th facial nerve palsy (forehead affected) hit the nucleus hyperacusis and altered sensation bc facial nerve innervates stapedium muscle and sensory role for taste of anterior two thirds of tongue tx: high dose prednisone + taper +/-acyclovir

areas of MS lesions

periventricular white matter juxtacortical regions corpus callosum cerebellar peduncles

prusuits

permit eyes to conjugately track a moving visual target (ipsilateral direction). originate in parieto-occipito-temporal area. connect to vestibular nuclei, cerebellum and PPRF.

absence of awareness or cognition; preserved sleep-wake cycles and maintenance of autonomic functions

persistent vegetative state

Parkinson's Dx

resting tremor, limited facial expression, decreased eye blinking, cogwheel rigidity, short stride with decreased arm swing while walking loss of DA neurons in substantia nigra + deposition of Lewy bodies in brain (neurodegenerative disorder) Lewy bodies-alpha synuclein protein tx: levadopa/carbidopa or DA agonist

perilymph fistula

results from disruption of lining of endolymphatic system. pt reports pop and time of sudden increase of middle ear pressure, followed by abrupt onset of vertigo.

pupillary constriction reflex

retina-->optic nerve-->optic chiasm-->optic tract-->pretectal nucleus-->EWN (bilateral)-->PNS fibers on CN III-->cilliary ganglion-->pupillary sphincter

left hemispatial neglect

right parietal cortex

treatment of ALS

riluzole - glutamate inhibitor

Epidural Hematoma

rupture of MMA, lens shaped, lucid interval, can cross falx/tentorium. CONVEX +/- lucid interval!!

subdural hematoma

rupture of the superior cerebral veins--bridging veins that drain into the superior sagital sinus. Cannot cross falx tentorium.

otolithic organs

saccule responds to verticle acceleration; utricle responds to horizontal linear acceleration

vestibular pathway

saccule/utricle--> vest nuc--> cerebellum/VPM/nuc of CN III, IV, VI--> vestibular cortex

West Syndrome

same as Infantile Spasms!! Keep it high on differentials if a baby has muscular jerks AND an uncle w the same issue!! Rx - ACTH esp.

low pressure HA

secondary headache that is worse in upright position and better when laying down. usually iatrogenic due to LP and persistent leak in CSF, but can arise spontaneously due to rupture of CSF pouch or cyst that surrounds nerve root. Tx: caffeine, fluid replacement, blood patch.

Cataracts

see GRADUAL loss of vision - glare - difficulty seeing at night. 1/2 of people over Age 75 have it. (compared to papilledema from ICP causing intermittent vision loss) *Unique - they can present w/ "Second sight" - no longer need reading glasses! COOL.

transient loss of consciousness..think?

seizure vs syncope pale, clammy, tachy-->syncope aura-->seizure not TIA-no LoC

"pt w/hx of rheumatic heart dz...sudden onset speech difficulty, paralysis on right face and UE. been sick,vegetations on echo" dx tx

septic embolization to MCA give antbiotcs!

Cervical Radiculopathy (C7)

severe neck pain radiating to eg. L shoulder and extending to arm, forearm, and dorsum of hand, qeakeness in triceps, finger extensors, and wrist flexors dx: MRI/EMG tx: analgesics with rest and possible cervical collar or cervical traction

ring enhancing lesions

show w/ contrast on MRI due to leaky blood vessels. brain tumors abscesses subacute infarct resolving hematoma MS plaque thrombosed aneurysm AV malformations radiation necrosis

incontinence with urgency

spastic bladder- UMN frontal lobe, pons, suprasacral cord decreased capacity and reduced compliance Tx- Tolterodine (anti-Ach) SE dry mouth

muscle weakness, sensory level, hyperrflexive

spinal cord compression

Myelopathy in thoracic region

spinal cord compression, could be neoplastic in origin, abscess tx: IV steroids acutely, decompressive surgery or debridement if abscess

abrupt flacid paralysis and loss of pain and temp sense below a level

spinal cord infarction generally due to Anterior spinal artery i.e. during thoracic aorta repair because this artery is dependent on radicular branches off the aorta

pure cerebellar ataxia, familial parkinsonism, hereditary spastic paraplegia, neuropathy, restless leg syndrome, impaired temperature discrimination in all limbs and trunk and face

spinocerebellar ataxia-3 or machado-joseph disease adult onset!

Radial nerve damage

spiral groove of humerus test wrist/finger extension and sensation of dorsolateral hand

CTS management

splinting, steroid injxns, surgery

Vascular dementia

step wise progression, control risk factors eg. HTN

tx of MG

steroids pyridostigmine is an acetylcholinesterase inhibitor which txs the symptoms

akinetic rigid gait

stopped posture w/ flexion of shoulders, neck and trunk. narrow based, shuffling and slow w/ small steps, decreased arm swing, difficulty w/ gait initiation w/ small steps at beginning. typical of parkinsons and other extrapyramidal disease.

causes of brain abcess

strep viridans in extended sinusitis staph aureus in iatrogrnic or trauma

Left superior quadrantanopsia

stroke affecting meyer's loop (optic radiations in temporal lobe carrying info from inferior retina) visual defects: inferior quadrantanopsia-optic radiations in parietal lobe baum's loop homonymous hemianopsia-optic tract lesion or occipital strok (macular sparing with PCA infarct of occipital lobe)

neuroimgaing in OCD

structural abnormalities in orbitofrontal cortex and striatum

Creutzfeldt-Jakob Disease

subacute dementia with startle myoclonus and gait incoordination, younger pts EEG: periodic generalized 1Hz discharges CSF: 14-3-3 protein life expectancy is 1 yr

TB or fungal meningitis

subacute in immunocompromised pts HA or cranial neuropathies dx: basal MRI enhancement + mononuclear pleocytosis in CSF TB-homeless, immigrant, hx of TB crypotocccus-soil/pigeon droppings histoplasma-ohio/mississippi river valleys, bird/bat droppigns coccidiomycosis-sw US blastomycosis-vertebral osteolytic lesions candida or aspirgillosis dx: crypto-india ink or crypto antigen, TB-positive PPD or CXR findings, candida-budding yeast + pseudohyphae tx: amphotericin for fungus

Increased Intracranial Pressure

subacute progressive HA-->intracranial mass pain worse in morning/middle of night after supine papilledema signs MRI mets to brain: lung, melanoma, breast if symptomatic mass effect: dextamethasone

47 yo presents 12 hours after sudden onset occipital headache with neck stiffness. no FND

subarachnoid hemorrhage!!!

HIV associated dementia

subcortical dementia. presents w/ cognitive impairment and psychomotor slowing. will show T2 hyperintensity and cerebral atrophy.

elderly comes for confusion. 2 months of falls labs are all mml. dx?

subdural hematoma

rexed lamina 2

substantia gelatinosa - Lissaur's then trigeminal spinal tract neurons leave here

tobacco-alcohol amblyopia

these can lead to insidious and painless loss of vision, and centrocecal scotoma.

Thenar atrophy

think Median N entrapment - hypothyroidism, pregnancy

"paresthesias of hands and feet and an unsteady gait that is worse in the dark"

think b12 def

unable to void, 7 days ago back pain started, progressive leg weakness, IVDU, fever, hyperreflexia of LE, sensation decreased below umbilicus, sensation to light touch pinprick proprioception and vibration decreased over LE - location of lesion

thoracic spinal cord - sensory level at umbilicus so must be thoracic lesion - since iVDU and fever likely spinal cord abscess?

DBS for Parkinson's

thought ti involve depolarization block (a reversible dysfunction of neurons near the tip of the electrode). target subthalamus in PD subthalamus or GP for dystonia. VIM of thalamus for essential tremor.

trigeminal neuralgia

thought to be caused by compression of nerve root @ cerebellopontine angle usually by aberrent vascular loop. Tx: carbamazepine refractory cases--percutaneous radiofrequency ablation or microvascular decompression

Depakote side effects

three black box warnings 1. hepatotoxicity 2. teratogenicity-neural tube defect 3. life threatening pancreatitis

causes of ischemic stroke

thrombosis, embolism, hypoperfusion

Carpal Tunnel Syndrome

tingling and numbness sensation in hand if it were in a dermatomal or myotome distribution then think radiculopathy aka nerve root dx: NCS to establish diagnosis and grade severity tx: wrist splints at night, surgery if really bad

lhermitte's sign

tingling, electric sensation down spine when pt flexes neck which is worse in heat (Uhthoff's phenomenon). indicative of MS.

Best diagnostic test for suspected glaucoma

tonometry

causes of torticollis

torticollis = dystonia of SCM muscle can be caused by typical antipsychotics, metoclopramide, prochlorperazine

Amaurosis Fugax

transient monocular blindness 2/2 emboli in central retinal artery of one eye image carotid artery tx: carotid endarterectomy + anti plts

rebound nystagmus

transient rapid horizontal jerk when eyes are moving to or from eccentric position. think cerebellum when you see this.

Neurocysticercosis

travel to an endemic area (Latin America), partial seizures + secondary generalization, HA, and enhancing cystic lesions MRI-scolex (head) of cysticerci dx: neuroimaging and serum ab tx: albendazole and praziquantel

steroids

treatment for muscular dystrophies. can slow decline but that is about all.

Normal Pressure Hydrocephalus

triad: apraxic gait, dementia, and urinary incontinence dx: CT o rMRI showing enlarged ventricles, high volume LP with pre and post gait assessment tx: ventricular shunting

treatment of the tremor in parkinsons

trihexyphenidyl

treatment of generalized dystonia (DYT-1)

trihexyphenidyl (artane) - anticholinergic but placement of deep brain stimulator is more effective

paroxysmal hemicrania

unusual trigeminal autonomic cephalgia. is unilateral w/ accompanying ANS symptoms that is shorter duration than cluster headache, but occur w/ greater frequency. responds to indomethacin.

corticobulbar input to CNVII

upper face: bilateral input lower face: contralateral input

post infectious cerebelitis

usally affects kids 2-7. follows varicella or other virus have acute onset of limb and gait ataxia and dysarthria. dx of exclusion. must r/o drug intoxication and mass lesions. lasts a few weeks and recovery is complete.

Cardioembolic dx ischemic stroke

use anticoagulant (coumadin or dabigatran) for secondary stroke prevention. IV heparin acute stroke.

1-2 weeks of rest

used for concussion treatment if there was LOC. need hospital eval. rest depends on duration of LOC. if 2nd event then they will need >1 month rest

1 week of rest

used for concussion treatment if there was NO LOC but concussion sxs lasted >15 min. evaluate and if sxs last >1 week and should be evaluated frequently for worsening sxs.

vagus nerve stimulation

used to treat partial and generalized seizures. stimulated L vagus nerve w/ preprogrammed electrical impulses

Cerebral edema w Ischemic Stroke

usu seen within 1-2 days.

spinocerebellar ataxia

usually AD. will have cerebellar ataxia. insidious onset w/ progressive impairment of gait and dysarthria different genotypes have differeing accompanying sxs. cognitive decline is late feature. usually CAG expansions. SCA-6 is allelic to EA-2 and P/Q VGCC.

alcoholic cerebellar degeneration

usually accompanied by alcoholic polyneuropathy. MCC of acquired cerebellar degeneration. vermis is usually injured most. causes progressive truncal and gait ataxia.

hypothyroid myopathy

usually affects proximal muscles. will have delayed relaxation on DTRs. can be ass. w/ distal polyneuropathy will have INCREASED CK

medulloblastoma

usually at medullary velum of the 4th ventricle. leads to hydrocephalus. is made up of primitive neuroectoderm. can spread via CSF to spinal cord and bone marrow. heterogenous contrast enhancing midline tumor compressing 4th ventricle on MRI. get CSF to look for malignant cells if possible, bone scan, bone marrow Bx. Tx: surg+rads+chemo. might need steroids for vasogenic edema.

neurosarcoidosis

usually causes cranial nerve neuropathy due to chronic basal meningitis. facial and optic nerves most commonly affected. can also have facial neuropathy w/ parotid inflammation. can have papilledema or meningoencephalitis, hypothalamic involvement, seizures, and focal deficits due to space occupying lesions. myelopathy can be due to infiltrating/focal granulmoas. symmetric distal polyneuropathy or mononeuropathy multiplex can also result. Dx: histology (usually from alternative site from brain), increased ACE, MRI (nodular meningeal enhancement, parenchymal mass lesions, white matter lesions, hydrocephalus). Tx: steroids (maybe methotrexate, azithioprine).

Brown Sequard

usually cervical region

vertigo

usually does not occur when there are symmetric bilateral changes in vestibular function or slow unilateral changes (like schwannoma). usually is due to acute asymmetry.

agnosognosia

usually due to R hemisphere (non dominant) causing pts to be unaware of their deficits. harder to rehabilitate.

uncal herniation

usually due to lateral mass. leads to medial shift of uncus by temporal lobe. causes III nerve palsy, decreased consciousness. hemiparesis ipsilateral due to contralateral cerebral peduncle compression. also can lead to PCA compression and duret hemorrhages.

Ballism

usually due to lesion in caudate or subthalamic nucleus. stroke is most common cause. also can be caused by increased glucose (HNKH).

transcortical sensory aphasia

usually due to lesions at the inferior portion of the L temporal lobe. can be caused by PCA infarcts and small temporal lobe hemorrhages and contusions.

drug induced mitochondrial myopathy

usually due to zidovudine. CK is usually normal. will be acute or insidious proximal muscle weakness.

nerve root disorders

usually innervate multiple muscles, and muscles usually are innervated by fibers from multiple of these structures. if single then = radiculopathy if multiple then = polyradiculopathy rare sensory loss but tingling and pain are common. EMG/NCS useful, MRI spine, LP if infectious etiology suspected.

When can you see an ischemic stroke on CT?

usually need to wait 6 hrs Hemorrhagic - shows up right away

primary CNS lymphoma

usually non-hodgkin B cell type. can present w/ occular lymphoma 1st. cannot resect. chamo+rads only. can show dramatic response to steroisd but usually recurr.

psychogenic gait

walking pattern/gait that has a range of abnormalities

Delirium

waxing and waning acute change in mental status common in pts with underlying neuro dx (strokes + dementia) and is commonly caused by infection, electrolyte imbalance, meds (anticholinergics) check urinalysis and electrolytes

Lambert Eaton

weakness in proximal LE that improves with repetitive movement , hyporeflexic, ptosis, impotence, dry mouth paraneoplastic syndrome associated with small cell lung carcinoma autoantibodies against calcium channels at presynaptic motor nerve terminal

primary muscle problem

weakness that is predominant in proximal muscles in symmetric pattern. distal becomes involved late in course. can have muscle pain if inflammatory. reflexes generally preserved. increase CK in some disorders. due to inflammation, toxic and congenital etiologies.

Valproic acid

weight gain, hair thinning, tremor, hepatotoxicity, pancreatitis, teratogenicity

ocular disturbances, AMS, ataxia

wernicke encephalopathy - thiamine deficiency

light-near dissociation

when pupillary constriction to accomodation is greater than constriction due to light. implies defect in light response. can be due to syphilis, adie's, dorsal midbrain lesion, severe bilateral vision loss.

parietal occipital cortex

where info about motion, spatial localization, depth, color, and form is configured. bilateral lesions of this area lead to simaltagnosia or impaired ability to perceive parts of the visual scene as a whole.

temporal occipital cortex

where information about what a particualar image is, is configured. lesions in this area lead to prosopagnosia, or inability to recognize people by their faces.

Leukoaraiosis

white matter hyperintensities are the nonspecific hyperintense changes in the cerebral white matter frequently seen on CT and MRI in aged individuals and even young adults sometimes. It is a condition routinely found in elderly people. These white matter changes are also commonly referred to as periventricular white matter disease, or white matter hyperintensities (WMH) due to their bright white appearance on T2 MRI scans. Many patients can have this without any associated clinical abnormality. However, underlying vascular mechanisms are suspected to be the cause of the imaging findings. Hypertension, smoking, diabetes, hyperhomocysteinemia, and heart disease are all risk factors for leukoaraiosis. has been reported to be an initial stage of Binswanger's disease but this evolution does not always happen. White matter hyperintensities can be caused by a variety of factors including ischemia, micro-hemorrhages, gliosis, damage to small blood vessel walls, breaches of the barrier between the cerebrospinal fluid and the brain, or loss and deformation of the myelin sheath. Multiple small vessel infarcts in the subcortical white matter can cause the condition, often the result of chronic hypertension leading to lipohyalinosis of the small vessels. Patients may develop subcortical dementia syndrome.

Multiple Sclerosis

white matter of cervical region asymmetric scanning speech, intention tremor, INO, nystagmus (SIN)

arcuate fasciculus

white matter tract that connects wernicke's area to broca's. lesions of this area lead to conduction aphasia (loss of the ability to repeat). pts will be unable to monitor speech, so will make subtle errors but won't correct.

subcortical aphasia

will also present with hypophonia. due to lesionsin left basal ganglia/thalamus. will have variable fluency, preserved repetition and variable comprehension.

gaze toward side of lesion

will be gaze preference in cerebral hemisphere insult that causes lesion in frontal eye fields. will have deficit contralateral to direction of gaze.