RITE exam combination 1

Best meds to treat neuropathy (14)

- Gabapentin - Lyrica - Lamotrigine - Carbamazepine - Oxcarbazepine - Topamax - Duloxetine - Amitriptyline - Nortryptiline - Topical capsaicin - Topical lidocaine - NSAIDS - Narcotics (cautious) - Mexilitene

Parts and function of the internal capsule

- Genu - contains CBT which carries UMNs from motor cortex to cranial nerve nuclei - Anterior limb - contains sensory fibers that run from thalamus to frontal lobe, fibers that connect lentiform and caudate nuclei, fibers that connect cortex to corpus striatum - Posterior limb - CST (anterior 2/3); sensory fibers mainly from thalamus, fibers of optical radiation headed towards occipital lobe, acoustic fibers headed to temporal lobe, fibers from occipital/temporal lobes to pons - Retrolenticular portion - carries geniculocalcarine tract of optic radiations - Sublenticular portion - part of auditory pathway from MGN to primary auditory cortex

Anterior choroidal artery supplies which areas (4)

- Globus pallidus - Putamen - Thalamus - Posterior limb of internal capsule **** Comes off the ICA****

Causes of triphasic waves

- Hepatic coma - Electrolyte disturbances - Toxicities - Degenerative disease - Head trauma - Post anoxic insult

Side effects of valproic acid/depakote

- Hepatotoxicity - very bad teratogen - pancreatitis - anemia - weight gain CYP450 inhibitor

Cymbalta side effects

- Hyperhyrdosis - GI upset - HTN

Side effect of carbamazepine / oxcarbazepine

- Hyponatremia (esp. oxcarb) - Hepatotoxicity - Pancytopenia - Teratogen -CBZ is autoinducer (causes metabolism of itself) - CBZ is 90% protein bound - meaning in someone who makes low amounts of protein (liver disease, malnutrition/hypoalbuminemia), more of it will be floating free in the blood = toxicity

Symptoms of Pompe disease? Cause?

- Hypotonia - Progressive muscle weakness - Macroglossia - Hepatomegaly Vacuolar myopathy on biopsy (due to excessive glycogen storage due to deficiency of acid-alpha-glucosidase / acid maltase)

Common causes of PLEDS (7)

- Vascular insults (most common) - Encephalitis (esp. HSV) - Abscess - Tumor - Following surgery for tumors or vascular disease - Head trauma - Subdural hematoma

Causes of BILATERAL ACA infarct

- Vasospasm due to complications from SAH due to rupture of ACOM/distal ACA aneurysms - Patient with only one ACA - so once it is blocked from athero, both sides get hit

Huge aneurysm on angio on a baby. Name? How does it present in neonate vs later in infancy?

- Vein of Galen aneurysm - Neonate: presents as CHF b/c more than 25% of cardiac output goes through this malformation - later in infancy: hydrocephalus (expanding cranium, bulging fontanelle)

Extramedullary extradural spinal tumors

- Vertebral hemangioma (honeycomb) - Osteoid osteoma - Metastases - Multiple myeloma - Lymphoma

Names of the 3 midbrain stroke syndromes

- Weber's - Benedikt's - Claude

Common manifestations of a temporal lobe seizure

- confusion - contralateral posturing of hand - automatisms - difficulty speaking

Corticobasal degeneration symptoms

- degeneration of cortex and basal ganglia, age 50-70yo - Parkinsonism - Alien hand syndrome - Apraxia (inability to repeat or mimic movements) - Aphasia - Dementia, depression, irritability, FTD

Intracranial capillary telangiectasias

- found in the pons, with a small amount of hemorrhage and gloss

Topamax 3 biggest side effects

- kidney stones - stupor/memory difficulties - somnolence

Meaning behind abnormal findings on NCS: - low amplitude - decreased conduction velocity - temporal dispersion - conduction block - repetitive stimulation

- low amplitude = axonal (motor or sensory) - decreased conduction velocity = demyelinating (motor or sensory) - temporal dispersion= demyelinating (motor or sensory) - Conduction block= demyelinating (motor or sensory) or compressive - Repetitive stimulation (motor only) with DECREMENT= myasthenia gravis - repetitive stimulation with increment / facilitation = LEMS

Benign rolandic epilepsy (benign childhood epilepsy with centrotemporal spikes - BCECTS)

- most common epilepsy syndrome in childhood - Starts ~3-13yo and stops around 14-18yo - "sylvian seizures" - b/c they start around central sulcus of brain (AKA centrotemporal area) On EEG you'll see signs of seizures around C3/C4 and T7/T8 - Unilateral facial sensorimotor symptoms - Oropharyngeal manifestations - speech arrest - hypersalivation

CMT1A

- most common form of CMT - Onset 10-20yo - Autosomal dominant - Duplication of PMP22 gene - EDX: demyelinating features w/ uniform decrease in conduction velocities (<38 m/s) - Pathology: onion bulb on nerve Bx

Pupil sparing CN 3 palsy

- most likely related to small brainstem infarct or diabetes - extrinsic masses, or pcomm aneurysm would NOT spare the pupil

Damage to the mesial frontal cortex can cause...

- mutism - gait disturbances - incontinence - salutatory seizures - alien hand syndrome - apraxia of gait or magnetic gait

What is the treatment for propionic acidemia?

- restricting protein from the diet and providing parenteral fluids for hydration -carnitine and biotin supplementation -dialysis -metronidazole may decrease production of propionate by enteric bacteria

Characteristics of NF2

-Schwannomas -Meningiomas -Ependymomas -Bilateral acoustic neuromas (hearing loss)

Damaged areas that can cause a Horner's syndrome due to stroke

-Thalamus -Lateral medulla (Wallenberg) -C8/T1 (where the 2nd order fibers begin as they head back up to the eyes) OTHER CAUSES: - Pancoast tumor (at apex of lung) - Thyrocervical venous dilation - Cervical rib - Thyroid cancer / goiter - Thyroidectomy - Thoracic aortic aneurysm - Demyelination - Syrinx

What structure is involved in motor initiation, goal directed behavior and motivation?

-dorsoMEDIAL prefrontal cortex -can cause depression

Clinically, how do those with Tangier's disease look?

-enlarged and orange tonsils, neuropathy (sensory), hepatomegaly

What is Miller-Dieker syndrome?

-form of lissencehaly, four-layers or classic lissencephaly -associated with microdeletions on chromosome 17 in the LIS1 gene -X linked lissencephaly results from mutations in the DCX gene

What are the phakomatoses?

-group of disorders that have dysplastic lesions and tendency for tumor formation -neurofibromatosis, tuberous sclerosis, Sturge-weber syndrome, epidermal nevus syndrome hypomelanosis of Ito

Tau is on chromosome...

17

Mutation that causes NF1 is on chromosome...

17 (makes protein called neurofibromin)

" NMO

4x in women. Absent oligoclonal bands. Necrosis in spinal cord. Antibodies to aquaporin 4 in cell membranes of astrocytes

Epilepsy Syndrome: Treatment for Dravet Syndrome, and what to avoid?

1st line: valproic, clobazam Avoid: sodium channel blockers

List the brain ventricles

2 lateral ventricles Foramen of monro (connects laterals to 3rd) Third ventricle Cerebral aqueduct (connects 3rd to 4th) Fourth ventricle

DLB

2 or more features: fluctuating attention, well formed visual hallucinations, REM sleep behavior disorder, or parkinsonism

What does EEG show in GLUT-1 deficiency?

2.5-4 Hz spikes and waves and interictal EEG improves with glucose

Mutation that causes NF2 is on chromosome..

22

Myelination of the frontal white matter is seen on MRI at what age?

4-6 months

Substance ABUSE criteria

1 of the following in 12mo period: - It's use causes you to miss major obligations (school, work) - Legal problems - Using despite possibility of causing physical harm - Using even tho it's causing social problems

Martin Gruber anastomosis

"15-30% of population. Ulnar motor nerves for first dorsal interosseous, adductor pollicis and abductor digiti minimi exti medial cord from median nerve then rejoin ulnar nerve. EMG showed reduction in ulnar motor amplitude at elbow compared to wrist

" Diffuse intrinsic pontine gliomas

"80% have H3 K27M mutation H3 G34R mtation - pediatric cerebral hemispheric gliomas BRAF V600E mutation- pediatric glial/glioneuronal tumors BRAF-KIAA1549 fusions- pilocytic/pilomyxoid astrocytomas in posterior fossa IDH1 R132H mutations- adult oligodendrogliomas, diffuse astrocytomas codeletion 1p/19q- adult oligodendrogliomas

" Atrophy of posterior cingulate cortex

"Alzheimer disease. Atrophy of anterior cingulate cortex seen in FTD Huntington chorea: atrophy of caudate

What % of patients with sarcoidosis develop neuro involvement?

5-10%

Nerve conduction velocities in a newborn are what percentage of the velocities of an adult? At what age do they reach adult levels?

50% 2yo

Ataxic dysarthria vs flaccis vs hyperkinetic

"Ataxic: excess, equal stress, slow rate from alcohol abuse Myastenia gravis: flaccid dysarthria, breathy voice, hypernasal quality, imprecise articulation Tourette syndrome: hyperkinetic dysarthria- sudden variations in loudness and harsh hypernasal quality

Genetic mutations: C9orf72, alpha synuclein, Notch 3, MAPT

"C0orf720 both FTD and ALS. Alpha-synuclein- families with Parkinson's Notch3- CADASIL MAPT (tau)- familial FTD Presenilin 1: AD

Cranial nerve exits

"CN 9,10,11 exit through jugular foramen. Ascending limb of spinal accessory nerve pass through foramen magnum.

Ankle weakness

"dorsiflexion eversion: common peroneal nerve tibila nerve ankle dorsiflexion and eversion. Combo is sciatic nerve or lumbosacral plexus. Sparing of hip abduction is against plexus

Landau-Kleffner syndrome

"epileptic aphasia" syndrome Syndrome that starts b/w 2-8 years old and results in progressive APHASIA 75% also have seizures

Alzheimer disease

"extracellular beta amyloid plaque depositions. CSF level of beta amyloid 1-42 is decreased due to reduced clearance from brain to CSF and plaque deposition in brain. Intraneuronal inclusions of microtubule assciated protein tau that is hyperphosphorlyated in AD, very high in CSF

Alexander disease

"fibrinoid leukodystrophy" Progressive and fatal neurodegenerative disease Affects midbrain and cerebellum mutation in gene for glial fibrillary acidic protein (GFAP) on Chr. 17 - macrocephaly - seizures - spasticity - hydrocephalus - IIH - dementia

A- delta vs C afferent fibers

"from bladder to spinal cord--> dorsal horn--> periaqueductal gray matter A-delta fibers: information about bladder filling C fibers: relays pain when ""something is wrong""--> project to VPL of thalmaus

Pathology of frontotemporal dementia

"frontal and anterior temporal lobes--> executive, language, memory parietal lobes are spared which include -->visuospatial calculation skills

Cowdry inclusions

"intranuclear inclusions in viral infections NF tangles, Lewy bodies, Lafora bodies, and Negri bodies are intracytoplasmic

Characteristics of NMO on imaging

"longitudinally extensive" transverse myelitis - lesion extending over 3 or more vertebral segments (especially lower C-sine/T-spine)

CNV muscles

"mastication: temporalis, masseter, medial and lateral pterygoids, mylohyoid, anterior belly of digastric, tensor veli palatini, tensor tympani muscles: CNVIII muscles: frontalis, corrugator, orbicularis oculi, nasalis, buccinator, orbicularis oris, mentalis, platysmas

falcarine fissue

"medial aspect of occipital lobe. Calcarin fissue seperates cuneus superiorly, lingual gyrus inferiorly

Recurrent artery; of Heubner

"medial striate artery" "long central artery" The largest perforating branch from the ACA

Midposition pupils

"midbrain lesions by interrupting both descending sympathetic pathways and exiting parasympathetic fibers from E-W nucleus. reactivity to light controlled by pretectal nucleus. Respiratory centers in midbrain causes centrl neurogenic hyperventilation

what AED can exacerbate some seizures?

"myoclonic seizures exacerbated by: carbamazepine, gabapentin, lamotrigine, pregabalin, tiagabine, vigabatrin. Absence seizures aggravated by gabpentin, tiagabine, vagabatrin. Broad spectrum AEDs include: valproate, lamotrigine, levetiracetam, topiramate, zonisamide

Buproprion

"no anticholinergic, sedating, sexul dysfunction or orthostatic hypotension side effects. Fluoxetine, citalopram, venlafaxine have risk of sexual dysfunciton. Mirtazapine is sedating

CHERISH study

"nusinersen improvement in motor function by 3 points at month 15% Nusinersin causes alternative splicing of SMN2 to increase functional SMN protein

Cheiro-oral syndrome

"pure sensory lacunar syndomre hemi outh and i/l fingertips numbness junction of VPL and VPM of thalamus

Marchiafava-Bignami disease

"seen in alcoholics, corpus callosum damage. Presents with sudden onset of stupor or coma and seizures callosal disconnection --> dementia, gait problems, psychiatric disturbances, incontinence, hemiparesis, aphasia, apraxia of left hand

" Primary progressive aphasia

"severe impairment comprehending meaning of words. Can't name things, pseech is empty, vocab ecomes simpler. Difficulty understanding what others are saying. Difficulty understanding the use of objects. MRI: asymmetric anterior temporal lobe atrophy with surrounding gliosis lack of truama or acuity. DDx: AD has early deficits in language but usually memory impairments first Logopenic variant: nonfluent, effortful speech with anomia no single word comprehension deficits. Agrammatic variant: loss of grammar leading to nonprogressive nonfluent speech, comprehension preserved

What are subcortical U-fibers?

"short association fibers" represent connections b/w adjacent gyri of the brain (they sort of line the bottom of a sulcus and make a "U" shape)

fast vs slow onset triptans

"slow onset: naratriptan, frovatriptan fast: sumatriptan, zolmitriptan, rizatriptan, almotriptan, eletriptan

Ballism

"subthalamic nucleus dyskinisia occurs c/l to lesion and has hypotonia. Subthalamic nucleus modules i/l basal ganglionic activity which modulated cortical motor outflow to c/l effector muscles

Diseases CATEGORY that causes grouping of all large/hypertrophic muscle fibers together and all small/atrophied muscle fibers together on histo

"type fiber grouping" Neurogenic/nerve damage/denervation

" Alemtuzumab

"used for RRMS, aginst CD52 glycoprotein depletes T and B cells Can cause delayed autoimmunity due to early B repopulation. Can cause autoimmune thyroid, ITP, nephropathy, herpes/listeria, thyroid papillary carcinoma melanoma

Vestibulospinal tract vs rubrospinal tract

"vestibulospinal tract: extensor muscle tone rubrospinal tract: flexor tone between the two lesions causes decerebrate posturing

Blood supply to internal capsule

Anterior limb: - Lenticulostriate branches (from MCA; superior half) - Recurrent artery of Heubner (from ACA; inferior half) Genu: - Lenticulostriate branches (from MCA) Posterior limb: - Lenticulostriate branches (from MCA; superior half) - Anterior choroidal artery (from ICA; inferior half)

Localization of semantic aphasia (unable to understand nouns)

Anterior portion of L temporal lobe

Significance of R temporal spikes

Anterior temporal / inferior frontal spikes are usually seen in adults 90-95% of people with these have seizures Increases during sleep (30-50% of ppl with temporal lobe epilepsy have spikes when awake --> up to 90% have them when they're asleep)

Which of the following muscles is most useful for distinguishing an L5 radic from peroneal neuropathy? - Anterior tibialis - Peroneus longus - Medial head of gastroc - Anterior tibialis

Anterior tibialis (foot inversion; tibial nerve/L5) NOT: - Anterior tibialis & peroneus longus (peroneal nerve/L5) - Medial head of gastroc (S1)

ADEM antibody

Anti MOG

NMO mutation

Anti-AQP4 Ab (autoimmune / auto-antibody)

Which of the following antibodies is associated with a sensory neuronopathy? - AChR Abs - Ca channel Abs - Anti-Hu Abs - Anti-Jo 1

Anti-Hu antibodies - also associated with lung cancer

Metachromic leukodystrophy is caused by a deficiency of which enzyme? Symptoms?

Arylsulfatase A Causes sulfatides to build up in multiple bodily tissues and destroys myelin - Developmental delay - Hypotonia - Difficulty walking - Seizures - Optic atrophy

Hallmark finding on imaging of patient with Huntington's disease?

Atrophy of caudate and putamen leading to enlargement of lateral ventricles.

Lafora disease

Autosomal recessive due to mutations in EMP2A and EMP2B Will see "lafora bodies" on histology - dark circles; composed of abnormal glycogen (polyglucosans) First develop Sx during adolescence: * Quickly developing, progressive and severe dementia - Seizures - Drop attacks - Myoclonus - Atalxia

Terminal branches of posterior cord of brachial plexus

Axillary nerve Radial nerve

Neurophysiologic (EMG) differences b/w axonal vs demyelinating neuropathies

Axonal: - Normal NCV/distal latencies - Low amplitude CMAP/SNAP - Denervation changes on EMG Demyelinating: - NCVs < 75% lower limit of normal - Conduction block - Temporal dispersion of CMAP - Prolonged distal latencies, F-wave latencies

Canavan disease - enzyme deficiency, symptoms

A leukodystrophy caused by deficiency of enzyme aminoacylase 2 (Chr 17). Symptoms appear from 3-6mo -- intellectual disability, loss of previously acquired motor skills, feeding difficulties, floppiness, hypotonia, seizures

A variety of impulse control behaviors, including gambling and other compulsive behaviors, have been reported in patients with _______ disease apparently related to dopamine agonist therapy. Patients should be informed of these behaviors prior to starting agonist therapy and should be monitored for this side effect during follow-up visits.

Parkinsons

Procedural memory problems are commonly reported in patients with________ ________ and affect tasks such as playing a musical instrument or golfing.

Parkinsons disease (PD)

SSRI with highest risk of "discontinuation syndrome" with missed doses?

Paroxetine (b/c short half life)

What type of seizures does Vimpat (lacosamide) treat?

Partial

Cavernous malformation (AKA cavernoma, cavernous angioma)

Popcorn appearance

Huge nasty cyst on outside of brain - looks like big hole in side of brain and goes all the way to the ventricles. Has little strings of arachnoid covering its opening.

Porencephalic cyst

The patient most likely suffers from _____ _______ ______, characterized by vivid dreams and abnormal motor activity during REM sleep. This is seen most commonly in concert with ________.

REM behavior disorder; parkinsonism;

First vertebrae you can count on MRI

C2 (it's super weird shaped and elongated) First normal shaped one is C3

Which nerve innvervates the rhomboid?

C5 nerve root

A patient's constellation of findings on the motor, sensory, and reflex examination all point to a lesion of the C6 nerve root rather than an individual peripheral nerve lesion. Her weak muscles are innervated by several different nerves but they all share a common innervation from the ___ _______ ________ .

C6 nerve root

Which affected dermatome causes dysesthesias in the little finger?

C8

Gene familiar frontotemporal dementia- ALS

C9orf17

NOTCH3 gene mutations cause....

CADASIL (cerebral AD arteriopathy with subcortical infarcts and leukoencephalopathy)

Initial neuropathy screening labs

CBC, renal, LFTs, glucose, 2hr GTT, A1c, ESR, folate, TSH/fT4, B12, MMA, homocysteine, SPEP (looking for elevated IgM, IgG, IgA), immunofixation/IFE, HIV If demyelinating: CSF protein If family Hx: genetic testing Autonomics: orthostatics Skin biopsy

Does GBS or CIDP get steroids?

CIDP

Cause of generalized bisynchronous periodic sharp waves?

CJD

What are the most common genes involved?

CLN1 and CLN2. CLN1 encodes for palmitoyl protein thioesterase 1 (PPT1), and CLN2 encodes for tripeptidyl peptidase 1 (TPP1).

Areas of the brain that degenerate in Huntington's disease

Caudate and putamen

Imaging finding for Huntington's disease

Caudate atrophy (makes lateral ventricles look much larger than normal)

Hirayama disease

Caused by structural a disorder if cervical spinal cord Distal hand motor weakness in one arm in young man sparing brachioradialis

HTLV1

Causes chronic progressive myelopathy (spastic parapareisis) in tropics

Alpha coma -- what is it? causes?

Clinical coma associated with alpha activity (8-13 Hz) on EEG that is unresponsive to external stimuli Caused by: - hypoxia - drug overdose - lesions in pontomesencephalic region

This is defined as a first demyelinating episode. A patient demonstrates only one lesion in time and space. The significance of this syndrome has been borne out in clinical trials which indicate that initiating a disease-modifying therapy (DMT) after this syndrome delays the time until a second clinical episode and a definitive diagnosis of multiple sclerosis (MS)

Clinically isolated syndrome (CIS)

This is endemic to parts of Southern Arizona, central California and Mexico, typically produces a subclinical pulmonary infection, though more severe forms of pneumonia may occur. Complicated pulmonary disease is rare, occurring in approximately 5%-10% of patients

Coccidioidomycosis

In an acute lumbar radiculopathy, what is the most likely EMG finding

Reduced recruitment (occurs from day 1-10) Fibrillations start at day 16-25

Only finding on EMG within the first few days of an acute lumbar radiculopathy

Reduced recruitment of motor unit potentials

Cause of cubital tunnel syndrome

Compression of ulnar nerve at flexor carpi ulnaris aponeurosis Compression of ulnar nerve at retroepicondylar groove is more COMMON (but the term "cubital tunnel syndrome" is named after the less common condition above)

This drug has class A evidence showing prolongation of survival in patients with definitive ALS

Riluzole

Open ring sign

Ring-enhancing lesions that have a "break" in the ring where it touches grey matter. Seen in tumefactive MS (Marburg disease, Schilder disease)

Age of bleed on MRI

T1/T2 2h: I / B (I brought) 2 days: I / D (iddy) 2 weeks: B / D (biddy) <2 months: B / B (baby) >2 months: D / D (doodoo)

Where does sympathetic outflow originate for bladder relaxation?

T11-L2

Armpit's dermatome

T2

Antidepressants that are most likely to cause anticholinergic side effects

TCAs (esp amitriptyline)

Presentation of CADASIL

TIA/Stroke + migraine with aura Progresses to subcortical dementia with pseudobulbar palsy and urinary incontinence Typically around 40-50yo

Epilepsy Syndrome: What is the treatment for Panayiotopoulos syndrome?

Treatment might not be needed if seizures are infrequent.

Susac syndrome

Triad: - Retinal branch occlusion - Hearing impairment - MRI changes that resemble MS (except these are more at midline of corpus callosum than at margins like MS)

An umbrella term for SUNCT/SUNA, paroxysmal hemicrania (PH), cluster headache, hemicrania continua (HC) is called?

Trigeminal Autonomic Cephalalgias

Sensory nerve for cornea

Trigeminal, V1 (ophthalmic) branch

Genetic mutation that causes Huntington's disease

Trinucleotide repeat amplification mutation in huntingtin gene on chromosome 4

20yo patient with atrophy in temporal region. What underlying chromosomal disorder do they have?

Trisomy 21 / Down Syndrome - b/c they can get early onset Alzheimer's

Pale "ash leaf" spot on skin

Tuberous sclerosis

This test, tests the ability to shift sets, while letter fluency primarily tests generation of new items

Trail making

Which of the following DOES NOT have impairments in repetition? Broca's aphasia Wernicke's aphasia Transcortical motor & sensory aphasia Conduction aphasia Pure word deafness

Transcortical motor and sensory aphasia

Aphasia: Non-fluent, Intact comprehension, repetition, reading and writing; impaired naming

Transcortical motor aphasia

Aphasia: Fluent, impaired comprehension, intact repetition?

Transcortical sensory asphasia

Positive occipital sharp transients of sleep (POSTS)

Transient sharp waves seen in the occipital region spontaneously during sleep

Group atrophy of skeletal muscle is seen in...

Werdnig-Hoffman disease

Aphasia: Fluent with paraphasic errors, impaired comprehension, impaired repetition, impaired naming, well formed writing?

Wernicke aphasia

Epilepsy Syndrome: Spasms of trunk and limb lasting 2-10 secs, often in clusters, often after waking. EEG shows very high amplitude asynchronous slow waves, multifocal spikes, poly-spikes that is more prominent in sleep. Most end up with intellectual disability. Which syndrome?

West Syndrome

Epilepsy Syndrome: What epilepsy syndrome has a triad of Epileptic spasms, hypsarrhythmia, and mental retardation?

West Syndrome

Hypsarrhythmia on EEG is seen in which disorder?

West Syndrome (infantile spasms)

"wet keratin" on histology - appearance? seen in what pathology?

Wet keratin is a big pink, pretty solid blob Seen in craniopharyngioma (adamantinomatous type)

What is logopenia?

Where is low word production with intact content

Aphasia: Fluent with hesitancy, intact comprehension, impaired repetition, impaired naming, inability to read aloud?

Conduction aphasia

Drug of choice for juvenile myoclonic epilepsy

Depakote (unless pregnant: keppra)

Fibrillation potentials and short duration motor units are most likely seen in which disorder?

Dermatomyositis

Types of sensory neuropathies (8)

Diabetic Paraneoplastic AIDS B12 deficiency Sjogren's Celiac Sprue Cisplatin, thalidomide Pyridoxine (too much B6) Inherited sensory neuropathies

This is the most common type of polyneuropathy. It is a distal axonal neuropathy and can be associated with autonomic features as well as pain. Improved glycemic control can prevent progression, but is not effective at controlling pain. There is level A evidence recommending pregabalin for symptomatic treatment of pain.

Diabetic sensorimotor neuropathy

Common peroneal nerve (branch of sciatic nerve) innervates which muscles

Divides into deep and superficial as well as lateral cutaneous N of calf Deep Mainly L5: Tibialis anterior (dorsiflexion) and Extensor hallucis Longus (toe dorsiflexion) Superficial: Peroneal muscles (Eversion)

EEG finding for benzodiazepine use

Excess beta-activity (so really high frequency > 13 Hz)

Damage to which area causes agraphia without aphasia

Exner's area (which is above Broca's area in frontal lobe)

2 types of spontaneous activity on EMG

Fibrillations - rhythmic firing of individual muscle fiber. Always abnormal: denervation or inflammatory myopathy Fasciculations - can be normal; common in motor neuron disease and neuropathy

Tumor with calcified psammoma bodies (onion skin looking) and background hypercellularity?

Fibroplastic meningioma

A patient has acute onset of abducens and hypoglossal nerve palsies, along with fatigue and weight loss what do they have?

Gottfredon's syndrome, suggestive of underlying malignancy, such as a metastatic tumor to the clivus

Where do metastases like to sit in the brain?

Grey-white junction

Concomitant use of which drug can increase lithium level by up to 40%?

HCTZ

treatment for cluster HA

HF O2/triptans (abort) and verapamil (ppx)

Common viral infection that people get after a bone marrow transplant?

HHV-6 encephalitis (on histo you'll see perivascular lymphocytes)

Infectious etiologies of neuropathy (6)

HIV Syphilis Lyme Leprosy (mycobacterium) HCV HBV

30% of patients with polyarteritis nodosa will have circulating antibodies to _________.

Hepatitis B virus

Nemaline rod myopathy

Hereditary (both AD and AR forms); rare In babies: hypotonia, lack of movement, dysphagia, difficulty breathing Swollen face Adults: respiratory symptoms; elongated faces, lower mandible

Findings on MRI of Wilson's Disease

Hyperintense basal ganglia, thalami, cortex, and cerebellum

Wernicke encephalopathy on MRI

Hyperintensity on DWI in bilateral thalami

Cause of a flow void

Loss of signal due to vigorously flowing fluid (vessel is definitely patent) (blood, CSF, urine)

Amitryptiline / nortryptiline side effects

Low doses: - Xerostomia - Xerophthalmia - Urinary retention High doses: - Prolonged QT

Intramedullary spinal tumors in children

Low grade glioma

Headache + diffuse dural enhancement...Dx?

Low-pressure headache (intracranial hypotension)

What nerve roots supply the lateral cutaneous nerve?

Lumbar plexus of L2-L3, supplies sensation to the lateral thigh

Mechanoreceptor in charge of sensing light touch

Meissner's corpuscles and Merkel's receptor

Brain Tumor Question: What benign brain tumor is associated with Psammoma bodies and whirled pattern of cell growth?

Meningioma

Prophalactic Frovatriptan is indicated for which type of migraine?

Menstrual

What is the alternative name to lateral cutaneous neuropathy?

Meralgia paresthetica

A 24 year old obese female presents with 3 months of progressively worsening headache and episodes of transient vision loss in both eyes lasting seconds. She is found to have bilateral papilledema. Workup should include?

MRI head w/wo, MRV and LP

Intelligence remains intact in which of the following: Alzheimers PSP Juvenile Huntingtons Wilson's Disease Machado-Joseph disease

Machado-Joseph disease (dystonia, athetosis, spasticity, rigidity, ataxia, LMN signs)

What is the most sensitive study for identifying carpal tunnel syndrome?

Median palmar study

Brain Tumor Question: What cerebellar tumor is a rapidly progressive, radiosensitive tumor that has Home-Wright Rosette on path (circle of cells around tangle of fibers)?

Medulloblastoma

Which neuro tumor is this: small blue round cells with tons of undifferentiated embryonal cells dispersed throughout. Can have Homer Wright rosettes

Medulloblastoma

Where to dopaminergic pathways arise?

Mesolimbic and mesocortical projections (all of which are dopaminergic fibers) arise from ventral tegmental area of midbrain and travel to cerebral cortex (prefrontal cortex), limbic structures (amygdala), and striatum (putamen, caudate)

Disease that presents on MRI as extensive white matter demyelination (especially periventricular), very symmetrical. Spares U-fibers

Metachromatic leukodystrophy (a lysosomal storage disease, autosomal recessive)

Weber's Syndrome

Midbrain stroke of substantia nigra, CST, CBT. Due to occlusion of paramedian branches of PCA or of basilar bifurcation perforating arteries - Ipsilateral CN III palsy - Contralateral hemiparesis

What is Miller-Dieker Syndrome?

Miller Dieker syndrome is a disorder of neuroblast migration causing agyria, intellectual disability, intractable seizures, and spasticity. Chromesome 17

Large deletions of LIS1 lead to?

Miller-Dieker Syndrome

What syndrome is anti-GQ1b associated with? 3 traits of this syndrome?

Miller-Fisher syndrome - Ataxia - Areflexia - Ophthalmoplegia

Sedating anti-depressant to avoid in truck drivers

Mirtazapine (even sedating at low doses)

Anencephaly

Missing most/all of brain and skull. Top of head is open

Aphasia: Non-fluent, impaired comprehension, intact repetition?

Mixed transcortical aphasia

two broad categories of peripheral neuropathy

Mononeuropathy (individual nerves such as median, radial, ulnar; mononeuritis) Polyneuropathy (distal symmetric polyneuropathy vs polyradiculoneuropathy)

Patient with Sx of pituitary dysfunction with anteriorly displaced pituitary on MRI?

Most likely Rathke cleft cyst (less likely craniopharyngioma)

Periventricular leukomalacia

Necrosis of white matter near ventricles in babies. Essentially strokes along lateral ventricles (on imaging to me it looks like how periventricular small vessel ischemic changes look in adults)

"Synnuclein-opathies" (7)

Parkinson's Dementia w/ lewy bodies Multiple system atrophy Hallervorden-Spatz (HVS) PKAN Pure autonomic failure Meige syndrome

Panayiotopoulos syndrome

child, pale flaccid, unresponsive. Ictal syncope or ictal syncoapl like episodes

At rest, neuronal membranes are most permeable to which ion?

chloride

" Treatment for vascular dementia

cholinesterase inhibitors such as donepezil, rivastigmine, galantamine

For what type of migraine is Botox indicated for?

chronic

The use of Indomethacin is indicated for which type of headache?

chronic paroxysmal hemicrania

Athetosis

a condition in which abnormal muscle contractions cause involuntary writhing movements. It affects some people with cerebral palsy, impairing speech and use of the hands

DaTscan

a contrast agent to be used with SPECT for detecting dopamine transporters Will be abnormal in Parkinson's and alpha synecleic diseases (MSA, PSP)

What is neuronal ceroid lipofuscinosis?

a group of autosomal recessive disorders characterized by progressive psychomotor retardation, seizures, and blindness, which can present in infantile, late infantile, juvenile, and adult forms

PSP vs CBD

all have core features of parkinsonisms. Both are tauopathies. PSP has symmetric axial rigidity. Alien limb syndrome favors CBD

Symptoms of bilateral ACA infarctions

apathy, lack of motivational intent, akinetic mutism, weakness of LE, urinary incontinence

Lamotrigine

approved for bipolar depression, aripiprazole as adjunctive therapy for resistant depression

Edaravone

approved treatment for ALS

ciliary ganglion

are postganglionic parasympathetic fibers of short ciliary nerve which innervate sphincter muscle of iris and ciliary muscle. Lesion would cause dilated pupils and loss of direct pupillary reflex

Circumventricular organs

area postrema, pineal gland, subcommissural organ, subfornical organ, organum vasculosum, median eminence, neurophypohysis.

Name the circumventricular organs?

area postrema, pineal gland, subcommisural organ, subfornical organ, neurohypophysis

IVIG, IV solumedrol and plasma exchange are appropriate treatment considerations for?

autoimmune encephalitis

tetrabenazine

used to treat chorea in huntington disease. Depletes monoamines such as dopamine from presynpatic terminal. Can cause parkinsonism and depression

Epilepsy Syndrome: What is the treatment for CSWS and Landau-Kleffner syndrome?

usually high dose benzo and steroids is first line, but can go on to others.

What are the clinical hallmarks of disease?

delayed motor development, progressive limb and neck rigidity with dystonia, choreoathetotic movements, facial grimacing, seizures, spasticity, and intellectual impairment. Aggressive behavior, self-mutilation, and progressive dementia are hallmarks of the neurologic form of the disease

Metamorphosis

delusion that people in environment swap identieis with each other

What will bone marrow biopsy show in Neiman pick A and B?

demonstrate vacuolated histiocytes with lipid accumulation called FOAM cells in which the sphingomyelin adopts the form of concentric lamellar bodies

Anton syndrome

denial of blindness

What is Anton's syndrome

denial of blindness

Anosognosia

denial of weakness

What are common behavioral side-effects of Levetiracetam?

depression and irritability

medial frontal cortex

detection of unfavorable outcomes and adaptation based on social cognition

What is the clinical presentation of patients with PKU?

developmental delay, cognitive impairment, microcephaly, seizures, hypotonia, and severe behavioral disturbance

Lhermitte-Duclos disease

dysplastic gangliocytoma of the cerebellum, a rare, benign, hamartomatous growth. It presents with headaches, diplopia, and tremors.occurs in pts w/ Cowden syn

This tremor is jerky and is often seen in extremities affected by dystonia. It is typically associated with abnormal or specific postures

dystonic

Where does the cingulate gyrus send fibers?

ventral striatum (limbic striatum) Ventral striatum includes the ventral caudate, nucleus accumbens and olfactory tubercle. Fibers from these regions then project to the dorsomedial nucleus of the thalamus, which then relay fibers back to the cortex. This circuit subserves motivation.

SUNCT/SUNA, paroxysmal hemicrania (PH), cluster headache, hemicrania continua (HC) how to differentiate?

frequency and duration

large motor unit potentisl

from collateral sprouting from adjacent axons

Muscles controlled by CN VII

frontalis corrugator orbicularis oculi nasalis buccinator orbicularis oris mentalis platysmas

A relatively young patient shows a progressive syndrome of language impairment with a gait and behavioral abnormality. The PET image shows a progressive metabolic decline particularly in the orbitofrontal region. This is most consistent with

frontotemporal lobar degeneration (FTLD)

What are the three enzymatic defects that account for galactosemia:

galactose-1-phosphate uridyltransferase deficiency, galactokinase deficiency, and uridine diphosphate galactose 4′ epimerase deficiency, from mutations in the GALT, GALK, and GALE genes respectively

What is the first layer of the retina (what light hits first)

ganglion cell layer

Function of the reticular nucleus of the thalamus

gates and temporally inhibits sensory output from the thalamus so that distracting sensations do not reach conscious awareness while attention is focused in another sensory modality basically it helps keep other sensations from distracting you when you're trying to focus on one

Juvenile myoclonic epilepsy EEG

generalized 4-6Hz polyspike and wave, triggered by photic stim

occipital intermittant rhythmic delta activity

genetic generalized epilepsies, childhood absence epilepsy

newborns brain with only one ventricle and one eye. Dx? Associated gene?

holoprosencephaly all of them have anosmia (due to absent olfactory bulbs and tracts) sonic hedgehog gene

What gene is associated with periventricular heterotopia?

human filamin A -> X linked periventricular heterotopia

Fatal familial insomnia

human prion disease. Loss of neuron and gliosis in thalamus (AV and DM)

Ocrelizumab

humanized monoclonal antibody depletes CD20 B cells, preserves capacity for B cell reconstitution and preexisting humoral immunity

Muscle cells with thick-rimmed vacuoles are seen in...

inclusion body myositis (will often also see an actual "inclusion body" floating around in the cell)

EMG effects with cold

increase latency, duration, amplitutde, area of motor and sensory nerve action potentijals

What are the most common histopathological finding in myotonic dystrophy?

increased number of internal nuclei

According to the most recent AAN Evidence-based guideline: Management of unprovoked 1st seizure in adults, there is an _____ 2 year risk of recurrent seizures in the following settings: prior brain insult or lesion as the likely cause of the seizure, EEG with either spikes or sharp waves, significant brain imaging abnormality suspected as the etiology for the seizure, and nocturnal seizure. In this case, it is possible to attribute the development of his seizure to a prior bout of bacterial meningitis. Immediate treatment with an antiepileptic drug (AED) in the setting of any of the aforementioned risk factors _____ the risk of recurrent seizure over the ensuing 2 years by about 35%.

increased; decreases

Treatment for PH/HC

indomethacin

How does GLUT-1 mutation present phenotypically?

infantile-onset seizures, developmental delay, microcephaly, and complex involuntary movements

What triggers an exacerbation in PDH deficiency?

infections, stress, or high-carbohydrate meals

EOM used to look up and inwards towards nose

inferior oblique

EOM used to look down and out

inferior rectus

Damage to which area causes prosopagnosia (failure to recognize facial features)

inferomedial part of temporooccipital region

fasciculation potnetials

irritation of alpha motor neuron

This is a generalized epilepsy that typically presents during adolescence or early adulthood. Patients typically describe a history of morning myoclonic jerks, and both the generalized seizures and the myoclonic jerks commonly are precipitated by sleep deprivation and alcohol.

juvenile myoclonic epilepsy

Midle meningeal artery- foramen spinosum" Weber's syndrome

lesions at base of midbrain (cerebral peduncle) i/l CNII palsy, and c/l hemiparesis (CST)

Serotonin

low levels associated with depression

C8 root

lower trunk/medial cord from ulnar nerve is derived

Question 221: There is an ovoid homogeneously enhancing mass with adjacent vasogenic edema. A hemorrhage, hemorrhagic infarction or demyelinating lesion would not have this pattern of homogeneous enhancement. A giant aneurysm would not show this degree of edema. This pattern is most frequently seen with ______

lymphoma

metachromatic leukodystrophy

lysosomal storage disorder affects both CNS and PNS. Has demyelinating sensorimotor polyneuropathy

What do you see on pathology of Fabrys disease?

lysosomal storage of birefringent lipids, with membrane-bound lamellar deposits on electron microscopy.

Neuro sx of B12 def

macrocytic anemia, pancytopenia. Paresthesias, peripheral neuropathy, demyelination of CST and dorsal columns (subacute combined systems disease)

Temporal lobe epilepsy (clinical presentation)

mc type of partial epilepsy Auras include viscerosensory, gustatory, olfactory, auditory, fear oral/hand Automatisms, altered consciousness, deja vu, complex partial seizures, olfactory hallucinations mesial temporal sclerosis

Where is the supplementary motor area located?

medial aspect of the superior frontal gyrus, thought to be a pacemaker for speech output

What cortical areas are critical for gait?

medial frontal region, paracentral lobule, supplementary motor cortex

Episodic memory localizes where?

medial temporal lobe-Papez circuit system

What is recommended for an initial therapeutic trial to treat the REM behavior sleep disorder; clonazepam may also be effective as a first-line therapy

melatonin

The pain that occurs with this is often widely distributed and can include the low back, buttock, anterolateral thigh, and lateral knee regions. It is often aggravated by standing and relieved by sitting. The syndrome is often associated with obesity or pregnancy, and symptoms disappear with weight loss or delivery.

meralgia paresthetica

Which syndrome is associated with amotivation?

mesial frontal

MCC of intractable temporal lobe epilepsy?

mesial temporal lobe sclerosis (also known as hippocampal sclerosis) - A specific pattern of hippocampal neuron cell loss (thinning of cortical ribbon, atrophy) - 65% of cases - Caused by age, vascular risk factors (stroke, HTN, small vessel disease), lower SES

side effects of anti emetics

metoclopramide, chlorpormazine, prochlorperazine, long QT

"ragged red fibers" surrounding muscle cells on histology represents...

mitochondrial encehalomyopathies (ocular system is commonly involved in mitochondrial disease)

Dorsolateral association cortex

monitors, judges, faciliates and inhibits activity of complex cortical networks "executive function" of frontal lobes

What does the neural plate form?

neural tube

This typically occurs due to severe central stenosis of the lumbar spine due to spondylosis (degenerative disease of the spine, ligaments, and discs). The MRI of the lumbar spine in this patient will likely show severe central stenosis at one or multiple lumbar spine levels, with resultant bilateral polyradiculopathie

neurogenic claudication

The combined eye and spinal cord involvement in a young woman is suggestive of?

neuromyelitis optica (NMO

Biopsy showing curvilinear bodies suggests what diagnosis?

neuronal ceroid lipofuscinosis

What can you see on brain MRI?

neuronal migration abnormalities such as pachygyria and or polymicrogyria as well as white matter lipid accumulation

electrocerebral inactivity

no cerebral EEG over 2 microvolts

Idiopathic Intracranial Hypertension (IIH) occurs most commonly in ______ women of childbearing age

obese

Martin-Gruber anastomosis (how common?, consists of?)

occurs in 15% to 30% of the population. Consists of a communicating branch from the median nerve (usually from the anterior interosseous nerve) to the ulnar nerve in the forearm. It consists of nerve fibers destined for the first dorsal interosseous, most commonly, followed by the adductor pollicis and abductor digiti minimi

WNV encephalitis

older patients, mental status changes, movement disorders, basal ganglia, thalmus. Can cause flaccid paralytic syndrome similar to polio

Patient presents with optic nerve dysfunction and ocular motility deficits, where does this localize?

oribtal apex as optic nerve goes through optic canal and the rest go through the superior oribital fissure

Prominent manifestation of congenital deficit of dopamine-B-hydroxylase?

orthostatic hypotension (due to high levels of dopamine and undetectable levels of norepinephrine)

This is characterized by a high frequency postural tremor in the legs which is present exclusively with standing. It is often difficult to see, even for patients, and is reported as a sense of imbalance which is relieved by movement

orthostatic tremor

Which of the hypothalamic nuclei has direct outptut to the sympathetic system?

paraventricular nucleus

This tremor is classically a resting tremor usually with a rotational component. It may involve the legs. Individuals with this may suffer from orthostatic tremor in addition to their this tremor, but will have other related findings such as rigidity, bradykinesia, and gait disorder.

parkinson's disease (PD)

Although valproic acid is also associated with significant weight gain, it is not associated with this edema.

pedal

Common adverse effects related to pregabalin are?

pedal edema and weight gain

What are risk-factors for developing cerebral hyperfusion?

perioperatively poorly-controlled blood pressures high-grade stenosis history of recent endarterectomy on the contralateral side. The syndrome is suspected to result from loss of cerebral autoregulation resulting in hyperdynamic blood flow through the revascularized blood vessel.

Need pictures

pleds CJD SSPE HSV

Rabies virus

temporal limbic lobes, momeory loss, personality changes, seizures, death

T/F Patients with fragile X-associated tremor ataxia syndrome (FXTAS) are not likely to have Friedreich ataxia, dentatorubral-pallidoluysian atrophy, or spinocerebellar ataxia type 3 given his age, absence of other clinical features, and the history of mental retardation in his daughter's son

true

T/F Platelet transfusions have not be shown to reduce hemorrhage outcome

true

T/F Seventy-four per cent of patients with thymomas and myasthenia have circulating serum striational antibodies in addition to antiAChR antibodies

true

Another term for absence seizures

Pyknolepsy (3 Hz spike-wave discharges)

What is propionic acidemia caused by?

-deficiency of propionyl-coa carboxylase, requires conenzyme biotic

What other organs are involved in Fabrys?

-dermatologic -cardiac -renal -vascular -corneal

What is Menke's disease due to?

-disorder of intracellular copper transport -X link recessive

what enzymes most abundant in liver?

"CYP3A4 used to metabolize most drugs including endogenous substrates, steroid, fatty acids. CYP2D6 not inducible Carbamazepine causes hepatic enzyme induction of CYP2C9, CYP2C19, CYP3A4 phenytoin, phenobarbital causes hepatic enzyme induction of CYP2C9, CYP3A4, BUT NOT CYP2C19 Carbamazepine causes auto indcution which increases its metabolism

Claustrum, Head of caudate,Globus pallidus pars externa,putamen,nucleus accumbens

"Claustrum: thin sheet just deep to insula, between extreme and external capsules head of caudate between lateral ventricle and internal capsule Gpe between Gpi and putamen putamen between Gpe and external capsule nucleus accumbens: base of forebrain where caudate and putamen are in continuity

Thrombectomy trials

"ESCAPE 53% paients within 12 hours had mRS 0-2 REVASCAT 44% mRS 0-2 within 8 hours. DAWN 2 point different in mRS up to 24 hours

Lenticular nucleus

"GP medially, putamen laterally posterior limb of IC traverses between GP and lateral subnuclei of thalamus

4-5 day history of HA, malaise, personality change, anterograde memory deficit, fever

"HSV-1 encephalitis until proven otherwise. Acyclovir IV 10mg/kg q8hr for 3 weeks usually medial temporal lobe and orbotalfrontal. Less commonly cingulate gyrus

Order of involvement of EOM in patients with Grave's ophthalmopathy?

"IM SLOW" inferior rectus medial superior lateral oblique (no w)

Lateral cutaneous n

"L2 and L3, numbness to lateral thigh, meralgia paresthetica. Weakness in knee extension and numnbess localizes to lumbar plxus of L2 and L3 roots

" Neuropsycho deficits in MS vs AD

"MS: subcortical dysfunction, information processing efficiency, learning of new information, working memory. AD: impairments in encoding information, visualspatial skills, sematic fluency, orientation to time

" ataxia, ophthalmoparesis, areflexia

"Miller-Fisher syndrome of AIDP. Anti-Gq1b antibody. DDx: Purkinje cell antigen- paraneoplastic cerebellar ataxia

" PDD vs LBD

"PDD: symptoms of PD more than 1 year before dementia. LBD: if seperated by less than one year

" treatment of levodopa induced dyskinesiase

"amantadine can increase dyskinesias when added to levodopa: pramipexole, ropinirole, apomorphine (direct DA agonists)

" Canavan disease

"aspartoacylase disorder: spongiform leukodystrophy only in the brain Krabbe disease (galactosylceramidase) and metachromatic leukodystrophy (arylsulfatase A) have deposits of sphingolipids

Myotonic dystrophy type 1 DM1

"autosomal domionant disorder, also affets endocrine abn, cataracts, sleep disturbancees, cardiac condition. Caused by CTG repeat expansion in 3' untranslated region of myotonic dystrophy protein kinase (DMPK) gene. Molecular mechanism for myotnia in DM1- reduction in both functional sarcolemmal CIC-1 and due to CUG repeat containing mRNA sequestering muscle blind like 1 (Mbn11) prtoeins required for proper CLCN1 premRNA splicing and chloride channel function

acute motor axonal polyneuropathy AMAN

"axonal form of GBS CMV associated with AIDP

Myelination

"begins in peripheral nervous system. Motor roots myelinate first. Before birth in CNS. Commissures continue after birth. Subcortical U fibers myelinate around 8 months of life, mature by 18 months

Musculocutaneous nerve, Anterior interosseous nerve, Posterior inerosseous nerve, Axillary n., Median n.

"biceps brachii flexor pollicis longus extensor indicis proprios deltoid pronator teres

central cord syndrome

"caused by syrinx, loss of pain and temperature at level of lesion 2/2 crossing spinothalamic fibers in ventral commisusure. Posterior columsn are spard large syrinx may compress anterior horn gray matter

Ocular tilt reaction

"compensatory movements of eyes and head in opposite direction when tilting body to one side. The ipsilateral utricle and anterior and posterior semicircular canals are activated--> AP in i/l vestibular nerve--> vestibular nucleus--> decussate and synapse on vertical eye movement centers in midbrain. i/l eye elevates and intorts vis SR. c/l eye will depress and extort via IR. lesions to vestibular nucleus will cause head tilt towards the lesion i/l eye will depress/extort, c/l eye will elevate and intort. CNIV palsy: elevation and extorsion of i/l eye, will have c/l head tilt

Vein of Labbe

"courses across temporal lobe connects superficial middle cerebral vein with transverse sinus basal vein of Rosenthal drains ventral surface of brain and joints great cerebral vein of Galen prior to formation of straight sinus

Anterior spinal artery

"descending corticospinal tracts and ascending spinothalamic tracts dorsal columns are preserved

inferior pareital lobule consist of supramarginal gyrus and angular gyrus

"dominant hemisphere lesions can cause Gerstmann syndrome dorsolateral frontal cortex: Broca's in dominant hemisphere superior parietal lobule: multimodal sensory projections: visuospatial orientation superior temporal lobe: language function on dominant side

CMAPS in lambert eaton syndrome

- Facilitation of CMAP after exercise - Facilitation of CMAP after repetitive stimulation (20-30 Hx) - Decrement of CMAP after slow repetitive stimulation (2-3 Hz)

AEDs for atypical absence, myoclonic, and atonic seizures

- Valproic acid - Lamotrigine - Topamax

Keppra side effects

- "keppra rage", mood issues. Avoid if bipolar - Renally eliminated

PFO management

- 20% of general population have a PFO - 50% of young stroke patients have it - Trial data has been inconclusive regarding closure vs medical management (antithrombotics) - except in setting of DVT or hypercoaguable states. - if a DVT is present, then it would be reasonable to consider tx of warfarin on the presumption of a paradoxical embolism

INO (what does it look like, cause)

- Affected eye cannot adduct - Unaffected eye abducts with nystagmus Due to injury to or dysfunction in the MLF (function is to connect the PPRF-CN VI complex of the contralateral side to CN III of ipsilateral side) Causes: MS, stroke

Symptoms of TCA overdose

- Anti-cholinergic: dry mouth, blurry vision, retention, tachycardia, constipation, confusion, delirium, seizures, fevers - Anti-histamine: sedation, dry mouth, N/V, appetite change, weight gain - Anti-adrenergic: orthostatic hypotension - Cardiac: Qt prolongation

Imaging findings for PSP

- Atrophy of midbrain tegmentum (hummingbird sign) - Atrophy of corpus callosum - Atrophy of anterior cingulate gyrus

Von Hippel Lindau - inheritance, symptoms/manifestations

- Autosomal dominant familial cancer syndrome - Spinal cord hemangioblastomas - Retinal angiomas - RCC - Pancreatic cysts - Epididymal tumors VHL-2: +pheochromocytoma VHL-1: no pheo

Which three arteries come off of the aortic arch?

- Brachiocephalic - Left common carotid - Left subclavian

Hypoglycemia and initial stroke management

- Can present with signs of focal neurologic dysfxn and mimic stroke - Should ALWAYS be checked before IV TPA administration - MRI, Echo and CXR and NOT required before TPA

AEDs that can CAUSE seizures in children with absence seizures

- Carbamazepine - Oxcarbazepine - Phenobarbital - Phenytoin - Tiagabine - Vagabatrin

AEDs that can CAUSE seizures in ppl with GTCs

- Carbamazepine - Phenytoin

AEDs that are enzyme inducers (3)

- Carbamazepine - Phenytoin - Phenobarbital

3 AEDs that are not removed by dialysis

- Carbamazepine - Valproic acid - Phenytoin

Phenytoin side effects? Where is it metabolized?

- Cardiotoxic - Hypotension - Ataxia - Nystagmus - Gingival hyperplasia - Decreased bone density (get annual DEXA) - metabolized by liver -CYP450 inducer (fosphenytoin is the metabolite of phenytoin; when checking levels of either, check phenytoin level)

Localize: Ipsilateral Horner and neck pain w/ contralateral hemiparesis

- Carotid dissection - trauma, fibromuscular dysplasia

What is the corpus striatum composed of (2)

- Caudate - Putamen

Superficial siderosis

- Deafness - Cerebellar ataxia Hemosiderin deposition in subpial layers of brain and spinal cord --> causes damage by slow bleeding into subarachnoid space MRI demonstrates hemosiderin deposition along upper cerebellar folia

Multiple system atrophy (Shy Drager syndrome) symptoms

- Death of neurons in substantia nigra, striatum, inferior olive, and cerebellum; possibly caused by a prion form of a-synuclein protein; ~50-60yo - Parkinsonism - Autonomic dysfunction (orthostatic hypotension, impotence, loss of sweating, dry mouth, retention) - Ataxia/cerebellar dysfunction

Causes of burst suppression

- Deep anesthesia - Drug overdose - Severe hypothermia (usually fatal)

Hyperintensity on DWI in bilateral thalami. DDx?

- Deep venous thrombosis (abnormal MRV) - Basilar artery ischemia (abnormal MRA) - Wernicke's/thiamine deficiency

Side effects/adjustments for gabapentin & pregabalin

- Drowsiness - Adjust for renal failure

Trial that demonstrated benefit of TPA b/w 3-4.5 hrs

- ECASS 3 trial - Addition of endovascular tx, IA TPA and thrombectomy have not demonstrated superiority to IV TPA alone (SYNTHESIS, MR RESCUE, IMS III)

Periodic generalized waves on EEG: - Every 4-15 seconds - Every 1 second

- Every 4-15 sec: SSPE - Every 0.5-1 sec: CJD (will also have sharp waves= myoclonus)

What is Alexander's disease?

-Pediatric disease, progressive disorder of astrocytes caused by mutations in the gene for glial fibrillary acidic protein

Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) symptoms

- Initial Sx: loss of balance, lunging forward when mobilizing, fast walking - changes in personality - Bradykinesia/Parkinsonism without tremor - Later: dementia, dysarthria, dysphagia - Scared/suprised look on face - Mild slowing of horizontal eye movements - Vertical ophthalmoplegia (cannot look up) - Slowing of downward eye movements - Axial rigidity (neck flexion/extension rigidity)

Treatment of acute cerebral venous thrombosis

- Initial anticoagulation with heparin is the standard treatment for acute CVT. This holds even in the setting of ICH or hemorrhagic stroke.

A lesion at the base of the midbrain would cause...

- Ipsilateral CN III palsy - Contralateral hemiparesis AKA Weber's syndrome

Broad spectrum AEDs (used for both focal and generalized onset)

- Keppra - Brivaracetam - Topamax - Depakote - Lamictal - Zonisamide - Clobazam - Felbamate - Perampanel - Rufinamide

Two renally excreted AEDs

- Keppra - Topamax (70%)

AEDs for focal/partial onset seizures

- Keppra (broad spectrum) - Lamotrigine (broad) - Valproic acid (broad) - Zonismide (broad) Works for focal only: - Carbamazepine - Oxcarbazepine - Phenytoin - Gabapentin - Pregabalin - Lacosamide (vimpat) - Phenobarbital - Tiagabine - Vagabatrin

Geniculate ganglion (from CN VII) innervates...

- Lacrimal gland (tears) - Taste - anterior 2/3 of tongue - Sublingual and submandibular gland

Which three AED's can trigger status epilepticus if used to treat absence seizures?

- Lamotrigine - Phenytoin - Carbamazepine

AED's for generalized seizures

- Lamotrigine (but can worsen JME) - Topamax - Valproic acid - Keppra

Signs of posterior circ stroke in pt w/ acute onset vertigo

- MRI may be normal within first 48 hrs - Presence of either skew eye deviation, normal head impulse testing, or direction changing nystagmus in eccentric gaze is highly sensitive and specific for acute brainstem or cerebellar stroke

DDx for ring-enhancing lesions on MRI (7)

- Metastasis - Glioma - Abscess - Infarction - Contusion - Demyelination - Radiation necrosis

Cingulate (subfalcine) herniation

- Most common - Innermost part of frontal lobe is scraped under part of falx cerebri (part of dura at top of brain that separates 2 hemispheres) - Occurs when one side of brain swells and pushes the cingulate gyrus past the falx cerebri - Can smash ACA vessels --> elevated ICP --> Can progress to central herniation

Topamax mechanism of action (4)

- Na channel blocker - Enhancement of GABA mediated Cl influx - Inhibits AMPA subtype glutamate receptor - Weak carbonic anhydrase inhibitor (good for pseudotumor/IIH)

Vimpat side effects

- Nausea (24%) - Dizziness (53%) - Headache (16%) - Diplopia (11%) - Scariest: arrhythias

CMT2

- Onset 20+ yo - milder Sx than CMT1 - Less involvement of upper extremities - EDX: axonal features

Lateral Medullary Syndrome

- PICA stroke - ipsilat facial anesthesia, contralateral body anesthesia - ipsilat horners - vertigo, dysarthria, hiccups - Ipsilateral cerebellar dysfxn

Optic neuritis presentation and exam

- Painful, unilateral, loss of visual acuity - Edematous optic disc (blurry margins)

B12 deficiency causes deterioration of which portions of spinal cord

- Posterior columns - Lateral CST

HNPP (hereditary neuropathy w/ predisposition to pressure palsy)

- Recurrent episodes of painless mononeuropathies - Peroneal (at fibular head), ulnar (at elbow), radial (at humerus), median (at wrist) - Autosomal dominant - PMP22 gene deletion

Epidermoid cyst on MRI

- Same color as CSF on all sequences, EXCEPT: bright on DWI (csf would be dark) - Has a subtle internal structure (compare to arachnoid cyst which would be same color as CSF in ALL sequences)

Extramedullary intradural spinal tumors

- Schwannomas - Neurofibromas - Spinal meningiomas

Prevention of DVT in stroke

- Stroke patients are at high risk due to age, immobility, and if they have risk for thrombosis. - Compression stockings, SCDs, anticoagulants (24 hrs after tPA)

Common exam/imaging findings in patients with Tuberous Sclerosis

- Subependymal giant cell astrocytomas - Cortical tubers - Subependymal glial nodules - Cutaneous angiofibromas - Cardiac rhabdomyomas - Pulmonary lymphangioleiomyomatosis - Renal angiomyolipomas

Which muscle is not innervated by the anterior interosseus nerve: - Pronator quadratus - Supinator - Flexor pollicis longus - Flexor digitorum profundus

- Supinator (deep branch of radial nerve, C5-C7)

How does MRI look for metachromatic leukodystrophy?

- T2 hyperintense signal changes in periventricular and subcortical white matter, SPARING the U fibers -cerebellum can be involved

Motor tracts of spinal cord (4) and their functions

- Tectospinal tract = reflex postural movements in response to visual stimuli (you de"tect" a visual stimulus and react) - Rubrospinal tract = flexor tone (rubro sounds like "red", like a man flexing and giving you a red rose showing off) - Vestibulospinal tract= extensor tone (wearing a vest keeps your back extended) - Reticulospinal tracts= withdrawal responses to noxious stimuli (tic bites hurt/noxious)

Delta sign

- Triangular / round area of hyper density at the posterior aspect of the superior sagittal sinus on a non contrast head CT. - indicator of sagittal sinus thrombosis. - Risk factors: OCPs, pregnancy, malignancy, hypercoaguable disorder - best tx: IV heparin, or LMW heparin - Warfarin does not have an acute anticoagulant effect

Central cord syndrome (manifestations, causes)

- UE > LE weakness (cannot move arms) - Hyperreflexia in LE - Variable decreased sensation below level of injury - Spares vibration and proprioception MCC: syringomyelia and spinal cord tumors

AEDs for absence seizures

- Valproic acid - Ethosuximide

Cerebral hyperperfusion syndrome

- seen after revascularization of an artery (surgery / stenting) - headache, neurologic deficit, seizure - Risk factors: periop poorly controlled BP, high grade stenosis, recent hx of CEA on contralat side. - Suspected to result from loss of cerebral auto regulation resulting in hyper dynamic blood flow through revascularized blood vessel.

Localize: ipsilateral CN V, VI, VII, Horner's syndrome

- spontaneous hemorrhage into tegmentum of pons

AED that is a big enzyme inhibitor

- valproic acid

Mural thrombus and stroke

-2-6% of pets with anterior MI - Usually within 2 weeks of MI

When does the adult onset start? What symptoms develop? What storage material accumulates?

-30 years of age -characterized by cognitive and behavioral disturbances; dementia ensues, ataxia, extrapyramidal symptoms (facial dyskinesias), myoclonic seizures -visual failure does not occur in the adult form -combination of fingerprint bodies, rectilinear profiles and granular osmiophilic deposits

LGS

-A variety of generalized seizures such as astatic seizures (drop attacks), tonic seizures, tonic-clonic seizures, atypical absence seizures, myoclonic (sudden muscle jerks), and, less frequently, focal seizures with impairment of consciousness. -Developmental delay/cognitive dysfunction -EEG showing slow spike-wave complexes of 2 Hz

How many types of Neiman-Pick disease are there?

-A: involves the CNS as well as other viscera and manifests in infancy with feeding difficulty, failure to thrive, hypotonia, head lag, inability to sit, and eventual psychomotor retardation with regression. + CHERRY RED SPOT, hepatosplenomegaly, usually do not survive past age 3 -B: ONLY visceral and does NOT affect the CNS, presenting with hepatosplenomegaly and interstitial lung disease. May present in mid-childhood and may survive into adulthood. -C: Filipin test positive (impaired ability of cultured fibroblasts to esterify cholesterol.)

A 2 year old girl has infantile spasms, speech delay and MRI shows absent corpus callosum, what is the disorder?

-Aicardi's syndrome -X linked dominant, lethal in males -agenesis of corpus, etinal lacunae, developmental disabilities

What is pseudoxanthoma elasticum?

-Gronblad-strendberd syndrome -connective tissue disorder in which yellowish xanthomas occur in various skin regions and mucous membranes -pigmentary changes in retina result in peu d'orange skin and can causes hemorrhages in the retina -autosomal dominant or recessive

What mutations are associated with septo-optic dysplasia?

-HESXI, homeobox, SOX genes -often associated with multiple endocrine abnormalities

What is another name for Menkes disease an what can it often be mistaken for?

-Kinky hair syndrome -for child abuse

Which pediatric disorders spare the U fibers?

-Krabbe, metachromatic leukodystrophy, adrenolukodystrophy Pelizaeus-Merzbacher disease

Characteristics of NF1

-Neurofibromas all over skin -Cafe-au-lait spots (brown) -Lisch nodules on iris -Retinal tumors/blind -Neurofibromas rarely turn into cancer

What is Lesch-Nyhan disease?

-X linked -caused by deficiency of the enzyme hypoxanthine guanine phosphoribosyltransferase, which participates in the salvage pathway of purine metabolism -encoded on HPRT1 on chromosome Xq26

How is adrenoleukodystrophy inherited?

-X linked -mutations in ABCD1 gene encoding for peroxismal membrane transporter (ABC transporter)

What is Fabry's disease?

-X-linked disorder -caused by deficiency of the enzyme α-galactosidase, resulting in accumulation of ceramide trihexoside in epithelial, mesenchymal, and neural cells.

What enzyme deficiency causes Niemann-Pick types A and B? What is its mode of inheritance?

-acid sphingomyelinase deficiency and accumulation of shingomyelin -autosomal recessive

How are the mucopolyssacharide diseases inherited?

-all AR minus Hunter's is X linked recessive

What is Osler-Weber-Rendu syndrome?

-also named hereditary hemorrhagic telangiectasia -autosomal dominant that cuses telangiectasias int he skin, mucus membranes, retina, GI tract, AVMs -mutation in HHT1 gene on chromosome 9 that encodes endoglin

The inferior parietal lobule is composed of what gyri?

-angular gyrus -supramarginal gyrus

Which gyrus causes Gerstmann's syndrome? What artery is it supplied by?

-angular gyrus of dominant parietal lobe -angular artery, branch of the MCA

How does propionic acidemia present?

-appear normal at birth but will develop symptoms in the early neonatal period, in infancy, or later in childhood -presents with feeding difficulty, hypotonia, dehydration, and attacks of metabolic acidosis with ketosis and hyperammonemia -can have seizures and coma -can have hepatomegaly, pancytopenia, and bleeding disorders including intracranial hemorrhage -Patients who survive have developmental delay and involuntary movements (resulting from basal ganglia involvement)

Metachromatic leukodystrophy is caused by defieciency in what enzyme?

-arylsulfatase A -accumulation of sulfatide resulting in demelination of the central and peripheral nervous system -prosaposin gene (PSAP gene), leading to deficiency of a sphingolipid activator protein (SAP-B or saposin B) that normally stimulates the degradation of sulfatides by arylsulfatase A

What neurotransmitter is secreted by the climbing fibers of the cerebellum?

-aspartate -they project from the inferior olivary nucleus located at the medulla to the deep cerebellar nuclei and Purkinjie cells

What is the mode of inheritance of tuberous sclerosis?

-autosomal dominant with incomplete penetrance -TSC1 codes for hamartin, chr 9 -TSC2 codes for tuberin, chr 16

What is the mode of inheritance of propionic acidemia

-autosomal recessive

What is maple syrup urine disease?

-autosomal recessive -caused by branched-chain (leucine, isoleucine, avline) α-ketoacid dehydrogenase complex deficiency

How is Tangier's disease inherited?

-autosomal recessive, causes a familial neuroapthy and caused by adenosine triphosphate cassette transporter mutation resulting in deficiency of high density lipoprotein and low serum cholesterol and high TG content

What are the neuro psych manifestations of NF1?

-behavior -learning disabilities -they do not have severe developmental delay

When does the juvenile form develop? What mutation is involved? What are the symptoms? What products accumulate on EM?

-between 4 and 10 years of age -CLN3 mutation -visual loss, seizures, myoclonus, psychomotor retardation, and focal neurologic deficits -fingerprint bodies, which are seen by electron microscopy.

What are the clinical features of Menke's disease?

-brittle coarse and lightly pigmented hair -hyperelastic skin -thin or absent eyebrows -seizures, developmental delay, cerebral vasculopathy, subdural hematomas or hygromas

What are the categories of malformations of cortical development?

-cell proliferation -migration -cortical organization cortical development not otherwise classified

What kind of deficits to patients with Zellwegers have?

-cirrhosis -polycystic kidney disease -chondrodysplasia punctata (bony stippling of the patella) -cardiac abnormalities (VSDs) -cataracts -retinal dystrophy -sensorineural hearing loss -severe hypotonia -decreased sucking and crying -hyporeflexia -deformities in flexion of the lower limbs with arthrogryposis -intellectual impairment -seizures

How does the infantile form of metachromatic leukodystrophy present?

-clumsiness, frequent falls, and slurred speech, and is associated with weakness and hypotonia -motor regression and UMN signs -vision and hearing loss -peripheral neuropathy -mental dysfunction -> vegetative state -> death

What do you see on EEG for infantile epileptic encephalopathy (Ohtahara syndrome)?

-combination of tonic spasms and burst suppression on EEG -this frequently evolves into West syndrome at about 4-6 months

What a\re the congenital disorders of glycosylation?

-group of genetic disorders inherited in an autosomal recessive fashion -***lipodystrophy with prominent fat pads in the buttocks and suprapubic area, inverted nipples*** <- hallmark -type 1: abnormal synthesis of glycans. Failure to thrive, developmental delay cerebellar atrophy, dysmorphic features, hypotonia, ataxia, weakness, retinitis pigmentosa, short stature with skeletal abnormalities, polyneuropathy, stroke-like episodes, liver dysfunction, hypogonadism, and multisystem involvement -type 2: abnormal processing and modifiication of glycans that lead to profound intellectual impairment but no cerebellar ataxia and peripheral neuropathy

What is Pelizaeus-Merzbacher disease?

-hypomyelinating leukodystrophy inherited in an X-linked recessive fashion -PLP gene

Earliest evidence of return of neurologic fun

-increase in tone - this occurs before improvement in strength or return of reflexes.

Uncal herniation

-innermost part of temporal lobe -Compression of CN III (blown pupil, eye down & out) -Contralateral homonymous hemianopsia (compression of PCA) -Ipsilateral hemiparesis (to due compression of contralateral cerebral crus CSTs)

How do you manage PDH?

-ketogenic diet (high fat with low carbohydrates) and thiamine supplementation. Carnitine, coenzyme Q10, and biotin supplementation may be given, but their efficacy is not well established -treatment with acetazolamide during attacks

What are two main features that make acute intermittent porphyria more likely than variegate porphyria and hepatic coproporphyria?

-lack of cutaneous manifestations -absence of increased coproporphyrin III in urine and stool

What are the three forms of metachromatic leukodystrophy?

-late infantile form with onset between 6 months and 2 years (most common) -juvenile form with onset between 3 and 16 years -an adult form with onset after 16 years of age.

Eosinophilic granular bodies (EGBs) are generally found in tissue preparations from which of the following conditions?

-low grade gliomas

What is the treatment of maple syrup urine disease?

-low-protein diet -start on thiamine

What is sialidosis?

-lysosomal storage disorder -autosomal recessive -accummulation of oligosaccharides, glycopeptides, glyoclipids

Smith-Lemli-Opitz syndrome presents how?

-micoencephaly, hypoplasia of frontal lobes, abnormal gyration, cerebellar hypoplasia -autosomal recessive, disorder of cholesterol synthesis

What is Kearns-Sarye syndrome?

-mitochondrial disorder by large scale DNA mitochondrial deletion, less commonly can be caused by duplication -diagnosis is made with triad of progressive external opthalmoplegia, onset before age 20 -either short stature, pigemntary retinopathy, cerebellar ataxia, heart block, increased CSF protein

Periventricular nodular heterotopia is a disorder of what?

-neuronal migration -nodules of gray matter lining the ventricles and extending to the lumen -most common presentation is seizures

AD nocturnal frontal lobe epilepsy is caused by a mutation in what?

-neuronal nicotinic acetylcholine receptor -autosomal dominant inheritance pattern

Dejerine-Sottas (CMT3)

-onset in childhood - motor developmental delay present - hypotonia - EMG: severe slowing on NCS (<10m/s) - Pathology: more pronounced demyelination and onion bulbs

What is Zellweger's disease? What are the mutations due to?

-peroxismal disorder in which white matter is involved -you find increased plasma very long chain fatty acids -mutations in the PEX genes, the majority with PEX1 resulting in abnormal peroxisomal biogenesis.

What is the enzyme deficiency in phenylketonuria (PKU)?

-phenylalanine hydroxylase deficiency -converts phenylalanine to tyrosine

What is the parasympathetic nervous system important for?

-pupillary constriction -bronchoconstriction -detrusor contraction -sphincter relaxation -penile erections

What are the other non-neurologic manifestations of NF1?

-renal artery stenosis due to renal artery dysplasia -> hypertension -pheochromocytoma -thinning of cortex of long bones and other long bone dysplasias -> fractrues and pseudoarthrosis, scoliosis, sphenoid wing dysplasia -of note: you can have moyamoya syndrome and other intracranial arterial abnormalities including intracranial aneurysms, macrocephaly, aqueductal stenosis

What opthalmologic manifestations occur with TSC?

-rentinal hamartomas (classic mulberry-like lesions near optic disc, including plaques or depigmented lesions) -retinal detachment or hemorrhage into the vitreous

What is treatment for Fabrys?

-replacement of α-galactosidase

What is the tetrad of chronic bilirubin encephalopathy?

-severe athetosis -high tone deafness -supranuclear palsy of upward gaze -enamel hypoplasia of the incisor teeth

What happens in incontinentia pigmenti?

-skin involvement occurs in stages including vesiculobullous lesions present at birth, verrucous lesions that appear at approximately 6 weeks of age, and then hyperpigmented lesions that follow the Blaschko lines (lines of skin development) -hyperpigmentation decreases over time, with tendency to disappear or become hypopigmented and atretic later in life -neurologic manifestations include intellectual impairment, pyramidal tract findings, and ocular abnormalities -X-linked dominant, only affects females, lethal in males -caused by mutation in the NEMO gene, which encodes a protein involved in the nuclear factor κ B pathway

What is the difference between spasmus nutans and opsoclonus

-spasmus nutans is a combination of dysconjugate, highly variable nystagmus, head nodding and head tilt without optho abnormalities -opsoclonus describes chaotic, conjugate saccades

The straight sinus receives blood supply from where?

-superior and inferior sagittal sinus -great cerebral vein of Galen (which gets blood supply from basal vein of Rosenthal)

What structures receive bilateral auditory input?

-superior olivary nucleus, lateral lemniscus, medial geniculate body, superior temporal lobe

What is the co factor for phenylalanine hydroxylase?

-tetrahydrobiopterin -deficiency causes hyperphenylalanimeia

What is the most prominent efferent pathway from the amygdaloid nuclear complex?

-the stria terminalis

How can you treat renal angiomyolipomas in regards to TSC?

-they are benign tumors consisting of vessels, smooth muscle, fat, can occur in more than half of patients with TSC -treat with rapamycin (inhibits their growth) -renal cell carcinoma can occur -renal cysts can also occur -surveillance with periodic renal ultrasonography is recommended

Latissimus dorsi is innervated by what nerve? It is a branch of what cord of the brachial plexus?

-thoracodorsal nerve -posterior cord

How many phenotypes of maple syrup urine disease are there? and what are they?

-three types. -classic: most severe, presents in the neonatal period with lethargy, poor feeding, hypotonia after ingestion of protein. Progressiv enecephalopathy developed with opisthotonus and abnormal movements, can have coma and respiratory failure, cerebral edema and seizures -> death -intermediate: late infancy with developmental delay, failure to thrive, ataxia, seizures. Exacerbations with protein intake or intercurrent infectious. Some response to thiamine -intermittent: normal between episodes, alteration of consciousness, ataxia

Can cardiac rhambdomyomas occur in tuberous sclerosis complex?

-yes, single or multiple can occur, they can regress over time -can have heart failure due to obstruction or cardiomyopathy, arrhythmias, stroke from cerebral embolization

Age of bleed on CT

1-2 days: bright 2 wk-2 months: isodense > 2 months: dark

Treatment of West Syndrome (infantile spasms) & what do these seizures look like

1. ACTH (first line) 2. Vagabatrin (2nd line) - big issue: macular edema, macular degeneration They are like 1 second flexion of body (if sitting up they will do a quick abdominal crunch/head will drop down; or arms can flail up in the air "Salaam attacks"; if laying down they'll lift both legs in the air). Occurs for a split second every like 5-10 seconds Can go away completely; 60% develop epilepsy later in life (sometimes turns into Lennox Gastaut)

two MCC of non-traumatic brain hemorrhage

1. Hypertensive hemorrhage 2. Cerebral amyloid angiopathy

This individual presents with typical clinical, MRI and CSF features for HSV encephalitis. Early recognition of this disease is imperative because of the availability of effective antiviral treatment. Spinal fluid HSV-PCR is ___% specific and ___% sensitive for HSV encephalitis, but may be falsely negative in the initial __ hours

100; 95; 72

Synnuclein is on chromosome...

14

absence seizures EEG

3 Herz spike and wave

Substance DEPENDENCE criteria

3 out of the following in a 12mo period: - Tolerance - Withdrawal - Using more/longer than intended - Trying to cut back - Large amount of time spent trying to obtain/using/ recovering from use - Continued use regardless of physical/mental health problems

Febrile seizures (incidence, risk factors, likelihood to develop into epilepsy, treatment)

3-5% of children age 6 months to 5 years 30% have second seizure Risk factors for 1st FS: family history of FS, prolonged neonatal ICU stay, developmental delay, day care Tx: supportive for one or more simple febrile seizure, may consider prophylaxis with diazepam, antipyretics (short-term) phenobarbital or VPA (long-term) if recurrent, prolonged, afebrile, complex, developmental delay

At what weeks does neurulation occur?

3-6 weeks

When does methemoglobin appear on imaging?

4 days after hemorrhage (lasts up to 2 months --> after that it's hemosiderin) (1 day: oxyhemoglobin) (2 days: deoxyhemoglobin)

Patients with stroke with elevated cardiac troponin levels had strokes involving what structures?

50% had lesions involving the right posterior, superior medial insula, right inferior parietal lobule -in right MCA strokes, the insular cortex was involved in 88% of patients with elevated serum cardiac troponin and only 33% had no elevation. "Neuroanatomic correlates of stroke-related myocardial injury Neurology 2006, Ay H, Koroshetz)

Likelihood of progressing to MS within 10 years after CIS?

56%

Average age of diagnosis of Parkinson's

62

A positive NMO IgG antibody (aquaporin-4) is____% sensitive and ___% specific for NMO

73; 91

which cranial nerves exit through the jugular foramen?

9, 10, 11

Different phases of sleep (NREM and REM)

90 minute cycles 4-6x per night NREM (3/4): - N1: drowsiness, slow eye movements alpha drops out, lower amplitude or replaced with theta, enhanced beta - N2: light sleep; K-complexes and spindles - N3: deep/slow wave sleep: delta predominance REM (1/4): - Low amplitude - Central sawtooth waves

Medial medullary (Dejerine) syndrome

<1 % of vertebrobasilar strokes; anteromedial branch of vertebral artery or ASA; basilar artery - Ipsilateral CN XII palsy - Contralateral hemiparesis - Contralateral lemniscal sensory loss Variable manifestations: - Isolated hemiparesis - Tetraparesis - Ipsilateral hemiparesis - Ipsi or contra facial palsy - Ataxia, vertigo, nystagmus, dysphagia

Somatic symptom disorder

>1 somatic symptoms that are distressing or result in a significant disruption of daily life. High level of anxieyt about health , persistnely symptomatic >6 months

" Down syndrome and AD

>75 of Down syndrome will develop AD due to amyloid precursor protein APP on chromosome 21. almost all will have amyloid plaques and tau tangles.hippocampal atrophy

Best view to see mesial temporal sclerosis on imaging?

Coronal view - Bright on T2 / T2 FLAIR

Temporal dispersion (on EMG) (definition? seen in?)

A reduction in proximal CMAP amplitude compared with distal CMAP amplitude when the proximal CMAP duration increases by >20% Seen in Guillain-Barre syndrome

Claude syndrome (cause, manifestations)

A type of midbrain syndrome due to infarction of RED NUCLEUS, brachium conjunctivum, and CN III - Ipsilateral CN III ***- Contralateral ATAXIA *** - Contralateral hemiparesis - Contralateral hemiplegia of the lower face, tongue, shoulder

Raymond syndrome

A ventral pontine syndrome Unilateral lesion of ventral medial pons --> affects ipsilateral abducens nerve + CST. Spares CN VII - CN VI palsy - Contralateral hemiplegia that spares face

Millard-Gubler Syndrome

A ventral pontine syndrome Unilateral lesion of ventrocaudal pons -- can involve basis pontis, VI, VII - Contralateral hemiplegia, spares face - Ipsilateral CN VI palsy - Diplopia that's worse when patient looks towards side of lesion - Ipsilateral peripheral CN VII paresis

What is the validated tool which accurately assigns the 7-day risk for severe stroke and recurrent transient ischemic attack (TIA) after an initial TIA. The studies validating the this showed a 7-day hazard ratio of 2.64 for major stroke 39 with TIAs of duration greater than 60 minutes. TIAs associated with age less than 60 years, systolic blood pressure less than 140; or diastolic BP less than 90 mmHg; diabetes; and clinical features of unilateral weakness or speech disturbance also confer an increased 7-day risk for major stroke.

ABCD2 score

Epilepsy Syndrome: Treatment for West Syndrome?

ACTH - but tuberous sclerosis is excluded Vigabtrin - choice of tuberous sclerosis Prednisone

" treatment to improve global cognitivei in Alzheimers and diffuse Lewy body disease?

AChEIs such as rivastigmine, donepezil, galantamine

Glut1 Deficiency Syndrome

AD, SLC2A1mutation in glucose transporter protein type 1 sx: seizures begin around first month tx: ketogenic diet

Generalized epilepsy with febrile seizures plus (GEFS+)

AD, has been linked to multiple genes including *SCN1A* (possible link to Dravet's?) Onset: Around 2 y.o. (but can range from 4mos-10yrs) Clinical Presentation: Afebrile and febrile seizures that continue beyond age 5. Afebrile sz types can include GTC, CPS, absence, myoclonic EEG with polyspikes or generalized spike-wave. No disease specific findings tx: Valproate Prognosis: Good in most children with resolution of seizures around 11 years of age. However, about 30% will have more severe epilepsy.

Occlusion of which artery can cause vertigo and deafness?

AICA (b/c it branches into internal auditory artery and anterior vestibular artery)

Types of motor neuropathies (7)

AIDP/CIDP Acute motor axonal neuropathy Multifocal motor neuropathy CMT Lead intoxication Porphyric neuropathy Diabetic lumbar radiculoplexopathy

Tonsillar herniation

AKA "downward cerebellar herniation" through the foramen magnum - Intractable HA - Head tilt - Neck stiffness (tonsillar impaction) - Decreased LOC - Flaccid paralysis AKA Chiari malformation Causes: - Decreased/malformed posterior fossa -Hydrocephalus, abnormal CSF volume - Ehlers Danlos syndrome

Pachygyria

AKA "incomplete lissencephaly" certain areas of the brain do not have gyri so they look very smooth/homogenous

Degeneration/pallor of CST in spine with sparing of posterior columns and spinocerebellar tracts. Dx?

ALS

Lesions to the anterior lobe of cerebellum cause

Ataxic gait

Olivopontocerebellar atrophy on MRI

Atrophy of belly of pons and cerebellum

Metachromatic leukodystrophy is caused by the deficiency of which enzyme?

Arylsulfatase A

Artery of percheron infarction

AOP is an uncommon anatomic variant -- a single dominant thalamoperforating artery supplies the bilateral medial thalami Occlusion causes: - altered mental status - vertical gaze palsy - amnesia If it also hits the rostral midbrain its called "thalamopendiular syndrome"

Progressive myoclonic epilepsy (Sialidosis)

AR deficiency of lysosomal enzymes, can be demonstrated in leukocytes and cultured fibroblasts, also high urinary sialyloligasaccharides Type 1: Deficiency of alpha-neuraminidase, action myoclonus, slowly progressive ataxia, vision loss and GTC in adolescents/adults, no mental decline or dysmorphism, positive CHERYY RED SPOT Type 2: Deficiency of N-acetyl neuraminidase and beta-galactosidase, myoclonus, coarse facial features, corneal clouding, hepatomegaly, skeletal dyplasia, learning disabilities, starts in neonatal period to second decade of life

Unverricht Lundborg Syndrome (Baltic myoclonic epilepsy)

AR, *EPM1* gene that encodes for cystatin B (CSTB) on chrome 21q22 mcc of the progressive myoclonus epilepsies. Onset ages of 6 and 16. Onset with myoclonic jerks and tonic-clonic sz , later various sz types, ataxia, tremor, cognitive decline. Often in wheelchair and death before the age of 24. EEG: 3-5 Hz spike-and-wave or multiple spike and wave activity; marked photosensitivity tx: Valproic acid (1st line) Levetiracetam, Clonazepam, topiramate, and zonisamide. avoid: Phenytoin (worsens szs & neurodegen) carbamazepine

Lafora body disease (What type of disease, what are Lafora bodies, where are they found, how is it diagnosed and treated)

AR, Mutation of *EPM2A* gene on Chr 6q24 (encodes laforin) Age 12-17 years Dysarthria, ataxia, emotional disturbance, dementia, multiple seizure types, death within decade. Fatal Dx: PAS positive Lafora bodies w/in apocrine sweat gland or DNA sequencing. inclusion bodies, (AKA Lafora bodies) in cytoplasm of cells composed of abnormal glycogen called polyglucosans. Found in heart, liver, muscle, and skin, Tx: Zonisamide and other AEDs.

Lafora progressive myoclonus Epilepsy

AR, child to adolescent, multiple seizure types, progressive dementia, Abnormal glycogen deposits in all organ systems, Lafora bodies

abetalipoproteinemia

AR, mutation in the microsomal triglyceride transfer protein, which is required for the normal absorption of cholesterol, fat, and fat-soluble vitamins (A, D, E, and K). symptoms are due to a deficiency of vitamin E. It presents in children with failure to thrive, diarrhea, steatorrhea (foul-smelling, fatty stools). Vitamin E deficiency leads degeneration of the spinocerebellar tracts and dorsal columns, accounting for the ataxia. Neurological symptoms develop between the ages of 2 and 10 years. Some patients develop skeletal abnormalities such as lordosis, scoliosis, and pes cavus. Vitamin K deficiency may lead to impaired blood coagulation and gastrointestinal bleeding. As the disease progresses, some patients develop progressive retinopathy due to vitamin A deficiency. Physical examination may reveal retinitis pigmentosa. Acanthocytes, (abnormal, star-shaped red blood cells) can be seen on peripheral smear. An intestinal biopsy will reveal lipid-containing vacuoles in enterocytes. It is treated with large doses of vitamin supplementation, especially vitamin E.

Imaging finding in MS patients that BEST predicts progression of disability?

ATROPHY (not number of lesions)

lesion on MRI with "salt and pepper" appearance (patchy areas with black flow voids)

AVM

" what group should testing be done on to evaluate fatal skin reactions SJ, and TEN

Aasian descent such as Hn Chinse, Indian, southeast asian ancestry. More common skin reactions in HLA B *1502

How to differentiate b/w ring enhancing TUMOR vs ABSCESS on MRI...

Abscesses have diffusion restriction - bright on DWI, dark on ADC. Also, abscesses often have smoother/thinner ring Tumors are the opposite

ABCD2

Accurately assigns the 7 day risk for severe stroke and recurrent TIA after an initial TIA - Higher risk if TIA > 60min - TIAs assoc w/ age > 60, SBP > 140 or DBP > 90, DM and clinical features of unilateral weakness or speech disturbance also have an increased 7 day risk for major stroke

31yo female 5 months pregnant presents with dull, pressure type headaches with a band-like distribution most severe bifrontal/bitemporal with 2-3 headache days per week. Most appropriate abortive medication? A) Acetaminophen B) Acetaminophen/butalbital/caffeine C) Ibuprofen D) Sumatriptan E) Codeine

Acetaminophen Bifrontal/bitemporal headaches, by description, are tension-type headaches. Acetaminophen is the first-line medication in pregnancy. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used in the first or second trimester, but acetaminophen is preferred. Butalbital, codeine and sumatriptan are Category C, whereas acetaminophen and ibuprofen are Category B. Sumatriptan use should be restricted to migraine and opiates are not encouraged for headaches. Acetaminophen is also the first line choice for breast feeding mothers.

Landau-Kleffner Syndrome

Acquired epileptic aphasia Onset 3-7 y/o Acquired auditory agnosia, Word deafness in the setting of normal hearing, receptive and expressive aphasia, EEG: Centrotemporal sharp waves that increase in sleep, Electrical status epilepticus in slow sleep (ESES) VPA and lamotrigine are usually effective for seizures, but speech recovery can be variable

ADEM

Acute disseminated encephalomyelitis - Autoimmune attack on brain and spinal cord; diffuse inflammation and demyelination - Presents like a rapid-onset MS - Young adults - Presents abruptly with headaches and fever, 2-12 days after a nonspecific URI - Resolves rapidly within a week Histo: inflammatory cells

50yo female developed a headache behind her right eye and blurred near vision. The next day she noticed her right pupil is larger than the left. On exam 2 months later, her right pupil does not react to direct or consensual light, but constricts to near effort. Instillation of dilute pilocarpine to both eyes results in constriction of the right pupil while the left remains unchanged. A) Adie's tonic pupil B) Horner's syndrome C) Physiologic anisocoria D) Syphilis

Adie's tonic pupil This patient's history is most compatible with a unilateral Adie's tonic pupil. An Adie's pupil is typically initially large although becomes smaller over time. It shows minimal reaction to light but normal constriction to near, ie, light-near dissociation. Unlike normal pupils, which will show no response to instillation of dilute pilocarpine, Adie's pupils will constrict to this agent due to denervation super sensitivity from ciliary ganglion dysfunction. Unlike Adie's pupils, Argyll-Robertson pupils due to neurosyphilis are typically bilaterally small. Adie's pupils are often associated with impaired muscle stretch reflexes; this combination of findings is referred to as the Holmes-Adie syndrome. Apraclonidine is used to detect a Horner pupil which should dilate in response to this agent.

This pupil is typically initially large although becomes smaller over time. It shows minimal reaction to light but normal constriction to near, ie, light-near dissociation. Unlike normal pupils, which will show no response to instillation of dilute pilocarpine, these pupils will constrict to this agent due to denervation supers ensitivity from ciliary ganglion dysfunction. These pupils are often associated with impaired muscle stretch reflexes; this combination of findings is referred to as the Holmes-___ syndrome.

Adie('s)

Electrical status epilepticus during slow-wave sleep (ESES)

Age 1-12 years, peak 4-5 Psychomotor retardation, multiple seizures during sleep Linked to Landau-Kleffner, but ESES has more global regression and more refractory to TH

Dandy Walker malformation

Agenesis/hypoplasia of cerebellum Cystic dilation of 4th ventricle Enlarged posterior fossa Basically: looks like a huge empty space in entire back of brain

Lissencephaly

Agyria, majority of patients have a mutation of either the LIS1 gene or the double cortin gene. Double cortin mutations cause anterior greater than posterior dysfunction, while LIS1 mutations cause posterior greater than anterior dysfunction. There is a variable clinical presentation with less severely affected patients having mild cognitive impairments and less severe epilepsy, while severely affected patients can have marked mental retardation, intractable epilepsy, and severe cerebral palsy. As much as 50% of patients have microcephaly.

The two "plates" that our brain develops from.... names and which functions they carry

Alar plate - will become sensory structures Basal plate - will become motor structures

Rosenthal fibers on biopsy

Alexander disease - mutation in gene encoding glial fibrillary acidic protein (GFAP)

Aphasia: Intact speech; intact comprehension, repetition, impaired naming, reading, writing;

Alexia with agraphia (left angular gyrus)

Drugs that cause peripheral neuropathy (>20)

Allopurinol ALmitrine Amiodarone Chloroquine Cisplatin Colchicine Dapsone Didanosine Disulfiram Doxorubicin Ethambutol FK506 Gold salts Isoniazid Metronidazole Misonidazole Nitrous oxide Nitrofurantoin Non-depolarizing NM blocking agents Paclitaxel Perhexiline Phenytoin Procainamide Pyridoxine/B6 excess Stavuldine Suramin Thalidomide Vinblastine Vincristine Valcitabine

Where in the brain do emboli typically go?

Along cortical ribbon

EEG waveform frequency names

Alpha: 8-13 (Normal awake) Beta: >13 (normal, augmented by benzos/barbs) Theta: 3.5 - 7.5 (sleep) Delta: < 3 (deep sleep)

"Tau-opathies" (6)

Alzheimer's Frontotemporal dementia/Pick disease PSP Myotonic dystrophy Corticobasal degeneration

Cell that reminds me of sperm (aka neurofibrillary tangle) is seen in...

Alzheimer's PSP

neurofibrillary tangles are seen in which disease?

Alzheimers

Episodic memory deficits are typical of which two diseases/syndromes?

Alzheimers disease and Balint's syndrome

. Semantic memory deficits are typical of?

Alzheimers disease and frontotemporal lobar degeneration (FTD)

This is characterized by a memory complaint; otherwise normal cognition, normal activities of daily living and objective memory impairment. It is thought to be a precursor to Alzheimer disease.

Amnestic mild cognitive impairment

Which area of the brain plays a crucial role in emotional responses?

Amygdala

Which area of the brain processes the emotional significance of pain?

Amygdala

Elderly with headache. SWAN shows lots of little bleeds in white matter areas. Cause?

Amyloid angiopathy (can also be caused by bleeding malignant melanoma, however less common)

Amyloid plaque vs neurofibrillary tangle

Amyloid plaque -- kind of brownish and round; seen in dementia w/ lewy bodies Neurofibrillary tangle -- little helical fragments tangled up inside a neuron (sperm-shaped); seen in Alzheimer disease, FTD, PSP, Down Syndrome

What is thalamic astasia?

An alert and awake patient with normal strength that cannot stand and sometimes sit unassisted for several days, they vary varying degrees of sensory loss, typically fall backwards or to side opposite of lesion

This is the most common cause of the syndrome characterized by language delay, seizures, hand-flapping, and an abnormally happy demeanor

Angelman syndrome maternally-derived; de-novo deltion of 15q11-13

Inferior parietal lobule is composed of... (2 gyri)

Angular gyrus Supramarginal gyrus

Aphasia: Fluent, intact comprehension, intact repetition

Anomic aphasia

Diffuse hyperintense T2 and DWI signal throughout cortex, mesial temporal lobes, thalami, and pons...Dx?

Anoxic brain injury

Symmetrical hyperintense signal in cerebellum and basal ganglia?

Anoxic injury (these areas are very sensitive to this)

What is used to detect a Horner pupil which should dilate in response to this agent?

Apraclonidine

MCC of increased head circumference at birth in the absence of other cerebral malformations

Aqueductal stenosis (at cerebral aqueduct of Sylvius b/w 3rd and 4th ventricle)

Insular cortex (definition)

Area of cortex that is folded deep inside the lateral sulcus (which is the fissure that separates the temporal lobe from the parietal/ frontal lobes)

Verocray body

Areas of nuclear pallasading alternating w/ anuclear zones seen in Schwannomas

These pupils due to neurosyphilis are typically bilaterally small

Argyll-Robertson

60yo male with poorly controlled diabetes presents with acute onset of painless vision loss in his left eye that has been progressively worsening for 3 days. On exam, there is decreased visual acuity in inferior visual field and papilledema of left eye only, but otherwise normal with BP 135/89. CT head and CTA head/neck are normal. Management? A) Corticosteroids B) IV labetalol C) Aspirin D) Hyperbaric oxygen E) Warfarin

Aspirin This patient has nonarteritic anterior ischemic optic neuropathy (NAION) resulting in optic nerve head ischemia. This is usually due to vascular occlusive disease of the posterior ciliary arteries which supply the optic nerve. Embolism from cardiac or large artery sources underlie few if any cases of NAION. In contrast to retinal ischemia, NAION is not associated with carotid occlusive disease. The primary risk factors are hypertension and diabetes mellitus. Most patients awaken with painless monocular visual loss presumably because of nocturnal hypotension. Corticosteroids, hyperbaric oxygen, and anticoagulation are not beneficial for this condition. Abrupt lowering of blood pressure may aggravate the ischemia. No treatment has been shown to improve clinical outcome in these patients but fortunately most will improve or stabilize over several weeks. Aspirin therapy is appropriate as it reduces the patient's future risk of cardiovascular and cerebrovascular disease. Vasculitic anterior ischemic optic neuropathy, a common presentation of giant cell arteritis, usually produces severe monocular or binocular visual loss. It often occurs in association with headache, systemic symptoms and elevated ESR.

Give 5 types of Glial Cells.

Astrocytes Microglia Oligodendroglia Ependymal Cells Schwann Cells

Brain Tumor Question: What stains with GFAP?

Astrocytes and related tumors

Keppra mechanism of action

Binds to synaptic vesicle glycoprotein (SV2A) and inhibits presynpatic calcium channels --> reduces neurotransmitter release and impedes nerve conduction across the synapse

This disease is characterized by subcortical foci of patchy T2 hyperintensities in white matter

Binswanger

Earliest EEG change in CJD?

Background slowing (most famous feature? - generalized bisynchronous sharp waves)

What vessel is a tributary of the vein of Galen?

Basal vein of Rosenthal

Common cancer in patients with ataxia telangiectasia

B-cell neoplasias (such as lymphoma)

Epilepsy Syndrome: Oropharyngeal motor seizures in half (motor or paresthesias), sometimes jacksonian march up to a generalized TC sz. Most often in first or last part of the night. Sleep activated centrotemporal spike and slow wave, uni or bilateral, with high amplitude. Has horizontal dipole.

BECTS Benign Epilepsy with Centrotemporal Spikes Benign Rolandic Epilepsy

Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE)

Begin in childhood and persist into adult life, mutations of the *nicotinic acetylcholine receptors* CNRNA4 (chrome 20) and CHRNB2 (chrome 1) Bizarre episodic behaviors in the context of hypermotor seizures (thrashing, jerking) Onset during non-REM sleep, patients awaken with motor manifestations, patients will be conscious and report epigastic or other aura Often misdiagnosed as pseudo seizure because of unusual appearance and normal EEG interictally Usually responds to carbamazepine

Benign Familial Neonatal Seizures

Begins in the first few weeks of life Focal, multifocal, or generalized seizures + Family Hx of similar neonatal seizures Autosomal dominant *(KCNQ2 and KCNQ3)* No EEG findings specific to the diagnosis GOOD PROGNOSIS - In most cases seizures resolve during infancy, normal development. However, 15% will have seizures in adulthood.

55yo male presents with episodic vertigo and imbalance for the past 2 months, particularly with changes in position like rolling over in bed or bending over. Each episode lasts 10-30 seconds. A dix-hallpike maneuver results in vertigo and nystagmus that resolve after 1 minute. Diagnosis? A) Benign paroxysmal positional vertigo B) Stroke C) Multiple sclerosis D) Labyrinthitis E) Vertebrobasilar insufficiency

Benign paroxysmal positional vertigo In benign paroxysmal positional vertigo, there is a latent period before the development of vertigo and nystagmus (vertical, torsional, or a combination) when the affected ear is in the down position on Hallpike-Dix testing and resolution of symptoms is within 60 seconds. The diagnosis is based on history and physical examination, with no need for further diagnostic testing in straightforward cases. Canalith repositioning treatment produces remission in 60% to 70% of cases and improvement in an additional 20% to 30% of cases.

Small sharp spikes (SSS)

Benign variant seen in adults (drowsiness and light sleep)

14- and 6-per-second positive spikes over posterior and temporal regions

Benign, seen in drowsiness and light sleep

Where is 4th ventricle on saggital view?

Between pons and cerebellum

Prosopagnosia is caused by lesions where?

Bilateral lesions to the fusiform (occipital temporal) gyri

Most common finding on CT head of someone with carbon monoxide poisoning?

Bilateral symmetric necrosis of globus pallidus (shows up as darker on CT; bright on T2)

Locked in syndrome

Bilateral ventral pons lesions Preserved consciousness and sensation, but paralysis of ALL movements except vertical gaze and eyelid opening - Quadriplegia due to bilateral CST involvement - Aphonia due to CBT involvement - Impairment of horizontal eye movements b/c of CN IV - Sparing of reticular formation -- fully awake - Supranuclear ocular motor pathways lie dorsally so pt can look vertically and blink

This causes pupil dilation due to its effect on both muscarinic and nicotinic cholinergic nerve terminals. Other disorders that may acutely produce this constellation of symptoms, for example myasthenia gravis, Guillain Barre syndrome and diptheria, are typically not associated with pupillary abnormalities. Autonomic dysfunction can be seen with Guilain Barre syndrome and diptheria but usually not early on.

Botulism

Randy travis drinks cold beer

Brachial plexus: Roots: anterior divisions of spinal nerves C5-T1 (posterior devisions innervate skin & musculature of trunk) Trunks (superior: C5/C6, middle C7, inferior C8/T1) Divisions (anterior & posterior) Cords (lateral, posterior, medial - name relative to their position to the axillary artery) Branches (musculocutaneous, axillary, median, radial, ulnar)

Transcalvarial herniation

Brain squeezes through a fracture or surgical site. "external" herniation

Anterior interosseous nerve (branch of?, what does it innervate)

Branch of the median nerve Innervates: - Flexor pollicis longus - Pronator quadratus - Flexor digitorum profundus to digits 2 & 3 (in a complete lesion they'd be unable to make the "ok" sign)

Aphasia: Nonfluent, intact comprehension, impaired repetition, impaired writing, impaired naming, ?

Broca aphasia (Posterior lateral frontal lobe)

Color of alpha-synulcein staining

Brown (ex - lewy bodies stain positive for this)

Anti-depressant that lowers seizure threshold

Bupropion

In episodic migraine patients, use of which medication is the most likely to result in medication overuse headache and is associated with this conversion at the fewest doses per month? A) Ibuprofen B) Prochlorperazine C) Sumatriptan D) Butalbital E) Opiates

Butalbital In a large, population-based study published in 2008, the use of barbiturates or opiates had the highest association with the development of medication overuse headache (MOH) in patients with a history of episodic migraine. However, butalbital was associated with this conversion at a dosing frequency of 5 days per month, whereas opioids required more frequent usage (8 days per month). Anti-emetics are not associated with MOH; sumatriptan is implicated in conversion to MOH, but at higher frequencies of use (10 days per month). Ibuprofen can be useful in preventing this condition if not taken very frequently (10-15 days per month).

Spinal tumor that almost always arises in the BONE (vertebral bodies)

Chordoma

The most common congenital infection in the US, this agent frequently causes hearing loss and calcification on MRI

CMV

Only cranial nerve that exits laterally?

CN IV

Facial colliculus

CN7 wraps around abducens nucleus before exiting brainstem at CP angle

Sturge Weber syndrome CT/MRI findings

CT -- calcification in a "serpantine" shape, usually parietooccipital MRI -- unilateral cerebral atrophy w/ enlargement of the ipsilateral subarachnoid spaces and ventricular cavities

40yo female with no PMH presents with 4 days of progressively worsening diffuse dull headache, maximal in bilateral occipital region, with associated photophobia, nausea and neck stiffness. This severely worsens with standing, but is nearly relieved after lying down. Which test will be most likely to yield the diagnosis? A) Meningeal biopsy B) Serum angiotensin converting enzyme C) CT myelogram D) Slit lamp exam E) CSF cytology

CT myelogram This patient most likely has orthostatic headache, secondary to spontaneous intracranial hypotension. The usual etiology is a CSF leak located in the spinal meninges. The leak is most readily identified now with MR spine imaging, or a CT myelogram. The other tests listed (meningeal biopsy, serum angiotensin converting enzyme, slit lamp examination, and CSF cytology) would not assist in the diagnosis of this syndrome.

Apperance of colloid cyst on CT vs T1 post-contrast vs T2 FLAIR

CT: bright T1 post-contrast: isointense, not enhancing FLAIR: bright Located in 3rd ventricle, adjacent to foramina of Monro

Appearance of colloid cyst on CT, T1, and T2 FLAIR?

CT: hyperdense (bright) T1: isointense, not enhancing FLAIR: hyperintense Located within 3rd ventricle adjacent to foramen of Monroe

Myotonic dystrophy mutation and chromosome

CTG repeats Chr 19 q13.3

Lesion that destroys C2 causing an "odontoid pannus" that smashes spinal cord

Can be due to rheumatoid arthritis or trauma (isointense on T1; bright on T2)

Air embolus

Can result form cardiopulmonary bypass, chest trauma, neurosurgical procedures in which the patient is operated with head above the heart - CT - air diffusely seen within cerebral vessels - causes global cerebral hypo perfusion

AED that causes autoinduction of hepatic enzymes (meaning it increases the activity of the enzymes that break it down --> decreases drug levels)

Carbamazepine

Lewy bodies are seen in Parkinson's disease and Lewy bodies dementia are?

synucleionopathies

Chiari 1 malformation

Cerebellar tonsils extend into foramen magnum. Often asymptomatic.

Chiari 3 malformation

Cerebellum and brainstem herniate through a hole in the back of the skull and protrudes outside the head

Chairi 2 (classic, Arnold-Chiari) malformation

Cerebellum and medulla extend through foramen magnum. - Often accompanied by myelomeningocele - Hydrocephalus/enlarged ventricles

CADASIL

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy - mutation in NOTCH 3 gene on chromosome 19 - often associated with progressive focal neurological deficits

Hypsarrhythmia (what does it look like, what causes it)

Chaotic admixture of continuous high amplitude multifocal spike & sharps alternating with arrhythmic slow waves Reflects severe cerebral damage in kids 4mo to 2yo

Patients with ______-______-______ disease may have postural and action tremor but should not have cerebellar ataxia.

Charcot-Marie-Tooth

Coronial MRI with separation between both sides of brain from top to bottom (absence of falx cerebri [dura]). Dx?

Chiari II malformation

Epilepsy Syndrome: Has brief and frequent staring spells with possibility of facial myoclonus with onset 5-7 yo. EEG reveals 3 Hz spike and slow wave discharge.

Childhood Absence Epilepsy

Pyknolepsy

Childhood absence in kids around 3-4 EEG: bilateral, synchronous, symmetric 3-Hz spike-and-wave discharge No distinct clinical or EEG features completely differentiate childhood absence epilepsy from juvenile absence epilepsy.

3-Hz spike and wave

Children 5-15yo, absence seizures

Brain Tumor Question: What non-neoplastic tumor can occur at the roof of the 3rd ventricle causing a ball-valve obstruction (dangerous)?

Colloid Cyst of the third ventricle

Turcot syndrome

Colon polyps and gliomas or medulloblastomas

Benign childhood epilepsy with centrotemporal spikes (Rolandic epilepsy)

Common (25% of childhood epilepsy), Onset: Typically begins bw 5-9 y/o, resolves mid-teenage years. Hemifacial sensorimotor sz with spread to i/L hand; progression to hemiconvulsion or GTC c in 50%, 70% only in sleep, 15% only awake. nL development and exam, nL imaging EEG: centrotermporal spikes on normal background activated by drowsiness and sleep Genetics: AD with variable penetrance; Possibly linked to 15q14; Tx: Most do not require AED. Carbamazepine (use only when prolonged or frequent seizures, wait for >2 before starting), stop after adolescence Prognosis: Excellent; most resolve before age 16 years. (only 5% continue to seize > 5 years of onset)

Distinguish simple and complex febrile seizures

Complicated if: 1. Recur within 24h 2. Prolonged (>15min) 3. Focal >15 min, focal features, abnormal exam, seizure recurrence in <24 hours, post-ictal signs (Todd's), more likely due to meningitis, encephalitis or underlying seizure disorder simple: 1. <15 min, 2. generalized, 3. no focality, 4, nl. exam, 5. no persistent deficits

Schizencephaly

Continuation of lateral ventricles towards extraaxial space with brain lined by gray matter/cortex in those contiguous canals of CSF (if it wasn't lined, it would be porencephaly)

Epilepsy Syndrome: Different types of seizures, deterioration in development, behavioral issues. EEG shows near continuous synchronous discharges during non-REM sleep.

Continuous spike and wave in slow wave sleep CSWS since behavioral issues (vs speech)

_______ deficiency can produce a clinical syndrome very similar to subacute combined degeneration. Clinical settings in which this occurs include malabsorption, including gastric bypass or as a consequence of excess zinc ingestion.

Copper

75yo female presents with 3 months of very severe stabbing, shooting pain radiating from in front of her left ear to her upper lip and along her jaw-line. This occurs 3 to 10 times per day lasting 30 seconds to 1 minute, particularly when brushing her teeth or eating. Neuro exam is normal. Most likely cause of her symptoms? A) SUNA syndrome B) Cranial nerve compression C) Demyelination D) Ischemia

Cranial nerve compression This patient's symptoms are most compatible with trigeminal neuralgia (tic douloureux). Most cases of trigeminal neuralgia are felt to be due to compression of the trigeminal nerve by a vascular loop. Imaging is indicated to exclude other, though less common, structural causes such as a meningioma, cerebellopontine angle neoplasm, and aneurysm. Multiple sclerosis can also be a cause of trigeminal neuralgia, but that is less likely in this patient due to her age and lack of other signs and symptoms. In the absence of long tract and cerebellar findings, stroke is extremely unlikely.

Brain Tumor Question: What childhood tumor is supratentorial, derived from Rathke's pouch, and has bitemporal hemianopsia?

Craniopharyngioma

Brain mass with globular calcification (so...bright on non-contrast CT) and separate cystic (black) components

Craniopharyngioma

Where does CN I exit the skull

Cribiform plate

Large empty dark area in brain with enhancing rim as well as a thicker white area attached to the rim?

Cystic astrocytoma

Cystic cerebellar mass that enhances

Cystic hemangioblastoma

Ring enhancing lesion in basal ganglia (with mass effect and edema crushing lateral ventricle) in immunocompromised patient?

Toxoplasmosis

Imaging finding for Canavan disease

DIFFUSE high signal intensity throughout white matter (spans from periventricular region to cortex; doesn't spare U-fibers)

Central herniation

Diencephalon (thalamus, hypothalamus) and parts of both temporal lobes are squeezed through a notch in the tentorium cerebelli (can herniate upwards or downwards) - Can shear basilar artery causing Duret hemorrhage (fatal) - Fixed dilated pupils - Paralysis of upward eye movement On imaging, if downward herniation you'll see obliteration of suprasellar cistern

Damage to which area causes gait apraxia (inability to initiate the process of walking)

Diffuse cerebral disease (like Alzheimer's)

What is a first-line oral agent for relapsing/remitting multiple sclerosis which was shown in two trials to be an effective agent in decreasing relapse rate in RRMS?

Dimethyl fumarate

Scary side effects of metoclopramide

Dopamine receptor antagonist - tardive dyskinesia - Neuroleptic malignant syndrome - Parkinsonism

Parinaud's syndrome is caused by a lesion where?

Dorsal midbrain, involving the quadrigeminal plate, pretectum, periaqueductal gray area

Which two nerves come off of the brachial plexus roots? Which roots supply them? Which muscle do they innervate?

Dorsal scapular (C5) - rhomboid major & minor, levator scapulae Long thoracid (C5-C7) - serratous anterior

Deep peroneal nerve (motor, sensory, roots)

Dorsiflexion (via anterior tibialis muscle) Webspace b/w 1st and 2nd toes Branch off of the common peroneal; L4-S1

Injury to which area of the thalamus can result in "frontal-like" symptoms such as disinhibition and poor executive function?

Dorsomedial (mediodorsal) nucleus

Epilepsy Syndrome: Prolonged hemi-convulsive sz with generalization that classically switches sides. Sometimes provoked by fever. Ends up with intellectual disability.

Dravet Syndrome

Eye of the tiger sign

Due to PKAN (pantothenate kinase-associated neurodegeneration). Accumulation of iron in globus pallidus

Manganese intoxication (cause? signs? imaging?)

Due to poor biliary excretion of manganese (biliary atresia, chronic liver disease) or due to high-dose manganese in TPN - Parkinsonism - Dystonia Doesn't respond to levodopa T1 MRI: hyperintensity in globus pallidus (main site of damage), striatum, midbrain

Most likely genetic abnormality in a child with findings of pes cavus, hammer toes, atrophy of the peroneal muscles, and absent muscle stretch reflexes

Duplication of PMP 22 (CMT 1A)

Brain with no gyri?

Either lissencephaly vs just super super premature birth (gyri haven't formed yet)

Damage to which area causes astereognosis (failure to recognize objects by touch)

Either parietal lobe

Neuronopathy

Dysfunction of the nerve cell body (dorsal root ganglion) Not length dependent Sjogrens is most common sensory neuronopathy

" Treatment for severe depression with cognitive deficits and pesudodementia

ECT

Epilepsy Syndrome: Tonic eye deviations, sometimes tonic clonic movements, freq headache after seizure. Prominent autonomic features usually at night. EEG might have spikes in one or both occipital lobes, and might have fixation-off sensivity.

Early Onset Benign Occipital Epilepsy Panayiotopoulos Syndrome same thing

Which of the following conditions associated with highest risk for arterial thrombosis?

Elevated factor VIII activity

Wernicke encephalopathy findings on MRI

Enhancement of mamillary bodies and dorsal aspect of midbrain

Vimpat mechanism of action

Enhances the SLOW inactivation of VG-Na channels without affecting fast inactivation (lacOOOOsamide= slOOOw)

What does a lewy body look like on histo?

Eosinophilic cytoplasmic inclusion (round) surrounded by melanin (little brown granules)

Brain Tumor Question: What childhood tumor can effect the fourth ventricle, frequently a/w Neurofibromatosis type 2 and forms perivascular pseudorosettes (circle of cell around a vessel)?

Ependymoma

Enhancing lesion that sits in the middle of the spinal cord

Ependymoma

Intramedullary spinal tumor in adults

Ependymoma

Tumor that starts in the spinal canal and spreads into the vertebral bodies?

Ependymoma (the opposite is caused by a chordoma - arises from vertebral body and grows into cord)

Tumors whose histology shows perivascular pseudorosettes

Ependymomas

Epidermoid vs arachnoid vs dermoid cyst on MRI

Epidermoid= bright on DWI and T2 Arachnoid = dark on DWI Dermoid = bright on T1 (high fat content)

Common cause of triphasic waves on EEG

Hepatic encephalopathy (or any kind of metabolic encephalopathy)

High amplitude single downward deflection on EEG

Eye blink (eye closing is downward, eye opening is upward)

Lateralizing signs in seizures

Eye deviation: contralateral frontal lobe Extended arm: contralateral supplementary moror area Dystonic arm: contralateral temporal lobe Gelastic: hypothalamus

AED that causes blue skin discoloration (lips, nails) and pigment changes in retina

Ezogabine

Extreme calcifications of basal ganglia (symmetrical, bilateral)

Fahr disease (idiopathic calcification of basal ganglia)

Ultrasound of entire plane of muscle can be used to detect...

Fasciculations

Potent AED (used for Lennox Gastaut) that can cause a fatal aplastic anemia and liver failure

Felbatol

Fingolimod

Fingolimod is a once daily pill that blocks lymphocyte egress from lymph nodes used in the treatment of relapsing-remitting multiple sclerosis There have been 19 cases thus far of progressive multifocal leukoencephalopathy (PML) in patients treated with fingolimod, and the medication has been taken by about 225,000 patients thus far. All patients had been treated for longer than 2 years. Immunity to the varicella zoster virus should also be documented prior to starting fingolimod, and appropriate vaccines given if needed. There have also been cases of herpes simplex encephalitis and over 30 cases of cryptococcal meningitis. There have been 9 deaths due to cryptococcal meningitis

SSRI with least risk of discontinuation syndrome

Fluoxetine (b/c longest half life)

Where does CN V3 and CN V motor branches leave skull?

Foramen ovale

Hereditary sensory neuropathies (3)

Friedrich's ataxia Abetalipoproteinemia (large fiber) Sensory ataxic neuropathy

28yo female with migraines reports good initial relief with her sumatriptan, but often has to take a second dose due to recurrence of her headache after a few hours. What is the best alternative triptan to potentially avoid this wearing off effect? A) Eletriptan B) Rizatriptan C) Zolmitriptan D) Frovatriptan E) Naratriptan

Frovatriptan Of all the oral triptan agents frovatriptan has the longest half-life. In this case, taking a longer-acting drug may provide more sustained relief and avoid the need for a second dose.

How to differentiate GBS from CIDP?

GBS should plateau by 4 weeks after onset

Intracerebral hemorrhage (ICH) Score

GCS (3-4=2 points, 5-12=1 point) ICH volume >30cm3 = 1 point Intraventricular hemorrhage = 1 point Intratentorial origin of hemorrhage = 1 point Age >80. = 1 point

Where is most of the body's serotonin located?

GI tract

Sign of CJD on EEG

GPEDs (generalized periodic epileptiform discharges)

What enzyme involved in galactosemia is the only one that causes intellectual impairment in patients?

Galactose-1-phosphate uridyltransferase deficiency

What one is most common?

Galactose-1-phosphate uridyltransferase deficiency

CJD EEG

Generalized periodic epileptiform discharges

Subacute sclerosing panencephalitis (SSPE)

Generalized periodic epileptiform discharges, but typically longer interval of 4-15 sec

Most common and most specific EEG pattern for NMDA receptor encephalitis

Generalized slowing, delta brush

Alpha coma (what does it look like, cause)

Generalized, invariant, monorhythmic patter in alpha frequency with little or no reactivity 2-3 days after cardiac arrest (poor prognosis - death or persistent vegetative state)

Brain Tumor Question: What tumor has pseudopalisading (snake-like), necrotic, often crosses midline?

Glioblastoma

Aphasia: Non-fluent, mute, impaired comprehension, repetition, naming, reading and writing?

Global aphasia

Placement of DBS for Parkinson's disease and dystonia?

Globus pallidus internus or subthalamic nucleus

Functions of CN IX

Glossopharyngeal nerve - Sensation of pharynx - Sensation and taste of posterior 1/3 of tongue - Carotid body and sinus

53yo male has intermittent, brief, sharp pains in the right side of his neck and throat, radiating internal to the angle of his jaw on the right. Occasionally this is associated with sudden loss of consciousness when he is upright, with rapid recovery when laid flat. Normal neuro/cardiac exams, no bradycardia or hypotension with carotid massage. Echocardiogram, EKG, MRI/MRA head/neck are unremarkable. On telemetry, has episode of 6 seconds asystole, followed by HR 30 and BP 70/50. Diagnosis?

Glossopharyngeal neuralgia

A patient has resolved ulnar neuropathy at the elbow, then has NCS/EMG which show diffusely low conduction velocities in multiple nerves; what disease does she likely have?

HNPP (Hereditary neuropathy with liability to pressure palsy)

MCC of lacunar infarctions

HTN (due to lipohyalinosis of arteries that are 50-200 microns in diameter)

Most important risk factor for stroke

HTN control is most important intervention to reduce risk of future stroke (over DM, smoking, HLD)

shield sign

Hallmark of length dependent process So sorry loss over chest and sternum

Brain Tumor Question: What is a/w von Hippel-Landau syndrome, foamy cells on histology, and can produce EPO (polycythemia)?

Hemangioblastoma

Treatment with chemotherapy and treatment with monoclonal antibodies are risk factors for progressive multifocal leukoencephalopathy. MRI reveals non-enhancing FLAIR abnormalities that involve the white matter more than the gray matter. Diagnosis of this is made by CSF demonstration of JC virus

Hematologic malignancies

Methanol poisoning on CT

Hemorrhagic necrosis of bilateral putamen with surrounding edema

Centrotemporal spikes

High amplitude, diphasic, blunt spikes with aftercoming slow wave tend to occur in clusters/trains over centro-temporal region (F7, T7, P7, F3, C3) EEG background otherwise normal Seen in: BCECTS/Benign Rolandic Epilepsy (symptoms: twitching of side of face and/or hand, tingling of tongue or cheek, motor speech arrest, drooling, mostly at night, can progress to generalized seizure) Easily controlled with AED; seizures usually gone in 2nd decade

hypsarrythmia

High voltage, disorganized, slow, multifocal epileptiform discharges (during nonREM sleep)

Suprascapular nerve - damage results in which issue?

Hits supraspinatus and infraspinatus muscles -- used for initial abduction and external rotation of shoulder

Motor unit recruitment

How quickly motor units are added during muscle flexion Decreased recruitment: neuropathy or motor neuron disease Rapid recruitment: myopathy

Huntington's disease chromosome

Huntingtin gene on Chr. 4

In a child, a non-cystic mass that grows in the floor of the 3rd ventricle...Dx?

Hypothalamic hamartoma - associated with precocious puberty and gelastic epilepsy (peculiar laughing seizures) (cystic, and can arise in this same area = pilocytic astrocytomas, craniopharyngiomas, teratomas)

Deficiency of this enzyme results in early dystonia followed by later self-injurious behavior.

Hypoxanthine-guanine phosphoribosyltransferase (Lesch-Nyhan Syndrome)

Infant (<24 months old) with high-amplitude slow waves with irregular spikes and variable rhythm

Hypsarrhythmia --> associated with infantile spasms/West syndrome

" Braintsem auditory evoked potentials BAEPS waves

I- distal CNVIII, II- i/l proximal CN VIII or cochlear nucleus, III- i/l superior olivary n. IV- n or axon of lateral lemniscus, V- inferior colliculus, VI- medial geniculate body, VII- thalamocortical pathways. VI and VII used for interpretation.

Which cranial nerves carry general somatic efferent fibers (carry motor impulses to skeletal muscle)

III IV VI XI XII

Which cranial nerves carry general visceral efferent fibers (carry motor impulses to smooth muscle, cardiac muscle, and glands)

III VII IX X

Treatment for acute psychosis

IM chlorpromazine and olanzapine

Treatment of neuroleptic induced acute dystonia

IM diphenhydramine or IM anticholinergic medication

A patient presents with an acute stroke and is evaluated rapidly in an emergency setting. She appears to be a potential candidate for receiving IV t-PA based on her stroke scale, time window, and absence of contraindications to the drug; however, her blood pressure exceeds the guidelines for use of t-PA what would be indicated instead? Hint: Heparin and dipyridamole/ASA would not be indicated in this acute setting.

IV labetalol

Neuropathies with significant autonomic features (8)

Idiopathic Diabetic Amyloid AIDP Porphyria HIV HSAN Toxic (vincristine)

Small fiber neuropathies (8)

Idiopathic Diabetic Amyloid HIV Hereditary sensory and autonomic neuropathy (HSAN) Fabry's Tangier Sjogren's

Common symptoms of this are include chronic headache, papilledema, transient visual obscurations and diplopia. Even in classic cases, neuroimaging must be done to exclude a structural lesion causing increased intracranial pressure. The presentation can also be similar to that of cerebral venous thrombosis and therefore an MRI and MR venogram should be ordered.

Idiopathic Intracranial Hypertension (IIH)

This occurs most commonly in obese women of childbearing age. Common symptoms include chronic headache, papilledema, transient visual obscurations and diplopia. Even in classic cases, neuroimaging must be done to exclude a structural lesion causing increased intracranial pressure. The presentation can also be similar to that of cerebral venous thrombosis and therefore an MRI and MR venogram should be ordered.

Idiopathic Intracranial Hypertension (IIH)

Juvenile myoclonic epilepsy (JME)

Idiopathic generalized epilepsy with adolescent onset. nL development, no gender preference sx: morning myoclonic jerks, tonic-clonic seizures, and possible absence seizures. Intellectually normal. EEG: generalized 4-6Hz polyspike and wave, triggered by photic stim Tx: VPA, also lamotrigine, levetiracetam, topiramate and zonsisamide, required lifelong Avoid: alcohol and sleep deprivation Contraindicated: Carbamazepine and phenytoin, and gabapentin

An aphasia is considered fluent if?

If word output per minute is high, there are five or more words per phrase, content per phrase is low, paraphasias are present, speech is nondysarthric with normal prosody

Most appropriate agent for ppx against recurrent stroke in afib

In the absence of clear c/i - warfarin

Leptomeninges

Includes the arachnoid and pia mater - between which circulates the CSF (where SAH's occur)

Chiari 4 malformation

Incomplete/underdeveloped cerebellum (cerebellar hypoplasia)

Upward herniation

Increased pressure in posterior fossa can cause cerebellum to move up through the tentorial opening AKA cerebellar herniation

"Onion bulb" appearance on histology ("hypertrophic neuropathy") is seen in...

Indolent demyelinating diseases such as CMT1a

32 year old female presents with 2 weeks of intermittent stabbing left temporal/supraorbital headache with 8-12 attacks daily each lasting about 20 minutes, associated with tearing and redness of left eye. Best abortive treatment?

Indomethacin

35yo male with no PMH p/w intermittent stabbing right supraorbital headache with associated redness of right eye and rhinorrhea. Each lasts about 5-20 minutes and has been occurring nearly daily for past 15 months with longest headache free period less than 2 weeks. Most appropriate treatment? A) Indomethacin B) High flow oxygen and sumatriptan C) Dilaudid D) Lamotrigine E) Topiramate and sumatriptan

Indomethacin This patient's headache fulfills the criteria for chronic paroxysmal hemicrania which is usually exquisitely responsive to indomethacin. Cluster headaches last 15-180 minutes without treatment and occur up to eight times in a day - so this patient's headaches are too short and too frequent.

Large infarct right next to anterior horn of lateral ventricle.... name?

Infarction of recurrent artery of Heubner (A1 segment of ACA) - supplies anteroinferior portion of caduate, putamen, and anterior limb of internal capsule

Pituitary apoplexy

Infarction or hemorrhage of pituitary HA, ophthalmoparesis, visual deficits, encephalopathy More common in patients with pituitary adenoma

EOM used to look up and out

Inferior oblique

The posterior cerebral artery supplies what?

Inferior temporal lobe, occipital lobe, splenium of the corpus callosum, and superior parietal lobule

Tetrad of severe athetosis, perceptive high tone deafness, supranuclear palsy of upward gaze, and enamel hypoplasia of the incisor teeth. Dx?

Kernicterus (chronic bilirubin encephalopathy)

Myoclonic Astatic Epilepsy EEG (Doose s/d)

Interictal 2-3Hz spike-and-wave discharges seen in

Lennox-Gastaut EEG

Interictal <2.5 Hz bilateral slow spike-wave complexes

EEG finding for Lennox-Gastuat syndrome

Interictal slow spike-wave discharges

Where do nerve roots leave the vertebral column?

Intervertebral foramina

Causes of gadolinium enhancement of meninges diffusely?

Intracranial hypotension Carcinomatosis Lymphoma

familial dysautonomia (Riley-Day syndrome)

It is caused by a mutation in the IKBKAP gene, which codes for a protein called IKK complex-associated protein (IKAP). It is inherited in an autosomal-recessive pattern, and occurs almost only in Ashkenazi Jews. There is widespread dysfunction of the autonomic nervous system and sensation. Patients present in infancy with hypotonia, feeding difficulties and failure to thrive, lack of tears, pneumonia, and difficulty controlling body temperature. Physical examination will reveal lack of fungiform papillae on the tongue, hyporeflexia, and a lack of overflow tears with crying

Where does CSF flow once it's in the 4th ventricle

It leaves one of the three outflow tracts (two lateral foramina of Luscka, one medial foramen of Magendie) and drains into the cisterna magna

Unique NCS/EMG finding in myasthenia gravis?

Jitter (measured with single fiber EMG)

Where do CN IX, X, XI exit the skull

Jugular foramen

Generalized fast polyspike and wave discharges + myoclonic jerks... Dx?

Juvenile myoclonic epilepsy

Disorder that affects predominantly adolescent males and is characterized by hyperphagia, hypersomnolence, cognitive changes, and mood disorder

Kleine-Levin Syndrome

Where does spinal cord (conus medullaris) end

L1/L2

spinal cord levels in infancy

L3 at newborn. Preterm at S1 at 24 weeks

2-3 generalized spike wave discharges

LGS

A patient's presentation is consistent with Parkinson disease (PD). 10 to 15% of patients with PD have a familial form PD with several known causative genes. _____ mutations are responsible for about 10% of familial and 5% of sporadic PD cases. However in North African Arabs about 30-40% of sporadic PD cases have mutations of this gene. These patients mimic idiopathic PD in the age of onset, severity and progression of their PD. The other genetic abnormalities listed are inherited as either autosomal dominant or recessive disorders, and are typically associated with young onset PD.

LRRK2

Brain Tumor Question: What are Psammoma bodies?

Laminated calcification (looks like rings on trees)

First line AED when pregnant?

Lamotrigine

what can trigger absence status?

Lamotrigine, phenytoin, carbamazepine

Epilepsy Syndrome: Different types of seizures, deterioration in development, speech issues. EEG shows near continuous synchronous discharges during non-REM sleep.

Landau-Kleffner syndrome, which is a subtype of CSWS that has progressive speech issues

Syringomyelia - what is it? symptoms?

Large cystic space that forms in spinal cord Interrupts crossing of PAIN fibers and damages anterior horn cells (muscle atrophy) Spares vibration, proprioception, soft touch

Motor unit SIZE (large vs small)

Large motor units: neuropathy or motor neuron disease (huge in ALS) Small motor units: myopathy

Major depressive disorder criteria

Lasts > 2 weeks Anhedonia/depressed mood + 4 of the following: SIGECAPS - Sleep changes - Impairment in functioning - Guilt - Energy lack - Cognitive decline - Appetite decreased - Psychomotor agitation (anxiety) or retardation (lethargy) - Suicidal thoughts

Which nerve supplies sensation to lateral aspect of forearm

Lateral antebrachial cutaneous branch of musculoskeletal nerve

Wallenberg syndrome

Lateral medullary syndrome Occlusion of distal vertebral artery vs. PICA Spinothalamic tract -- hemisensory loss of pain/temp; hiccups CN V -- loss of pain/temp in face; facial pain Restiform body & cerebellum -- ataxia arm, leg, gait Vestibular nucleus -- nystagmus, N/V, vertigo Nucleus ambiguus - hoarseness, dysphagia Descending sympathetics -- Horner syndrome

Alexia without agraphia is most likely due to infarct in which artery?

Left PCA, can also see right homonymous hemianopia and achromatopsia and/or color anomia

Damage to which area causes writing apraxia or dysgraphia (difficulty performing the movements needed to write)

Left angular gyrus

Normal nerve conduction velocities (arms and legs) velocities in demyelination

Legs: 40-45 m/s Arms: 50-65 m/s Demyelination: 13-20's m/s

Elderly patient with HA, ataxia, weight loss, and enhancement of leptomeninges around cerebellum. Dx?

Leptomeningeal carcinomatosis (basilar meningitis appears similarly but doesn't fit Hx of wt loss and no fever)

Areas of brain involvement of neurosarcoidosis?

Leptomeningeal enhancement Parenchymal enhancement Pituitary stalk Hypothalamus Can be small rounded lesions and mimic MS Can be super tiny lesions Can be bigger lesions Can be diffuse (kind of like a lymphoma)

This Leptomeningeal metastases can present with severe headaches, nausea and vomiting indicating increase in intracranial pressure. Obstruction of CSF re-absorption can lead to a communicating hydrocephalus. Often, this is too subtle to appreciate on neuroimaging. Removal of CSF results in significant clinical improvement.

Leptomeningeal metastases

What is the earliest and most common finding in true Neurogenic thoracic outlet syndrome?

Lesion at the lower trunk of the brachial plexus Earliest finding: on EMG, absent or small medial antebrachial subcutaneous SNAP (this nerve is a branch from the medial cord of the brachial plexus)

interposed nuclei (globose and emboliform)

Lesion here gives you titubation, action tremor, rapid alert sting movements off, dysmetria

85yo female with 2 separate dark areas on SWAN in subcortical regions of R frontal and occipital lobes. Dx?

Likely amyloid angiopathy - punctate hemorrhages (if it were instead a hemorrhagic infarct it would affect cortical ribbon)

Damage to the lateral frontal lobe (frontal convexity syndrome)

Linked to motor structures: - Apathy - Indifference - Psychomotor retardation - Moter perseveration - Impersistence - Poor abstract thinking

Infant brain without gyri

Lissencephaly (Agyria) - neuronal migration abnormality

Type of hemorrhage caused by cerebral amyloid angiopathy

Lobar hemorrhage (which can extend into leptomeninges and appear as a SAH over the convexities)

Fibers of ciliary ganglion (sympathetic vs parasympathetic)

Located in the orbit receives input from ophthalmic nerve, carotid plexus, and oculomotor nerve parasympathetic fibers (change shape of lens for accomodation and pupil constriction) and Sympathetic fibers for pupillary dilation run through this ganglion but do not synapse here

Cell with rounded, targetoid inclusion in patient with epilepsy? Formal name for this type of epilepsy?

Lofora body - located in cerebellum of patient with Lofora body disease (myoclonic epilepsy)

Lateral pontine syndrome (Marie-Foix syndrome)

Long circumferential branches of basilar or AICA Lesion in lateral pons (includes middle cerebellar peduncle) - Ipsilateral arm/leg ataxia - Contralateral weakness (UE/LE) (CST) - Contralateral hemisensory loss of pain/temp (spinothalamic)

How to determine if a lesion is proximal or distal to dorsal root ganglion?

Look at sensation: If proximal (AKA radiculopathy) - sensation will be spared If distal to ganglion (AKA at a plexus) - sensation will be affected

How to see vasculitis on angio?

Look for "beading" or "sausage link" appearance

EEG findings in early Alzheimer's

Loss of alpha and beta frequency activity (so basically all the frequencies above 8 Hz)

Triphasic waves

Waves that cross the baseline twice. Non-specific, but common in metabolic encephalopathies (hepatic encephalopathy)

Neuronal ceroid lipofuscinosis (NCL)

Lysosomal storage disease, generally *AR* disorders Accumulation of autofluorescent lipopigments resembling ceroid and lipofuscin. Made up of fats and proteins and build up in neurons, liver, spleen, myocardium, and kidneys. Variable onset, yet progressive, sx, including *sz, dementia, visual loss, and/or cerebral atrophy* 4 types 1. Early infantile variant (INCL or Santavuori-Haltia): nL birth, early visual loss --> complete retinal blindness by 2 yrs, vegetative state by 3 yrs, death at 4. 2. Late infantile variant: 2-4 years get sz and deterioration of vision. Death 10-12 yrs. 3. Juvenile NCL (JNCL, Batten Disease, or Spielmeyer-Vogt): ~4-10 yrs get vision loss d/t retinal dystrophy, with sz, psychological degeneration, and eventual death 20s or 30s. 4. Adult variant NCL (ANCL or Kuf's Disease): milder sx ~30 yrs, death ~10 years later.

Progressive myoclonic epilepsies

Lysosomal storage disorders or mitochondrial disorders Tx: VPA (avoid if mitochondrial mutation due to liver failure), clonazepam, levetiracetam, topiramate, zonisamide, lamotrigine

Which Parkinson's agents are possibly the only disease modifying?

MAO-B (Selegiline) Exercise is best

" what in GMB can predict response to treatment?

MGMT promoter methylation

" Creutzfeldt-Jakob disease

MRI DWI/ADC hyperintensity cortical ribboning, caudate, and thalamus. CSF: 14-3-3 protein

46yo female presents with dizziness for 1 year that started immediately after a 7 day Caribbean cruise, describing this as a constant swaying sensation since she left the ship. This improves while she is driving her car, but immediately returns after she gets out of the car. It is much worse when sitting still at her desk at work. She denies nausea/vomiting and vertigo. MRI head is normal. Diagnosis? A) Vestibular migraine B) Motion sickness C) Mal de debarquement syndrome D) Chronic subjective dizziness E) Naviculam permanens syndrome

Mal de debarquement syndrome The history is consistent with Mal de debarquement syndrome, described by patients usually as a feeling of motion, rocking or swaying when there is no physical motion. This can be persistent and is most often provoked by an ocean voyage. The syndrome is notable for typically lacking nausea, vomiting, vertigo or other common vestibular symptoms as well as an absence of hearing symptoms. Patients usually feel some relief by being in motion themselves, either by driving or going back out on the ocean, and they feel most symptomatic when they are sitting still. Naviculum permanens is latin for permanent boat and is not a real disease.

People with central core myopathy are at increased risk for which condition?

Malignant hyperthermia (similar mutation / same chromosome?)

Right thalamus infarct causes

Mania

Vein of Galen malformation

Manifest as high output CHF and neurologic symptoms secondary to cerebral venous congestion and abnormal CSF flow. Tx: urgent endovascular embolization

Muscles supplied by CN V

Masseter Temporalis Medial/lateral pterygoids Mylohyoid Ant. belly of digastric Tensor veli palatini Tensor tympani

Which tracts are involved in withdrawal responses to noxious stimuli?

Medial and lateral reticulospinal tracts

Lesion in which part of thalamus can cause behavior and mood change?

Medial dorsal - because it has connections with amygdala, orbitofrontal region, and temporal lobe Lesions here can cause disinhibition, mania, memory loss

6 parts of the peripheral nervous system

Motor neuron & DRG Roots Plexus Nerve NMJ Muscle

Glial cytoplasmic inclusions are also related to abnormalities in alpha-synuclein and are seen in which disease?

Multiple system atrophy

Hot crossed bun sign

Multiple system atrophy ("+" shape in pons)

Lesions of lateral cord would affect muscles innervated by which nerves?

Musculocutaneous Median Lateral pectoral

Huntington's disease mutation and chromosome

Mutation in HTT gene on chromosome 4 that makes protein called huntingtin. Causes increased # of CAG trinucleotide repeats

Leprosy occurs due to _______ ______, a neurotropic bacterium. This diagnosis is supported by the distribution of this patient's sensory loss, which includes his ears and nose, and the fact that he has emigrated from a country endemic for leprosy.

Mycobacterium leprae

Symptoms of serotonin syndrome

Myoclonic jerks Tremor HTN Diarrhea Confusion Fever tachycardia Coma Death (can be caused by SSRI's, mirtazapine, trazodone)

MR Spectroscopy: which peak should be the tallest in a normal spectrum

N-Acetyl-aspartate (NAA) - it represents neuronal activity

Tumor in NF1 vs NF2

NF1: Neurofibroma NF2: Schwannoma

Sandifer syndrome

NOT a seizure. Spastic torticollis or dystonic movement associated with reflux

Defective gene in CADASIL

NOTCH3 gene (Chr 19) - can check for this via skin biopsy (of small artery in skin)

Methanol poisoning causes what change on imaging?

Necrosis of putamen

Disease that presents very similar to Huntington's Disease?

Neuroacanthocytosis

Mass outside of spinal canal and outside of meninges that enhances with contrast

Neurofibroma

Do patient's have normal head size in Alexander's disease?

No, megalencephaly

This is characterized by cognitive difficulties, gait apraxia and urinary incontinence. Imaging typically demonstrates a communicating hydrocephalus. Gait abnormalities are the most likely to improve after ventriculoperitoneal shunting

Normal pressure hydrocephalus

EEG finding for juvenile myoclonic epilepsy (JME)

Polyspike wave, 4 - 4.5 Hz

CADASIL gene

Notch3

Area of brain involved in addiction, substance abuse, reward

Nucleus accumbens

Nucleus ambiguus, Dorsal motor nucleus of vagus, Nucleus tractus solitarius

Nucleus ambiguus- branchial motor outflow to laryngeal and phargngeal muscles, Dorsal motor nucleus of vagus: parasympathetic outflow to viscera, Nucleus tractus solitarius: afferent visceral sensory information

Drug used to treat spinomuscular atrophy

Nusinerson - antisense oligonucleotide

Don't get urinary invontinence w ALS because

ONIF spinal nuclei are spares

Lesion of which nerve causes hip adductor weakness

Obturator nerve

If normal flow void (dark) is replaced with a bright area... Dx?

Occlusion/thrombus of that vessel

14 Hz + 6 Hz positive spikes

Occur in posterior head regions during light sleep in adolescents

Cerebral amyloid angiopathy

Often sporadic Highly assoc w/ dementia 5-15% of nontraumatic brain hemorrhage Incidence increases with age

Where do medulloblastomas spread?

Often spread to CSF. Can spread to systemic organs.

Where is a comfortable sodium level for oxbarbazepine (per Dr. Privitera)

Okay as long as Na>125, that means it's working (125 is ideal)

Which of the following diseases is most likely if the only abnormality on NCS/EMG is large motor units firing rapidly? - CIDP - HNPP - Old polio - MG

Old polio

Brain Tumor Question: Which tumor has a slow progression, mostly in frontal lobes, often calcification, and on path has clear halo of cytoplasm (fried egg)?

Oligodendroglioma

Tumor cells with "fried egg" apperance

Oligodendroglioma

Picture of bottom of brain where pons and cerebellum are super small... Dx?

Olivopontocerebellar atrophy (which is a component of MSA)

Myotonic discharge on EMG

Only Pompe and myotonic dystrophy

Early Onset Idiopathic Occipital Epilepsy (Panayiotopoulos Syndrome)

Onset 4-8 years sx: Tonic eye deviation, *vomiting,* autonomic sx, visual auras, *GTC in sleep* EEG: occicpital spikes in 1-3 Hz bursts when eye closure Treatment not required, resolves in a few years

Lennox-Gastaut Syndrome

Onset: 2-8 years Triad of seizures of multiple types -tonic, atonic, atypical absence, myoclonic. 1/3 have h/o infantile spasms. - a/w MR. 50% nL cognitively before sz's, but all show severe psychomotor retardation after onset of disease EEG: Interictal <2.5 Hz bilateral slow spike-wave complexes tx: VPA, lamotrigine, topiramate. ketongenic diet. Often refractory, can consider rufinamide or felbamate in those cases

West Syndrome

Onset: 4-7 months Triad: 1. Infantile spasm 2. hypsarrythmia 3. developmental arrest Multiple etiologies: hypoxic-ischemic, brain malformations, structural abnormalities, congenital or acquired infections, chromosomal abnormalities, inborn errors of metabolism, cryptogenic in 30% Think tuberous sclerosis (TS) and get MRI px: poor, can progress to LGS tx: ACTH (corticotropin) or high dose prednisone. If d/t TS treat with vigabatrin

Myoclonic Astatic Epilepsy Doose Syndrome

Onset: Preschool age (peak onset age of 2-6 yrs) sx: Previously nL child develops multiple sz types (GTC, absence, myoclonic, atonic astatic, myoclonic astatic) Usually characterized by difficult to control and possibly several seizures daily. EEG: Interictal 2-3Hz spike-and-wave discharges tx: VPA, Ethusoximide, keppra, benzos. May use LTG for GTCs. ketogenic diet can be the most effective treatment. Avoid: CBZ, vigabatrin Prognosis: VARIABLE - Some resolve completely and others develop intractable epilepsy with impaired cognition.

Early Infantile Epileptic Encephalopathy (EIEE) Ohtahara Syndrome

Onset: starting age 1 day to 3 months sx: Tonic seizures, possible infantile spasms. Severe developmental delay. EEG: Ictally with tonic sz, there is a high-voltage slow wave followed by fast activity. Interictally there is a *burst suppression* pattern. Treatment: Standard AEDs. Ketogenic diet Prognosis: Poor, 50% die during infancy. Others can progress to West syndrome or LGS.

What innervates the dura within the cranial vault?

Ophthalmic branch of CN V

Most common CNS tumor in patient with NF1?

Optic glioma

area of the brain damaged in people with socially inappropriate behaviors, disinhibition, and poor impulse control

Orbitofrontal cortex

The use of this drug can result in significant chronic hyponatremia, especially in the elderly

Oxcarbazepine

2 AEDs for people with seizures and bipolar disorder?

Oxcarbazepine & lamictal are good mood stabilizers

" side effects of VPA in women?

PCOS

5yo with developmental delay, progressive dysarthria, blepharospasm, torticollis, clumsiness, and retinal degeneration. Dx?

PKAN Caused by changes in PANK2 gene (deficiency of pantothenate kinase enzyme) - Clumsiness - Frequent falls - Retinal degeneration "eye of the tiger sign"

Which EEG finding occurs in most cases of HSV encephalitis?

PLEDs

HSV encephalitis EEG

PLEDs (periodic lateralizing epileptiform discharges)

Progressive Myoclonic epilepsy

PMEs are a collection of disorders characterized by progressive cognitive decline and seizures. Myoclonic seizures are most common, though tonic-clonic seizures may occur. Specific examples include Unverricht-Lundborg disease (Baltic myclonus), myoclonus epilepsy and ragged red fibers, Lafora disease, neuronal ceroid lipofuscinosis, and type I sialidosis. The most common of these is Unverricht-Lundborg disease, which is AutoRecessive, begins at ages 6 to 18, with seizures and progressive cognitive decline over the course of several decades.

This syndrome is associated with high levels of serum vascular endothelial growth factor and low levels of erythropoietin. Serum levels of VEGF normalize with response to therapy.

POEMS

Meaning of the term "POSTS" on EEG

POSTS= positive occipital sharp transients of sleep

Globose (kind of round) neurofibrillary tangles in substantia nigra, brainstem tegmentum, putamen, and select cortical and subcortical areas... Dx?

PSP

Mechanoreceptor in charge of sensing vibration

Pacinian corpuscles

Nucleus ambiguus contains motor fibers that supply muscles of which areas?

Palate Pharynx Larynx Disruption of this nucleus will impair phonation

School-aged children with this syndrome may have spells of vomiting followed by flaccid loss of consciousness

Panayiotopoulos syndrome A benign focal epilepsy of childhood Autonomic symptoms predominate. In about 1 in 5 seizures the child will become flaccid and unresponsive (ictal syncope). While ictal EEG shows occipital spikes, intraictal EEG often shows multifocal spikes. Conventional seizures with eye deviation or motor manifestations also occur. Many children will go on to develop BECTS.

Brown iron-containing pigment in globus pallidus and pars reticularis of substantia nigra. Dx?

Pantothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease)

Acquired sensory neuropathies (8)

Paraneoplastic Abs (anti-Hu) Sjogrens Acute sensory neuropathy (Anti GD1 Abs) Pyridoxine/B6 intoxication Cis-plantinum Doxorubicin Idiopathic sensory neuropathy

Autoimmune etiologies of neuropathy

Paraproteinemic neuropathy (SPEP, monoclonal gammopathy/IgM/DADS) Connective tissue disease associated (lupus, RA, Sjogrens) Vasculitis (polyarteritis, ANCA, nerve Bx)

Infection that causes multiple "bubbly" cysts in white matter, caudate, and putamen?

Parenchymal involvement of cryptococcal meningitis

On gross specimen of brain: multiple collections of bubbly cysts in white matter. Dx?

Parenchymal involvement of cryptococcal meningitis

What to tell parents with kids who had febrile seizures?

Parents should be reassured after a simple febrile seizure that there is no negative impact on intellect or behavior, and no increased risk of death. Routine laboratory tests, electroencephalography, and neuroimaging are not recommended in patients with simple febrile seizures. Use of long-term continuous or intermittent antiepileptic medication after a first simple febrile seizure is not recommended because of potential adverse effects. Use of antipyretic agents at the onset of fever is not effective at reducing simple febrile seizure recurrence.

conjugate upgaze paresis, nystagmus retractorius, and unreactive pupils.... syndrome?

Parinaud syndrome

Absence seizures

Peak age 6, more often in girls, usually normal neuro exam Multiple daily spells for a few seconds, begin and end abruptly, mild jerks of eyelids, eyes, eyebrows at onset, then blank stare, automatisms like lip smacking, nose rubbing, picking at clothes, provoked by hypoglycemia and hyperventilation. Thalamus implicated with low-threshold *T-type calcium channels* Tx: Ethosusximide (inhibits calcium channels), also may use VPA, lamotrigine, topiramate and zonisamide) AEDs can be discontinued in adolescence if seizure free 1-2 yrs and normal EEG, overall good prognosis, remissions in 90% Avoid: Lamotrigine, gabaergic drugs (activate calcium channels)

S2,3,4 keeps the 3P's off the floor

Penis Poo Pee

TPA and ICH

Per AHA/ASA guidelines, any history of ICH - CANNOT receive IV tPA because of risk for recurrent bleeding.

Pathognomonic histologic finding for dermatomyositis

Perifascicular atrophy (also common in juvenile dermatomyositis)

PLEDs meaning

Periodic lateralized epileptiform discharges - repetitive/rhythmic lateralized or focal spike, spike-wave, or sharp wave complexes recurring at regular or nearly regular intervals. Seen with acute/subacute cerebral dysfunction - infection (16%, commonly HSV), stroke (34%), abscess, subdural hematoma, tumor (13%), anoxia, metabolic

Brain Tumor Question: What is most common childhood tumor and statins GFAP?

Pilocytic astrocytoma (Glioma)

Brain Tumor Question: Which childhood tumor has slow growth, excellent prognosis, Rosenthal Fibers (corkscrew eosinophilic statining fibers)?

Pilocytic astrocytoma (Glioma)

Drug used to treat tics that can cause tardive dyskinesia?

Pimozide

Structure at most distal end of corpus callosum on saggital view?

Pineal (if enlarged and causing hydrocephalus = pineoblastoma)

What are the most common tumors in children?

Pliocytic astrocytoma, meduloblastoma, ependymoma

Fever, weight loss, myalgias in patient with HTN and polyneuropathy. Dx?

Polyarteritis nodosa

New enhancing lesion in brain of patient who was treated with radiation... cause?

Possibly radiation necrosis

Arterial supply of pineal gland

Posterior choroidal artery

Which part of brain does EEG lead F7 correlate to?

Posterior frontal lobe / anterior temporal lobe (on left)

Damage to which area causes speech apraxia

Posterior part/inferior frontal gyrus

Fornix

Principal output pathway of the hippocampus (big arch that connects hippocamus to mamilary bodies, septal area, and hypothalamus) Divided into two fiber bundles: 1. Pre-commisural fibers - innervate the septal area & anterior hypothalamus 2. Post-commusural fibers - innervate the mamillary bodies

45yo male presents with severe stabbing right orbital and temporal headache with associated right facial sweating, rhinorrhea and right eye tearing. He has had these 2-5 times daily for the past 10 days each lasting 1-2 hours. What medication would be best to break this headache cycle? A) Propranolol B) Prednisone C) inhaled oxygen and sumatriptan D) Verapamil

Prednisone This patient's symptoms and signs are most consistent with cluster headache. Prednisone is most helpful in breaking the cluster (transitional prophylaxis). Sumatriptan and inhaled oxygen would be most helpful as acute symptomatic treatments once the headache has developed. Verapamil may prevent future episodes (longterm prophylaxis) of cluster headache, but would be unlikely to stop his present cluster. Propranolol is usually not helpful in treating cluster headache. Ipsilateral greater occipital nerve block is another option for transitional prophylaxis in cluster headache

62yo male with poorly controlled diabetes presents with decreased sensation in both legs below the knee and painful pins and needles sensation in both feet. Which is most likely to help his pain? A) Improved glycemic control B) Nortriptyline C) Pregabalin D) Oxycodone E) Acupuncture

Pregabalin

Friedreich's ataxia (age of onset? symptoms?)

Presents b/w 5-15yo, but can present in the 20s/30s - Weakness in arms/legs - Ataxia - Vision & hearing impairment - Slurred speech - Scoliosis - Pes cavus (high arches) - Diabetes - HCM / Afib - Loss of vibration/proprioception - nystagmus - hyporeflexia

Hereditary neuropathy with liability to pressure palsy (HNPP)

Pressure on the peripheral nerves causing numbness, pain, weakness, atrophy In normal ppl an "asleep" limb goes back to normal quickly. In these people it lasts minutes up to months Deletion or mutation in PMP22 gene -- decreased motor and nerve conduction velocities Most commonly affected areas: - Peroneal neve at fibular head - Ulnar nerve at elbow - Median wrist at palm

Types of peripheral polyneuropathy (9 broad categories)

Primarily motor Primarily sensory Mixed small vs large fiber Autonomic involvement Distal > proximal Distal = proximal Distal < proximal Axonal vs demyelinating

Elderly patient with CD20+ staining mass with monotonous cytologic features. Dx?

Primary CNS Lymphoma

Spongiform change in cerebral cortex (little white holes everywhere like swiss cheese). Dx?

Prion disease

What are 4 tauopathies that are characterized by abnormal deposits of Tau proteins, Pick bodies and ballooned neurons

Progressive Supranuclear palsy corticobasal degeneration Pick's disease Frontotemporal dementia

Subacute sclerosing panencephalitis

Progressive brain inflammation due to persistent infection with measles (hx of primary measles infection prior to age 2) No cure, often fatal Gradual, progressive psychoneurological deterioration (personality change, seizures, myoclonus, ataxia, photosensitivity, spasticity, coma)

Hummingbird sign

Progressive supranuclear palsy (saggital view; atrophy of midbrain)

What is the earliest NCS/EMG finding in Guillain Barre syndrome?

Prolonged F waves Characteristically prolonged F responses in demyelinating illness Very sensitive in GBS because measures along very extensive segment of nerve

What are the three segments the neural tube forms?

Prosencephalon, mesencephalon and rhombencephalon -prosencephalon subsequently forms telencephalon which gives rise to cerebral hemispehres as well as diencephalon which forms hypothalmaus and thalamus -mesencephalon gives rise to midbrain -rhombencephalon gives rise to ponts, medulla and cerebellar hemispheres

Prosidy (definition, where is it produced, where is it processed)

Prosidy - the ability to produce and understand the emotional quality of speech Production of prosody depends upon non-dominant dorsolateral frontal lobe Comprehension of prosody is a function of the non-dominant temporal lobe

Aphasia: Intact speech, intact comprehension, repetition, writing, impaired naming, reading?

Pure alexia without agraphia

Alexia without agraphia

Pure word blindness Left occipital infarction with involvement of splenium of corpus callosum (losing visual input to language area) - Right hemianopia - They can write - Cannot read written words

Posterior cord lesions affect muscles innervated by which nerves?

Radial Axillary Thoracodorsal Subscapular

which nerves supply sensation to dorsal aspect of hand

Radial nerve: Back of thumb, digits 2-3, and half of 4th digit from furthest knuckle to wrist Ulnar nerve: 5th digit, half of 4th digit to wrist Median nerve: tips of thumb, digits 2-3, half of tip of digit 4

Sensory nerve action potentials are preserved in which of the following: - Neuropathy - Radiculopathy - Plexopathy

Radiculopathy

One and a half syndrome

Rare opthalmoparetic syndrome - Conjugate gaze palsy in one direction (neither eye can look to that side) - INO in the other direction - Can have hemiparesis and facial palsy too Unilateral lesion of dorsal pontine tegmentum (involving ipsi PPRF, fibers of ipsi MLF, and CN VI)

Rassmussen's Syndrome (Etiology, clinical presentation, Tx)

Rare, severe, inflammatory brain disorder Progressive unilateral hemispheric atrophy, progressive hemiparesis and cognitive decline, intractable focal seizures Antibodies to *glutamate receptor 3* Consider hemispherectomy

Side effect of lamotrigine

Rash

Oblique vs rectus eye muscles

Rectus looks laterally (up and out is superior rectus, pure lateral is lateral rectus, down and out is inferior rectus) Obliques look towards nose (superior oblique looks down towards nose; inferior oblique looks up towards nose)

Tolosa-Hunt Syndrome

Recurrent episodes of severe unilateral headaches with orbital pain + weakness/paralysis of certain eye muscles

Area of brain that maintains consciousness

Reticular formation (located in tegmentum of brainstem, basal forebrain, and thalamus) Diffuse bilateral hemispheric cortical lesion

Reticular activating system is made up of?

Reticular formation Locus ceruleus (norepi) Raphe nuclei (serotonin)

The suprachiasmatic nucleus receives direct input from where?

Retina, involved in circadian rhythm

What do the vertebral arteries branch off of?

Right and left subclavian arteries

Which tract maintains FLEXOR tone

Rubrospinal tract

Epilepsy Syndrome: What mutation is associated with Dravet Syndrome?

SCN1A

Gene mutation in generalized epilepsy with febrile seizures plus (GEFS+; seen in infants)

SCN1A mutation

What gene is GLUT-1 encoded by?

SLCA1 on chromosome 1p34.2

Genes linked to spinomuscular atrophy

SMN (survival motor neuron) NAIP (neuronal apoptosis inhibitory protein)

spinal muscular atrophy

SMN1 gene mutation, AR disorder. Proximal hypotonia and motor neuron/nerve process

Cause of repetitive high voltage slow wave complexes every 4-15 seconds?

SSPE (subacute sclerosis panencephalitis) - degenerative disease in kids thought to be 2/2 measles virus (motor spasms, cognitive decline)

First line treatment for OCD?

SSRIs

55yo male presents with attacks of stabbing left temporal and supraorbital pain lasting about 1 minute and occurring from 20 to >50 times per day, with associated tearing of his left eye. Can be triggered when brushing his teeth or chewing. His wife has noticed his left eye is red during attacks and sometimes his left eyelid becomes swollen. Diagnosis? A) SUNCT syndrome B) SUNA syndrome C) Cluster headaches D) Trigeminal neuralgia E) Paroxysmal hemicrania

SUNCT syndrome

≥1 ipsilateral autonomic Sx (conjunctival injection, lacrimation, rhinorrhea, eyelid edema, facial sweating, miosis, ptosis)

SUNCT/SUNA

TPA in dissections

Safety profile of tPA in ischemic stroke due to dissection is similar to other causes of ischemic stroke

Brain Tumor Question: What is the tumor a/w S-100 and has association with neurofibromatosis type 2 when bilateral?

Schwannoma

Frontal Lobe Epilepsy

Second most common type of focal epilepsy Semiology can be bizarre - hypermotor behavior (e.g. bicycling), may also have vocalizations Brief (typically <30sec), can be clustered, often no post-ictal confusion

Most common presentation of cavernoma

Seizure

Myocolonic epilepsy with ragged red fibers (MERRF)

Seizures, myoclonus, ataxia, myopathy. maternally-inherited point mutation at position *8344 in the mt genome disrupts gene for tRNA-Lys and synthesis of proteins essential for oxidative phosphorylation (in 80%) "Ragged Red Fibers" - clumps of diseased mt accumulate in the subsarcolemmal region of the muscle fiber and appear as "Ragged Red Fibers" when stained with modified Gömöri trichrome sx: progressive myoclonic epilepsy, short stature, ataxia, hearing loss, lactic acidosis, exercise intol, poor night vision tx: symptomatic, High doses of Coenzyme Q10 and L-Carnitine

"SNAP" on nerve conduction studies

Sensory nerve action potential: summated action potentials of large & medium sized myelinated fibers (NOT small unmyelinated fibers -- that's why small fiber neuropathies can have normal NCS)

Dravet Syndrome

Severe myoclonic epilepsy of infancy due to sodium channel mutation *(SCN1A)* with developmental delay and neurologic abnormalities. Febrile seizures start first year of life followed by other seizure types (myoclonic, atypical absence, GTC. Tx: VPA, topiramate, zonisamide and ketogenic diet. Avoid: Phenobarbital, phenytoin, carbamazepine and lamotrigine

Carbidopa/Levodopa

Side effects: nausea Start with 25/100 TID dosing and titration up

When EEG has hemispheric asymmetry (in the ABSENCE of a skull defect), how to determine which hemisphere is abnormal?

Side with lower alpha activity is the ABNORMAL one

Myoedema

Slow, prolonged electrically silent mounding caused by percussion of a muscle in a patient with hypothyroid myopathy (painful cramps, elevated CK, normal reflexes)

Mnemonic for which CN's are sensory vs motor vs both

Some say money matters but my brother says big boobs matter more I = Sensory II = Sensory III = Motor IV = Motor V = Both VI = Motor VII = Both VIII = Sensory IX = Both X = Both XI = Motor XII = Motor

History of fluctuating lower thoracic myelopathy that's worse with Valsalva and exertion. Spinal cord edema on imaging. Dx?

Spinal AV fistula

Tethered cord syndrome

Spinal cord conus extends down into sacral region. Cannot see cauda equina b/c its so low. Conus adheres to posterior aspect of spinal canal within the sacrum

Vimpat dosing

Start at 50-100 bid Increase weekly by 100 mg daily Usual maintenance dose: 200 - 400 mg/d Max dose: 600 mg daily

Anti-amphiphysin antibodies are associated w/ which disease?

Stiff man syndrome

Inhibition of unwanted or inappropriate responses falls under the rubric of executive function. What test is used when subjects are presented with names of colors written in colors other than the one spelled out. After reading the words, subjects then must report the color of the word seen, rather than its meaning (e.g., the word red written in green ink), necessitating inhibition of the immediate response to read the word as written.

Stroop

Generalized periodic long-interval discharges on EEG that recur every 4-15 seconds... Dx?

Subacute sclerosis panencephalitis

28yo male with no PMH p/w intermittent left sided throbbing headaches with associated nausea and photo/phonophobia, 2 headache days per month. Most appropriate management? A) Propranolol B) Topiramate C) Topiramate and sumatriptan D) Sumatriptan

Sumatriptan This patient's symptoms are most compatible with migraine. Although he may ultimately require preventative migraine therapy in an attempt to reduce the frequency, duration, or severity of attacks, his current frequency of two headaches per month warrants an initial trial of abortive therapy with a migraine specific agent (e.g. triptan) for his attacks. If his headaches do not respond well to abortive therapy, if he is intolerant of side effects of abortive medication, or if his headaches increase in frequency, then a prophylactic agent would be appropriate.

Two arm supinator muscles

Supination of the forearm is done primarily by the supinator muscle. However, when the forearm is flexed and pronated, the biceps muscle is the most powerful forearm supinator.

Cause of N/V during initiation of Sinemet therapy

Supoptimal dosing It takes 100-150 mg of carbidopa per day to saturate the peripheral aromatic AA decarboxylase ezyme to prevent peripheral side effects of levodopa

Which nucleus in the brain controls circadian rhythm

Suprachiasmatic nucleus of hypothalamus (regulates melatonin secretion by pineal gland)

Entrapment of what nerve causes shoulder pain, weakness of shoulder abduction, and sparing of the sensation about the shoulder?

Suprascapular nerve

Which muscle initiates the abduction of a fully adducted arm?

Supraspinatus

This is in the differential diagnosis for patients who present with foot pain. Pain around the medial malleolus is the most common symptom with associated paresthesias. Pain is often worse at night and with weight bearing. The syndrome is due to entrapment of the distal branch of the tibial nerve at the medial ankle (the medial plantar nerve). Causes include trauma, degenerative bone disease or rarely, mass lesions.

Tarsal Tunnel Syndrome

Which tract mediates reflex postural movements in response to visual stimuli?

Tectospinal tract (arises from superior colliculus and descends to the upper cervical regions - C1-C4)

Exploring objects orally is a feature of ______ dysfunction

Temporal lobe

Superior gluteal nerve innervates which 3 muscles

Tensor fascia latae Gluteus medius Gluteus minimus

Which circuit is important in sequential hand movements and simple rhythm tapping?

Test of frontal lobe function, affecting frontal-subcortical circuits (frontal cortex to basal ganglia to thalamus and back to frontal cortex)

Nutritional/vitamin deficiencies that cause neuropathy

Thamine B12 Vitamin D Copper

Pituitary apoplexy

The infarction of a pituitary adenoma with resultant rapid swelling and pressure exertion on neighboring structures Will be isodense to brain tissue but will have contrast enhancement around the ring. Will see a darker area inside of it corresponding to the necrosis

How does the circadian rhythm work

The suprachiasmatic nucleus inhibits the paraventricular nucleus of the hypothalamus, which decreases paraventricular driving of the superior cervical ganglion, thereby decreasing sympathetic drive onto the pineal gland

Muscles used for shoulder abduction (4)

The supraspinatus helps abduct the shoulder through the first 15 degrees. With deltoid contraction, the arm is abducted (raised laterally) to the horizontal plane. Elevation above the horizontal plane is assisted by contraction of trapezius and serratus anterior.

In which ganglion do CN IX fibers synapse?

These parasympathetic fibers derived from the inferior salivatory nucleus synapse in the otic ganglion prior to reaching the parotid

What are Microglia?

They are a type of glial cell that are CNS phagocytes.

What are ependymal cells?

They are a type of glial cell that line CSF filled ventricles and spinal canal.

What are oligodendroglia?

They are a type of glial cell that myelinate cells of CNS. They look like fried egg on pathology. They myelinate many axons.

What are astrocytes?

They are a type of glial cell that provide physical support, repair injury, create scar tissue (reactive gliosis), and feet form layer of BBB

What are schwann cells?

They are a type of glial cell that that myelinate cells of peripheral origin. Schwann cells only myelinate one axon.

What are Nissl bodies?

They are the rough ER in neurons.

What causes contrast enhancement on MRI?

Things that break down the blood brain barrier: - Malignancy - Infarction - Encephalitis - Hemorrhage - Abscess

Systemic/metabolic diseases that cause neuropathy (4)

Thyroid Diabetes Renal failure/uremia Porphyria

The use of this drug often engenders reversible mental slowing, memory disturbance, weight loss and paresthesias in the extremities.

Topiramate

Which of the following has Class A evidence for use in prevention of episodic migraine? A) Topiramate B) Botox C) Verapamil D) Gabapentin E) Frovatriptan F) Indomethacin

Topiramate According to AAN guidelines, there is Class A evidence supporting the use of topiramate for migraine prophylaxis. Botox is indicated for chronic migraine rather than episodic migraine. Frovatriptan can be used as prophylaxis, specifically for menstrual migraines. Indomethacin is used for chronic paroxysmal hemicrania. Gabapentin can be helpful with headaches that have a stabbing type quality. There is currently no Class A evidence for the use of verapamil in this setting.

Benedikt syndrome (cause, manifestations)

Type of midbrain syndrome. Due to occlusion of PCA or penetrating branches of basilar - affects red nucleus, CST, CNIII nucleus, brachium conjunctivum, and superior crebellar peduncle - Ipsilateral CN III palsy ***Contralateral tremor *** *** Contralateral choreoathetotic movements *** - Contralateral ataxia - Athetosis (involuntary writhing movements) - NO HEMIPLEGIA

Transient global amnesia (how long?)

Typically lasts < 12 hours (can't recall recent events; impairs new learning - but do have immediate recall) produces no other focal neuro deficits disappears w/o residual effects

Benign Idiopathic Neonatal Seizures

Typically occurs around fifth day of life "Fifth day fits" Neurologically normal infant, no family history Partial clonic seizures can progress to status epilepticus No EEG findings specific for the diagnosis GOOD PROGNOSIS - Resolves in approximately 24 hours, normal development, not at increased risk of seizures as adults.

Rizatriptan half life and onset

T½ 2-3hrs, onset 30-120m

Zolmitriptan half life and onset

T½ 2.5-3hrs, onset 45m

Frovatriptan half life and onset

T½ 25hrs, onset 120-180m

Sumatriptan half life and onset

T½ 2hrs, onset 20-30m (tablet)

eletriptan half life and onset

T½ 3.6-5.5hrs, onset 30m

Naratriptan half life and onset

T½ 5.0-6.3hrs, onset 60-180m

Lesions of medial cord would affect muscles innervated by which nerves?

Ulnar Median ALSO sensory N; medial cutaneous N of the forearm Medial Cutaneous N of the arm

Primary Lateral Sclerosis (PLS)

Upper motor neuron for at least 3 years (no lower motor neuron signs)

Area of the spinal cord that is most vulnerable to ischemic insult

Upper thoracic (T1-T4) because it's in a vascular boundary zone b/w ascending and descending sources of blood supply

Prothrombin Complex Concentrate (PCC)

Used to reverse factor Xa inhibitors Corrects INR rapidly and completely Superior to FFP

What does the brain MRI look like in those with Alexander's disease?

Usually anterior/frontal lobes WITH INVOLVEMENT of U fibers

Which cranial nerves carry special visceral efferent fibers (carry motor impulses to pharyngeal arches)

V VII IX X XI

Which cranial nerves carry general somatic afferent fibers (conduct impulses of pain, touch, and temp from surface of body back into brain)

V - receives contributions from facial, glossopharyngeal, and vagal nuclei but output via trigeminal nerve VII

Which cranial nerves carry general visceral afferent fibers (conduct sensory impulses such as pain from internal organs, glands, and vessels to the CNS)

VII IX X

Which cranial nerves carry special visceral afferent fibers (carry special senses of smell and taste)

VIII IX X

What AED is shown to cause lower IQ in patients when used in utero?

VPA

Muscle biopsy with large white holes in each cell

Vacuolar myopathy due to extensive glycogen storage --> Pompe disease

This virus resides in the dorsal root ganglia and, upon activation, utilizes axonal transport machinery to reach the skin surface. It is also capable of traveling centrally to the cord resulting in a myelopathy.

Varicella Zoster Virus (VZV)

This a common presentation of giant cell arteritis, that usually produces severe monocular or binocular visual loss. It often occurs in association with headache, systemic symptoms and elevated ESR.

Vasculitic anterior ischemic optic neuropathy

Cytotoxic vs vasogenic edema

Vasogenic: blood brain barrier is disrupted. Extracellular edema. Mainly affects white matter due to leakage from capillaries. Seen around: brain tumors, abscesses, cerebral contusion, hemorrhage. Bright on T2/FLAIR, no restricted diffusion. Cytotoxic: BBB intact. Occurs when extracellular water passes into cells, causing cells to swell. Seen in ischemia. +diffusion restriction

Which of the following muscles is NOT innervated by the L5 root? - Gluteus medius - Vastus medialis - Tibialis posterior - Peroneus longus

Vastus medialis (L2-L4) - one of the quadriceps muscle (knee extension)

Which SNRI can cause HTN?

Venlafaxine

32yo male has severe sharp left supraorbital headaches lasting 30m-2.5hrs with associated restlessness, left miosis and rhinorrhea. He has had these for the last 2 years, occurring 1-5 times per day, with a headache-free period of no more than one month. He develops a headache within 1-2 hours anytime he consumes alcohol. What is the most appropriate prophylactic medication for his frequent headaches? A) Atenolol B) Verapamil C) Imipramine D) Ergotamine

Verapamil The patient described has cluster headache and is being considered for prophylaxis of this condition. Ergotamine is an abortive treatment. Atenolol has little CNS penetration and is not recommended for headache prophylaxis; imipramine is more appropriate for migraine prophylaxis. Verapamil, a calcium channel blocker, is currently the preferred agent for prophylaxis of cluster headache.

Which tract maintains EXTENSOR tone

Vestibulospinal tract

Drug used to treat refractory complex partial seizures & epileptic spasms/west syndrome that can cause permanent visual field loss?

Vigabatrin

Basilar invagination

When the base of the skull folds inwards and can crush the brainstem due to migration of the top of the C2 vertebra (odontoid process)

Imaging finding in adrenoleukodystrophy

White matter changes in occipital and parietal regions that spares U-fibers (less commonly involves frontal lobes)

What test is used as a measure of prefrontal cortical function

Wisconsin card sorting test, it assesses visual conceptualization and set shifting.

Physical manifestation of a posterior interosseous nerve lesion. Where does this nerve get trapped?

Wrist drop (weakness of finger extensor muscles) Radial wrist deviation on extension (b/c extensor carpi radialis will be intact but extensor carpi ulnaris gets knocked out) PIN is a purely motor branch of radial nerve Gets trapped at Arcade of Frohse (part of the supinator muscle)

This cramp a form of focal dystonia, is an activity-specific movement disorder associated with discomfort and abnormal posturing without more generalized symptoms.

Writer's

Pyruvate dehydroxylase deficiency is inherited in which fashion?

X linked

Aicardi syndrome

X-linked dominant syndrome - agenesis of corpus callosum - retinal lacunae - developmental disabilities - infantile spasm

skeletal contraction, sarcomere

Z discs move closer together. Actin and myosin slide past each other but do not shorten. H zone is middle fo sarcomere occupied by only myosin without overlaping actin, shrinks during contraction

Disease characterized by weakness, hypotonia, seizures in an infant with a high forehead, hypoplastic supraorbital ridges, and midface hypoplasia with elevated serum very long chain fatty acids.

Zellweger Syndrome (autosomal recessive peroxisomal disorder)

45 year old male with PMH of CAD s/p stents and chronic migraines. Which medication should not be prescribed? A) Topiramate B) Propranolol C) Nortriptyline D) Zolmitriptan

Zolmitriptan

There is marked widening of the cerebellar folia with atrophy within the brain stem demonstrated, along with very decreased gradient T2* weighted signal layered along the cerebellar folia and other structures. There is also decreased signal on the T2- FLAIR weighted views layered along the cerebellar folia. The pattern and signal characteristics are that of extensive hemosiderin deposition, characteristic of systemic siderosis. This is generally seen associated with chronic subarachnoid hemorrhages of multiple etiologies. This syndrome is often associated with ataxia, neural sensory hearing loss, and dementia.

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What is Wyburn-Mason syndrome?

a neurocutaneous disorder that causes multiple AVMs of the face, retina, and intracranially

acute confusional state

abrupt onset and short duration of attentional alteration. Impaired, fluctuatin, reduced attention. Can have but not defined by cognitive deficits such as memory/comprehension, motor abn, hallucinations, delusions, mood alterations, sleep wake cycle disturbances, neurovegetattive alterations

What is Joubert syndrome?

absence or underdevelopment of the cerebellar vermis

What is the first-line medication for tension-type headaches in pregnancy?

acetaminophen

Acute organophosphate poisoning is secondary to __________ _______ and results in pinpoint pupils, excessive salivation, weakness and respiratory collapse

acethylcholinesterase inhibition

Landau-Kleffner syndrome

acquired epileptic aphasia. In healthy children who acutely or progressively lose receptive and expressive language ability, with paroxysmal EEG changes in sleep, mediated by inappropriate reinforcement of synaptogenesis

This can occur by dopamine receptor blockade by metoclopramide. This adverse effect is most likely to occur in younger patients within the first 48 hours of starting antidopaminergic medications. The appropriate therapy is an anticholinergic agent such as benztropine or diphenhydramine

acute dystonic reaction

Posterior corpus callosum lesion results in what?

alexia without agraphia

Skin bronzing and adrenal insufficiency are hallmarks of what disorder?

adrenoleukodystrophy

Risk factors for IVIG induced renal failure

age, renal impairment, DM

Anterior corpus callosum lesions result in what?

akinetic mutism or tactile anomia

Treatment for neurpathic pain in diabetic distal symmetric polyneuropathy

amitriptyline (anticholinergic cardiac toxicity), gabapentin, pregabalin, duolextine(nausea), venlafaxine

Isolated lesion in the anterior nucleus of the thalamus can cause...

amnesia

What structure may be related to the development of GAD?

amygdala

Kluver busy

amygdala impairment--> inappropriate sexual behaviors, mouthing objects, loss of normal fear or anger

What is the pulvinar?

an association nucleus in the thalamus involved in visual processing

Semantic memory localizes where?

anterior and inferior temporal lobes

Lesion of what nerve impairs the ability of the patient to make an OK sign with the thumb and index finger?

anterior interosseous nerve

In the Papez circuit, the mammillary bodies project to where?

anterior nucleus of the thalamus

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome

as well described by the name, is characterized by very brief attacks of periorbital pain with ipsilateral tearing and eye redness. The attacks of this syndrome resemble those of cluster headaches but are primarily differentiated by the much briefer attack duration and greater frequency in this than cluster headache. This can also resemble V1 trigeminal neuralgia but the latter should not have the prominent autonomic features of this.

F wave responses

assess entire lengthof motor axon, can be absent or delayed in demyelinating such as AIDP

This is a common complication in patients with Spinal Cord Injury above the level of T6. Episodes are characterized by headaches, diaphoresis, hypertension and bradycardia. They are caused by noxious stimuli below the level of injury such as bladder distention, fecal impaction and pressure sores.

autonomic dysreflexia

How is GLUT-1 deficiency inherited?

autosomal dominant

What is the mode of inheritance of Alexander's disease?

autosomal dominant

How are most of the porphyria's inherited?

autosomal domnant

How is PKU inherited?

autosomal recessive

Pantothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease)

autosomal recessive progressive neurodegenerative disease with neuroaxonal dystrophy Buildup of iron in the brain Parkinsonism, dystonia, dementia, and ultimately death Onset prior to 15yo Mutation in PANK2 gene Brown iron-containing pigment in globus pallidus and pars reticularis of substantia nigra -- "EYE OF THE TIGER" sign

Metachromatic leukodystrophy

axial T2-weighted images demonstrate symmetric, striped, hyperintensity of the periventricular white matter. The postcontrast axial T1-weighted image demonstrates enhancement of the optic nerves (blue arrow) and oculomotor nerves (red arrow). Metachromatic leukodystrophy is a lysosomal storage disorder that is inherited in an autosomal recessive pattern. It has three forms: late infantile, juvenile, and adult. The severity of symptoms depends on the age of onset. In the commonest form, children develop symptoms between 12 and 18 months. Both motor and language skills regress in affected children. Involvement of the peripheral nervous system leads to sensory loss. Patients develop progressive visual and hearing loss and dysphagia. Most patients develop seizures. They become progressively demented, weak, and rigid, leading to death by age 5. On MRI, there are symmetrical hyperintensities of the periventricular white matter, which is said to show a "tigroid pattern" due to dark stripes that appear within the hyperintense area. Enhancement of cranial nerves may be seen.

what causes prosopagnosia

b/l fusiform (occipitotemporal) gyri lesion

This individual has problems with procedural memory, that is the ability to acquire (with practice) cognitive and behavioral skills that subsequently operate automatically. Procedural memory is associated with lesions of the?

basal ganglia-cerebellum supplementary motor area system

What does the adult form of metachromatic leukodystrophy have that the infantile and juvenile form does not?

behavioral changes, psychosis, and dementia.

Capgras delusion

belief that close relative or spouse has been replaced by imposter

Mirror sign

belief that one's reflection in mirror is some other person

subjective doubles

belief that ther is a double of the patient that is carrying out independent actions

early onset benign childhood occipital epilepsy (Panayiotopoulos type)

benign childhood epilepsy with predominantly autonomic features, such as a nausea with vomiting, hypersalivation, pallor, and tachycardia. It presents in children at around age 5 years. Most of the seizures are prolonged and half of them last more than 30 minutes thus constituting autonomic status epilepticus More generalized seizures present with cognitive dysfunction and hemi- or generalized convulsions. In some cases, the seizures are often sustained, and focal status epilepticus may occur. An EEG will show multifocal high-amplitude sharp and slow wave complexes that often shift regions. Occipital spikes are the most common abnormality.

what can worsen autoimmune myastehenia gravis?

beta-blockers, NM blocking agents, anticholinergics, antibiotics such as aminoglycosides

Although the diagnosis of normal pressure hydrocephalus can be problematic, some studies have stratified both positive and negative predictors of outcome from shunting. Patients whose initial presentation is gait disorder have a _____ chance of successful outcome (if other confounding diagnoses, such as parkinsonism, are excluded), while those with dementia of greater than 2 years' duration, dementia preceding the gait disorder, or other factors which are more consistent with Alzheimer dementia (aphasia and loss of hippocampal volume) predict a ______ outcome from shunting. Presence of urinary incontinence is not predictive of improvement from shunting.

better; poorer

Lesion where would cause a locked in syndrome BUT responds to pain?

bilateral frontal (akinetic mutism)

Pseudobulbar affect localizes where?

bilateral lesions that disconnect the corticobulbar tracts from the brainstem cranial nerve nuclei

manganese disposal

biliary excretion. Patients with biliary atresia, chroic liver disase or high exposure to managenes during TPN can develop managnese intoxication. Features: parkinsonism and dystonia that do not respond to levodopa. MRI T1 shows hyperintneisyt of globus pallidus, striatum, midbrain

PSP vs CBD

both have parkinsonism. PSP has truncal/axial rigidity, tend to fall backwards. Others have appendicular rigidity. PSP can't look down. Both have frontotemporal dementia and obsessive compulsive behaviors. CBD has appendicular rigidity with forward and side falls. MSA falls due to dysautonomia

Infantile Spasms

breach rhythm

Treatment for neuroleptic malignant syndomre

bromocriptine

How does glucose cross the blood-brain barrier?

by GLUT-1, membrane-bound protein

The superior visual fields project to the inferior lip of the contralateral ________ sulcus.

calcarine

oxcarbazepine

can produce hyponatremia due to SIADH

If you're in a bind you can substitute oxcarbazepine for carbamazepine.. what's the ratio?

carbamazepine : oxcarb 200 : 300

Treatment for trigeminal neuralgia

carbamazepine, baclofen, lamotrigine, pimozide

Bilateral necrosis of globus pallidus is due to __________

carbon monoxide intoxication

AEDs that have risk for kidney stones?

carbonic anhydrase inhibitor- zonisamide, topiramate

What syndrome is characterized by ipsilateral Horner syndrome and neck pain with contralateral hemiparesis?

carotid dissection

Syncope associated with glossopharyngeal neuralgia results from activation of the _____ ______ branch of the glossopharyngeal nerve, resulting in bradycardia and hypotension due to peripheral arterial vasodilatation.

carotid sinus

What sleep symptom is most specific for narcolepsy?

cataplexy

Drug induced parkinsonism

caused by high potency neuroleptic antipsychotic such as haloperidol. Low potency chlorpromazine and atyypical olanzapine, aripriprazole, quetiapine, can cause it too

What location is preferred for ependymomas?

center of the conus medullaris

Rolandic epilepsy EEG

centrotermporal spikes on normal background activated by drowsiness and sleep

Most frequent site of CNS metastases

cerebellum

CADASIL (what it means, what it affects, histo staining)

cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Most common hereditary stroke disorder; a type of leukodystrophy Affects small/medium-sized arteries of white matter Abnormal vessels stain PAS-positive

In patients with hepatic encephalopathy-induced coma, up to 80% develop ________ _______ , which is the leading cause of death in these patients.

cerebral edema

The full-blown of this syndrome may be seen after revascularization of an artery (either with surgery or stenting) and consists of headache, neurologic deficit and seizures.

cerebral hyperperfusion

What is a serious complication of subarachnoid hemorrhage typically occurring between the 4th and 14th day after hemorrhage. It often presents with focal neurologic deficits and drowsiness.

cerebral vasospasm

ICA segments

cervical, petrosal, cavernous, supraclinoid. ICA emerges from supraclinoid segment

High flow oxygen is effective for _____ headaches, which have a male predominance, are longer lived than PH and SUNCT, often occur at night, and are not responsive to indomethacin

cluster

Prophalactic verapamil is indicated for which type of headache?

cluster

Idebenone

co enzyme q10 analog that can improve respiratory function in DMD

Cerebral amyloid angiopathy

common cause of lobar intracerebral hemorrhage in elderly. - Implicated as a potential cause of chronic micro bleeds on gradient echo MRI

myopathies from statins

common clinical problem, muscle toxicity, myalgias, myopathy with weakness, elevated CK greater than 10x normal.

paroxysmal tonic limb spasms

common in MS. Due to spontaneous neuronal discharge in demyelinating lesions in spinal cord. Painful, brief, 30-120 seconds, occur in clusters, other paroxysmal events include ataxia, dysarthria, pruritis. Can be treated with carbamazepine

CN4

completeley decussates before it exits from dorsal brainstem,--> SO--> depression/intorsion of i/l eye

Alien limb syndrome occurs to lesions where?

contralateral anterior cerebral artery territory, involving the corpus callosum or supplementary motor area

How is plavix metabolized

converted to active metabolite by cytochrome P450. genetic variants of CYP are poor metabolizers and have increased risk for stroke

Male with tall stature, marfinoid habitus, intellectual disability, ectopic lentis. Plasma homocysteine levels elevated and methionine levels elevated. What is this caused by?

cystathionine-β-synthase deficiency.

Cause of a central cord lesion which produces pain and temperature dysfunction in a bilateral "shawl" or "cape" distribution

due to involvement of crossing fibers for these modalities in the anterior (aka ventral) white commissure

meralgia paresthetica

damage to the lateral cutaneous nerve of the thigh

Laminar necrosis

death of cells in the cortex of the brain in a band-like pattern. Relative preservation of cells immediately adjacent to the meninges. Caused by ischemic insults

Norepinephrine

decrease in depression, increase linked to mania

How do you treat PKU?

dietary restriction of phenylalanine, should also have low protein diet and phenylalanine free feeding formula

What does MRI show in Pelizaeus-Merzbacher disease?

diffuse hypomyelination characterized by T2 hyperintensity and loss of white matter with relative thinning of the corpus callosum

This test, tests working memory, while the CVLT is a test of episodic memory.

digit span subset

Hemispatial neglect

does not respond or fails to notice one half of space (usually left)

Aphemia localizes to where?

dominant operculum (frontal lobe)

Ideomotor apraxia is caused by lesions where?

dominant parietal cortex, around superior marginal and angular gyrus

What is the source of parasympathetic outflow to most of the viscera?

dorsal motor nucleus of the vagus

What structure is involved in the planning of motor activity and behavior, executive functioning, judgement and problem solving?

dorsoLATERAL prefrontal cortex

Botulism

double vision, blurred vision, drooping eyelids, slurred speech, dysphagia, dry mouth, muscle weakness 18-36 hours after

generalized beta activity

drug use, benzo barbs.

Common manifestations of ataxia telangiectasia

due to defect in ATM gene - recurrent infections - conjunctival & malar telangiectasia Later: - nystagmus - ataxia

What embyronal layer gives rise to the nervous system?

ectoderm

What are the symptoms of galactosemia?

eeding difficulties, vomiting, diarrhea, and jaundice. They also have hepatomegaly, failure to thrive, lethargy, and hypotonia. Cataracts also occur and are caused by an accumulation of galactitol. Late neurologic sequelae include developmental delay, cognitive impairment, ataxia, and tremor, with brain MRI demonstrating white matter changes and cortical and cerebellar atrophy.

What do you expect on labs with PDH deficiency?

elevations of lactate and pyruvate levels, with a low lactate:pyruvate ratio

Orbitofrontal cortex lesions

emotion and reward with decision making, creation and extinction of behaviors based on reward or punishment

superior temporal cortex

emotion with facial stimuli

Bradycardia is associated with a first-dose effect of?

fingolimod

" alphaglucosidase alfa

enzyme replacement for Pompe's disease (acid amltase def)

West syndrome

epileptic infantile spasms and hypsarrhthmia.

Those with PDH deficiency have what symptoms?

episodic or progressive ataxia, nystagmus, dysarthria, lethargy, weakness with areflexia, hypotonia, and psychomotor retardation

Braca aphasi

expressive impairments, deficits in comprehension of short connector words and passive speech.

Severely suppressed immune status, as seen in patients with AIDS, lymphoma and on rheumatological therapies such as antiTNF drugs confers a much greater risk of dissemination to________ sites: an estimated 30-50% of infections that disseminate are found in these individuals

extrapulmonary

This is even more rare than complicated pulmonary disease i, estimated to occur in fewer than 0.5% of infections in Caucasians. However, it is felt to be several times higher in persons of Filipino and African American descent

extrapulmonary spread

Most common genetic risk factor for thrombophilia

factor V leiden (prevalence in AA is quite low)

What do patient's with PKU look like?

fair, with blond hair, blue eyes, and pale skin given the lack of tyrosine and melanin pigment production

T/F Hypnagogic hallucinations and sleep paralysis cannot be experienced by normal individuals

false

T/F Oral corticosteroids have been shown to be useful in treating MS exacerbation

false

T/F Embolism from cardiac or large artery sources underlie many cases of NAION

false. Few, if any.

T/F Prophylactic antiepileptic drugs are usually advised in acute ICH

false. Not advised

T/F Corticosteroids, hyperbaric oxygen, and anticoagulation are beneficial for this NAION

false. Not beneficial

T/F Triptans are effective to abort episodes of cluster headache, PH, or SUNCT

false. Usually do not abort episodes of PH or SUNCT

T/F Pulmonary or extrapulmonary complications are uncommon among diabetics, older people and men

false. more common

Are neural tube defects more common in males or females?

females

Lumbar plexus vs femoral neuropathy

femoral would not involve adductors. Obturator would not affect vastus medialis, lateralis and iliopsoas

anterior interosseous nerve

flexor digitorum profundus to digits 2 and 3, FPL, prontor qudratus,. Weakness flexing distal phalanges of thumb and index finger and forearm pronation.

aphasia, fluency

fluent if word output high per minute, >5 words per phrse, content is low. Paraphasias, nondysarthria speech, normal prosody. Logopenia is low word production with intact content

One of the classes of antibiotics that has been reported to cause seizures is the ___________,which exert their CNS effects by primarily interacting with GABA receptors. The co-administration of non-steroidal anti-inflammatory agents further potentiates this effect.

fluoroquinolones

What is the most common side-effect of Dimethyl fumarate? The drug is also, to a slightly lesser extent, associated with gastrointestinal upset and abdominal pain, and less commonly, with diarrhea.

flushing, treated with aspirin

Where does CN V3 exit the skull

foramen ovale

Where does CN V2 exit skull

foramen rotundum

The best predictor to assess the need for intubation in patients with neuromuscular respiratory failure (NMRF) is the?

forced vital capacity (FVC)

What is the main efferent fiber system from the hippocampal formation?

fornix

How many different types of metachromatic leukodystrophy are there?

four

Glutaric aciduria is caused by a deficiency of which enzyme? Sx? Tx?

glutaryl-CoA dehydrogenase Can't break down lysine, hydroxylysine, & tryptophan - Metabolic acidosis - Macrocephaly - +/- hypotonia - If they get sick they can develop hyperkinetic movement disorder and seizures - Tx: supplement with carnitine (can help)

Lacrimal gland innervation

greater pretrosal branch of CN7

When contrast is administered, subacute infarcts commonly enhance with a ______ pattern

gyriform

Most common non-neurologic manifestation of a Vein of Galen aneurysm?

heart failure

Dopamine

helps with symptoms of depression, deficiency doesn't lead to depression

A lesion where can cause apraxia of the non-dominant limb with preservation of all motor and language?

lesion at the corpus callosum

Patients who develop liver failure acutely are more prone to development of ______ encephalopathy.

hepatic

Thinning of corpus callosum with age (esp by 30yo) is seen in...

hereditary spastic paraparesis

What dysmorphic features are characteristic of Zellweger's?

high forehead, large fontanelles, flat supraorbital ridges, hypertelorism, epicanthal folds, broad nasal bridge, micrognathia, and flat occiput

essential tremor

high frequency posturla dn action tremor of UE. Tx with propranolol and primidone

femoral nerve

hip flexion and knee extension. Sensory is territory of saphenous nerve below knee

What are the primary risk factors for NAION?

hypertension and diabetes mellitus

Which two things can provoke absence seizures?

hyperventillation hypoglycemia

zonisamide side effect

hypohidrosis, can lead to heat stroke

idiopathic Parkinson disease

hypophonic, hypokinetic, monotone

Post-cardiac arrest anoxic-ischemic brain injury is a major cause of morbidity and mortality. Two pivotal trials in 2002, demonstrated that therapeutic _______ can ameliorate brain injury, reduce mortality, and significantly improve neurological outcome. The other options have not been shown to significantly modify neurologic outcomes in this setting.

hypothermia

This may present with a dementing illness and systemic symptoms including weight gain, hoarseness, lethargy, and cold intolerance. Screening for this is considered standard for patients presenting with dementia.

hypothyroidism

When schizencephaly is sporadic, what is it due to?

hypoxic or ischemic insult

This disorder typically presents with severe pain followed by weakness and sensory loss in the upper extremity. Weakness may occur within 24 hours, but its onset is more commonly delayed two weeks or longer. This disorder is more common in young adults and sometimes follows physical exertion or other physical stress, including upper respiratory tract infection, vaccination, surgery, and childbirth. A small percentage of patients have an autosomal dominapresents with severe pain followed by weakness and sensory loss in the upper extremity. The prognosis for eventual recovery is good. The role of corticosteroids is not certain, although they often help pain considerably.

idiopathic brachial neuritis

Wernicke aphasia

impair comprehension for 4 step pointing command

anterior insula impairment

impairments of language function and addiction to substances

" symptoms of CTE

imparied attention, executive fxn, memory plus behavior imuplsivity, explosive anger. Tauopathy but NF tau tangles are in perivascular regions and depts of cerebral sulci

SUNCT

injection AND tearing (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing)

SUNA

injection OR tearing, but NOT BOTH (Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms)

suprachiasmatic n

inputs from retina. Invovled in circadian rhythms, melatonin secretion

Where do CN VII and VIII exit the skull

internal acoustic meatus

In high cervical cord lesions, ipsilateral diminished pain and temperature sensation in the preauricular area of the face is due to ...

involvement of cells in the substantia gelatinosa which is the distal continuation of the descending trigeminal nucleus and tract.

How do you treat GLUT1 deficiency?

ketogenic diet (improves seizure control and abnormal movements -less effective for psychomotor impairment

Treatment for SUNCT/SUNA

lamotrigine

Toxoplasmosis histology

large cell with tons of nuclei

In the context of a normal neurological examination and normal motor units, spontaneous fasciculations can occur in more than half of people, with a smaller subset of patients have frequent and widespread fasciculations. MND typically presents with painless, asymmetric weakness. Fasiculations tend to be a ____ finding.

later

Which syndrome is associated with dysexecutive symptoms?

lateral convexity

The hypocretin/orexin neurons of what part of the hypothalamus innervate the cholinergic and monaminergic cell groups of the brainstem?

lateral hypothalamus

Echopraxia (involuntary imitation of observed motions) is associated with what?

lateral orbital frontal cortex

The imitation of gestures (echopraxia) and mimicking of use of objects in the environment (utilization behavior) are seen after lesions to the...

lateral orbitofrontal cortex

Homunculus of precenral gyrus order from medial to lateral

leg, arm, face, pharynx/larynx

"dural tail sign"

little tail that sticks off of a meningioma

Norepinephrine is the majory neurotransmitter of which nuclei?

locus ceruleus

What do you see on CSF analysis for patients with GLUT-1 mutation?

low CSF glucose with normal serum glucose

Bizarre Astrocytes are seen in progressive disease?

multifocal leukoencephalopathy

What does path show for Alexander's disease?

multiple Rosenthal fibers, which are elongated eosinophilic fibers seen with hematoxylin and eosin stains, and they are diffusely distributed throughout the brain with clusters in the subpial, subependymal, and perivascular areas

Where do myotonic potentials originate from? - Anterior horn cell - Nerve root - Muscle membrane - NMJ

muscle membrane

What nerve does the biceps innervation?

musculocutaneous nerve, which arises from the lateral cord of the brachial plexus

Mutation for hereditary spastic paraparesis

mutation in SPG11 gene on chromosome 15

Hereditary Spastic Paraplegia (HSP)

mutation in the SPG4 gene accounts for about 40% of cases. It codes for a protein, spastin, a member of the ATPases associated with microtubules and endosomes. It is inherited in an autosomal-dominant pattern. Treatment is symptomatic. Imaging findings are variable and often normal, but potential abnormalities include atrophy of the midbrain and cerebellar vermis, a thin corpus callosum, and spinal cord atrophy. White-matter hyperintensities are common, often affecting the corticospinal tract in the posterior limb of the internal capsule

Genes for SMA

mutations that delete exon 7 in both SMN1 gene. Some have extra SMN2 which can help replace some SMN protein loss

________ are associated with interferon use

myalgias

Junvenile myoclonic epilepsy

myoclonic jerks with retained consciousness and generalized tonic clonic seizure

Most common seizure induced by photic stimulation

myoclonic seizure

Myoclonus dystonia

myoclonus arms, neck, trunk, triggered by stress or loud noises, followed by dystonia 50% of time. dystonia can be focal or more generalized. legs are involved less frequently. Psychiatric symptoms such an depression, anxiety, and obsessive-compulsive disorder are common as well. movements often temporarily improve w alcohol. presents in childhood or early adolescence. mutation of the SGCE gene, which codes for a protein known as epsilon (ε)-sarcoglycan, AD, autosomal dominant pattern, RX benzodiazepines, anti-convulsants, and anticholinergic medications, though few patients have full symptom relief. Botulinum toxin injections and deep brain stimulation can be used in treatment of refractory patients. Some patients experience a spontaneous remission, while others experience worsening.

Critical illness myopathy predominantly affects what muscle protein?

myosin

Carotid dissections often occur in the setting of ____ trauma and disorders of______ _____ including fibromuscular dysplasia.

neck; connective tissue

What does neuro-imaging show on GLUT 1 deficiency

no specific abnormalities

Cocaine

non selective reuptake inhibitor of NE, DA, serotonin. Binds to pre synpatic DAT inhibiting DA re uptake in nucleus accumbnes

Amelodia or affective motor aprosodia localizes to where?

nondominant posterior inferior frontal gyrus

What is the source of brachial motor outflow to the laryngeal and pharyngelal muscles?

nucleus ambiguus

What nucleus is the site of termination of afferents fromt he baroreceptors?

nucleus tractus solitarius

what receives afferent visceral sensory information?

nucleus tractus solitarius

ENDEAR study

nusinersen had better mootor milestone

What innervates the dura within the cranial vault?

ophthalmic branch of the trigeminal nerve

In a child, what is the most common cause of orbital pain and a partial 3rd nerve palsy?

opthalmoplegic migraine

Describe Balint's syndrome

optic ataxia, ocular apraxia, and simultagnosia

Balint syndrome

optic ataxia, ocular apraxia, simultanagnosis

What is the Balint's syndrome triad?

optic ataxia, ocular dysmetria and asimultagnosia

Where does CN II exit the skull

optic canal

Which syndrome consists of socially inappropriate behaviors and poor impulse control with disinhibition?

orbitofrontal

What structure is involved in judgment, inhibition of socially inappropriate behaviors, emotional and visceral functions?

orbitofrontal cortex

Which part of the brain receives input from organs that are in pain (visceral nociceptive input)

organs --> ventromedial posterior nucleus of thalamus --> insular cortex

This is one of the autonomic-based headaches comprising the trigeminal autonomic cephalalgias (TAC). It is side-locked, repeats multiple times daily (on average about 11), lasts under 30 minutes, and is associated with autonomic phenomena and pain in a V1 distribution. In contrast to cluster headache and SUNCT (which lasts for less than 10 minutes and reoccurs up to 100 times daily), it is more common in women and typically very responsive to indomethacin.

paroxysmal hemicrania (PH)

What does the neural crest cells form?

peripheral nervous system, chromaffin tissue of the adrenal medulla and melanocytes

neurological symptoms with SLE

peripheral neuropathy, cerebritis, neuropsychiatic symptoms such as depression, mania, psychosis

What does the superficial peroneal nerve innervate?

peroneus longus and brevis

Which metabolite causes musty odor of sweat and urine in PKU patients?

phenylacetic acid

what AED are protein bound?

phenytoin

What is the reasonable next step in patients with acute exacerbations of MS who fail to improve with IV methylprednisolone?

plasma exchange (Steroid-refractory acute exacerbations in MS have been shown to have better outcomes with plasma exchange when compared to those who received sham exchanges)

MME

polyspikes are specific. Myoclonic jerkes with retained consciousnes and generalized tonic clonic seizures

Damage to which area causes dressing apraxia (difficulty performing the movements needed to dress)

posterior R frontal lobe

Balint's syndrome localizes to _____ _____ _______

posterior parietal regions

Working memory localizes to?

prefrontal cortexsubcortex-parietal-association cortex system

How does Fabry's disease present?

presenting with dysesthesias, lancinating pain, and episodes of burning sensation from small fiber neuropathy, which also may be associated with autonomic dysfunction

X-linked infantile nystagmus

presents at birth or develops prior to age 6 months. Horizontal eye movements are most common, but some children have vertical and rotary nystagmus. Anxiety, fatigue, or fixation may worsen the nystagmus and some children turn or tilt their heads as a compensatory measure. Some children have reduced visual acuity and poor balance, but are otherwise healthy. It is caused by a number of mutations in the FRMD7 gene, which makes a protein that is abundant in the brainstem, cerebellum, and retina.

Which areas are crucial for response inhibition?

presupplementary motor area and ventrolateral prefrontal cortex

Borderline personality disorder

problems with interpersonal relationships, fear of abandonment, waxing and waning between idealizing and devaluing people close to them, impulsive outbursts of anger, suicidal gestures, associated depression, dissociatives states

Alexander disease

profuse Rosenthal fibers densely compacted glial intermediate filaments made of GFAP

mamillary bodies

projects to anterior n. of thalamus as part of circuit of Papez

Function and innervation of pronator quadratus

pronation of arm when elbow is flexed innervated by anterior interosseous nerve (branch of median nerve)

These are required for a diagnosis of what? I. Papilledema II. Normal neurological examination except for cranial nerve abnormalities III. Neuroimaging: normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion and no abnormal meningeal enhancement on MRI, with and without gadolinium, for typical patients (female and obese), and MRI with and without gadolinium, and magnetic resonance venography for others; if MRI is unavailable or contraindicated contrast-enhanced CT may be used IV. Normal CSF composition V. Elevated lumbar puncture opening pressure (>/= 250mm CSF in adults and >/= 280mm CSF in children [250mm CSF if the child is not sedated and not obese]) in properly performed lumbar puncture.

pseudotumor cerebri syndrome

Since Orthostatic tremor slowly worsens over time it is often misdiagnosed as what?

psychogenic

Catatonia features

psychosocial withdrawal, stupor, mutism, immobility, analgesia, posturing, waxy flexibility, mannerisms, rituals, grimacing, shoulder shrugging

What is the treatment of Lesch-nyan disease?

purine-restricted diet, hydration to prevent kidney stones, allopurinol to decrease the production of uric acid, and supportive care to prevent self-inflicted injuries and control of abnormal movements -Levodopa and tetrabenazine have been tried for the neuropsychiatric manifestations.

Which nerve innvervates the brachioradialis?

radial nerve, right arises from the posterior cord

This syndrome refers to the finding of MRI lesions typical in location and character for MS in the absence of symptoms suggestive of MS. NMO is characterized by longitudinally extensive spinal cord lesions (3 or more vertebral segments).

radiologically isolated syndrome

Decompression illness

rapid ascent to surface. Joint pain, paresthesais. Tx with hyperbaric O2

What is the only thalamic nucleus that does not project to the cerebral cortex?

reticular nculeus is part of the ventral thalamus

The ophthalmic artery is a branch of the internal carotid artery and supplies the _____.

retina

Dressing apraxia localizes where?

right parietal lobe

Parasympathetic ganglia originate where in the spinal cord?

sacral

lesion to lower cauda equina/conus medularis

saddle anestheisa, with loss of sphincter tone and erectile dysfunction. Sensory abrnoamality in S2-S5 dermatomes. No weakness so not affecting lumbar/lumbosacral plexi. Onuf's nucleus in S2-S4 affects only urinary symptoms. Can be due to central disc herniation

The fourth cervical dermatome lies adjacent to what dermatome?

second thoracic dermatome

Pimavanserin

selective serotnonin 5-HT2A inverse agonist without dopaminergic, adrenergic, histaminergic, or muscarinic affinity. Used to treat hallucinations and delusions associated with psychosis in patients with PD.

Artery of Percheron

single thalmoperforating artery from PCA. Cause b/l paramedian thalmic infarction

How to diagnose CADASIL?

skin-muscle Bx with thickening of smooth arteriopathic muscle cells that eventually degenerate electron microscopy --> granular osmophilic materials in arterial smooth muscle

What is a secondary causes of glossopharyngeal neuralgia?

skull-based tumors

Excessive daytime sleepiness and sleep fragmentation can be seen in a number sleep disorders such as?

sleep aponea idiopathic hypersomnia depression sleep deprivation states drug and alcohol dependence restless leg syndrome

A patient's signs and symptoms of sensory loss to pinprick with preservation of vibratory sense and reflexes, is most consistent with a _____ _____ ____, such as leprosy.

small fiber neuropathy

What does the tibial nerve innervate?

soleus and tibialis posterior

Fingolimod

sphingosine-1 phosphate receptor modulator for RRMS. Reduces lymphocyte recirculation from lymph nodes. Can activate third subtype in heart and prolong PR interval, effects max after 1st dose, but can persist for 2 week.

Which nerves come off of the brachial plexus trunks?

suprascapular (C5-C6, from upper trunk) - supraspinatus, infraspinatus to subclavius (from upper trunk) - subclavius

What other organs can you see foam histiocytes?

spleen, lymph nodes, hepatic sinusoids, and pulmonary alveoli.

EEG in increased ICP

srhythmic slow activity in theta-delta frquency range

Lesions to CN VII after stylomastoid foramen?

stapedius is given off before it passes and would be preserved. Innervation and sensory to posterior auricular nerve would be affected

What nerve innervates the supraspinatus?

suprascapular nerve from the upper trunk

A patient is presenting with chronic daily headache (CDH) most likely due to medication overuse (headache more than 15 days/month plus regular overuse of 3 or more months of one or more analgesic drugs/ergotamine/triptans/opioids). It is unlikely that either acute or preventive headache treatments will be effective before she is weaned from her overused drug, in this case butalbital-acetaminophen-caffeine. Medication overuse headaches are often accompanied by nonrestorative sleep, nasal stuffiness and post nasal drip which improve after weaning the offending agent. What can be used to help mitigate the weaning process, and are unlikely to be contributing to her CDH?

steroids

Appearance of central pontine myelinolysis on gross specimen

stringy/linear appearance of pons in cross section. Almost looks like long nasty tapeworms in pons

What area is the site of neuropoiesis in the adult brain?

subependymal (subventricular) zone, where neural stem cells reside

optimal doses of carbidopa

suboptimal causes n/v. takes 75-150mg of carbidopa per day to saturate the peripheral aromatic amino acid decarboxylase enzyme to prevent peripheral side effects. Decreasing may reduce n/w how ever may result in less symptomatic improvement

Lesion of which area causes ballism/hemiballismus (flinging movements)

subthalamic nucleus

Cataplexy (what is it, symptoms, treatment)

sudden transient loss of muscle strength precipitated by emotion (esp. laughter) associated with narcolepsy bilateral often partial (i.e. buckling of knees,facial sag, jaw drop) loss of DTR recall intact treat with SSRI, TCA, sodium oxybate

Prolonged P100 latency

suggest dysfunction of ipsilateral optic nerve. Retinal disorder would have absent response i/l

EOM used to look down and towards nose

superior oblique

Where do CN III, IV, V1 and VI exit the skull

superior orbital fissure

What is the treatment of Neiman Pick disease?

supportive management

VACTERL

syndrome consists of vertebral anomalies, anal atresia, cardiovascular anomalies, trachea-esophageal fistula, renal anomalies, limb defects.

What is the treatment of neuronal ceroid lipofuscinosis?

symptomatic

Placement of deep brain stimulator for essential tremor

thalamus (ventrointermediate nucleus)

What nerve innvervates the deltoid?

the axillary nerve which comes from the posterior cord

Anterior choroidal artery is a branch off of...

the internal carotid artery

What nerve innervations the pronator teres?

the median nerve from lateral cord of brachial plexus

Wernicke-Korsakoff syndrome

thiamine deficiency, amnesti disorder with memory and learning abnormalities. Ophthalmoplegia and gait ataxia.

Electron micrograph/ histologic findings in CADASIL

thickening around arterioles/arteries (granular osmiophilic material around them)

Preganglionic neurons of the sympathetic system are located where in the spinal cord?

thoracic and lumbar regions

What does the deep peroneal nerve innervate?

tibialis anterior, extensor digitorum brevis

Cause of central pontine myelinolysis

too rapid correction of hyponatremia

T/F Paresthesias and hypesthesia usually involve a much more limited area in the anterolateral thigh

true

detrusor hyperreflexia wihout outlet obstruction or urinary retention

treatment with anticholinergic including oxybutynin

These are contraindicated in the presence of known coronary artery disease due to the risk of precipitating vasospasm and coronary ischemia

triptans

Which is the only cranial nerve to complete decussate prior to exiting the dorsal aspect of the brainstem?

trochlear

T/F A negative HSV-PCR early on, in a patient with clinically compatible HSV encephalitis and no alternative etiology, should not delay antiviral therapy. Fosphenytoin should not be started unless there are clinical or EEG documented seizures

true

T/F Abrupt lowering of blood pressure may aggravate the optic nerve head ischemia

true

T/F Aspirin therapy is appropriate for NAION as it reduces the patient's future risk of cardiovascular and cerebrovascular disease.

true

T/F Both enhanced physiologic and essential tremors are postural but not exclusive to standing. In addition they are also usually kinetic and typically do not involve the legs.

true

T/F Forced vital capacity accurately predicts diaphragmatic dysfunction and the development of atelectasis due to diminished cough. A drop in FVC is an early indicator of respiratory failure and the need for possible intubation. Drops in arterial oxygen saturation and hypercarbia occur late in the setting of NMRF. Although anti-MAG antibodies and repetitive nerve stimulation studies may assist with the diagnosis of demyelinating polyneuropathy and myasthenia gravis, neither predicts the clinical severity of disease or the need for intubation.

true

T/F In patients with orthostatic tremor, postural tremor may also be present in arms

true

T/F Most patients with NAION awaken with painless monocular visual loss presumably because of nocturnal hypotension

true

T/F No treatment has been shown to improve clinical outcome in NAION patients but fortunately most will improve or stabilize over several weeks

true

T/F Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used in the first or second trimester, but acetaminophen is preferred. Butalbital, codeine and sumatriptan are Category C, whereas acetaminophen and ibuprofen are Category B. Sumatriptan use should be restricted to migraine and opiates are not encouraged for headaches.

true

T/F Only one molecular marker in anaplastic gliomas has been shown to predict response to chemotherapy. Patients with anaplastic oligodendroglioma and loss of heterozygosity at chromosomes 1p and 19q have a significantly better response to chemotherapy and overall improved survival. MGMT hypermethylation is likely predictive of response to alkylating chemotherapy in Glioblastoma, but this remains unclear in anaplastic oligodedrogliomas.

true

T/F Oxycodone may provide pain relief for cluster headache, PH, or SUNCT but is not an effective headache abortive agent

true

T/F Several studies have now demonstrated no increased bleeding in patients with intracerebral hemorrhage (ICH) while taking aspirin at doses under 650 mg. A patient is at high risk of ischemic cardiovascular and cerebrovascular disease and therefore lowering the risks of these disorders with aspirin outweighs any potential for aspirin to exacerbate ICH. High dose aspirin does not add to its vascular risk reducing effects and increases the incidence of adverse effects such as GI bleed. There is no need to hold BP lowering medication as a BP of 160/90 is felt to be generally safe, and may even improve prognosis in ICH.

true

T/F Statin agents have recently been shown not to increase risk of ICH

true

T/F Symptomatic therapy for glossopharyngeal neuralgia is the same as that used for trigeminal neuralgia

true

T/F The presence of elevated CSF myelin basic protein, elevated CSF IgG index, abnormal visual evoked potentials and urinary retention are all consistent with demyelinating disease but are not specific and can be seen in a number of demyelinating and inflammatory CNS disorders

true

T/F There is not an established role for the use of intravenous immunoglobulin (IVIG), cyclophosphamide, or natalizumab in the treatment of acute MS exacerbations

true

T/F Working memory deficits can result from multiple cerebrovascular events AD, FTD, PD, dementia with Lewy Bodies, and traumatic brain injury.

true

T/F rash is not generally seen with existing MS treatments on the market

true

T/F retinal ischemia, not NAION is associated with carotid occlusive disease

true

What type of meningitis in toddlers can progress to hydrocephalus ad infarcts in region of basilar perforators?

tuberculous meningitis

Subependymal nodules on MRI are characteristic of...

tuberous sclerosis

Reduplicative paramnesia

type of delusional misidentification syndrome- can be seen in stroke, trauma, neurodegernative syndromes. Patient will think that a strange place simultaneously exists in 2 or more locations despite information to the contrary. Localizes to right frontal, right temporal, or right parietal region

Simultanagnosia

unable to simultaneously perceive more than one stimulus item or more than one part of a complex pattern. Seen in Alzheimer disease

6 types of herniation

uncal central cingulate transcalvarial upward tonsillar

" ethambutol

used for TB. Optic neuropathy higher risk for renal function

Eteplirsen

used for DMD with particular exon deletions

Alopecia, obesity and pharmacologic tremor are adverse effects associated with chronic use of this

valproic acid

A patient has nonarteritic anterior ischemic optic neuropathy (NAION) resulting in optic nerve head ischemia. This is usually due to _____ ______ ______ of the posterior ciliary arteries which supply the optic nerve.

vascular occlusive disease

Triptans are contraindicated in the presence of known coronary artery disease due to the risk of precipitating ________ and coronary ischemia

vasospasm

The notochord gives rise to what structure?

vertebral column

What is the clinical presentation of neuronal ceroid lipofuscinosis

vision loss, seizures and dementia

Gomez-Lopez-Hernandez syndrome

which includes scalp alopecia, numbness in the distribution of the trigeminal nerve, cognitive impairment, short stature, craniosynostosis, and dysmorphic facial features

Where do the majority of axons of cerebellar Purkinje cells synapse?

with neurons in the deep cerebellar nuclei

" Orbitofrontal cortex lesions

witzelsucht (inappropriate joking), can also have disinhibition and antisocial behavior

dorsolateral prefrontal cortex or posterior cingulate lesion

working memory, attention, cognitive slowing, depression

" Vit B 12 def

"combined system degneration, damage to posterior and lateral columns DDx: AMLS- neuroaxonal loss of upper and lower motor neurons sparing sensory columns. ASA thrombosis sparesa posterior columns. Neurosyphilis produces tabes dorsalis demyelination and axonal loss of posterior columns. Vit E defi dystrophic axons in posterior columns

Cowden disease

"multiple hamartoma syndrome, AD, oral mucosa fibromas, trichilemmomas, hamartomatous colon. NCS dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos disase DDx: VHL- hemangioblastomas, medulloblastomas seen in Gorlin or Turcot syndomres. Schwannomas in NF2, SEGA in Tuberous sclerosis

" Familial frontotemporal dementia

"mutation on chromosome 17 for tau protein. DDx: Dementia with lewy bodies- alpha synuclein. Presenilin from familial Alzheimer dementia

" Cerebral malaria

"parasitized RBC in cereral blood vessels causing petechial hemorrhage in white matter ring hemorrhages with central necrotic blood vessel. Hemozoin bodies are malarial pigment produced by plasmodium as biproduct of hemoglobin breakdown Negri bodies in rabis. Cowdry A inclusions in herpes.

NMO antibodies

Aquaporin 4

Anticholinergics for parkinsons

Artane and Cogentin Avoid in older people- delirium

Progressive Supranuclear Palsy

Falling backwards (as opposed to Parkinson's classically fall forward or to the side) Loss of eye movements up and down Does not respond as well to Sinemet (but it's still the best medicine)

Riluzole mechanism of action?

Inhibition of glutamate release Check liver enzymes

This causes focal cerebritis rather than diffuse edema in the temporal lobes.

Toxoplasmosis

Cardinal features of Parkinson's

Tremor-resting pull rolling Rigidity-cogwheel **Bradykinesia-slowing down Postural instability-falls *NEEDS BRADYKINESIA for diagnosis

Hypnic headache

Tx: caffeine, lithium every bedtime Wakes patient up from sleep between 1-3 AM every night and lasts for up to 3 hours. Rare. Usually in women Pain B/L

Question 236: The CT provided demonstrates hematomas within the right frontal region, along with subarachnoid hemorrhage overlying the right hemisphere. The pattern is typical of _____ which arise in the interface between the bone surfaces. In addition, there is an obvious right parietal soft tissue hematoma. This pattern of hemorrhage with contusion is not typical of an aneurysmal rupture, nor would encephalitis present with this pattern of intracranial hemorrhages and subarachnoid hemorrhage. There is evidence of subdural hemorrhage; however, no epidural hematoma is seen in this view.

contusions

nuclei of the cerebellum

clusters of neurons in the interior of the cerebellum that send axons to motor-controlling areas outside the cerebellum Lateral to medial: DEGF- Dentate, emboliform, globose, fastigial

Flocculonodular lobe responsible for

Equilibrium and eye movements

Risk of first time hemorrhage from AVM annually

2-4%

Parkinson's diagnosis

3 of 4 cardinal symptoms At least 2 years No other cause Responds to medicine

Question 218: Toxoplasmosis is caused by an obligate intracellular parasite Toxoplasma gondii. Patients who develop _____ are at particularly high risk of disseminated toxoplasmosis which most frequently involves the CNS. Toxoplasmosis is the most common focal mass lesion in this and it most commonly involves the _______ ______.

AIDS; basal ganglia

Question 227: There is cerebellar tonsillar ectopia (approximately 1 cm below the foramen magnum) with an associated cervical syrinx, findings consistent with ______ malformation. The contents of the syrinx correspond to CSF (i.e. T1 hypointense and T2 hyperintense). The lesion has distinct margins and displaces rather than infilatrates adjacent spinal cord. Astrocytomas are infiltrating tumors. Basilar invagination refers to narrowing of the foramen magnum due to upward migration of the top of the C2 vertebrae. Multiple sclerosis causes demyelinating lesions of the brain and spinal cord and is not associated with tonsillar ectopia. Chordomas are tumors of notochord remnants which most often occur in the clivus and sacrococcygeal regions.

Chiari 1

dentate nucleus of cerebellum

Controls dexterity

fastigial nucleus of cerebellum

Controls walking and stance

Question 229: There is restricted diffusion involving the left occipital and left lateral temporal cortices. This patient had sporadic ______-_____ disease, a progressive neurodegenerative disorder resulting from misfolded prion proteins. The imaging abnormality does not respect vascular territories, making ischemic infarction unlikely. Hypoxic brain injury typically causes symmetric abnormalities. The limbic, or medial temporal, structures appear normal. Hashimoto's encephalopathy more commonly results in lesions in the white matter and/or brain stem.

Creutzfeldt-Jakob

Left thalamus infarct causes

Depression

Question 231: This patient had gradual dementia onset and no focal full thickness cortical loss. This is often is familial wutg frontal and temporal atrophy as seen in the patient images are supportive features for diagnosis of this, though absence of atrophy does not exclude the diagnosis. It is characterized by a strong gradient of atrophy from anterior to posterior along the temporal lobe, as seen in the patient images.

Frontotemporal dementia

Tolosa-Hunt Syndrome

Granulomatous Inflammation of cavernous sinus that can affect CN 3, 4, 6 (painful ophthalmologist)

HIV related vacuoles myelopathy

HIV patient with ataxia, spastic parapets is and sensory loss befor waist Lateral and posterior column demyelination with microvacuolar changes

This is an acute febrile illness. Common early symptoms include change in consciousness (confusion, then stupor, then coma), fever, headache, and seizures. Inferior frontal lobe and medial temporal lobe involvement with edema, restricted diffusion and contrast enhancement in the acute phase, and tissue destruction later are usual findings.

Herpes encephalitis

CARASIL mutation

HtrA serine protease Cause of small vessel disease

Most important intervention for prevention of recurrent stroke or TIA

Hypertension (over tobacco, statin, etc)

3 cerebellum cortex layers

Molecular, purkinje, granule cell layer (MPG- mikes per gallon)

2 sources of input to cerebellum

Mossy and climbing fibers 1 missy fiber excites thousands ofpurkinje cells 1 climbing fiber excited 10 purkinje cells

Acute intermittent porphyria

Motor symptoms more prominent than sensory

Which needle EMG FEATURE is useful to differentiate radiculopathy vs mononeuropathy

Muscle distributions

Systemic vascular is most associated w polyneuropathy

Polyarteritis nodosa (small to medium blood vessels)

Which sensory modality can be evaluated by standard nerve conduction studies?

Position sensation (not temperature, pain, or baroreception) Can assess axons 6-12 um. The others are too small

posterior lobe of cerebellum

Responsible for precise movement

Question 246: This case demonstrates Absent septum pellucidum, small optic nerves/chiasm compatible with ________ _____ 75-90% have brain abnormalities; 45% have pituitary insufficiency. Child with short stature, endocrine dysfunction, Normal or color blindness, visual loss, nystagmus, strabismus mental retardation, spasticity, microcephaly, anosmia.

Septooptic Dysplasia

Intraventricular thrombosis (alteplase)

Significant Decrease in mortality by nearly half compared to EVD alone Less risk of bacterial ventriculitis, no statistically significant safety events compared to EVD alone

Which channelopathy associated w intense pain, redness, swelling. Worse in warmth, relieved by cooling

Sodium. Gain of function mutations in SCN9A

Question 255: Type 1 odontoid fractures involve the tip of the dens and are ______ fractures.Type 2 odontoid fractures occur at the base of the dens and are considered _____. Type 3 odontoid fractures involve the base of the dens as well as the facets lateral to the odontoid process, and can be either stable or unstable.

Stable; unstable

Amantadine

Starting dose 100mg BID, max dose 400 mg Helps with walking and balance- prevents falls Helps with dyskinesias Side effects: hallucinations, vivid dreams, livedo reticularis, leg edema

Neurosarcoid

affects mostly leptomeninges at skull base causing cranial neuropathies

3 lobes of cerebellum

anterior, posterior, flocculonodular Flocculonodular

Question 226: The patient has markedly enlarged lateral and third ventricles on axial views, with a small (or absent) cerebral aqueduct on sagittal views. These findings are most consistent with ______ _____. In ________ there is dilation of the atrium of the lateral ventricles, as in this patient, but not of the temporal horn, present here. In Dandy-Walker syndrome there is partial absence of the vermis of the _________, not present here. The Miller-Dieker syndrome includes lissencephaly, not present here. In this age group normal pressure hydrocephalus is not as frequent as this. In addition, a communicating hydrocephalus of this kind would be accompanied by some dilation of the ______ ventricle.

aqueductal stenosis; colpocephaly; cerebellum; fourth

CADASIL

cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Small artery disease, early age of stroke on site, increased migraine with aura, white matter lesions on MRI

Question 238: This study demonstrates a linear fracture defect which includes all three vertebral columns extending horizontally through the vertebral body, the pedicles, and through the facet joints. This lower thoracic fracture is termed a ______ ______, which is a fracture occurring most commonly in the lower thoracic or upper lumbar regions from T11 to L2. It is a result of hyperflexion of the back, most commonly seen in motor vehicle accidents, in belted patients, resulting in a wedge shaped compression fracture which includes posterior elements of the vertebra. Half of these patients also have an abdominal injury. This should be considered an unstable fracture until proven otherwise. Spondylolisthesis without spondylolysis is incorrect as there is no subluxation demonstrated. The fracture margins are sharply demarcated and not the pattern of chronic fractures.

chance fracture

Question 248: There is gross traumatic injury to the spinal column with disruption of the C4-C5 ligamenta flava, interspinous ligaments, and posterior longitudinal ligament. There is fracture deformity of C5 vertebra consistent with a flexion teardrop fracture and fracture of C6. There is prevertebral soft tissue edema, and the cord has T2 hyperintense signal at the C5 and C6 level consistent with this and with some intramedullary hemorrhagic component. Neuromyelitis optica, ependymoma, abscess, and sarcoid myelitis are not the best choices because the extensive vertebral column injuries are not consistent with the typical presentation of any of these entities.

cord contusion

Question 245: Sagittal T1-weighted images demonstrate a loculated fluid collection in the posterior epidural space, extending for several levels in the mid-thoracic spine. The collection causes significant compression and anterior displacement of the spinal cord. Note on both sagittal and axial images that the subarachnoid space is also compressed, placing this in the epidural, not intradural space (excluding meningioma). This is a typical appearance of a loculated, _______ ______ _____. A 10-day-old hematoma should be hyperintense on T1-weighted images. Epidural metastases and myeloma are of soft tissue signal, generally nearly isointense to neural tissue and usually are anteriorly located, extending posteriorly from vertebral body lesions.

encapsulated epidural abscess

Infarction typically has an acute onset of neurologic deficit and on imaging there will be a ____ _____defect.

focal cortical

Question 243: There is a single, large infiltrative multicystic enhancing mass with vasogenic edema. This appearance is most frequently seen with a ________ _______. No vascular structures are seen as expected in an arteriovenous malformation (AVM). An infectious process, such as toxoplasmosis and a metastasis, would more likely have a ring pattern of enhancement and not the large complex multicystic pattern seen here.

glioblastoma multiforme

Question 242: The images show bilateral mesial temporal and hippocampal edema, and the clinical history supports diagnosis of this. This disease often leads to elevated RBC count in CSF due to hemorrhagic changes. JC Virus is seen in PML, a white matter disease. HTLV antibodies are seen in HTLV-associated Myelopathy/TSP.

herpes simplex encephalitis

Question 254: The CT study demonstrates near complete loss of cortical sulcation and diffuse decreased density in all hemispheres including the cerebellum along with generalized edema. These changes are in no specific vascular distribution and are typical of a severe _____ insult. The relative increased density within the fissures is a manifestation of contrast to the severe hypodensity of the remainder of the brain. Petechial hemorrhage due to necrosis may also be present. There were, however, no imaging findings to suggest a primary subarachnoid hemorrhage. Lissencephaly would not be associated with marked mass effect and edema as seen in this study; nor would hypertension encephalopathy.

hypoxic

Question 249: The combination of the patient's physical exam and neuroimaging findings is consistent with a right anterior choroidal artery infarction. The anterior choroidal artery is a branch of the internal carotid artery and supplies the posterior limb of the _______ ______ and the lateral _____.

internal capsule; thalamus

Question 230: The MRI shows an intradural extramedullary mass which is fairly homogenous and is T1 hyperintense and STIR hypointense. The STIR sequence suppresses fat signal, suggesting that this mass is composed of fat. The other answer choices are lesions which are either not composed of fat or not found in the intradural extramedullary space. This is most suggestive of a?

lipoma

Germinal matrix hemorrhage

located in walls of lateral ventricles, common complication of premature birth

Question 251: The MRI findings show the "Hot Cross Bun" sign in the pons, as well as hyperintensity in the cerebellar peduncles. These findings are most suggestive of ______ ______ ______. Parkinson disease, postencephalitic parkinsonism, and prolonged metoclopramide exposure typically would not have characteristic MRI findings. NPH would demonstrate enlargement of the ventricles along with transependymal flow.

multiple system atrophy

Question 241: MRI of the brain demonstrates markedly enlarged optic nerves, the left greater than the right, typical of _____ ____ _____. The coronal plexus study demonstrates an ovoid homogeneous enhancing lesion typical of a nerve sheath tumor. The combination of peripheral nerve sheath tumors and this is seen in conjunction with neurofibromatosis type 1 and is not a feature of the other choices. NF type 2 and schwannomatosis are not associated with these.

optic nerve gliomas

The ______ thalamic artery is a branch of the posterior cerebral artery and supplies the medial thalamus.

paramedian

Question 235: The study demonstrates increased density within the pituitary fossa extending to the cavernous sinuses bilaterally with an expansive lesion, which is demonstrated on the FLAIR and T1 view areas of both increased and decreased signal. There is an ovoid mass throughout the suprasellar cistern compressing the optic chiasm. It appears as that of a large pituitary mass, likely a _____ _____ ____. There is a perimeter of increased T1 weighted signal in the non-contrast view. A compilation of these features would be that of hemorrhagic transformation i.e. pituitary apoplexy of the pituitary tumor mass. A pituitary tumor of this size can be expected to result in bitemporal hemianopia. However, in the absence of hemorrhagic transformation, one would not expect to the tumor's presence to result in coma as seen with this patient. A colloid cyst would not be expected in this location and generally arises as an intraventricular lesion adjacent to the foramen of Monro in the third ventricle. A metastatic lesion within the sella would be in the differential of a tumor mass; however, again, it would not be the best clinical explanation for the patient's clinical presentation of sudden coma.

pituitary macro adenoma.

Question 219: The mass lesion with isointense signal on T1- and bright T2-weighted signal posterior to the dens is most frequently due to pannus. The inflammatory pannus involving the dens is often seen in association with _______ _______.

rheumatoid arthritis

Question 224: The study depicts a marked increase of fat tissue within the epidural space most marked from L4 to the sacral region, which results in _____ ______ as seen with a small triangular-shaped thecal sac at L5-S1. The signal characteristics are those of subcutaneous fat tissue and not CSF. Congenital for of this is a result of short pedicles is not seen on this study. The patient does have mild disc disease at L4-5 and L5-S1. They are inadequate to produce this and result in spinal claudication, as seen with this patient. The conus is not well demonstrated, but is not low-lying; nor are there any imaging features to suggest tethering of the cord.

spinal stenosis

Elevated _______ _____ levels can be seen in CNS Lyme Disease, which causes multifocal white matter lesions.

spirochete IgM

In Alzheimer disease, atrophy occurs throughout this lobe.

temporal

Question 237: The figure demonstrates widening of the CSF space posterior to the spinal cord in the mid thoracic region, resulting in anterior displacement and compression of the cord at that level best seen on the T2 weighted view with somewhat ovoid appearance. This is also well demonstrated as a region of isointense signal to CSF on the T1 weighted views and shows no enhancement. The most common etiology of this appearance is that of a ______ _____ cyst. An epidermoid cyst is an additional, although less likely, etiology and not one of the choices. A lipoma in this location would have increased T1 weighted signal similar to subcutaneous fat tissue not seen here. Similarly, a meningioma would not show the very bright T2 weighted signal and the hypodense signal on T1 weighted view. Myelomalacia within the cord may develop, although is not depicted on this study. The signal of characteristics are not hemorrhage as would be seen in an intrathecal hematoma.

thoracic arachnoid

Question 217--- The CT of the head demonstrates a hypo dense segment in the left cerebellum typical of an acute infarction, and the CTA axial view demonstrates a segment of decreased density within the left vertebral artery typical of a ______. The carotid and right vertebral artery are well demonstrated and are normal. A thrombus can occur due to atrial fibrillation, that would not be the only etiology. Similarly, trauma can result in a vertebral dissection, but again, that would not be the only possible etiology. The study demonstrates normal appearance on CT of the sigmoid sinus bilaterally and does not suggest a sigmoid sinus thrombosis. Similarly, subclavian steal is associated with posterior circulation symptoms or infarction; however, a segment of decreased density within the left vertebral artery is not the appearance seen with reversal of vertebral arterial flow.

thrombus

Question 255: Spinal immobilization and neurosurgical/orthopedic evaluation is essential for _____ odontoid fractures given their instability. In this case, the fracture results in slight anterior displacement of the odontoid fracture (seen on the sagittal image). The greater the displacement, the greater the risk of non-union. This patient underwent surgical placement of an odontoid screw for fixation of the fracture.

type 2

" what does corticosteroid do to muscle?

type 2 myofiber atrophy

Question 216. Figure 3.-- The diffusion-weighted views demonstrate multiple focal areas of acute infarction. These involve both the deep gray and white matter along with scattered cortical areas of infarction. These regions of infarction are in a multifocal vascular distribution atypical for branch large vessel disease and similarly atypical for small vessel infarctions or a cardioembolic etiology, which tend to be cortical. An MRA scan of the head performed concomitant with the study illustrated demonstrated marked beading along numerous arterial structures typical of ________. This is best seen and illustrated in Figure 3 on the conventional cerebral angiogram

vasculitis