Brain/Neuropathology

NO!! This loss of caudate/putamen in the gross specimen is typical for HUNTINGTON'S!! Clinical presentation is classic MS

30 year old white woman presents with loss of vision in one eye, inability to move her other eye past the midline, and urinary continence. Does this gross specimen match this presentation?

YES!!! This presentation is classic MS, and SO IS THIS GROSS IMAGE!! WE GOT DEMYELINATED AREAS OF WHITE MATTER UP IN HERE!!

30 year old white woman presents with loss of vision in one eye, inability to move her other eye past the midline, and urinary continence. Does this gross specimen match this presentation?

This is classic presentation for MS (optic nerve demyelination, INO, and autonomic demyelination leading to bladder dysfunction). Confirm with MRI which will show periventricular plaques Just for fun, you could do a spinal tap which would have CSF that shows >1 oligoclonal bands, increased IgG, and high myelin basic protein

30 year old white woman presents with loss of vision in one eye, inability to move her other eye past the midline, and urinary continence. What do you suspect might be going on here? How would you confirm?

A. It's a HIGH pressure headache because it's worse when she lies down so that tosses out C. It's not a migraine because of the fever, that also rules out pseudotumor. The high fever suggests infectious/inflammatory process like encephalitis or meningitis.

35 yo woman notes mild fever and malaise last night, she couldn't lie flat in bed due to her headache. She felt okay when she sat up. In the morning, she developed neck discomfort and a higher fever. Which of the following could be the diagnosis? A) Encephalitis B) Migraine C) Low pressure headache D) Pseudotumor Cerebri

Krabbe's disease, an AUTOSOMAL RECESSIVE mutation in galactocerebrosidase enzyme, deficiency causes buildup of galactocerebrosides in macrophages that destroys myelin. Presentation=INFANTS WITH MICROCEPHALY, also absent reflexes, developmental delay, and progressive motor/sensory problems

5 month infant + Galactocerebrosidase deficiency + microcephaly + absent reflexes EQUALS what?

B- Alpha Synuclein deposits in LEWY BODIES in LEWY BODY DEMENTIA!! These hallucinations are CLASSIC for Lewy body dementia, small formed people (not large scary people, it doesn't bother them). A describes Alzheimer's, C describes meningiomas, D describes Crudzfelt-Jacob Disease (a prion disease), and E describes pilocytic astrocytoma (most common brain tumor in kids)

72 year old man reports seeing a small man in the corner of the room. Histology of the cortex is likely to show which of the following? A) AB peptide amyloid plaques B) Alpha Synuclein Deposits C) Psammoma bodies D) 14-3-3 protein E) Rosenthal Fibers

This is likely Charcot-Marie-Tooth disease, a VERY RARE autosomal dominant condition with defective production of proteins a/w myelin or nerve structure in PERIPHERAL nerves. A/w foot deformities as seen here, foot drop from PERONEAL nerve neuropathy, claw hand, FOOT DEFORMITIES (like seen here), skinny legs from muscle wasting, and clumsiness/falls NOT FROM CEREBELLAR ATAXIA Typically presents in late childhood/adolescence

A 10 year old child presents with clumsiness and many falls. On physical exam, he has muscle weakness, foot drop, claw hand, unsteady gait, skinny legs, and a foot that looks like this picture. What is likely going on here?

Juvenile Myoclonic epilepsy which is common in children, usually ghey get absence seizures first (described here), then they can progress to myoclonic seizures later (with characteristic high yield jerking movements after waking up from sleep), progressing eventually to Grand-Mal seizures.

A 15 year old patient who has a history of spacing out for 10 seconds at a time multiple times a day has been waking up from sleep and jerking both arms irregularly. Recently, he had a grand-mal seizure. What condition is this likely to be?

Likely Pseudotumor Cerebri (NOT A TUMOR) AKA idiopathic intracranial hypertension. Almost ALWAYS in a female, obese patient of childbearing age. Worse when patients lay flat (it's a high pressure headache), commonly presents with tinnitus, diplopia (6th nerve palsy), and Papilledema from increased ICP. Diagnosed with a lumbar puncture (CSF is normal, elevated pressure) Treatment: Carbonic anhydrase inhibitor (acetazolamide), lumbar puncture (to relieve ICP), shunts if necessary.

A 30 year old morbidly obese female patient presents with headaches that are worst in the mornings. She reports tinnitus and diplopia with blurring of her eyesight. She has no family history of neurological disorders. On opthalmic exam, this image is obtained of her retina. What condition is this likely to be?

D- this is HUNTINGTON'S DISEASE, an autosomal DOMINANT trinucleotide CAG expansion disorder in the HTT gene (displays genetic anticipation). Causes chorea/athetosis described here, ataxia described here, and Parkinsonism can also be seen. Onset typically in 20s/30s, BASAL GANGLIA DEGENERATE, PARTICULARLY THE CAUDATE/PUTAMEN! Creates appearance of hydrocephalus on MRI. Psychiatric symptoms include aggression, anxiety/depression, apathy, psychosis. Patients at high risk of suicide A is hemiballism which would present with wild flinging movements on ONE SIDE ONLY B is essential tremor, not chorea C is Parkinson's, there's INCREASED dopamine here, not decreased E is Wilson's disease, a rare AR genetic defect in the ATP7B gene which impairs copper metabolism/transport and has systemic symptoms like liver damage

A 33 year old adopted patient recently developed wild-flinging movements of both arms, writhing movements of his hands/fingers, and has begun walking as though he looks drunk. Further examination is likely to reveal: A) A prior infarction of the subthalamic nucleus from a Lacunar stroke B) Improvement of symptoms with either alcohol, Propranolol, or Primidone C) A resting pill-rolling tremor, 4-6 hz and Lewy bodies in the Substantia Nigra pars Compacta D) Degeneration of the caudate nucleus on MRI E) Decreased levels of Ceruloplasmin

CLUSTER HEADACHES (commonly asked about) which are RARE but happen in clusters for a week on/off then remit for months at a time. There's a circadian rhythm to it, typically occurring at the same time of day (usually middle of night). AUTONOMIC FEATURES MUST BE PRESENT like pupil dilation, lacrimation, or runny nose etc...

A 35 year old man has severe, stabbing pain in his orbit/temple multiple times in 1 week, then this pain goes away for months, then it comes back. It's on/off for a week typically. He notes getting a runny nose and tearing up when he gets his headaches, which are are often in the middle of the night. What disorder is this?

B- This is Crudzfeldt-Jacob Disease (CJD) from misfolded prion proteins (in the PrPsc formation which is a beta sheet and not the PrPc formation which is an alpha helix) causing spongiform encephalopathy (see the sponges on histo?). CJD is the most common cause of spongiform encephalopathy. Presentation- patient is VERY YOUNG to have other forms of dementia, and the RAPID PROGRESSION gives away CJD along with the STARTLE MYOCLONUS described here. Unfortunately progresses to death within a year. Specific findings- you get 14-3-3 protein in the CSF and spike-wave complexes on EEG. A could describe normal pressure hydrocephalus, which wouldn't be so rapidly progressive or have startle myoclonus C describes Alzheimer's, D describes Parkinson's or Lewy Body Dementia, and E describes Huntington's

A 43 year old man presents with impaired cognition and impaired memory, which has been getting noticably worse since its onset two months ago. He also has unsteady gait and has exaggerated startles in response to small touches and noises. A brain biopsy is obtained and is shown on the left, right is normal for comparison. This patient will likely also have: A) Urinary incontinence B) 14-3-3 protein in his CSF C) Amyloid plaques and neurofibrillary tangles D) Alpha Synuclein Deposits E) Loss of GABAergic neurons in the Caudate Nucleus

A- this is viral meningitis based on the CSF analysis (normal glucose, mostly lymphs in CSF). Therefore, it's NOT the bacteria on this list. Note that Coxsackievirus, Echovirus, and Poliovirus are COMMON BOARDS causes of viral meningitis which are ALL ssRNA viruses transmitted through fecal-oral route (high yield)!! HSV is another common cause in real life

A 5 year old child presents with a fever of 102, confusion, and refuses to bend his head forward due to pain. He requests that the lights be turned down in his hospital room even though it's 3:00 PM. CSF analysis reveals glucose of 50, protein of 57, and an increased white blood cell count (predominantly lymphocytes). Which of the following is NOT likely to be causing the patient's symptoms? A) E. coli B) Coxsackievirus C) Echovirus D) Poliovirus E) Herpes Simplex Virus

B- could be bacterial or fungal meningitis

A 50 year old man presents with cough, fever of 102 F, severe headache, and nuchal rigidity with altered mental status. LP shows high pressure (25 cm water, normal 10-20), 2000 WBCs (elevated), 90% neutrophils, glucose of 20 mg/dL (serum glucose 120 mg/dL), and CSF protein of 170. Which of the following is this likely to be? A) Normal CSF B) Bacterial Meningitis C) TB meningitis D) MS E) Subarachnoid hemorrhage

This is likely Temporal Giant Cell Arteritis, an inflammatory vasculitis affecting ELDERLY patients (>50 yo). Common presentation includes unilateral headache with a pronounced temporal artery, jaw claudication (pain in jaw with chewing), proximal muscle pain/weakness (polymyalgia rheumatica), transient blindness, and inflammatory signs/symptoms like fever, weight loss, and anemia. Complications: Can include permanent blindness and increased risk of aortic aneurysm if untreated! Treatment: STEROIDS IMMEDIATELY if you suspect, even before the biopsy you should start steroids!

A 60 year old man presents with frequent right-sided headaches, pain in his jaw when he chews as well as shoulder pain/weakness, episodes with transient blindness, fevers, and unexplained weight loss. A CBC reveals mild anemia. What condition is this likely and what complications is this patient at risk of developing? What treatment(s) can be given?

The patient likely has Childhood Absence Epilepsy, characterized by multiple absence seizures daily (10 seconds at a time they stare off blankly into space, no post-ictal confusion) On EEG, the classic finding THAT YOU MUST KNOW is 2.5-5 Hz spike wave activity superimposed on a normal EEG background. The pharm treatment is Ethosuximide, which blocks thalamic T-type calcium channels, and is very effective!

A 7 year old girl is doing poorly in school, her parents say that she just stares off blankly into space for a few seconds and that this happens a lot. She's taken to a neurologist for evaluation. What condition does the child likely have? What is the neurlogist likely to find on EEG during one of the patient's episodes? What pharmacologic treatment will reduce the frequency of the patient's episodes?

A- this "ballistic" pattern of unilateral movement is HEMIBALLISM, resulting in damage to the subthalamic nucleus! B is Essential tremor (bilateral) C is Parkinson's, the age fits but it would be pill-rolling tremor at rest D is Huntington's which would have manifested earlier ahd have BILATERAL chorea/athetosis E is Wilson's disease, an AR defect in the ATP7B gene causing defective copper metabolism, would manifest MUCH YOUNGER and have other systemic symptoms like liver disease.

A 72 year old patient recently developed wild-flinging movements of his right arm and right leg. Further examination is likely to reveal A) A prior infarction of the subthalamic nucleus from a Lacunar stroke B) Improvement of symptoms with either alcohol, Propranolol, or Primidone C) A resting pill-rolling tremor, 4-6 hz and Lewy bodies in the Substantia Nigra pars Compacta D) Degeneration of the caudate nucleus on MRI E) Decreased levels of Ceruloplasmin

Normal pressure hydrocephalus which presents with the CLASSIC TRIAD of: 1) Urinary incontinence 2) Cognitive Impairment 3) Gait disturbances (from enlarged ventricles stretching the fibers of the corona radiata) Mnemonic=WET, WACKY, and WOBBLY Treatment=LP to improve symptoms (removing CSF) or VP shunt (also removing CSF)

A 78 year old woman presents with symptoms of dementia (her cognition has deteriorated), and is unable to walk in a straight line or walk without assistance. She has recently begun wearing adult diapers. What disease is this likely to be?

Medulloblastoma, which is the only primary brain tumor that spreads to spots outside the CNS. Reminder- Medulloblastoma is derived from neural ectodermal embryonic tissue, arises in the cerebellum, and can spread to the cortex, spinal cord, and occasionally bone/lymph nodes

A Brain tumor has metastasized to the bone and regional lymph nodes. What tumor is it likely to be?

CRANIOPHARYNGIOMA which is the MOST COMMON non-glial brain tumor in kids. It's benign. Embryologically derived from REMNANTS of Rathke's pouch (oral ectoderm) that fails to regress

A child presents with bitemporal hemianopsia. What neoplasm are you most concerned about? What is its embryological derivation?

E- Neurofibromatosis type 2

A patient presenting with bilateral vestibular schwannomas most likely has: A) Neurofibromatosis type 1 B) Tuberous sclerosis C) Von Hippel-Lindau syndrome D) Li-Fraumeni syndrome E) Neurofibromatosis type 2

B- This is describing a Schwannoma, a benign tumor of the peripheral nervous system. Since cranial nerves 3-12 are myelinated by Schwann cells, any of them could theoretically present with tumors, but Schwannomas are most common on the vestibular portion of the 8th nerve, causing unilateral hearing loss/tinnitus. Schwannomas are S100 positive!!! Anatomically, the most common location will be on the vestibular (8th) nerve at the cerebellopontine angle It's not malignant (A), glioblastoma (C), a metastasis (D) or an astrocytoma/glioblastoma (E)

A patient presents with a unilateral mass on MRI and presents with unilateral hearing loss. This tumor is likely to be: A) Grade 4 B) S100 positive C) Positive for pseudopalisading necrosis on histology D) A metastasis from another primary tumor elsewhere in the body E) A proliferation of astrocytes Bonus: Where is this tumor likely to be anatomically?

A- this is describing Cauda Equina syndrome caused by compression of the cauda equina. LMN lesions are seen BUT NOT UMN because compression is after the level of the CST. So plantar response (no babinski), loss of pain/temp/innervation to the saddle area is common (sacral dermatome), bowel/bladder dysfunction (autonomic loss). Prostate cancer loves to go to the BONE to metastasize so boards love to ask about metastatic bone lesions causing cauda equina syndrome!

A patient presents with loss of sensation in his "saddle" area and fecal and urinary retention. On his neuro exam, lower extremity reflexes are absent (upper extremity is fine) and his anal wink reflex is also absent. His toes are down-going (plantar response). Which of the following is he also likely to have? A) Metastatic prostate cancer B) Pernicious Anemia C) A sexually transmitted disease caused by a spirochete D) Type 2 diabetes mellitus E) An autoimmune attack on Schwann cells after a resolved GI infection

This is hippocampal sclerosis AKA Mesial Temporal Sclerosis, which makes the temporal lobe the most common location for partial seizures. Characterized by loss of neurons, shows up white on MRI

A patient presents with recurrent partial seizures of the temporal lobe. An MRI (shown here) shows neuronal loss in the patients temporal lobe. What is this condition?

The MI is likely causing hypoperfusion to the brain causing global ischemia. This is likely causing BILATERAL infarctions of the watershed areas of the brain that have dual blood supplies. In the ACA/MCA border zone territory, it includes the primary motor cortex that innervates proximal muscles of the shoulder, trunk, and thigh, leading to the "MAN IN THE BARREL" SYNDROME!!!

A patient with an MI now presents with bilateral weakness of his shoulders and thighs that progresses to full-blown paralysis, though strength is normal in his face, hands and feet. What is likely happening to this patient?

D- This describes Alzheimer's, which classically presents with memory deficits and global changes such as visuospatial dysfunction. A describes frontotemporal dementia (AKA Pick syndrome) B describes Parkinson's (remember TRAP mnemonic for presentation) C describes Huntington's E is nonspecific and could describe a ton of different things

A patient with diffuse cortical atrophy who died in a nursing home after 10 years of a neurodegenerative disorder had this brain biopsy. What would be likely have been the patient's clinical presentation? A) Personality changes and executive function B) Tremor and bradykinesia C) Chorea and dementia D) Memory and visuospatial dysfunction E) Psychomotor slowing

This patient has Progressive Multifocal Leukoencephalopathy (PML) caused by immunosuppression and JV polyomavirus. Typical presentation= AIDS patient with CD4<200 or immunosuppressed patient with new and progressive onset of weakness/visual changes/difficulty speaking. MRI shows multiple NON-ENHANCING lesions. Different from MS because the lesions would be smaller and often are contrast enhancing (acute plaques)! Treatment=stop the immunosuppression for this patient!

A patient with multiple sclerosis has been well managed with Natalizumab. However, over the last few weeks she has experienced new onset of visual changes and difficulty with speech that has not regressed and has progressively worsened. An MRI of her head was obtained (shown here). What might be going on here and what treatment(s) can be given?

E- this gross specimen comes from a patient who had Huntington's (loss of the caudate and putamen), characterized by a mutation in the HTT (Huntingtin) gene which causes a CAG trinucleotide repeat. Net result is loss of inhibitory GABA-ergic neurons in the caudate/putamen leading to decreased inhibition of the striatum on the subthalamic nucleus. Overactive subthalamic nucleus inhibits the inhibitory GPi interneurons that synapse on the motor thalamus. Net result-DISINHIBITION of the motor thalamus, hyperexcitability of the cortex, and a HYPERKINETIC movement disorder! A describes Parkinson's, B and D describe Alzheimer's, C describes ALS.

A patient with with this brain section would have been likely to: A) Have spasticity and an expressionless face B) Present initially with memory loss C) Have significant denervation atrophy D) Have the APOE4 allele E) Have a mutation and a CAG trinucleotide repeat

A- though there's both UMN and LMN degeneration, the UMN degeneration "wins out" here and you see hyperreflexia with extensor plantar response.

ALS patients have: A) Hyperreflexia B) Hyporeflexia C) Neither D) Mixed, depends on where

APP (amyloid precursor protein) gets cleaved by alpha/beta/gamma secretase. When beta secretase is overactive or when there's excess APP, AB peptide is formed by beta secretase cleavage of APP. AB peptide is what accumulates to form amyloid plaques that entrap neuritic processes

APP pathway in formation of plaques in Alzheimer's disease?

C- absence seizures usually arise in children and last about 10 seconds with brief staring spells.

Absence epilepsy is characterized by which of the following findings: A) Onset is usually in young age B) Seizures usually last 1-2 minutes C) Multiple, brief staring spells D) Automatisms E) EEG showing focal temporal spikes

Chronically degenerative disorder of the MACULA (central vision) in elderly, smoking is significant risk factor. Pathogenesis- Drusen deposition leading to geographic atrophy of retinal photoreceptor cells and pigmented epithelium, both dry and wet variants

Age-Related Macular Degeneration?

C- DOOOOOOOOO NOTTTTTTTTTTTT GIVE STEROIDS TO GBS/AIDP PATIENTS!!!

All of the following are indicated treatments for Guillain-Barre Syndrome EXCEPT: A) Intravenous Immunoglobulins (IVIG) B) Plasmaphoresis C) Corticosteroids D) Mechanical Ventilation

ONLY MOTOR degeneration of both UMN and LMN. Anterior horn involvement=LMN lesions with denervation atrophy/fasciculations. CST involvement=UMN findings like spasticity/hyperreflexia/Babinski. HIGH YIELD genetic association for some familial cases- SOD1 mutation (a zinc-copper superoxide dismutase mutation), though most cases are sporadic

Amyotrophic Lateral Sclerosis (ALS)?

Metachromatic Leukodystrophy, a lysosomal storage disease, most common type of leukodystrophy, 3 types that can present at different ages. Genetic defect- AUTOSOMAL RECESSIVE mutation in arylsulfatase which causes buildup of sulfatides that leads to impaired myelin production/breakdown, DEMYELINATING DISEASE that presents with central AND peripheral demyelination with ATAXIA AND DEMENTIA

Ataxia + Dementia + arylsulfatase deficiency equals what?

B- lambert eaton but NOT myasthenia

Autonomic dysfunction is common in A) Myasthenia Gravis B) Lambert Eaton C) Neither D) Both

Lesion to subthalamic nucleus (lack of excitation to the inhibitory GPI)

Ballism or large flinging movements=what lesion on boards?

Becker isn't as severe as duchenne, it's not a deletion. However, proximal muscle weakness still manifests. Also XLR inheritance, also high CK levels!

Becker Muscular Dystrophy?

As the picture shows, blood supply to the spinal cord is MOSTLY from the anterior spinal artery (one of them, makes it more susceptible to an ASA stroke from occlusion). You'd lose EVERYTHING EXCEPT posterior column function (vibration/position sense) because the ASA does everything else. Posterior spinal arteries- there's TWO of them so it might be harder to knock out vibration/position sense bilaterally. Artery of Adamkiewicz- supplies the lower 2/3 of the anterior spinal cord as branch off the aorta, so AORTIC SURGERY/AAA are risk factors for infarcts caused by compromise of the artery of Adamkiewicz!

Blood supply to spinal cord and Artery of Adamkiewicz? Hint: Use this picture

COMPLETE hemisection of the spinal cord on one side. Causes=trauma, typically bullet or knife wound. Findings- At the level of the lesion: UNILATERAL COMPLETE LOSS OF SENSATION AND MOTOR WITH FLACCID PARALYSIS. Below the level of the lesion- CONTRALATERAL pain/temp loss (Spinothalamic tract crossed over) with ipsilateral motor loss (flaccid paralysis), and ipsilateral loss of vibration/position sense

Brown-Sequard Syndrome findings and causes?

Rifampin

Close contacts of a patient with N. meningitidis meningitis should receive what prophylaxis?

E is false (surgical evacuation not always needed) A and B are true, abscesses most often present with mass lesion effects and not very often prevent with infectious symptoms like fever C and D are true, while the most common cause is hematogenous spread, trauma/surgery/Otitis media can definitely cause brain abscesses too F is true. Prolonged antibiotic therapy because you really want to nail this abscess!!!

Concerning Brain abscesses, all of the following are true EXCEPT: A) May present as a mass lesion with headache and focal neurological deficits B) Fever seen in 50% of cases only C) The source is usually hematogenous D) Otitis media and head trauma/surgery can be other causes E) Surgical evacuation is indicated in all cases F) Prolonged antibiotic treatment (4-6 weeks) is recommended

B- Lambert eaton gets incremental response by flooding the presynaptic terminal with calcium, releases more ACH and gets greater response!

Deep tendon reflexes get incrementally more exaggerated in: A) Myasthenia Gravis B) Lambert Eaton C) Neither D) Both

Simple partial=no loss of consciousness, only one part of brain affected Complex partial=only one part of brain affected, WITH loss of consciousness Absence seizures=children transiently lose awareness of surroundings, often genetic and happens hundreds of times per day. Tonic Clonic (Grand Mal) seizures= EXTREME muscular rigidity, abrupt loss of consciousness, blank face, respiration inhibited, falls are common. Rhythmic jerking/writhing (clonic phase), tongue biting and post-ictal confusion often with bowel/bladder incontinence Atonic "Drop" seizures= patients become flaccid and collapse, can mimic syncope from cardiac problem Myotonic seizure= Rhythmic jerking of one body part few times, not as severe as tonic-clonic Secondary generalized= becomes generalized in all parts of brain after starting off partial in only one part of brain

Define each of the following: Simple Partial Seizure Complex Partial Seizure Absence seizures Tonic Clonic (Grand Mal) seizures Atonic "Drop" seizures Myotonic seizure Secondary generalized seizures

N. meningitidis is inhaled, enters through the nasopharynx where its pilus allows it to attach to the mucosal surface while IgA protease helps it evade host defense (by cleaving IgA at its hinge point. From the nasopharynx, it enters the bloodstream and spreads hematogenously to the CNS where it then infects the meninges.

Describe the entry route, virulence factors, and infection route for N. meningitidis meningitis?

Type 4 hypersensitivity reaction against CNS myelin and oligodendrocytes, specifically against myelin antigens in the CNS (especially myelin basic protein). These CD8 T cells release IFN-g which RECRUITS MACROPHAGES!

Describe the pathogenesis of MS in terms of what you'll need for step 1?

In Down's syndrome, you have 3 X chromosomes. Since the APP gene is on chromosome 21, extra breakdown of APP leads to increased AB peptide which causes EARLY ONSET ALZHEIMERS <40 yo IN DOWN'S PATIENTS!

Down's and early onset Alzheimer's?

Dry- Drusen buildup with yellow/white spots on retina with NO ANGIOGENESIS Wet- ANGIOGENESIS!! Increased vessels seen, possibly with hemorrhages, still have Drusen buildup. Can inject Bevacizumab (anti-VEGF mAb) to prevent neovascularization Dry macular degeneration usually leads to wet (neovascularization)

Dry vs Wet age-related macular degeneration?

DUchenne=DUletion. Frameshift deletion of the dystrophin gene producing symptoms of proximal muscle weakness from a young age (symptoms evident by age 3), kids do the GOWER maneuver! Kids get calf pseudohypertrophy, the calf muscles get destroyed and REPLACED BY FAT which makes them look strong but they aren't! Inheritance=XLR!! CRAZY HIGH CK levels!

Duchenne Muscular Dystrophy? Inheritance pattern?

A- ONLY dementia has a normal EEG. Delirium (the acute process) has abnormal EEG.

EEG will be normal in A) Dementia B) Delirium C) Neither D) Both

Essential tremor- WORSE with intentional movement (action tremor), usually SYMMETRIC tremor, symptoms may improve with ALCOHOL (high yield) Parkinson's- BETTER with intentional movement (resting tremor), usually ASYMMETRIC tremor, onset typically in 60s/70s

Essential tremor vs parkinson's?

Childhood absence epilepsy, which is really the only condition that Ethosuximide is effective at treating! Reminder- it's a thalamic T-type calcium channel blocker

Ethosuximide is effective at treating what condition?

Werdnig-Hoffman disease (AKA Spinal Muscular Atrophy type 1) which is anterior horn degeneration EVERYWHERE for poor infants. Survival is 2-3 years on average, BUT the new miracle drug Nusinerin has shown dramatic improvement in disease course!

Floppy baby syndrome=what?

AR genetic inherited disease, unstable trinucleotide repeat (GAA) in the FRATAXIN GENE (high yield to know that it's essential for MITOCHONDRIAL iron regulation, leads to free radical damage when gene is lost). Presents in early childhood, patients typically wheelchair bound by age 10. Degenerative disorder of the cerebellum AND the spinal cord tracts. A/W HYPERTROPHIC CARDIOMYOPATHY! USUAL CAUSE OF DEATH! Also a/w diabetes! Friedreich is Fratastic (frataxin): He's your favorite frat brother, ALWAYS STAGGERING AND FALLING but he has a sweet, big heart!

Friedreich's Ataxia?

Trisomy 21 (3 copies of the APP gene leading to increased AB peptide from increased breakdown), most Down's patients get alzheimer's by age 40 Presenilin 1 is the key mutation in familial Alzheimer's which is rare and leads to early onset Alzheimer's.

Genetic risk factors for Early Onset Alzheimer's?

Mutation with abnormal expansion of CAG trinucleotide repeats in the Huntingtin gene (HTT)

Genetics of Huntington's?

1) Campylobacter jejuni 2) CMV ONLY IF HOSTS WERE IMMUNOCOMPROMISED AND HAD SYMPTOMATIC INFECTION!!

Guillain-Barre Syndrome commonly arises after infection with what two pathogens?

Albuminocytologic dissociation is PATHOGNOMONIC for GBS (elevated protein with normal WBC count). Also normal glucose and increased protein!

Guillain-barre spinal tap

Tropical spastic paraparesis with predelection for females>males. RARE though many people carry the virus. Will see signs on MRI with slow progression over years of spasticity and muscle weakness AKA myelopathy

HTLV-1?

Buildup of ammonia which goes to the brain causing delerium and can be fatally toxic! The TIPS proceduce can cause encephalopathy from releasing ammonia in cirrhosis/liver failure patients!!

Hepatic encephalopathy?

Headache/focal neural deficits present more often that infectious symptoms (fever). On imaging see a single lesion with a CONTRAST-ENHANCING RIM!!!

How are brain abscesses likely to present?

Most patients get: Blood work (to look for electrolyte abnormalities like low/high Na+, hypoglycemia, hypocalcemia, hypomagnesemia) EKG (to differentiate cardiac syncope from seizure) EEG Brain imaging to look for tumor/stroke Sometimes lumbar puncture if meningitis/encephalitis is suspected

How are seizure patients often worked up?

LEFT abducens nucleus lesion: Won't be able to voluntarily move both eyes to the left LEFT frontal eye field lesion: Since frontal eye fields control voluntary gaze to the CONTRALATERAL side, voluntary gaze to the RIGHT will be impaired and the eyes will point to the side of the lesion (to the left, EXACTLY like hypoglossal nerve/nucleus lesions!) In summary- abducens nucleus lesions and frontal eye field lesions produce deficits in looking to DIFFERENT sides!!

How are the presentations of a LEFT abducens nucleus lesion and a LEFT frontal eye field lesion different?

Timecourse of onset: Strokes present SUDDENLY AND RAPIDLY Tumors present gradually over weeks to months

How can a brain tumor be differentiated from a stroke clinically?

CONTRAST ENHANCING lesion on MRI with contrast (gadolinium) because of ALTERED permeability of the BBB

How can you detect an acute attack of MS on MRI?

Afterwards, many seizures (particularly Tonic-Clonic) will be followed by a post-ictal state of confusion/decreased awareness. However, a syncopal episode of cardiac etiology will NOT be followed by a post-ictal state.

How can you differentiate cardiac syncope from a seizure without EEG/EKG?

Some differentiators include: Post-ictal confusion (and/or Todd's paralysis after seizures, post-syncope patients are generally okay), shaking (more common in seizure though happens in both), Prodrome (Deja Vu is pathognomonic for seizure, syncope is commonly tachycardia, PALLOR, sweating), duration (syncope typicall short, seizures last longer), incontinence (can happen in both), cyanosis (much more common in seizure), tongue biting (more often in seizure), trigger (peeing for orthostatic syncope for example), past medical history (seizures in the past? Autonomic problem? Dehydration/hypovolemia?)

How can you differentiate seizure vs syncope by taking a history?

1) Broca's patients are aware of their deficits, Wernicke's patients aren't which means that... 2) Broca's patients can follow commands and comprehend you, but Wernicke's patients aren't able to comprehend or follow commands 3) Broca's is likely to present with right hemiparesis of the arm/head/trunk (not leg) but Wernicke's commonly presents with right hemianopsia or right superior quadrantopia b/c it's so close to the temporal visual radiations 4) Broca's: Left frontal lobe (near primary motor cortex) Wernicke's: Left temporo-parietal region

How do Wernicke's and Broca's aphasia differ in terms of: 1) Patient awareness of problem 2) Ability to comprehend and follow commands 3) Associated neurological findings 4) Anatomical location affected

Hyperphosphorylation of the Tau protein (necessary for stabilization of microtubules) leads to DISORDERED TANGLES OF MICROTUBULES WITH HYPERPHOSPHORYLATED TAU WITHIN THESE TANGLES!! Damn, look at these tangles! The disordered microtubules running through the cytoplasm leading to the "flame shape" of the neurons seen here!

How do these neurofibrillary tangles form in Alzheimer's disease?

Right-sided (contralateral) hemiparesis of the arm/face/trunk (not the leg/foot) and Aphasia (speech is left-sided in vast majority of patients)

How might a Left-sided MCA stroke present?

Loss of vision, particularly homonymous hemianopsia of the contralateral side with macular sparing b/c the macula has a dual blood supply in the occipital lobe! Also PCA supplies the THALAMUS, so sensation could be lost on the contralateral side (entire side b/c the entire somatosensory thalamus is affected!)

How might a PCA stroke present?

Left-sided (contralateral) hemiparesis of the arm/face/trunk (not the leg/foot) and Left-sided hemineglect (can't recognize objects presented on the side they neglect)

How might a Right-sided MCA stroke present?

Acute loss of contralateral hip/leg/foot movement because the humunculus has the knee draping over the bend in the medial brain! Also sensory loss of the contralateral leg/foot b/c of damage to the same region in the somatosensory cortex (decreased pin prick/vibration sensation). (ACA also supplies the caudate)

How might an ACA stroke present?

LOCKED IN SYNDROME from central pons KO (including CST/CBT damage) but sensation, vision, and eye movements are fine!!

How will a Basilar artery occlusion manifest?

AICA stroke=Lateral Pontine syndrome- hits the vertebral system (nystagmus, vertigo), lose spinothalamic tract, spinal nucleus of V (CROSSED PAIN AND TEMP LOSS), Horner's syndrome (ipsilateral)...

How will an AICA occlusion manifest?

Anterior Spinal Artery stroke=Medial Medullary syndrome- CST gone (UMN contralateral lesion) so contralateral hemiparesis, CN12 is whacked (tongue deviates to side of lesion), loss of proprioception/vibration on contralateral side (medial lemniscus gone)

How will an Anterior Spinal Artery occlusion manifest?

PICA stroke=Lateral Medullary syndrome AKA Wallenberg's Syndrome- vestibular nuclei gone (nystagmus, vertigo), Horner's, crossed pain&temp loss, nucleus ambiguus lost (hoarseness, dysphagia, decreased gag reflex). NOTE THAT MIDLINE STRUCTURES LIKE HYPOGLOSSAL NUCLEUS ARE FINE!

How will an PICA occlusion manifest?

AICA stroke=Lateral Pontine syndrome- hits the vertebral system (nystagmus, vertigo), lose spinothalamic tract, spinal nucleus of V (CROSSED PAIN AND TEMP LOSS), Horner's syndrome (ipsilateral)... PICA stroke=Lateral Medullary syndrome AKA Wallenberg's Syndrome- vestibular nuclei gone (nystagmus, vertigo), Horner's, crossed pain&temp loss, nucleus ambiguus lost (hoarseness, dysphagia, decreased gag reflex). NOTE THAT MIDLINE STRUCTURES LIKE HYPOGLOSSAL NUCLEUS ARE FINE! Anterior Spinal Artery stroke=Medial Medullary syndrome- CST gone (UMN contralateral lesion) so contralateral hemiparesis, CN12 is whacked (tongue deviates to side of lesion), loss of proprioception/vibration on contralateral side (medial lemniscus gone)

How will the following manifest: AICA occlusion PICA occlusion Anterior Spinal Artery occlusion

Generalized seizures (unresponsive) where you drop to the ground and have myoclonic jerks that aren't necessarily rhythmic (but often are). NOT a tonic-clonic seizure tho which is more severe (RIGID/STIFF)

How would myoclonic seizures typically present?

Triad of HTN, proteinuria, edema is late pregnancy/early post-partum is PRE-ECLAMPSIA. Toss in seizures and you have ECLAMPSIA MgSO4 treats seizures in pre-eclampsia, does not treat seizures in ANY OTHER condition!

Hypertension, proteinuria, and edema in a pregnant woman in late pregnancy/post-partum period is called what? What's it called if you add in seizures? How are the seizures treated?

Neurofibromatosis type 2

In what condition are bilateral schwannomas of the 8th cranial nerve seen?

SMALL CELL LUNG CANCER! (neuroendocrine tumor, derived from Kulchitsky cells, Chromogranin+, NSE+, L-myc amplification)

Lambert Eaton. What else is going on?

A- neovascularization in the tumor has an abnormal structure for brain vessels in that they LACK a BBB, causing leakage of contrast into the interstitium

MRI imaging of a glioblastoma usually shows abnormal enhancement following administration of a contrast agent (like gadolinium). The best explanation for this is: A) Abnormal structure of vascular endothelial cells present in the tumor B) Altered permeability of blood vessels due to cytokine production by tumor cells C) Destruction of blood vessels by invading tumor cells D) Vascular congestion due to slowing of blood flow within the tumor E) Vascular supply of tumor derived from extracranial vessels normally lacking a blood brain barrier

Oligoclonal bands>1, IgG index is INCREASED (highest sensitivity and specificity for MS diagnosis), increased myelin basic protein, normal/increased mononuclear cells, protein is increased, glucose is normal!!!

MS spinal tap?

Viral/Aseptic meningitis!!!

Meningitis + Normal CSF glucose equals what?

ONLY eclampsia during pregnancy (no other condition)

MgSO4 would be effective at treating seizures of what etiology?

A- ONLY oligodendrocytes because MS is ONLY a CNS problem (doesn't affect PNS)

Multiple sclerosis affects A) Oligodendrocytes B) Schwann cells C) Neither D) both

THYMOMA!

Myasthenia Gravis- what thoracic disorder?

STEP 2 LOVES THIS!! Optic neuritis PLUS transverse myelitis (like MS, only you NEVER have brain lesions!). HIGH YIELD a/w anti-aquaporin 4 antibody. harder to treat than MS!

Neuromyelitis Optica?

Normal Pressure Hydrocephalus is a rare cause of dementia that presents with the CLASSIC TRIAD of: 1) Urinary incontinence 2) Cognitive Impairment 3) Gait disturbances (from enlarged ventricles stretching the fibers of the corona radiata) Mnemonic=WET, WACKY, and WOBBLY Treatment=LP to improve symptoms (removing CSF) or VP shunt (also removing CSF)

Normal Pressure hydrocephalus? What treatment can improve symptoms?

Common viruses (KNOW THAT THEY'RE ssRNA VIRUSES TRANSMITTED THROUGH THE FECAL-ORAL ROUTE) include coxsackievirus, Echovirus, and Poliovirus for boards purposes. Again, ALL ssRNA, ALL FECAL-ORAL! Rare viral causes include HSV, HIV, WNV, VZV

On boards, which viruses are likely to cause viral meningitis and how would you characterize their genomes/transmission routes?

Open angle: Flow itself is okay, it's an IMBALANCE of production/drainage of aqueous humor. More common, more insidious (more common cause of vision loss), not emergent. Closed angle: Angle itself is CLOSED from ciliary body compressing the trabecular meshwork/canal of Schlemm. CAN'T REABSORB!! More emergent, leads to blindness if untreated. Very painful too, more acute presentation (also much more rare) GLAUCOMA LEADS TO BLINDNESS IF UNTREATED!

Open angle vs Closed angle glaucoma? What happens if both go untreated?

Pick's disease AKA FrontoTemporal Dementia is an atrophy of the frontal/temporal lobes specifically (it PICKS the frontal and temporal lobes). Spares the Occipital/Parietal lobes mostly. Earlier onset (age 50-60), and a HUGE association with ALS Presentation- apathy and PERSONALITY CHANGES WITH NO SOCIAL AWARENESS (due to frontal lobe loss) that eventually progresses to dementia. Histologically, you'll see round aggregates of Tau protein in neurons of the cortex (remember that ALZ has neurofibrillary tangles with hyperphosphorylated Tau). DIFFERENT FROM ALZ BECAUSE ALZHEIMER'S IS IN ALL AREAS OF THE BRAIN, PICK'S IS FOCAL

PIck's disease?

ALS is a selective degeneration of MOTOR NEURONS, both upper and lower. Typically sporadic, though genetic causes=SOD1 mutation or repeat expansion of C9orf72 gene.

Pathophysiology of ALS?

Huntingtin gene (HTT) has trinucleotide repeats (CAG) which leads to LOSS OF INHIBITORY GABA-ERGIC NEURONS which leads to decreased inhibition of the striatum, then dis-inhibiting the subthalamic nucleus to send more inhibition to the GPi to send less inhibition to the motor thalamus. Net effect=DISINHIBITION OF THE MOTOR THALAMUS, HYPEREXCITABILITY OF THE CORTEX, AND HYPERKINETIC MOVEMENT DISORDER

Pathophysiology of Huntington's?

CHECK FOR UNDERLYING MALIGNANCY!! Dermatomyositis can be a paraneoplastic syndrome!

Patient has dermatomyositis. What do you do doc?

Complex partial seizures because there's LOSS OF AWARENESS! Myoclonic seizures wouldn't have loss of awareness and would likely be in both arms. Simple partial seizures wouldn't have loss of awareness!

Patient presents with brief stereotyped movements of right arm only with lip smacking without loss of consciousness, though the patient cannot remember these episodes afterwards. They are responsive during these episodes. What kind of seizure is this?

Xanthochromia from RBC breakdown! When you spin down CSF, it'll look yellow to the naked eye (confirmed on spectroscopy)

Patient who had a subarachnoid hemorrhage 12 hours ago should have CSF that looks like what?

BLOODY WITH INCREASED RBCs IN TUBES 1-4 CONSISTENTLY

Patient who had a subarachnoid hemorrhage 2 hours ago should have CSF that looks like what?

This is Guillain-Barre syndrome, a demyelinating disease of the PERIPHERAL nervous system attacking Schwann cells in peripheral nerves, specifically in the endoneurium Treatment- 1) Respiratory support 2) Plasmaphoresis 3) IVIG

Patient with ascending paralysis shows elevated protein and normal cell counts on his CSF. He recently got over a "GI bug" last week. What might be going on here and what treatment(s) can he be given?

STATINS causing myopathy!! Proximal muscle weakness

Patient with hyperlipidemia and cant' comb hair. What's going on?

2 things on the differential=Tertiary syphilis (Tabes Dorsalis) or subacute combined degeneration (B12 deficiency). You'll want to work them up for both- first take careful history including sexual history. Then maybe get a blood smear which may or may not show megaloblastic RBCs if there's a B12 deficiency (it doesn't have to!) Then maybe get blood tests like anti-FTA antibody test for syphilis

Patient's got a positive Romberg sign- What do you do doc?

NO, you expect that for every 700-1000 RBCs in the CSF you'd have about 1 WBC. So you'd expect about 4-5 WBCs!

Patient's lumbar puncture has 4200 RBCs and 5 WBCs. Are her white counts elevated?

YES! You expect that for every 700-1000 RBCs in the CSF you'd have about 1 WBC. So you'd expect about 4-5 WBCs!

Patient's lumbar puncture has 4200 RBCs and 60 WBCs. Are her white counts elevated?

Herpes encephalitis which presents with symptoms of viral meningitis and HIGH RBCs because herpes encephalitis likes to go to the temporal lobes and cause hemorrhage, leading to BLOOD in the CSF!!!

Patients presents with altered mental status, fever, nuchal rigidity. Spinal tap shows CSF with normal glucose, 68 WBCs (mostly lymphs), and 3000 RBCs. What clinical picture does this look like?

Polio=ssRNA virus, fecal-oral transmission. Vaccine has eradicated it! LMN lesion only. Anterior horns of LMN cell bodies get knocked out, creating an asymmetric flaccid paralysis, usually spreading to other limbs in a clockwise/counterclockwise distribution. On boards, it'll be an UNVACCINATED CHILD who FIRST gets febrile illness and 4-5 days later gets asymmetric flaccid paralysis in the legs (more than the arms)

Poliomyelitis?

C- Absence seizures don't get post-ictal confusion, but partial complex and tonic/clonic do!

Post-ictal confusion can be seen after all of the following EXCEPT: A) Partial complex seizure B) Generalized tonic clonic seizures C) Absence seizures D) All of the following have post-ictal confusion

Epstein-Barr virus causes primary CNS lymphoma in AIDS patients

Primary CNS lymphoma in a patient with a CD4 T cell count <200 is likely due to what etiology?

AR inherited disease of JUST LMNs. So flaccid paralysis, often fasciculations ESPECIALLY of the tongue. Presents similar to polio (but might be more symmetrical) Type 1 is AKA Werdnig-Hoffman disease. Floppy babies with respiratory drive decline, usually death within 2-3 years. SMA 2/3 presents later but more manageable disease course. Nusinerin (intrathecal) has DRAMATICALLY improved the course of patients with SMA!!

Spinal Muscular Atrophy? New exciting treatment?

Most commonly Staph Aureus, usually spreads hematogenously from skin/soft tissue. Can compress the spinal cord and present with fever and spinal pain as most common findings (sometimes focal neuro deficits) Treatment=DECOMPRESS THE SPINAL CORD IMMEDIATELY through surgery

Spinal cord abscess?

Meningiomas can cause injuries, so can Ependymomas which are IN THE SPINAL CORD ITSELF

Spinal cord tumors?

B12 deficiency causes Subacute Combined Deneration, a chronic demyelinating disorder involving the posterior columns, CST, and dorso-spinocerebellar tract. Findings= weakness and ataxia (it's possible they may not show megaloblastic RBCs!!), POSITIVE ROMBERG, spastic paresis in legs with hyperreflexia in lower extremities and POSITIVE BABINSKI! It's basically Tabes Dorsalis PLUS UMN damage Dentists can be at risk for SCD because inhaling Nitrous Oxide (NO) can cause a B12 deficiency!

Subacute Combined Degeneration? What causes it? Why are dentists at increased risk?

Subarachnoid hemorrhage (Xanthochromia is sign of bleeding into CSF)

Sudden onset severe headache plus xanthochromia on CSF sample equals what?

Syringomyelia occurs from a "syrinx" AKA cyst in the spinal cord, usually in the spinal canal, and usually in the C8-T1 level. Creates a Cape-Like distribution of pain/temp loss in the arms/back bilaterally by knocking out the anterior white commissure at the level of lesion. Can also expand to create LMN weakness by impacting the anterior horns or causing an ipsilateral Horner's syndrome. Posterior columns/CST are untouched! Causes- 1) Chiari malformation type 1 2) trauma 3) surgery HIGH YIELD ASSOCIATION=CHIARI MALFORMATION TYPE 1!! ALSO KYPHOSCOLIOSIS!!!

Syringomyelia? Association?

Caused by TERTIARY SYPHILIS. Posterior column degeneration and posterior root degeneration, leading to ABSENT REFLEXES, ATAXIA, POSITIVE ROMBERG SIGN, ARGYLL-ROBERTSON PUPILS (small pupils that accomodate but don't react to light). Classic patient is a patient with OTHER STDs and difficulty walking, 5/5 strength in legs and arms, + Romberg, - knee/ankle reflexes bilateral, Argyll-Robertson Pupils

Tabes Dorsalis?

C- temozolomide is commonly used as first-line treatment for high grade gliomas like Glioblastoma

The chemotherapeutic agent that is most appropriate for use in a patient with a HIGH GRADE glioma is: A) Vincristine B) Acyclovir C) Temozolomide D) Dexamethasone E) Herceptin

B- Autoantibodies against the ACH-R causes INTERNALIZATION by endocytosis, DECREASING the density of ACH-R on the postsynaptic surface!

The density of Nicotinic ACH receptors on the post-synaptic region will be __________ in myasthenia gravis A) Increased B) Decreased C) Unchanged

C- These neurofibrillary tangles are typical of Alzheimer's disease which contain hyperphosphorylated tau leading to disordered microtubules. A describes Lewy bodies (seen in Parkinson's and Lewy Body Dementia) B describes Creutzfeldt-Jacob Disease (CJD) which is a form of Spongiform encephalopathy. No sponges seen here on histo! D- S100 positivity should make you think of a Schwannoma. I don't know what that looks like but it ain't this!

The purple arrows point to areas with: A) Alpha synuclein deposits B) Aggregates of proteins in the PrPsc formation C) Hyperphosphorylated Tau D) S100

F- Epidural hematomas like this one are often from ruptures of the middle meningeal artery, which is a direct branch off of the MAXILLARY artery! While the maxillary branches off the external carotid, the middle meningeal doesn't directly branch off the external carotid!

This arterial rupture arose from a direct branch off of which of the following arteries? A) Internal Carotid Artery B) Basilar Artery C) Middle Cerebral Artery D) Anterior Cerebral Artery E) Posterior Cerebral Artery F) Maxillary Artery G) External Carotid Artery H) Temporal Artery

A- this is an EPIDURAL HEMATOMA (football/lens shape) which is a high pressure bleed and most often a rupture of the middle meningeal artery. Often presents initially with neurological symptoms, then has a LUCID PERIOD after initial loss of consciousness, where the patient appears okay, then the patient takes a downward turn and increased intracranial pressure from mass effect can lead to a LETHAL BRAIN HERNIATION! B would lead to subdural hematoma from ruptured bridging veins in elderly patients C would be likely to form berry aneurysms which could rupture to give a subarachnoid hemorrhage (star sign on head CT) D and E would lead to strokes (hemorrhagic and embolic, respectively)

This is likely to be associated with: A) Fracture of the temporal bone B) A fall in an elderly patient C) Ehlers Danlos Syndrome D) Malignant hypertension E) Atrial fibrillation

B- would lead to subdural hematoma (crescent-shaped bleed on head CT) from ruptured bridging veins in elderly patients or in shaken baby syndrome. Could lead to mass effect and lethal brain herniation, but would occur later on in timecourse than the higher pressure epidural hematomas. A would lead to middle meningeal artery rupture which would lead to football shaped bleed. Would also get lethal brain herniations if not evacuated surgically in time. C would be likely to form berry aneurysms which could rupture to give a subarachnoid hemorrhage (star sign on head CT) D and E would lead to strokes (hemorrhagic and embolic, respectively)

This is likely to be associated with: A) Fracture of the temporal bone B) A fall in an elderly patient C) Ehlers Danlos Syndrome D) Malignant hypertension E) Atrial fibrillation

C- This is subarachnoid hemorrhage which happens most often from ruptured berry aneurysms (think PCKD, Ehlers Danlos, Marfan's). Acute complications if you survive SAH include Cerebral vasospasm after 2-3 days (give nimodipine, a CCB), and seizure after 2-3 weeks (prophylaxis/treatment with valproate or leviteracetam) A would lead to middle meningeal artery rupture which would lead to football shaped bleed. Would also get lethal brain herniations if not evacuated surgically in time. B would lead to subdural hematoma from ruptured bridging veins in elderly patients C would be likely to form berry aneurysms which could rupture to give a subarachnoid hemorrhage (star sign on head CT) D and E would lead to strokes (hemorrhagic and embolic, respectively)

This is likely to be associated with: A) Fracture of the temporal bone B) A fall in an elderly patient C) Ehlers Danlos Syndrome D) Malignant hypertension E) Atrial fibrillation

TRICKED YOU!!! You can't tell what disease this is without more information like histopathology or history. All you know is that there's diffuse cortical atrophy!!

This patient had: A) Alzheimer's Disease B) Lewy body dementia C) Chronic Traumatic Encephalopathy (CTE) D) Vascular Dementia E) Pick Disease

A- this is primary CNS lymphoma, a subtype of diffuse large B cell lymphoma. It's CD20+ and caused by EBV, most common in the immunosuppressed. IT IS NOT S100 POSITIVE, THIS DESCRIBES A SCHWANNOMA

This tumor is likely to be all of the following EXCEPT: A) S100 positive B) CD20 positive C) Infected with Epstein-Barr Virus D) Most common in the immunosuppressed E) Visible on MRI with a single mildly ring-enhancing lesion

D- this is a meningioma which shows a "whorled" appearance to the tumor cells. It's benign (typically grade 1), an extra-axial tumor, derived from meningeal cells (not glial and usually from arachnoid mater), and also commonly seen with Psammoma bodies on histo as well! E is wrong, describes Glioblastoma Multiforme

This tumor is likely to be: A) Malignant B) Derived from glial cells C) Intra-axial D) Associated with psammoma bodies on histology E) Associated with pseudopalisading necrosis on histology

D- this is a meningioma, a BENIGN tumor of the meninges (usually arachnoid mater) and the MOST COMMON non-glial tumor. On histo, you'll see a whorled appearance to cells and can see psammoma bodies of calcification too. Pseudopalisading necrosis on histo describes GLIOBLASTOMA

This tumor is likely to be: A) Malignant B) Derived from glial cells C) Intra-axial D) Associated with psammoma bodies on histology E) Associated with pseudopalisading necrosis on histology

C is correct. This is medulloblastoma, a highly malignant proliferation of embryological neural ectodermal tissue. It classically arises in the cerebellum (in the granular cells) and is typically a high grade (usually grade 4) tumor, found in children (not adults). Though it arises in the cerebellum, it can spread via CSF/ventricular system to cortex and spinal cord, and is the only primary brain tumor that can spread outside the CNS (to bone and lymph nodes)

This tumor is likely to be: A) Contained to the cerebral cortex B) Benign C) Derived from embryological tissue D) A grade 1 tumor E) Found in the elderly

D- this is Glioblastoma Multiforme which pseudopalisading necrosis. It's derived from astrocytes (not embryological tissue like medulloblastoma), poorly differentiated aggressive cells, and OFTEN CROSSES OVER THE CORPUS CALLOSUM TO INVOLVE BOTH SIDES OF THE BRAIN! This is why it's often called a "butterfly" glioma Note: There's often also a proliferation of endothelial cells in glioblastoma, creating an abnormal BBB and highly permeable increased vascularity

This tumor is most likely: A) located in only one hemisphere of the brain B) Derived from remnant embryological tissue C) Well Differentiated D) Highly necrotic and cellular

C- this is a pilocytic astrocytoma which is the MOST COMMON brain tumor in kids. It's benign. On imaging, see that cyst (piloCYSTIC) with mural nodule against the wall. A/w pink ROSENTHAL fibers on histo A- this describes craniopharyngioma which would likely present with bitemporal hemianopsia and show a mass in the PITUITARY, not the cerebellum B- This describes medulloblastoma, which is less likely overall than a pilocytic astrocytoma D- this describes an Oligodendroglioma which usually presents with seizures and the "fried eggs" on histo E- This describes meningioma

This tumor was found in a child. It is likely to: A) Be derived from remnants of remnants of Rathke's pouch B) Be derived from granular cells of the cerebellum C) Show Rosenthal fibers on histology D) Show "Fried Egg" cells on histology E) Show a whorled apparance with psammoma bodies on histology

D- This is a pilocytic astrocytoma (benign proliferation of astrocytes) which is MOST COMMON CNS TUMOR IN CHILDREN!!! It's usually found in the cerebellum with a cystic lesion (piloCYSTIC) with a mural nodule growing on its wall. On histo, those pink things you see are ROSETHAL fibers (high yield) and these tumors are GFAP+!! A- WRONG, remember that MOST PEDIATRIC BRAIN TUMORS ARE INFRATENTORIAL!!! B- wrong, this describes medulloblastoma C- wrong, pilocytic astrocytomas are benign! E- wrong, this describes a Schwannoma which often causes 8th nerve palsy and presents with unilateral hearing loss

This tumor was found in a child. It is likely to: A) Be found in the cerebral cortex B) Be derived from granular cells of the cerebellum C) Be malignant D) Be GFAP positive E) Be S100 positive

MYASTHENIA GRAVIS!

Thymoma. What neurological disorder?

A- Myasthenia often has underllying THYMUS abnormalities (like thymoma with loss of immune tolerance leading to sensitization against the nicotinic NMJ ACH receptor). Thymectomy can improve symptoms of Myasthenia!

Thyroid/Thymus abnormalities are common in A) Myasthenia Gravis B) Lambert Eaton C) Neither D) Both

Segmental spinal cord injury from acute inflammation. Etiology unclear (might be MS/viral/autoimmune from RA/SLE). Almost always in thoracic cord affecting 1-2 segments. Treatment=acute steroids (to make symptoms better quicker), usually get complete resolution of disease

Transverse Myelitis?

FALSE, triptans are serotoning agonists that induce vasoconstriction!

True/false: Serotonin antagonists are used as first line treatment to treat migraines?

Unilateral- think Parkinson's itself Bilateral- think drugs/iatrogenic cause! Drugs that can cause parkinsonism- dopamine receptor blockers, anti-emetics (prochlorperazine, promethazine, metoclopramide), Lithium, Valproate, Ca++ channel blockers, cholinergics, chemotherapeutics

Unilateral vs bilateral symptoms of parkinsonism should make you think of what?

Vascular dementia is ischemia caused by multiple strokes or diffuse small vessel ischemia (microvascular disease from lipohyalinosis/HTN, vasculitis, or atherosclerosis). Dementia occurs during this cerebral global ischemia because the pyramidal cells of cortex layers 3, 5, and 6 are highly vulnerable to ischemia!

Vascular dementia?

When ALCOHOLISM or starvation leads to THIAMINE DEFICIENCY!! Without thiamine, ATP production goes down so tissues that need energy like the heart/brain are affected first and HIT HARD!! This is why you give thiamine before glucose. Classic triad= Confusion, ophthalmoplegia (from CN3 damage, may present as double vision), and ataxia In brain, Thalamus and mammillary bodies get hit first. If it affects the heart, it can become WET BERIBERI causing high output HEART FAILURE (dilated cardiomyopathy)!!

Wernicke's Encephalopathy?

Causes- 1) Low perfusion to entire brain (atherosclerosis for example in internal carotids) 2) Acute decrease in blood flow (like in shock/MI) 3) Anemia=chronic hypoxia 4) REPEATED EPISODES OF HYPOGLYCEMIA (high yield) like from an insulinoma Moderate global ischemia infarcts the WATERSHED AREAS and also highly vulnerable areas like pyramidal neurons of cortex (layers 3, 5, 6). Creates a line of necrosis called CORTICAL LAMINAR necrosis

What are 4 causes of global cerebral ischemia and what are the sequellae?

"Suicide headaches" that typically occur in MALES, AT NIGHT, lasting 15 min-3 hours. MUST HAVE AUTONOMIC FEATURES PRESENT (like pupil dilation, runny nose/teary eyes). VERY RARE in real life (but common on boards) They tend to occur in clusters of about a week on/off, then remit for months, then come back

What are Cluster Headaches?

1) Multi-systems atrophy (MSA) with earlier onset, has more predominant autonomic features (like horrible orthostatic hypotension, horrible constipation) and often cerebellar dysfuntion (ataxia/loss of balance). Imaging shows "Hot Cross Buns" sign of the pons from DEGENERATION OF THE CEREBELLOPONTINE TRACTS 2) Progressive supranuclear palsy (PSP) with supranuclear gaze palsy (can't look up from damage to the superior collicul vertical gaze center), they fall all the time and have frontal release figns (feel sad but laugh for example). Imaging reveals "hummingbird sign" with atrophy of the midbrain with PRESERVATION OF THE PONS' BIG FAT BELLY!

What are Multi-Systems Atrophy and Progressive Supranuclear Palsy?

Optic nerve involvement=blurred vision in ONE eye (monocular), If brainstem is involved they'll have vertigo and slurring speech (sounds like they're drunk. If the MLF is involved in the brainstem, you get INO!!! Symptoms develop symptoms gradually over days, improve over days to weeks, then RELAPSES can occur with symptoms MOST COMMON IN REAL LIFE=Sensory loss in limbs

What are common clinical presentations of MS (for boards purposes)?

Myelin ovoids are seen in AXONAL DEGENERATION in which myelin remnants are taken up by phagocytes.

What are myelin ovoids and when might you see them?

A partial seizure involves only one discrete part of the brain (unlike generalized seizures that involve all parts of the brain). Simple partial seizure=NO alteration of consciousness, maybe involuntary muscle contractions or altered sensations Complex partial seizure=ONE discrete part of brain PLUS alteration of consciousness

What are partial seizures? How are they further classified?

Optic neuritis (like vasculitis, inflammatory, genetic), myelitis/myelopathy (nutritional causes, ischemic/vascular causes, infectious causes), Brainstem syndromes (like stroke, tumor, vasculitis), Cerebral white matter lesions (like small vessel disease from HTN/Hyperlipidemia, vasculitis from SLE/Sjogren's/Behcet's, Sarcoidosis)

What are some other diseases on the differential with MS that should be ruled out?

Fever (children get febrile seizures) Lack of sleep Trauma Drugs/alchohol Hypoglycemia Tumors (REMEMBER THIS LINK) Strokes Infections like meningitis/encephalitis Epilepsy=multiple unprovoked seizures

What are some things that can provoke seizures?

MOST COMMON=Group B strep (intrapartum transmission, can be prevented with intra-partum ampicillin), which is CAMP TEST POSITIVE!!!!! 2nd MOST COMMON=E. coli which has the K1 capsular antigen that INHIBITS COMPLEMENT AND EVADES THE IMMUNE SYSTEM! E. coli grows on Blood agar, MacConkey agar (ferments lactose), and eosin methylene blue agar (ALL HIGH YIELD)

What are the 2 most common causes of neonatal meningitis? Describe some distinguishing features of both?

1) Cytotoxic edema: Caused by lack of sufficient energy (ATP) to maintain ion pumps, happens in hypoxic-ischemia, stroke, severe hypoglycemia, and some toxins 2) Vasogenic edema: Caused by abnormal increases in permeability of the blood brain barrier which lets fluid enter the extracellular space. Occurs in brain tumors (with neovascularization), abscesses, inflammatory conditions (like MS), and in the later stages of stroke 3) Interstitial edema: Develops when there's abrupt obstruction to flow of CSF in brain (like in hydrocephalus). CSF under excessive pressure moves from ventricles between gaps in ependyma into the ECF of the white matter Brain edema is BAD because can cause increased ICP and herniations of the brain like transtentorial or tonsillar herniation, which can both be fatal.

What are the 3 types of cerebral edema? Why is cerebral edema bad?

NOTE THAT ALZHEIMER'S IS A CLINICAL DIAGNOSIS!! CSF will show low AB42 and increased total tau

What are the CSF findings consistent with Alzheimer's?

Most common=trauma Second most common=drugs/alcohol

What are the most common etiology of seizures (without prior history) in young adults in their 20s?

Contraindications: Space-occupying lesions (concern for herniation) so get a CT/MRI if you can, Bleeding/clotting disorders (get platelet count/coagulation studies before LP), infection/abscess at site of injection Potential complications: Post-LP headaches (CSF-hypotensive headaches), RARE potential complications include bleeding, infection, brain herniation

What are the potential complications and contraindications of a Lumbar Puncture?

MS (top of differential) and lyme (second on differential)

What are the two most likely culprits for diagnosis when CSF shows increased BANDS?

looks like viral meningitis but with MORE BLOOD! Glucose NORMAL, WBCs high (mostly lymphs), HIGH RBCs because herpes encephalitis likes to go to the temporal lobes and cause hemorrhage, leading to BLOOD in the CSF!!!

What are the typical CSF findings for Herpes encephalitis?

1) Lead (check for lead lines) 2) Reye Syndrome- kids who get aspirin and have a viral infection can get Reye Syndrome which consists of ENCEPHALOPATHY! It's fatal!

What are two main causes of delirium in children?

Hyposmia (loss of smell) and REM sleep behavior disorder are two of the earliest things!

What are two of the earliest symptoms of Parkinson's (in the pre-motor stage)

Hippocampus, which is why memory is lost early on in Alzheimer's!

What area of the CNS is typically the first one affected in Alzheimer's disease?

Epigastric "rising" sensation, sweating, piloerection (skin stands up), pupillary changes (particularly dilation)

What autonomic symptoms/signs are common in seizures?

Use the Iliac Crest as a landmark for the L4/L5 vertebral level (safely below the level of the conus medullaris where the spinal cord ends, and where the cauda equina is). C- you do NOT pierce the pia in a lumbar puncture (high yield), the CSF is in the SUBARACHNOID SPACE!

What bony landmark is used for lumbar punctures? Which of the following is NOT pierced during an LP? A) Dura mater B) Arachnoid mater C) Pia mater

This is ALS, you can see the CST (UMN TRACT) which has atrophied as the demyelinated sections on this myelin stained spinal cord section

What disesase is this and what's going on here?

Blue=Meningitis/encephalitis Black=traumatic tap (will get progressively less blood as time/drainage progresses), Subarachnoid hemorrhage (will stay consistently bloody or Xanthrochromic with each tube) Red=CNS infections, tumors, demyelinating disorders (like MS), or traumatic tap Yellow=Meningitis (most often bacterial with VERY low glucose counts), but NOT viral/aseptic meningitis (glucose would be normal) Green=Demyelinating disorders (like MS), viral encephalitis, HIV, lyme. Purple- with BANDS, think MS first!!!! Also could be Lyme (think second)!!

What do you think about in each of the following colored boxes?

Increased protein in CSF (>50 mg/dL protein) is a sensitive but not specific sign that reflects breakdown in the BBB b/c protein isn't supposed to leak into CSF normally!

What does increased protein in CSF mean?

Oligoclonal bands (unique ones in your CSF compared to your serum) are highly suggestive of MS (high sensitivity) though other CNS infections can cause oligoclonal bands in CSF too (Lyme, HIV, syphilis)

What does it mean to have oligoclonal bands in your CSF? What disease state should this make you think of?

This is papilledema (swelling of optic disk from increased ICP). DO NOT give these patients a lumbar puncture, could cause lethal brain herniation through foramen magnum by altering pressure gradients outside the skull!

What does this mean? Should this patient get a lumbar puncture?

Temozolomide which is an alkylating agent that readily crosses the BBB with relatively low toxicity

What drug is first-line for a high grade glioblastoma?

APOE4 allele carries increased risk of ALZ, while APOE2 allele carries DECREASED risk of ALZ!

What genetic factors can influence someone's risk of developing SPORADIC (not familial) Alzheimer's?

Charcot-Marie-Tooth disease is a VERY RARE autosomal dominant condition with defective production of proteins a/w myelin or nerve structure in PERIPHERAL nerves. A/w foot deformities as seen here, foot drop from PERONEAL nerve neuropathy, claw hand, FOOT DEFORMITIES (like seen here), skinny legs from muscle wasting due to peripheral demyelination, and clumsiness/falls NOT FROM CEREBELLAR ATAXIA Typically presents in late childhood/adolescence

What is Charcot-Marie-Tooth disease?

Lysosomal storage disease, most common type of leukodystrophy, 3 types that can present at different ages. Genetic defect- AUTOSOMAL RECESSIVE mutation in arylsulfatase which causes buildup of sulfatides that leads to impaired myelin production/breakdown, DEMYELINATING DISEASE that presents with central AND peripheral demyelination with ATAXIA AND DEMENTIA

What is Metachromatic Leukodystrophy?

PML is caused by JC virus, a naked dsDNA virus with a circular genome. It's a demyelinating disorder that typically presents with symptoms like weakness, visual changes, difficulty with speech, and is progressive over the course of weeks. IT ONLY AFFECTS THE IMMUNOSUPPRESSED! Either AIDS patients with CD4<200 or patients taking immunosuppressants (like MS patients on Natalizumab) On imaging, appears with multiple NON-ENHANCING LESIONS throughout the brain. Note- almost 50% of population is infected with JC virus, but doesn't cause symptoms or PML until you become immunosuppressed!

What is Progressive Multifocal Leukoencephalopathy and what causes it? How does it appear on imaging and how does it present?

A SERIOUS, LIFE-THREATENING condition where a seizure either doesn't stop after 30 minutes or it recurs less than 30 minutes after the first one. It's a medical emergency because it can lead to arrhythmias, lactic acidosis, hypertension, and more!

What is Status Epilepticus?

Demyelination of the posterior columns/roots of the spinal cord in TERTIARY SYPHILIS (accompanied by Argyll-Robertson Pupils that accommodate but DO NOT REACT to light) Findings=Loss of proprioception with POSITIVE ROMBERG sign, Loss of vibration sense, Loss of discriminative touch with ABSENT DEEP TENDON REFLEXES! Patient also has pain all over and odd gait from loss of proprioception

What is Tabes Dorsalis?

In alcoholics withdrawing, usually happens 2-4 days AFTER their last drink. Pink elephants are the classic story for hallucinations. DANGEROUS!! Because of weird autonomic functioning (can lead to tachycardia...) or STATUS EPILEPTICUS WHICH IS FATAL IF UNTREATED WITH BENZOS!

What is delirium tremens?

Multiple unprovoked seizures

What is epilepsy?

3 Hz spike wave on EEG!!! WILL BE ON BOARDS. Treatment=Ethosuximide (it SUCKS to have absence seizures)

What is pathognomonic for Absence seizures? What is the treatment?

Loss of dopaminergic neurons in the substantia nigra compacta leads to INCREASED activity of the indirect pathway and DECREASED activity of the direct pathway leading to HYPOKINETIC movement disorder.

What is the pathogenesis of Parkinson's?

Apnea (not breathing), nystagmus, pupils are midline and reactive

What is the toxidrome associated with Sedatives/hypnotics?

fever, tachycardia, flushing, mydriasis (think atropine)

What is the toxidrome associated with anticholinergics?

Hypothermia, bradycardia, apnea, pinpoint pupils

What is the toxidrome associated with opiates?

This is a Lewy body with Alpha synuclein aggregating to form the pink blob and pushing aside the pigmented neuromelanin (brown stuff around the pink blob). Lewy bodies are seen in BOTH Parkinson's and Lewy body dementia. This particular image is probably from the SNc, where neuromelanin is located.

What is this and what disease(s) might it be seen in?

Blue=Bacterial (HUGE increase in WBCs, HUGE decrease in glucose relative to serum, >90% polys!) Orange=Viral/Aseptic (increased WBCs but <1000 unlike bacteria, lymphocytic predominance, NORMAL GLUCOSE!!!) Green=Fungal/AFB/Granulomatous (Increased WBCs but not >1000 like bacterial, low glucose but not bacterial low, and LYMPHOCYTIC PREDOMINANCE!!)

What kinds of etiologies fit into each box?

Cell count (WBC/RBC count, <5 for each is normal), and chemistry (glucose/protein levels). Then specific things to look for based on what you suspect (bands for MS/lyme? Myelin basic protein for demyelinating diseases? IgG or albumin?) Could smear and gram stain/fungal stain/AFB stain or culture it. Could do cytology too to look for leukemia/lymphoma/metastasis though you need a TON of CSF to do this.

What labs are ALWAYS obtained from a CSF sample? What are some tests that might be added in and what are they looking for?

Anticholinergics (like Diphenhydramine AKA Benadryl), Opioids, Benzos, Beta blockers, Digoxin... PRETTY MUCH ALL OF THEM!!

What meds can predispose someone for a delirious state?

Mannitol (a diuretic that pulls fluid out of brain and into the toilet) and hypertonic saline- pulls fluid out of cells and into ECF

What meds can treat increased intracranial pressure?

For TBI, physical exam might include checking level of consciousness and using Glasgow Coma Scale (GCS), Cranial nerve exam to evaluate brainstem/CN function, checking behind the ears for Hemotympanum (blood which could indicate temporal bone fracture and epidural hematoma), Raccoon eyes, "Battle Sign" (bleeding over the mastoid process)

What physical exam components are important for evaluation of a patient with traumatic brain injury?

Ciliary body epithelium produces, trabecular meshwork/canal of Schlemm at iridocorneal angle drain it through nasolacrimal duct

What produces aqueous humor? What drains it?

This is a neuritic plaque seen in Alzheimer's disease. PLAQUES AND TANGLES!! 1) AB amyloid plaques (the pink extracellular proteinaceous material) that entrap neuritic processes 2) Neurofibrillary tangles formed by misfolded Tau from hyperphosphorylation

What two key features are seen on histopathology of this neurodegenerative cause of dementia?

1) Huntington's (onset typically in 20s-30s 2) Acute Rheumatic Fever (the S in JONES!)

What two things cause chorea?

Extra-axial tumors are much better to treat surgically because you don't need to remove brain parenchyma. Intra-axial are more difficult, but can be done for well-circumscribed tumors like metastases/low grade gliomas

What type of brain tumor can be treated surgically?

3 common presentations 1) Optic neuritis with loss of vision and pain in one eye 2) MLF (can't move one eye past midline 3) Bladder dysfunction (spastic bladder/overflow incontinence treated by catheter or pharm with bethanechol) or bowel/sexual dysfunction from autonomic demyelination

What would be a common presentation of Multiple Sclerosis for a boards question?

Watershed infarcts from global ischemia (that might classically be seen post-MI for example from hypoperfusion) can lead to infarction of the ACA/MCA border zone which causes BILATERAL paralysis of the shoulder, trunk, and thigh (think about how the humunculus is oriented) but NOT the face, arm/hand, or lower leg and feet!!

What's "Man in the Barrel" syndrome and name a scenario when it might develop?

Problem with both Adrenal gland AND WHITE MATTER. Impaired addition of Coenzyme A to long-chain fatty acids, so fatty acids accumulate which damages adrenals AND white matter!

What's Adrenoleukodystrophy?

A reaction of neuronal cell bodies to axonal injury. You see increased volume/pinkness of cell bodies with Nissl substance pushed to the periphery. It's a sign of increased production of new protein for axonal sprouting/regeneration! You'll also see death of the axon DISTAL to the site of injury (AKA Wallerian Degeneration)

What's Central Chromatolysis?

Alzheimer's patients can get CEREBRAL AMYLOID ANGIOPATHY which are amyloid deposits around the blood vessels in the brain which weakens the vessel walls increasing the risk of hemorrhage, HIGH YIELD ASSOCIATION!

What's Cerebral Amyloid Angiopathy and in what disease might you see it?

Edrophonium is an ACHE inhibitor which is used to test for clinical improvement in Myasthenia Gravis patients. This is the TENSILON TEST.

What's Edrophonium and what is it used to test for?

AUTOSOMAL RECESSIVE mutation in galactocerebrosidase enzyme, deficiency causes buildup of galactocerebrosides that destroys myelin. Presentation=INFANTS WITH MICROCEPHALY, also absent reflexes, developmental delay, and progressive motor/sensory problems

What's Krabbe's Disease?

Preceded by muscle jerking from seizures originating in frontal (motor) cortex)

What's a Jacksonian Seizure?

Atypical Parkinson's is like Parkinson's, but with RED FLAGS such as LACKING A TREMOR! Boards LOVE these. 3 diseases are 1) Multi-systems atrophy (MSA) with earlier onset, has more predominant autonomic features (like horrible orthostatic hypotension, horrible constipation) and often cerebellar dysfuntion (ataxia/loss of balance). Imaging shows "Hot Cross Buns" sign of the pons from DEGENERATION OF THE CEREBELLOPONTINE TRACTS 2) Progressive supranuclear palsy (PSP) with supranuclear gaze palsy (can't look up from damage to the superior collicul vertical gaze center), they fall all the time and have frontal release figns (feel sad but laugh for example). Imaging reveals "hummingbird sign" with atrophy of the midbrain with PRESERVATION OF THE PONS' BIG FAT BELLY! 3) Corticobasal deneration- Unilateral focal rigiditiy, apraxia, aphagia

What's atypical Parkinson's? What are the 3 diseases??

Anterior Ischemic Optic Neuropathy (AION) which is a lacunar stroke of the optic nerve head (>50 yo patients). It's acute/subacute PAINLESS monocular vision loss with hyperemic disc swelling seen here (papilledema). Two types of causes: arteritic (temporal arteritis) and non-arteritic

What's going on here?

Central retinal artery occlusion (from Temporal arteritis or embolism causing occlusion), ISCHEMIA of retina causing white appearance with a macular "cherry red spot". It's acute and painless monocular vision loss. May be preced by amaurosis fugax (transient monocular blindness)

What's going on here?

Spinal stenosis is compression of the spinal cord by usually disc herniation, fracture, or long-standing stenosis (most common causes). Two types: 1) Central stenosis where something is pushing in to compress the SC centrally 2) Foraminal stenosis where you just compress ONE NERVE ROOT ON ONE SIDE for unilateral symptoms (from narrowing of a foramen)

What's spinal stenosis? What are the two types?

Strep pneumo! Gram positive lancet-shaped diplococci! MOST COMMON CAUSE! MOPS mnemonic!

What's the #1 cause of bacterial meningitis?

Genetic: HLA-DR2 is biggest genetic association, and LATITUDE AWAY FROM THE EQUATOR is a/w MS (probably because lower vitamin D) and EBV is biggest infectious cause

What's the biggest genetic risk factor and biggest environmental risk factor for Multiple Sclerosis? What about infectious?

Fever, stiff neck (AKA Meningismus), and altered mental status (lethargy/obtundation/coma)

What's the classic triad of acute bacterial meninigitis?

Triad=fever, headache, meningismus (AKA neck stiffness from the pain of meningeal inflammation) Viral/aseptic meningitis is the etiology most likelyt to present with photophobia.

What's the classic triad of symptoms for meningitis? What etiology is most likely to present with photophobia?

Mnemonic=TRAP T=Tremor (resting, pill-rolling, better with intentional movements) R=Rigidity (spasticity) A=Akinesia/Bradykinesia (with expressionless face) P=Postural instability/shuffling gait

What's the clinical presentation of Parkinson's?

Lewy body dementia has dementia diagnosed EARLY in the disease course (within a year of diagnosis) Parkinson's has LATE DEMENTIA with lewy bodies forming and hypokinetic state developing way before dementia kicks in

What's the difference between Lewy body dementia and Parkinson's?

Ophthalmic artery

What's the first artery to branch off the internal carotid?

Lorazepam (or any benzo)

What's the first line treatment for status epilepticus?

Metastases from lung cancer (50% of total brain tumors) and breast cancer (20% of total brain tumors) SECONDARY (metastatic) more common than PRIMARY brain tumors!

What's the most common cause of malignancy in the brain?

Alzheimer's

What's the most common neurodegenerative cause of dementia?

Brain abscesses, also capable of forming a dense fibrous scar (while other processes form a glial scar with liquefying necrosis)

What's the only pathologic process that can form granulation tissue in the brain?

E- this "wing-beating" tremor is CLASSIC for Wilson's disease, a rare AR genetic condition from a mutation in the ATP7B gene. Wilson's is a disorder of inadequate hepatic copper excretion. Diagnostic findings=decrease in ceruloplasmin (copper is added to aceruloplasmin to make ceruloplasmin, and impaired transport makes this impossible), so copper builds up in the cornea (corneal rings), cirrhosis, depression, BASAL GANGLIA DEGENERATION (seen here with the CLASSIC WING-BEATING TREMOR WHICH BOARDS LOVE), and Parkinsonian symptoms at an early age (20s-30s). Treatment is with penicillamine and supplemental zing (to chelate and prevent uptake of dietary copper, respectively) A is wrong, describes hemiballism which is UNILATERAL B) This describes essential tremor, which is typically an intention tremor, not wing-beating C) This describes Parkinson's, which typically manifests in 60s/70s and doesn't have wing-beating D) This is Huntington's, which would have chorea and athetosis but NOT WING-BEATING TREMOR. Note that the patient's age does fit with Huntington's tho!

When a 24 year old patient is asked to clasp his hands at chest height with his elbows out, his elbows repeatedly beat up and down. Further examination is likely to reveal A) A prior infarction of the subthalamic nucleus from a Lacunar stroke B) Improvement of symptoms with either alcohol, Propranolol, or Primidone C) A resting pill-rolling tremor, 4-6 hz and Lewy bodies in the Substantia Nigra pars Compacta D) Degeneration of the caudate nucleus on MRI E) Decreased levels of Ceruloplasmin

After lumbar puncture (after 30% of them!), usually 48 hours after them.

When do orthostatic headaches classically develop?

TRICK QUESTION! PRIMARY BRAIN TUMORS DO NOT TYPICALLY METASTASIZE OUTSIDE THE CNS!!

Where is the most common site of metastasis of primary brain tumors?

1) Pinpoint=Pons 2) bilateral dilated=midbrain (both CN3) 3) Only one CN3

Where must a lesion be to see 1) Bilateral pinpoint pupils 2) Bilateral dilated pupils 3) Unilateral dilated pupil

Adults: typically supratentorial Children: mostly neuroepithelial tumors infratentorially

Where to children/adults tend to develop brain tumors anatomically?

Pyramidal cells in the hippocampus (loses memory), and purkinje cells of the cerebellum (leads to ataxia)

Which cell types in the brain are MOST susceptible to hypoxic damage?

YOU FOOL!!! You don't get focal neural deficits early on in Alzheimer's!

Which focal neural deficits are commonly seen early in Alzheimer's disease course?

EPSTEIN-BARR VIRUS (EBV). We could prevent a LOT of MS cases if we had an EBV vaccine!

Which infectious agent is associated with Multiple Sclerosis?

Peripheral vision goes first in glaucoma.

Which is lost first in glaucoma: Peripheral or central vision?

E- Metoclopromide is used to BREAK acute seizures, NOT to prevent like TCAs, topiramate, valproate, and propranolol

Which of the following is NOT used as seizure prophylaxis: A) Propranolol B) Topiramate C) Valproate D) TCAs E) Metoclopromide

A- ENDONEURIUM (containing Schwann cells) SURROUNDS EACH INDIVIDUAL AXON and is affected in Guillain-Barre Syndrome, and ASCENDING paralysis!

Which of the following is affected in Guillain-Barre Syndrome: A) Endoneurium B) Perineurium C) Epineurium D) Fasciculus E) Axons

A- nothing else would do both!!

Which of the following is most likely to be associated with papilledema and left hemiparesis? A) Brain tumor B) CSF hypotension syndrome C) Migraine D) Pseudotumor cerebri E) Temporal arteritis

C- Lamotrigine is broad spectrum and least teratogenic (less than topiramate, MUCH less than Valproate/Phenytoin) and Ethosuximide wouldn't work for anything that's not an Absence seizure! Remember Lamotrigine carries a risk for Stevens-Johnson syndrome, which is why you start with low dose and gradually increase

Which of the following meds would be safest and most beneficial for a woman who plans to become pregnant with Mesial Temporal Sclerosis? A) Topiramate B) Valproate C) Lamotrigine D) Phenytoin E) Ethosuximide

C- Medulloblastoma is derived from granular cells of the cerebellum which are neural ectodermal tissue (boards love asking about this). Classically arises in the cerebellum in CHILDREN. It has a poor prognosis (though 50% 5 year survival), grows rapidly, and enters into the 4th ventricular space to spread via CSF to spinal cord (called a drop metastasis) Histologically, it's possible you'll see Homer-Wright rosetttes.

Which of the following tumors are derived from embryological tissue: A) Glioblastoma multiforme B) Meningioma C) Medulloblastoma D) Schwannoma E) Astrocytoma

E- while A, B, and C are all currently used MS treatments, corticosteroids are best to resolve the symptoms of acute flares because of their ANTI-INFLAMMATORY PROPERTIES!! Recall that MS is an autoimmune type 4 HSR against oligodendrocytes

Which of the following would be best to resolve the symptoms of an acute MS flare? A) Interferon Beta B) Natalizumab C) Dimethyl fumarate D) Levetiracetam E) Dexamethasone

B- Complex partial seizures have post-ictal confusion, Absence seizures DO NOT!

Which of the following would have Post-Ictal Confusion? A) Absence seizures B) Complex partial seizure C) Neither D) Both

D- meningiomas are typically benign (grade 1) and don't infiltrate into surrounding tissues

Which one of the following brain tumors has a circumscribed, non-infiltrative pattern of growth? A) Glioblastoma B) Medulloblastoma C) Meningial carcinomatosis D) Meningioma E) Primary CNS lymphoma

B- remember that medulloblastoma arises in the cerebellum from neuroectodermal PRECURSOR cells

Which one of the following tumors may originate from neuroepithelial cells located in the roof of the 4th ventricle? A) Glioblastoma B) Medulloblastoma C) Meningioma D) Low-grade astrocytoma E) Schwannoma

They can get CEREBRAL AMYLOID ANGIOPATHY which are amyloid deposits around the blood vessels in the brain which weakens the vessel walls increasing the risk of hemorrhage, HIGH YIELD ASSOCIATION!

Why are Alzheimer's patients at increased risk of hemorrhage?

FATIGUE IS A HUGE AND COMMON PROBLEM FOR MS PATIENTS. After a hot shower, there's less nerve conduction, so less activity exacerbates their symptoms like fatigue!

Why do MS patients feel fatigued after a hot shower?

Leviteracetam (Keppra) is the MOST COMMONLY PRESCRIBED anti-epileptic medication, because it's so broad spectrum and has no real contraindications (including pregnancy!) MofA: Decreases glutamate release into synapse by binding to SV2A protein on synaptic vesicles.

Why is Leviteratam called "Vitamin K"?

A) Subarachnoid hemorrhage B) post-exertional headache C) Carotid dissection (can also present with Horner's, referred pain to neck)

Worst headache of your life PLUS A) Loss of consciousness B) Happens after sex C) Happened after chiropractic neck manipulation/yoga EQUALS WHAT?

It's actually DEXAMETHASONE! B/c the inflammatory effects in meningitis are harmful, you need to decrease swelling in the brain! This is why dexamethasone (a glucocorticoid, anti-inflammatory like cortisol) reduces mortality in bacterial meningitis. Then proceed swiftly to antibiotics like ceftriaxone +/- vancomycin

You suspect a patient has meningitis. What's the first drug you give?

A- altered vascular permeability in active but not inactive plaques leads to ring-enhancing appearance in active plaques!

____________ are contrast enhancing in Multiple Sclerosis: A) Active plaques B) Inactive plaques C) Neither D) Both