Divine Intervention: All Neurology

• Brain tumor that causes loss of “vertical gaze” → pinealoma (causes Parinaud’s syndrome)

  • Pathophys? Compression of superior colliculus, which is the vertical conjugate gaze center

• 13 mo child with history of hypopigmented macules + seizures. → tuberous sclerosis

  • Hypopigmented macules = ash leaf spots

  • Associated seizure syndrome? West syndrome (infantile spasms)

    • EEG finding? Hypsarrhythmia

    • Tx? ACTH or vigabatrin

• Most common primary brain tumor in kids → pilocytic astrocytoma

  • Histology? Rosenthal fibers

  • Marker? GFAP (glial cell marker)

• Medulloblastoma 

  • Location? Cerebellar vermis

  • Histology? Homer-Wright rosettes

• Brain tumor presenting as hydrocephalus → ependymoma

  • Histology? Perivascular pseudorosettes or ependymal rosettes

• Brain tumor causing visual problems in kid → craniopharyngiomas 

  • Derived from? Rathke’s pouch

  • Imaging? Calcified suprasellar mass

  • Complications?

    • Bitemporal hemianopsia (2/2 optic chiasm compression)

    • Anterior pituitary hormone deficiencies (2/2 pituitary stalk/gland compression)

• MC cause of brain tumor in adults → metastasis

  • Location? Grey-white junction

  • Imaging? Multiple well-circumscribed lesions

• Most common primary brain tumor in adults → GBM

  • Imaging? Butterfly-shaped mass that crosses corpus callosum + edema + central necrosis

  • Marker? GFAP (glial cell marker)

  • MC brain tumor excluding mets

• Bilateral acoustic neuromas 

  • Pathophys? Schwannoma of CN8

  • Location? Cerebellopontine angle

    • #1 MC tumor at cerebellopontine angle → acoustic neuroma

    • #2 MC tumor at cerebellopontine angle → meningioma

  • Tumor marker? S-100

  • Associated syndrome? NF-2

• Brain tumor that presents as parasagittal mass along falx cerebri → meningioma

  • Histology? Psammoma bodies (“laminated calcifications”)

• 2 yo child with abdominal mass that crosses midline + myoclonus + weird eye movements + calcified mass on imaging → neuroblastoma

  • Alternate location: posterior mediastinum

  • Associated syndrome?

    • NF-1

    • Beckwith-Weideman syndrome 

      • Neuroblastoma / Wilms tumor / hepatoblastoma

      • Hemihypertrophy

      • Macroglossia

      • Enlarged abdominal organs

      • Abdominal wall defects

      • Hypoglycemic seizure in newborn 

        • Pathophys? hyperplasia of beta cells of pancreatic islets

• 2 yo child with abdominal mass that does not cross midline + no calcifications → Wilms tumor

• Frontal lobe tumor → oligodendroglioma

  • Histology? fried egg appearance

• Brain tumor that produces EPO → hemangioma blastoma

  • Associated syndrome? VHL syndrome

  • Treat because they can cause a life-threatening hemorrhage! 

• Recent viral illness + vertigo + tinnitus → labyrinthitis

• Feeling like room is spinning with positional changes + nystagmus with provocative maneuvers → BPPV (caused by otolith displacement)

  • Diagnostic maneuver? Dix-Hallpike

  • Tx? Epley / Semont maneuver 

• Anesthesia over medial thigh + weak thigh adduction → obturator nerve injury

  • Nerve roots? L2-L4

• Anesthesia over lateral thigh → lateral femoral cutaneous nerve injury

• 7 yo kid with ataxia + hypertrophic cardiomyopathy + LE hyporeflexia → Freidrich’s ataxia

  • Pathohpys?

    • GAA repeats → LOF mutation in frataxin gene (iron binding protein) → iron overload causes oxidative damage

      • Destruction of dorsal columns of spinal cord → hyporeflexia

      • Cerebellar destruction → ataxia

  • Inheritance? AR

    • Unlike other trinucleotide repeat disorders, which are AD

  • Repeat? GAA

  • MC cause of death? CHF 2/2 hypertrophic cardiomyopathy

• 35 yo F with a BMI of 35 with visual difficulty and severe intermittent headaches. She takes tetracycline for acne. → idiopathic intracranial hypertension (“pseudotumor cerebri”)

  • Imaging/Procedure Mgmt:

    • 1st step? Fundoscopic exam → shows papilledema

    • 2nd step? Head CT → is normal

    • 3rd step? LP → shows elevated OP

  • Tx?

    • Weight loss

    • Acetazolamide (carbonic anhydrase inhibitor)

    • Serial LPs

    • VP shunt

  • Tx for severe visual difficulties? optic nerve fenestration (relieves pressure)

  • Triggers?

    • Tetracyclines (i.e. female patient getting acne tx)

    • Vit A derivatives

• Management of ischemic stroke (caused by blockage w/i blood vessel)

  • Initial imaging? Non-contrast head CT to DDx ischemic stroke vs. hemorrhagic stroke b/c blood looks just like contrast ☺

  • Next imaging? MRI

  • Time window for tPA administration? 3-4.5 hrs 

    • Up to 6 hrs if you can inject tPA directly into involved vessel

  • HY contraindications to TPA administration

    • Bleeding disorder

    • GI bleeding

    • Recent brain surgery

  • Blood pressure management?

    • Permissive HTN (BP up to 220/120) in patients with ischemic strokes only in pt’s that cannot NOT get TPA

      • Why? Increased BP keeps vessels perfused, which optimizes blood flow to ischemic penumbra of brain

    • If getting tPA, BP < 185/110

    • If hemorrhagic stroke with high BP, can lower BP with labetalol, nitroprusside, nicardipine

• 78 yo has 15 min episode of complete vision in one eye that has since resolved. → amaurosis fugax (TIA)

  • Classic presentation? Painless loss of vision in one eye, “curtain coming down”

  • Dx?

    • Non-contrast head CT

    • Brain MRI

    • Echo (to look for origin of clot)

    • Carotid US

  • NBSIM? Probably carotid US on shelf exam

  • Prevention of further episodes?

    • Aspirin

    • Aspirin + dipyridamole 

    • If aspirin contraindicated, clopidogrel 

  • Indications for carotid endo

    • >70% stenosis 

    • Symptomatic 

  • #1 modifiable RF for a stroke? HTN

• 6 yo M with difficulty walking + uses arms to “walk up” his legs/thighs (Gower’s sign) + hypertrophy of calves bilaterally. → Duchenne muscular dystrophy

  • Gene? DMD

  • Mutated protein? Dystrophin (stabilizes sarcolemma to cytoskeleton)

  • Inheritance? X-linked recessive

  • Dx?

    • Increased creatine kinase levels

    • Genetic testing for dystrophin gene mutation

  • Tx?

    • Steroids

    • High-calorie nutrition

  • MC cause of death? Respiratory failure or cardiac causes

• Muscular dystrophy + intelligence + life expectancy in the 50s → Becker muscular dystrophy

  • Pathophys? Have some functional dystrophin

  • Gene? DMD

  • Mutated protein? Dystrophin

  • Inheritance? X-linked recessive

• Ataxia + confusion + ophthalmoplegia → Wernicke encephalopathy

  • Reversible 

  • Imaging? Hemorrhagic infarct of mamillary bodies

  • Pathophys? Vitamin B1 deficiency 

  • Function of which enzyme affected? Transketolase in pentose phosphate pathway

    • Decreased transketolase activity

  • Tx? IV thiamine then glucose

  • Note: “ophthalmoplegia” means eye problem; pt’s with Wernicke-Korsakoff can have any eye problem, e.g. nystagmus, lateral gaze palsy, etc.

• Complication of untreated Wernicke encephalopathy? Korsakoff syndrome

  • Irreversible

  • Sxs: ataxia + confusion + ophthalmoplegia + amnesia + confabulation

• 40 yo F with diplopia or droopy eyelids worse at end of day + difficulty swallowing + weird speech → myasthenia gravis

  • Pathophys? Antibodies to post-synaptic nicotinic ACh receptor

  • Dx? anti-AChR or anti-MUSK Ab testing

    • NOT edrophonium test/Tensilon test (no longer standard of care)

  • Tx? AChE inhibitors (e.g. pyridostigmine)

  • Associated malignancy finding? Thymoma (anterior mediastinal mass)

• NBSIM for patients with myasthenia gravis? CT chest to look for thymoma

  • b/c removal of thymoma can resolve myasthenia gravis

• Smoker with proximal muscle weakness that improves with use → LEMS 

  • Pathophys? Antibodies to presynaptic voltage-gated Ca++ channel

• Lateral medullary syndrome (Wallenberg syndrome) causes dysphonia, dysphagia, and loss of gag reflex

  • Blood vessel occluded? PICA occlusion

  • Loss of pain and temperature on left face → L trigeminal nerve 

  • Loss of pain and temperature on right side of the body→ L spinothalamic tract

    • Located in lateral brainstem

    • Decussates at anterior white commissure → contralateral sxs

  • Absent gag reflex, dysphonia, dysphagia → CN9/10 

    • Medulla

  • Vertigo → CN8

    • Pons/Medulla

  • Ptosis and miosis on left (Horner’s) → L sympathetic tract to superior cervical ganglion

    • Located in lateral brainstem

• Medial medullary syndrome causes tongue deviation to ipsilateral side

  • Blood vessel occluded? Anterior spinal artery occlusion

  • Right sided paralysis → L corticospinal tract

    • Runs through medial brainstem

    • Decussates at level of medullary pyramids

  • Tongue deviation to the left. → L CN12

    • Tongue deviation to ipsilateral side

• A 35 yo African American female with 3-day history of eye pain. Funduscopic exam is notable for conjunctival erythema and miosis. CBC is notable for increased ACE levels and Ca2+ of 12.9. CXR with bilateral lymphadenopathy. What is the cause of her eye symptoms? → optic neuritis 2/2 sarcoidosis

  • Exam finding? APD + pain with eye movements (this is optic neuritis!)

  • Another condition a/w optic neuritis? Multiple sclerosis

• 35 yo F with tremors in her hands bilaterally that are worsened by stretching out her hand. Better with alcohol. → benign essential tremor

  • Inheritance? AD

  • Tx?

    • Beta blocker (e.g. propranolol) 

    • Barbiturates (e.g. primidone) 

      • Mechanism? Increase duration of opening of chloride channels → hyperpolarization

.

Central scotomas, straight lines look wavy = age related macular degeneration

• 2 types - wet (exudative/neovascular) and dry (atrophic)

• Wet: more progressive, but also more treatment options offered

  • Tx: VEGF inhibitors (Ranibizumab, Bevacizumab) or laser

• Dry: drusen on fundoscopy; more common than wet

  • Tx: Vitamins C, E, beta-carotene, zinc slow progression

  • Note: (careful in smoking pt as increased mortality rate from lung cancer with Vit E and beta carotene)

• MCC of vision loss in developing world = macular degeneration

• Other risk factors = smoking, aspirin (chronic use)

• Buzz word = neovascularization 

• Lose central vision (macula) (whereas in glaucoma lose peripheral vision)

2. Punched in eye + floaters and bright streaks of light = retinal detachment

• Risk factors = trauma, cataract surgery

  • Note: others metabolic disorders, myopia, degenerative diseases, vascular disorders

• Treatment strategies = laser photocoagulation (buzzword to remember)

• Ophtho emergency 

3. Newborn with R eye white reflex = retinoblastoma

• Associated with = Osteosarcoma 

  • Sunburst (codman’s triangle) on Xray

4. Amblyopia vs strabismus

• Amblyopia = cortical blindness -> eye normal but brain doesn’t recognize is

  • MCC = strabismus

  • Others: anything that obstructs light - cataracts, glaucoma 

• Strabismus = misalignment of eye

  • Treat if persists past 3 months with patching of unaffected eye - makes the brain work the affected eye to align itself; eyepatch or “paralytic” drops with atropine

  • Corneal light reflex is uncentered in affected eye (not symmetric)

  • Writers note: red reflex will be brighter in affected eye

5. Asian male with sudden onset severe eye pain + nausea and vomiting following nasal decongestant use = acute angle closure glaucoma

• Alpha one agonist - phenylephrine will cause mydriasis

• Treatment = laser iridotomy (ophtho emergency), clonidine, muscarinic agonist (pilocarpine), mannitol or acetazolamide, latanoprost, beta-blockers

• Risk factors = African American, diabetes, steroid use (can decrease outflow at anterior chambers)

  • Closed (aCute) think Asians, Open (chrOnic) think African Americans

• MCRF = increase in intraocular pressure *Keep in mind

• Dx: Tonometry  

• Fundoscopic exam = increased optic cup:disc ratio 

• Ciliary epithelium makes aqueous humor made by ciliary epithelium (driven by sympathetic NS via beta2 receptors) → post chamber of eye → ant chamber ni→ drain via canal of Schlemm/ trabecular meshwork (M3 receptors) & some (~25%) through the uveoscleral outflow tract (controlled by prostaglandins)

• Treatment targets the different points in the pathway:

  • Aqueous humor production

    • Beta blockers (Timolol): decrease aqueous humor synthesis (Beta2)

    • Alpha2 agonist (briminodone)- (Gi coupled inhibit adenylate cyclase) inhibit presynaptic release of NE 

      • NOT in closed angle glaucoma - can precipitate closed (acute)

    • Carbonic anhydrase inhibitor (acetazolamide) - decrease the bicarb at the ciliary epithelium will decrease the Na+ and H20 transport and thus dec synthesis of aqueous humor

  • Uveoscleral outflow (note: his mechanism is a little off here so I’m adding the correct mechanism for PG agonists): increase outflow through the uveoscleral tract by increasing permeability with prostaglandin agonists 

    • SE: Permanent discoloration of iris

  • Trabecular outflow - increased drainage of meshwork/canal with increase episcleral vasculature with M3 agonists (Carbachol, Pilocarpine)

6. 2 days of severe R eye pain + blurry vision + sinusitis taking Benadryl + pain with eye movement and 20/200 vision and difficulty moving EOM = orbital cellulitis

• Dx: clinical +/- ocular CT scan

  • Differentiate from preseptal cellulitis by involvement of EOM and pain with movement in orbital cellulitis - orbital cellulitis is preseptal cellulitis + other worsening symptoms

• Path: polymicrobial infection

• Tx: clindamycin + penicillin family

• Big risk factor = sinusitis

7. female with severe L eye pain, can barely see, worse after hot shower = optic neuritis due to multiple sclerosis

• Diagnostic imaging = MRI

• Tx = IV steroid (po steroid increases risk of recurrence of optic neuritis) Very high yield info

• Most likely sequelae = resolve over time

8. 76 yo F difficulty seeing while driving at night and reading road signs = cataracts

• Path = opacification of the lens

• Metabolic RF = diabetes (aldose reductase converts glucose to sorbitol, lens lacks sorbitol dehydrogenase to convert it to fructose so sorbitol accumulates in lens)

• Drug associated RF = steroids

• Infectious RF = congenital syphilis, congenital CMV, congenital toxo

• Newborn with a bilateral presentation = classic galactosemia (Galactose-1-phosphate uridyltransferase deficiency or GALT) - galactitol accumulates in lens and opacifies it 

  • WNote: red reflex = dark, dull, white

  • Most common cause of death in newborns with galactosemia = e. coli sepsis**HY 

9A. 

• MCC conjunctivitis in first 24 hours of life = chemical conjunctivitis from silver nitrate (not used much anymore)

• -       5 day old newborn with “super” purulent conjunctivitis 

  • Bug = gonorrhea - within 1st week of life - most dangerous for blindness in US

  • Tx = IV cefotaxime (3rd gen cephalosporin) Ceftriaxone causes kernicterus and cholestasis in newborns 

  • Prophylaxis = topical macrolide (erythromycin) 

    • §  DOES NOT PPX AGAINST CHLAMYDIA

• -       12 day old newborn with “watery” discharge conjunctivitis and eyelid swelling 

  • Bug = chlamydia (serovars A-C) - appears 1-2 weeks after birth - leading cause of preventable blindness worldwide 

  • Tx = PO (oral!) erythromycin 

    • Use an oral treatment to eliminate eye infection and possible nasopharyngeal infection otherwise will develop PNA at 1-3 months

    • Topical erythromycin does NOT ppx against chlamydia though remember 

  • Prophylaxis relationship to presentation 2 = topical erythromycin does NOT ppx against chlamydia conjunctivitis 

  • Pneumonia presentation = newborn with “staccato cough”

  • “down the line” nonbilious vomiting after tx (pear shaped mass) = pyloric stenosis from erythromycin 

9B.  Most likely Conjunctivitis 

• Glued eyes in am (unilateral) + water discharge + rhinorrhea = viral 

  • Tx = warm/cold compress 

  • o   HY bug = adenovirus “pharyngoconjunctivitis”

    • Remember adenovirus causes gastroenteritis and hemorrhagic cystitis 

  • MCC conjunctivitis = viral 

• Glued eyes in AM (unilateral then becomes bilateral) + purulent discharge + no rhinorrhea = bacterial

  • MCC bacterial conjunctivitis = s. aureus

  • Tx: antibiotic eye drop (erythromycin except contact-wearers use fluoroquinolone drops to cover for possible pseudomonas)

• -       Hx of asthma + BL itchy red eye = allergic conjunctivitis

  • o   Tx: antihistamine eye drops (Olopatadine, Azelastine)

10. Eye pain + worsened in light in 23yo male with hx of chronic LBP and morning stiffness = ankylosing spondylitis = anterior uveitis 

• Pulmonary disease association = sarcoid

• Pediatric rheumatology association = oligoarticular JRA 

  • If given JRA pt and asked next best step - slit lamp 

• If determine caused by HSV - give acyclovir +/- topical steroid vs. cause is autoimmune phenomenon give topical steroids

11. 70 yo F with difficulty reading books. Has to move books way from her eyes to make out the words = presbyopia

• Path = lens loses elasticity with age so can’t accommodate (just like skin loses elasticity)

12. Differentiate central retinal artery occlusion from central retinal venous occlusion - HY*

• CRAO = acute, painless, monocular vision loss

  • Amaurosis fugax – curtain over eye

    • Note: assoc with Temporal arteritis, carotid a stenosis; presents as transient loss of vision for several minutes and then eventually present with persistent vision loss (aka CRAO)

  • Dx: ocular u/s + fundoscopic exam = thin retinal vessels, fundal pallor (swelling of the retina)* helps distinguish from venous occlusion

  • Tx: ocular massage and high flow oxygen while en route to hospital; once at hospital give TPA

• CRVO =  rapid, painless loss of vision of varied severity 

  • Dx: fundoscopic exam = optic DISC swelling (vs retinal swelling in CRAO)thick retinal vessels, blood and thunder retina, cotton wool spots, hard exudates

    • Fluorescein angiography not u/s if suspect venous occlusion

  • Tx: VEGF-I, photocoagulation 

• Don’t forget about these both presenting with cherry red spot on fundoscopy:

  • Tay-sachs (hexosaminidase A deficiency) losing milestones and impaired startle, no HSM

  • Niemann-pick (sphingomyelinase deficiency) - similar to Tay Sachs but with hepatosplenomegaly 

• Diabetic Retinopathy - presents very similar to CRVO **note similar descriptions

  • Nonproliferative: dot and blot hemorrhages, hard exudates, retinal edema, microaneurysm 

  • Preproliferative: cotton wool spots

  • Proliferative: neovascularization (buzzword), macular edema 

  • Do fundoscopic exam - repeat every year; to make definitive diagnosis do fluorescein angiography to confirm diagnosis (r/o CRVO)

  • Tx: same as CRVO - VEGF-Inhibitor, laser photocoagulation

13. 19 yo F with pain and a “foreign body sensation” in the eye + wears contact lenses = corneal abrasion 

• Dx = fluorescein slit lamp exam - can’t see with naked eye  

• Tx = REMOVE contact, topical broad spec antibiotic coverage with pseudomonas coverage (fluoroquinolone) topical NSAID drop for eye, eye patch (he says you can but avoid patching it)

  ---

• HSV meningitis or HSV encephalitis

  • CSF findings?

    • Elevated WBCs (lymphocytic predominance)

    • Lots of RBCs (but < 1000)

      • Note: If 4000-5000 RBCs → think SAH instead

    • Elevated protein

    • Normal glucose

  • Location? Temporal lobe

  • Dx? HSV PCR of CSF

  • Tx? IV acyclovir

• Young adult patient + fever + headache + neurologic deficits + no nuchal rigidity→ brain abscess

• Headache + high fever + sudden-onset nuchal rigidity + → bacterial meningitis

  • CSF findings?

    • Elevated WBCs (neutrophilic predominance)

      • Note: in contrast, Tb meningitis is the only bacterial meningitis with lymphocytic pleocytosis 

    • HIGH protein

    • LOW glucose
      
      

• #1 cause bacterial meningitis in children through middle-aged adults? Strep pneumo

  • Tx for bacterial meningitis? Ceftriaxone + vancomycin + steroids
    
    

• #1 cause bacterial meningitis in neonates? Group B Strep (Strep agalactiae)

  • Tx for neonatal meningitis? Cefotaxime + vancomycin + ampicillin

    • Cefotaxime covers GBS

    • Vancomycin covers Staph aureus & resistant Strep pneumo

    • Ampicillin covers Listeria
      
      

• Special bacterial cause of meningitis seen in neonates & elderly only? Listeria monocytogenes

  • Tx? Add ampicillin to standard bacterial meningitis tx

• What pathogen causes bacterial meningitis in young adult sxs of skin petechiae? Neisseria meningitidis 

  • Tx? Ceftriaxone

  • PPX for close contacts? Rifampin, ciprofloxacin, or ceftriaxone (“RCC”)

  • Prevention? Neisseria meningitidis vaccine
    
    

• Pt with Neisseria meningitidis meningitis becomes profoundly hypotensive + hypoglycemic + hyperkalemic + bleeding from mucosal sites. Dx? → Waterhouse-Friedrichson syndrome 

  • Pathophys? adrenal hemorrhage → primary adrenal insufficiency

    • Cortisol production impaired → hypoglycemia & hypotension

    • Aldosterone production impaired → hyperkalemia & non-AG metabolic acidosis (can’t excrete H+)

    • Type 4 RTA
      
      

• Populations at risk for Neisseria meningitis?

  • Asplenic patients (e.g. sickle cell disease)

  • Terminal complement deficiency or eculizumab use (tx for paroxysmal noctural hematuria)

    • Terminal complement = C5-C9

    • Eculizumab = C5 inhibitor

• 25 yo M having generalized tonic-clonic seizures. Began intranasal desmopressin (ADH analog) 5 days ago after water deprivation test was conducted at local hospital. Dx? → hyponatremic seizures (i.e. due to increased water reabsorption)

  • Pathophys? Desmopressin = ADH analog acts on V2 receptors of principal cells to cause increased water reabsorption → this causes dilutes serum Na+ 

  • Tx? Hypertonic saline

    • To raise serum Na+ levels

    • Note: cannot increase Na+ levels > 12 mEq over 24 hrs

• What neurological complication results if hyponatremia is corrected too quickly? Osmotic demyelination syndrome aka central pontine myelinolysis aka “locked in” syndrome
  
  

• What neurological complication results if hypernatremia is corrected too quickly? Cerebral edema → transtentorial herniation
  
  

• In general, do you use a slow or fast general management strategy to manage electrolyte imbalances? SLOW management strategy to correct electrolyte imbalances

• Bizarre dreams on awakening (hypnopompic hallucination) or on falling asleep (hypnagogic hallucination) + history of 3 major accidents from falling asleep while driving + sudden falls from LE weakness (cataplexy). Dx? → Narcolepsy

  • Diagnostic test? Polysomnography (will show decreased sleep latency, i.e. pt goes straight into REM sleep)

  • Tx?

    • Frequent naps

    • Modafinil (stimulant)

    • Sodium oxybate to treat cataplexy

  • CSF findings? Decreased hypocretin/orexin levels

• What drug is given to tx insomnia? Suvorexant 

  • MOA? hypocretin receptor antagonist

• Severe headache + multiple extraocular muscle deficits + recent otitis media infection. → cavernous sinus thrombosis

  • Pathophys? CN III, IV, VI, V1, V2 go through cavernous sinus

    • Note: loss of CN VI function occurs first (lateral gaze palsy)

  • Ophthalmic veins anastomose with facial veins & veins that drain sinuses 

  • MC organism? Staph aureus

  • Tx? IV abx + heparin

• Severe headache + necrotic sinusitis in patient with blood glucose of 300 + pH of 7.13 + HCO3- of 15. → mucormycosis in context of DKA

  • Tx? Debridement + amphotericin B

• Asymmetric resting tremor + cogwheel rigidity + bradykinesia/festinating gait + postural instability → Parkinson’s disease 

  • Associated handwriting finding? Micrographia (small handwriting)

  • Associated BP finding? Orthostatic hypotension

  • Pathophys? Destruction of dopamine-producing cells in substantia nigra pars compacta (part of the midbrain)

  • Gross pathological finding? Loss of pigmentation of substantia nigra

  • Histology of Parkinson disease? Lewy bodies (eosinophilic inclusions made of alpha-synuclein)

  • Tx?

    • Start with:

      • NMDA receptor antagonists (e.g. amantadine; increases dopamine release in CNS) 

OR

• Dopamine agonists (e.g. bromocriptine, ropinerole, cabergoline, pramipexole)

OR

• MAO-B inhibitors (e.g. rasagiline, selegiline) 

OR

• COMT inhibitors (e.g. entacapone, tolcapone)

  • Entacapone = acts peripherally b/c does not cross BBB 

  • Tolcapone = acts centrally b/c crosses BBB

• Last line tx? Levodopa-carbidopa

  • Why last line? b/c only works few years then becomes less effective due to on-off phenomenon
    
    

• What drugs or drug class cause drug-induced Parkinsonism?

  • Dopamine receptor antagonists

  • Metoclopramide

    • 2 indications for metochlopramide? antiemetic, gastroparesis
      
      

• What anticholinergic drug reduces tremors of Parkinson’s diseases? Benztropine (muscarinic receptor antagonist)
  
  

• Parkinsonism in a young patient that took a “drug” at a party? MPTP-contaminated heroin

• DDx Parkinson disease vs essential tremor 

  • Parkinson’s disease tremor = asymmetric resting tremor + improves with activity

  • Essential tremor = symmetric + worsens with activity + responds to propranolol
    
    

• What 2 drugs tx acute dystonias caused by pt taking dopamine antagonists? Benztropine, diphenhydramine 

  • Pathophys? Dopamine antagonists cause Ach-dopamine imbalance → results in unopposed Ach overactivity)

  • Note: diphendramine works to tx acute dystonias b/c it has significant anticholinergic activity

• Tx of focal dystonia? Botox injection

• Tx for psychosis in pt with Parkinson’s disease?

• Reduce dose of carbidopa-levodopa 

• Quetiapine (atypical antipsychotic with weak dopamine receptor antagonist activity)

• What Parkinson’s disease drug causes hypertensive crisis? MAO-B inhibitors

  • Tx? Phentolamine (alpha-1 blocker)

• 35 yo F + BMI of 35 + visual difficulty + intermittent severe headaches + being treated for acne vulgaris. → idiopathic intracranial hypertension

  • Risk factors?

    • Obese female

    • Vit A derivatives

    • Tetracyclines (can be used as acne tx, as in this pt)

  • Ocular finding? Papilledema 

  • Diagnostic? CT scan plus LP

    • CT scan → normal

    • LP → elevated opening pressure (>250)

  • Tx?

    • Acetazolamide

    • Serial LPs

• 41 yo M with 1 year of forgetfulness + behavioral changes. His father had similar presentation before dying at 47 y.o. → Huntington’s disease

  • Pathophys? Atrophy of caudate (part of striatum)

  • Trinucleotide repeats? CAG

  • Chromosome? 4

  • Inheritance? AD

    • With anticipation!

  • Tx? Anti-dopaminergic drugs

    • Haloperidol

    • Tetrabenazine (VMAT inhibitors)

• Worst headache of patient’s life + bilateral flank masses. → SAH in pt with ADPKD

  • Pathophys? Rupture of berry aneurysm in circle of Willis 

  • MC aneurysm location? ACom aneurysm (anterior communicating artery)

  • Associate heart murmur? MVP

  • NBSIM? CT head non-contrast (because blood will show up as hyperdense)

    • If CT is negative → do LP (looking for xanthochromia)

  • Tx? Lower pt’s BP to 140/90

  • What drug is given to prevent superimposed ischemia? nimodipine
    
    

• Cluster headache

  • Sxs? Unilateral tearing/conjunctival injection/rhinorrhea, occurs for several days 

  • Tx? 100% O2 and triptans
    
    

• Tension headache

  • Sxs? Bilateral + high-stress environment + worse at end of day

  • Tx? NSAIDs
    
    

• Migraine headache

  • Sxs? Unilateral + pulsatile + photophobia & phonophobia +/- aura

  • Abortive tx? 

    • Sumatriptan (5-HT receptor agonist)

      • Contraindications? variant angina aka Prinzmetal angina, CAD, CREST syndrome

        • Can trigger serotonin syndrome

      • Ergotamine 

  • PPx?

    • Propranolol

    • Topiramate

    • TCA

      • Contraindications? Elderly pt’s, glaucoma pt’s

        • Avoid with glaucoma (anticholinergic mydriasis)

    • CCB’s

• Analgesic rebound headaches

  • Sxs: use of analgesics at least 8-10x in last month; headache returns once analgesic effects wear off

  • Tx? Wean off analgesics
    
    

• Headache + mental status changes in patient using fireplace to warm house in winter → CO poisoning

  • Diagnostic? Carboxyhemoglobin levels

    • Note: Pulse O2 cannot distinguish between oxyhemoglobin and carboxyhemoglobin

  • Tx? High-flow O2 / hyperbaric O2 

  • Imaging? Hyperintense lesions in globus pallidus on MRI
    
    

• 35 yo smoker + right-sided headache + occurs everyday at same time for past month + PEx notable for rhinorrhea & pupillary miosis on right → cluster headache

• A child is not doing well in school. His teacher often observes him staring into space with no awareness of his surroundings. → absence seizures

  • EEG pattern? 3 Hz spike and wave

  • Tx? Ethosuximide (T-type Ca++ channel blocker)

• 55 yo F presents with a 6 mo history of feeling like the room is spinning around her that lasts for about 3 days at a time. She has also had difficulty hearing at home. She also complains of a “ringing sound” in her ear. → Menierre’s disease

  • Triad: vertigo + tinnitus + sensorineural hearing loss 

  • Pathophys? Endolymphatic hydrops, poor reabsorption of endolymph in ear → high pressures

  • Tx?

    • Dietary Na+ reduction

    • For vertiginous sxs:

      • scopolamine (anticholinergic) 

meclizine (antihistamine, anticholinergic)

• diphenhydramine (antihistamine, anticholinergic)

• Diuretics

• Definitive tx? Gentamicin injection to ablate CN8

  • Causes of permanent sensorineural hearing loss

• Vertigo triggered by changes in position, NO hearing loss → BPPV

  • Pathophys? otoconia/otoliths in semicircular canals

  • Dx? Dix-Hallpike maneuver

  • Tx? Epley maneuver

• Recent viral URI + constant non-positional vertigo, lasts for days to weeks before spontaneous resolution, NO hearing loss → Vestibular neuritis 

• Recent viral URI + constant vertigo + hearing loss → Labrynthitis 

• Hit in the head with a bat, passed out for < 30s, finished out the game, now presents with severe headache and somnolence. → epidural hematoma

  • Pathophys? Fracture of frontal bone → tearing of the middle meningeal artery

  • Dx? Non-con head CT → lens-shaped hematoma

    • Do NOT perform an LP because increased ICP will cause herniation

  • Tx?

    • Neurosurgical evacuation

    • Reduce ICP

      • Elevate head of bed

      • Hyperventilate pt (CO2 down → cerebral vasoconstriction)

      • Mannitol

    • What is the quickest means of reducing ICP acutely? Hyperventilation

• Old person on warfarin with multiple falls, acting out-of-it recently OR alcoholic → subdural hematoma

  • Pathophys? Brains shrink with aging or alcoholism, stretches the bridging veins, causing greater susceptibility to shearing

  • Dx? Non-con head CT → crest-shaped 

• Brain bleed in shaken baby → subdural hematoma

• Super tense muscles and T of 105 after intubation. → malignant hyperthermia

  • Pathophys? Mutation in the ryanodine receptor → increased release of Ca++ from the sarcoplasmic reticulum → hypercontractile state that generates heat

  • Inheritance? AD

  • Tx? Dantrolene (ryanodine receptor antagonist)

  • Electrolyte disturbance? hyperkalemia → peaked T waves → wide QRS → sinusoidal pattern

    • Tx?

      • 1st calcium gluconate

      • Then insulin/glucose, albuterol, or sodium bicarb

      • Kayexalate (helps excrete K+)

      • Furosemide (loop diuretic with hypokalemia as side effect) 

  • Why elevated Cr? 

    • Rhabdomyolysis → myoglobin release → damages kidney

    • Tx? IVF

• If same presentation after starting fluphenazine (1st gen high-potency antipsychotic) → neuroleptic malignant syndrome

  • Tx? 

    • Dantrolene

    • Dopamine agonist (e.g. bromocriptine/cabergoline, amantadine) 

• Hx of depression + Tx for S. Aureus bacteremia (or migraines) presents with high fevers, diarrhea, clonus → serotonin syndrome

  • Drugs that can trigger

    • Any antidepressant

    • Linezolid

    • Ondensatron 

    • Triptans

    • MAO-B

    • MDMA/ecstasy

  • Tx?

    • Cyproheptadine (antihistamine with powerful serotonin receptor blocking activity)

    • Benzo

• Tremors 6 hrs after the successful completion of a AAA repair in a business executive. → alcohol withdrawal

  • Ppx? Long-acting benzo (e.g.chlordiazepoxide, diazepam)

• Tremors after starting Indapamide (thiazide-like diuretic) in a patient with a history of “episodes” where they spend tons of money/jump into severe depression. → lithium toxicity in pt with bipolar disorder

  • Pathophys? Diuretics can raise lithium levels → lithium toxicity, which manifests as tremors

  • Causes of lithium toxicity? anything that causes RAS activation (aldosterone acts on principal cell to increase Na+ reabsorption → Li+ can allow be reabsorbed through this channel)

• Child presents with bulging fontanelles and somnolence. What is the most likely region of stenosis responsible for the presenting hydrocephalus? Cerebral aqueduct of Sylvius

• 70 yo F that is forgetful. She staggers into the exam room. She has a history of recurrent perianal sores from urinary incontinence. → “wet wobbly wacky” → normal pressure hydrocephalus 

  • Dx? Non-con head CT with enlarged ventricles

    • LP → normal opening pressure

  • Tx? VP shunt

• Tuberous sclerosis

  • Inheritance? AD

  • Mutated genes? TCS1 & TCS2 (tumor suppressor genes)

  • Mutated proteins? Hamartin & tuberin

  • Brain findings? Subependymal tumors (usually calcified)

  • Renal findings? Angiomyolipoma

  • Cardiac findings? Rhabdomyoma

  • Skin findings 

    • Hypopigmented = ash leaf spots

    • Hyperpigmented = Shagreen patch

  • Kind of infantile seizure? West syndrome = infantile spasms

    • EEG findings? hypsarrhythmia

    • Tx? ACTH or vigabatrin 

• Mom has a history of inconsistent condom use. She delivers a stillborn fetus with no brain. → anencephaly 2/2 Zika 

  • Pathophys? Anterior neuropore fails to close

    • Polyhydramnios because no swallowing center

• Dimple or tuft on hair in lumbosacral area → Spina bifida occulta

  • Pathophys? Failure of the spinous processes to fuse around the spinal cord

  • Usually causes no problems

• Meningocele 

  • Pathophys? Failure of fusion of the posterior neural arch → herniation of meningeal tissue and CSF 

  • Associated deficiency in mom? folate

  • Tx? Surgery

  • Good prognosis after surgery

• Myelomeningocele 

  • Pathophys? Failure of neural tube to close → herniation of meninges and spinal cord tissue through defect 

  • Associated deficiency in mom? folate

  • Tx? Surgery

  • Worse prognosis

  • Associated dz? Chiari 2 malformation

• Type 1 Chiari malformation

  • Pathophys? Cerebellar tonsils herniate through foramen magnum

  • Associated dz? Syringomyelia (“Syr1ngomyelia”)

• Type 2 Chiari malformation

  • Pathophys? Cerebellar tonsils herniate through foramen magnum

  • Associated dz? Lumbosacral myelomeningocele 

• Serum marker for NTD/spinal dysraphism? Elevated maternal serum AFP

  • Also elevated ACh esterase 

  • What if msAFP is decreased? Down syndrome

• Subacute combined degeneration of spinal cord

  • Vitamin deficiency? B12

  • Loss of dorsal columns → loss of vibration, touch, proprioception

  • Loss of lateral corticospinal tract → UMN signs

  • Differentiate folate & B12 deficiency with serum markers

    • Megaloblastic anemia in both

    • Homocysteine high in both

    • MMA high in ONLY B12 deficiency

• Cafe au lait spots + brown “pigments” in the axilla + “tuber like” skin growths + episodic headache and severe HTN. → NF-1

  • Eye malignancy? Optic nerve gliomas

  • Iris finding? Lisch nodules

  • Brain malignancy? Meningioma

  • Neuroendocrine malignancy? Pheo

  • Ear malignancy? Bilateral acoustic neuromas/vestibular Schwannomas in NF-2

    • Location? Cerebellopontine angle

  • Inheritance? AD

  • Gene?

    • NF-1 → neurofibromin

    • NF-2 → merlin

  • Chromosome

    • NF-1 → 17

    • NF-2 → 22

• Student in a lab is unknowingly touching a lighted bunsen burner in the chemistry lab until his hand is pulled away by the TA. PE is notable for loss of pain and temperature sensation in the UEs bilaterally and mild hand muscle weakness. → syringomyelia

  • Pathophys? Obstruction in the central canal of the spinal cord → cystic expansion of central canal caudally → compression of anterior while commissure → knocks of spinothalamic tract

  • Tracts involved?

    • Spinothalamic → loss of pain + temperature bilaterally

    • If left untreated, will expand and affect the ventral horn → motor sxs (UE first because these tracts are most medium)

  • Dx? MRI

• Nuchal rigidity in a recent immigrant presenting with hemoptysis and high fevers. There is marked “enhancement” at the base of the brain. → TB meningitis 

  • Dx? 

    • LP

  • CSF findings?

    • Lymphocytic predominance

    • Protein HIGH

    • Glucose LOW

    • High OP

  • Tx? RIPE regimen + Vit B6

  • Differentiating meningitis from encephalitis

    • Encephalitis → more neuro deficits, may not have nuchal rigidity

    • Meningitis → nuchal rigidity 

• Most common neurologic sequelae of meningitis in kids → hearing loss

• Top 3 causes of meningitis in neonates

  • GBS

  • E. coli

  • Listeria

• Empiric tx for neonatal meningitis? Cefoxamine + vanc + ampicillin

  • Ceftriaxone can cause intrahepatic cholestasis in neonates

• CSF studies in bacterial, viral, and fungal meningitis.

  • Bacterial

    • Neutrophilic predominance

    • Protein HIGH

    • Glucose LOW

  • Fungal

    • Lymphocytic predominance

    • Protein HIGH

    • Glucose LOW

  • Viral

    • Lymphocytic predominance

    • Protein high

    • Glucose low/normal

  • Patient with symmetric ascending paralysis after a recent bloody diarrheal infection → GBS after Campylobacter infection

    • CSF finding? Albuminocytologic dissociation

      • Usually WBC & protein go up together

      • In this case, high protein but new WBCs

  • 30 yo F with an afferent pupillary defect and other neuro findings → MS

    • CSF finding? Oligoclonal bands

    • Dx? MRI

  • CSF with a ton of RBCs

    • HSV encephalitis

    • Xanthochromia 2/2 SAH

• General treatment cocktail for bacterial meningitis. 

  • Ceftriaxone + vancomycin + steroids (CVS)

• What should be added in the elderly, immunocompromised, or super young (infants/neonates)? ampicillin

• CSF studies with normal glucose, slightly elevated protein, and a lymphocytic pleocytosis → viral meningitis 

• Classic imaging and CSF findings in HSV meningitis OR encephalitis → enhancement of temporal lobes

• 25 yo M presents with a T of 103, severe headache, and a 2 day history of profound LE muscle weakness. He was recently treated for Otitis Media. → brain abscess

  • Triad: fever + HA + neurologic deficit 

    • No nuchal rigidity 

  • Dx? MRI

  • Tx? Drain abscess + steroids to decrease swelling + abx

  • What is the bug that is classically associated with meningitis with MRI enhancement at “the base of the brain”? TB

• 35 yo zoologist is brought to the ED by ambulance after becoming unresponsive at home. He studies cave dwelling animals. Over the past 2 weeks he has resisted attempts by his parents to give him water or get him to the shower. → rabies

  • Prevention? Rabies immunoglobulin + vaccine (at two different sites)

  • Pathognomonic histologic finding? Negri bodies

• Bilateral acoustic neuromas → NF-2

  • Inheritance? AD

  • Gene? Merlin

  • Chromosome? 22

  • Involved cranial nerve? CN8

  • Classic brainstem location? Cerebellopontine angle

  • Dx? MRI

• Port wine stain + glaucoma + seizures + ID + tram track CT calcifications → Sturge-Weber syndrome

  • Inheritance? Non-heritable! Sporadic!

  • Gene? GNAQ activating mutation

• Woman with trouble sleeping due to weird sensations in legs → restless legs syndrome

  • Associated dz? IDA

  • Tx? 

    • pramipexole/ropinirole

    • primidone 

• Pt that has paresthesias over palmar aspect of hand (+ thenar atrophy → carpal tunnel syndrome

  • Associations

    • RA

    • Pregnancy

    • Hypothyroidism 

  • PE maneuvers 

    • Tinnel’s sign (tapping over carpal tunnel)

    • Phalen sign (flexed wrists)

  • Tx?

    • Wrist splint

    • NSAID

    • Inject steroids

    • Carpal tunnel release

  • What should be done to confirm the dx before pursuing surgery? Nerve conduction study!

• 32 yo Asian M presents with severe R eye pain. PE reveals a “rock hard” unreactive pupil. → acute-angle glaucoma 

  • Dx? Tonometry (eye pressures)

  • Tx?

    • Usually requires surgery: laser iridotomy

    • Medical management

      • Mannitol (lows IOP by drawing water out of the vitreous humor of the eye and into the intravascular space)

      • Acetazolamide (decreases aqueous humor production)

      • Timolol (decreases aqueous humor production)

      • Pilocarpine (muscarinic agonist, constricts pupil)

• A 3 yo F is brought to the ED by her dad. She has been using the potty continuously for the last 3 hrs. PE is notable for pupillary miosis and profuse sweating. She played in the farm this morning. → organophosphate poisoning

  • Pathophys? Organophosphates inhibit ACh esterase → ACh levels rise → parasympathetic overactivation 

  • Tx? Atropine (blocks AChR) + pralidoxime (regenerated AChE) 

• Flaccid paralysis after consuming home canned goods. → botulism

  • Pathophys? Botulinum toxin cleaves SNARE proteins → prevents release of ACh from presynaptic neuron

  • Tx?

    • Botulinum immune globulin 

    • Intubate for respiratory support

  • Contrast to tetanus, which will have a spastic paralysis 

    • Pathophys? tetanus toxin prevents the release of glycine & GABA (inhibitory NTs) from Renschaw cells → spastic paralysis

• Difficulty getting out of a chair in a patient with a 45 pack year smoking history that improves with muscle use. → LEMS

  • Pathophys? Ab to the presynaptic voltage-gated Ca++ channel

  • Increment in muscle contraction with repetitive nerve stimulation

  • Associated malignancy? Small cell lung cancer

• Difficulty swallowing and droopy eyelids in a 35 yo F with an anterior mediastinal mass → myasthenia gravis in s/o thymoma

  • Pathophys? Ab against nicotinic AChR

  • Decrement  in muscle contraction with repetitive nerve stimulation

  • Dx? anti-AChR antibodies

    • Not Tensilon test anymore

  • Tx?

    • AChE inhibitors (e.g. pyridostigmine)

    • Resection of thymoma

  • In addition to tx with AChE what is NBSM? Chest CT (look for thymoma)  

  • How is this condition differentiated from ChAT deficiency? 

    • ChAT = choline acetyltransferase 

    • ChAT deficiency sxs will not improve with AChE therapy

• Anticholinergic toxidrome = “hot as a desert, dry as a bone, red as a beet, blind as a bad, mad as a hatter”

  • Classic meds:

    • Diphenhydramine

    • TCAs

    • 1st gen low potency antipsychotics (e.g. chlorpromazine)

• Tongue fasciculations in a 6 mo. → SMA (spinal muscular atrophy)

  • Inheritance? AR

  • Pathophys? Pure LMN disease

  • Mutated gene? SMN1 

  • Chromosome? 5

  • Viruses that affect anterior horn cells?

    • Polio

    • West nile

• Tongue fasciculations in a 65 yo M with asymmetric weakness. → ALS

  • UMN + LMN problems

  • Pathophys? Destruction of the UMNs (corticospinal/corticobulbar tract), LMNs (anterior horn of spinal cord), and cranial nerves 

  • Genetic mutation in familial cases? SOD1, C9orf72 

  • What is spared in ALS? Sensation, bowel/bladder function, extraocular muscles

  • EMG findings? Chronic denervation, fibrillation potentials

  • Tx that improves survival?

    • Riluzole (NMDA receptor antagonist, reduce glutamate excitotoxicity)

    • Bipap 

  • Tx for spasticity?

    • Baclofen (GABA receptor agonist)

    • Tizanidine (alpha-2 agonist)

    • Botox injection

• Dermatomyositis/polymyositis

  • Ab? Anti-Jo1, anti-Mi-2, anti-SRP

  • Dx?

    • 1st muscle MRI

    • 2nd muscle biopsy

  • Lab findings? Elevated creatinine kinase

  • Associated malignancy? Lung cancer (especially small cell)

• Dysarthria + truncal, gait, and limb ataxia in a patient that is subsequently found to have lung cancer → paraneoplastic cerebellar degeneration

  • Associated malignancies?

    • Lung 

    • Breast

  • Ab? Anti-Hu, anti-Yo

  • Pathophys? Tumors express cerebellar proteins, immune system tries to attack tumor but also mounts response against cerebellum 

• Confusion, ophthalmoplegia, and ataxia in an alcoholic. → Wernicke’s syndrome

  • What if there’s also forgetfulness & confabulation → Korsakoff’s syndrome

  • Tx? IV thiamine (Vit B1)

• Pharmacological management of hepatic encephalopathy

  • Lactulose (convert ammonia to ammonium, which you poop out)

  • Rifaximin (a lot of ammonia comes from bacteria in GI tract)

• Simple partial seizures = focal seizure w/o loss of awareness

  • Is there a loss of consciousness? NO

  • 4 types: motor, sensory, autonomic, psychic

    • Rigidity or jerking of extremity → motor 

    • Hissing sound or can’t see or smell burnt rubber → sensory 

    • Sweating, mydriasis, rising sensation in abdomen → autonomic 

  • Auras that localize to temporal lobe

    • Smelling burnt rubber

    • Deja vu

    • Rising sensation in abdomen

• Required imaging before recommending sports in a 12 yo with a history of Trisomy 21. Why? High risk of atlanto-axial instability/subluxation

  • Dx? Lateral neck XR

  • Another patient population at risk?

    • RA

    • Ankylosing spondylitis

• 25 yo M goes into respiratory failure. He recently recovered from a 7 day episode of bloody diarrhea. PE is notable for pronounced areflexia in the LE bilaterally. His parents report that he had muscle weakness that “started in the legs and progressed upward”. He initially complained of leg tingling and numbness before his other sxs started. → GBS

  • Alternative term? AIDP (acute inflammatory demyelinating polyneuropathy)

  • Pathophys? Peripheral demyelinating disease 

    • Cells affected? Schwann cells

  • Classically associated bug? Campylobacter jejuni

  • CSF findings? Albuminocytologic dissociation 

  • Tx? 

    • IVIG

    • Plasmapheresis 

• GBS-like presentation + nystagmus + ataxia? Miller-Fisher syndrome

• Pt that has syncope or neuro deficit when they use their arms → subclavian steal syndrome

  • Pathophys? Proximal subclavian stenosis → low pressure system distal to stenosis → reversal of blood flow in vertebral arteries → “stealing” blood from basilar → hypoperfusion to brainstem

• Multiple neuro deficits in a kid after getting the VZV vaccine or after an URI. → ADEM (acute disseminated encephalomyelitis) 

  • Pathophys? Inflammatory demyelinating lesions in brain & spinal cord

  • Dx? MRI

  • Prognosis? Full recovery

• Multiple Sclerosis

  • Classic presentations? Female in her 30s with disparate neuro deficits

    • Uhthoff's phenomenon = sxs worse in heat

  • CN2 pathology? Optic neuritis

    • Presentation? Unilateral eye pain + decreased visual acuity (e.g. 20/200) + afferent pupillary defect

  • Associated Vit deficiency? Vit D

    • Higher prevalence in places farther from the equation

  • Dx? MRI brain & spinal cord

    • Multiple demyelinating lesions separated in space & time

  • LP findings? Oligoclonal bands

  • Exam findings? 

    • APD

    • Lhermitte's sign = electric shock with neck flexion

  • Tx acute exacerbation? Very high-dose corticosteroids

  • Tx chronic/DMARDs?

    • Fingolimod

    • Natalizumab

      • Mechanism

      • AE? JC virus reactivation → PML

    • Glatiramer 

    • Rituximab

    • Interferon-beta

  • Tx urge & overflow incontinence?

    • Urge → oxybutynin, trospium, darifenacin/solifenacin, tolterodine

      • Detrusor muscles are hyperactive, so quiet them down with an antimuscarinic 

    • Overflow → bethanechol, neostigmine 

      • Alternative? Intermittent self-cath

      • Destrusor hypotonia, so activate detrusor with muscarinic agonists or AChE-inhibitors

      • High PVRs

  • Tx of spasticity?

    • Baclofen (GABA-B receptor agonist)

    • Tizanidine (alpha-2 agonist → decreases norepi release)

    • Dantrolene (CCB)

    • Benzos (GABA-A receptor agonist)

    • Botulinum toxin for localized spasticity
      
      

• Pt sees blood or has emotional stressor then passes out → vasovagal syncope

  • Dx? Tilt table test

  • Tx? Midodrine (alpha-1 agonist)

• Most common cause of death in patients with Factor 8/9 deficiencies? Hemorrhagic stroke

  • Pathophys? Defect of secondary hemostasis

  • Inheritance? X-linked recessive

• Stepwise diagnostic testing in stroke management. 

  • 1st dx step? Non-con head CT

    • Why? Differentiate b/t hemorrhagic & ischemic stroke

  • If NCCT is negative? Diffusion-weighted MRI 

  • Additional diagnostics?

    • Carotid artery duplex US of internal carotid arteries

    • Echo to look for LA appendage thrombus or PFO

  • Initial tx for ischemic stroke?

    • Aspirin

    • If aspirin isn’t an answer choice, choose another antiplatelet agent 

      • Clopidogrel

      • Dipyridamole

    • Do NOT give anticoagulant unless A-fib

  • If high suspicion for SAH but NCCT negative? LP → look for xanthochromia  

    • Tx for SAH? Lower BP + nimodipine

• Paralysis of ipsilateral upper and lower facial muscles, dry mouth, loss of lacrimation → LMN CN7 lesion

  • Pathway?

    • CNs (except CN2) are LMNs

    • UMNs comprise the corticobulbar tract (connect cerebral cortex to CNs)

      • Decussate prior to synapsing with CNs

    • CN7 also carries parasympathetic fibers

  • Why ipsilateral? Decussation happens upstream to CN7

• Paralysis of lower facial muscles, sparing of upper face→ contralateral corticobulbar tract lesion

  • Why contralateral? corticobulbar tract decussates BEFORE synapsing on CN7 nucleus

  • Why upper face sparing? Bilateral corticobulbar pathways that synapse on the CN7 nucleus (so you have to knock out both corticobulbar pathways to affect the upper face)

• Loss of facial sensation, jaw deviation to the ipsilateral side, impaired corneal reflex. → CN5 (trigeminal)

  • Pathways?

    • Facial sensation is carried by CN5

    • Corneal reflex

      • Afferent = CN5

      • Efferent = CN7

    • CN5 provides motor innervation to muscles of mastication (e.g. masseter, pterygoids, temporalis)

• Anosmia → CN1

  • Associated syndrome? Kallman’s syndrome 

    • Presentation? Anosmia + hypogonadotropic hypogonadism

    • Pathophys? Failure of GnRH neurons & olfactory neurons to migrate

• Mydriasis with the eye deviated down and out. → CN3 (oculomotor)

  • Pathways?

    • CN3 innervates all muscles of eye except superior oblique (CN4 trochlear) & lateral rectus (CN6 abducens)

    • CN3 also carries parasympathetic fibers to go to pupillary constrictors

  • Why down & out?

    • Lateral rectus → abduction 

    • Superior oblique → depression

  • Causes of CN3 lesions?

    • PCom aneurysm

    • Uncal herniation 

  • For compression lesions, mydriasis often happens before eye deviation

    • Why? Parasympathetic fibers run on the outside of CN3 (so more susceptible to compression)

  • For ischemic lesions (e.g. due to DM), eye deviation will happen first

• Bitemporal hemianopsia (“tunnel vision”), afferent pupillary defect. → optic chiasm compression

  • Causes?

    • Pituitary adenoma

    • Craniopharyngioma

• Contralateral lower facial weakness with forehead sparing and loss of taste sensation with the anterior ⅔ of the tongue. → UMN CN7 lesion

  • Pathways?

    • General sensation anterior ⅔ tongue → CN5

    • Taste anterior ⅔ tongue → CN7

• Horizontal diplopia with failed abduction on lateral conjugate gaze. → CN6 lesion

• Vertical diplopia with the jaw tilted towards the side of the lesion + difficulty going down stairs. → CN4 (trochlear) lesion

  • Pathway?

    • Trochlear emerges dorsally & crosses to opposite side

    • It’s the only CN that decussates

  • Note that the head tilt if TOWARDS the side of the lesion

• Sensorineural hearing loss, vertigo, and abnormal caloric test results. → CN8 (vestibulocochlear nerve)

  • Caloric test results → COWS mnemonic (cold opposite, warm same)

• Deviation of the protruded tongue to the ipsilateral side. → CN12

  • Mnemonic “Lick your wounds”

  • Pathway? Innervates all intrinsic muscles of the tongue except palatoglossus (innervates by CN10)

• Weakness in shoulder shrug + problems turning the head to the opposite side. → CN11 (spinal accessory nerve)

• Loss of taste sensation in the posterior ⅓ of the tongue + dysphagia + absent gag reflex. → CN9 or 10

  • Pathways?

    • Taste posterior ⅓ of tongue → CN9

    • General sensation posterior ⅓ of tongue → CN9

    • Taste extreme posterior tongue → CN10

    • General sensation extreme posterior tongue → CN10

    • Gag reflex

      • Afferent limb → CN9

      • Efferent limb → CN10

• Innervates the superior oblique muscle → CN4 (trochlear)

• Innervates the lateral rectus → CN6 (abducens)

  • Location? medially

• Anosmia + hypogonadotropic hypogonadism → Kallmann syndrome

• Differentiating between an afferent and efferent pupillary defect

  • Assume R-sided lesion

  • APD 

    • Shine light into R eye → nothing

    • Shine light into L eye → bilateral constriction

  • EPD

    • Shine light into R eye → only L constriction

    • Shine light into L eye → only L constriction

    • Eye with the defect doesn’t constrict regardless

• Most common pediatric brain tumor → pilocytic astrocytoma

  • Location? Posterior fossa

• Hemorrhagic lesion in the cerebellum with a path specimen revealing some kind of rosette (perivascular, Homer Wright) → medulloblastoma

  • 2nd MC pediatric brain tumor

  • Location? Cerebellum

  • Presentation? Ataxia + elevated ICP

  • Associated syndrome? Turcot syndrome (colon cancer + brain tumors)

• Brain tumor that drains “motor oil fluid” and is calcified/could present with tunnel vision → craniopharyngioma 

  • Presentation? Bitemporal hemianopsia 2/2 optic chiasm compression

  • Embryologic origin? Rathke’s pouch

  • Location? Sella turcica

  • Imaging? Calcified

• Kid with abdominal mass that crosses midline with calcifications on imaging → neuroblastoma

  • Alternate location? Posterior mediastinum

  • Presentation? Opsoclonus myoclonus syndrome (“dancing eyes, dancing feet”)

• Neck pain radiating to the arm with specific dermatomes affected and multiple peripheral nerves involved → cervical radiculopathy

  • Pathophys? Problem with the nerve ROOT

    • Versus myelopathy, which is a problem with the spinal cord

• Complete paralysis of the face, arms, and legs with no sensory losses and contralateral “clumsiness” → internal capsule lesion (subcortical lesion)

  • Pathway? Motor fibers from the cortex condense and form the posterior limb of the internal capsule

  • Artery involved? Lenticulostriate arteries

• Inability to calculate + eyes looking towards the side of the lesion + facial paralysis contralateral to the side of the lesion + UMN signs → cortical stroke

  • Acalcula → dominant parietal lobe lesion 

    • Contrast with nondominant parietal lobe (often R) lesion → L-sided hemineglect

  • Eyes looking toward side of the lesion → frontal eye field lesion

    • R front eye field → L PPRF→ controls L abducens and R oculomotor

    • R frontal eye field lesion → no communication to L PPRF → R PPRF unopposed → R deviation

• Complete sensory loss on the left + severe pain on the right → lesion of the thalamus (subcortical lesion)

• Ataxia, past pointing, impaired rapid alternating movements (dysdiadochokinesia), and intention tremor → ipsilateral cerebellar lesion

• Bowel/bladder dysfunction + UMN and LMN findings + sensory level → spinal cord compression

  • E.g. cauda equina syndrome

  • UMN findings below level of lesion

  • LMN findings at level of lesion

• Trouble swallowing + problems with eye adduction on conjugate gaze + sensory loss on the left face + sensory loss on the “right body” → lateral medullary syndrome (Wallenberg syndrome)

  • Pathophys?

    • Sensory loss L face → L spinal trigeminal nucleus

    • Sensory loss R body → L spinothalamic tract

    • Trouble swallowing → nucleus ambiguus lesion (supplies CN9/10)

  • Affected arteries? PICA

• Hemiballismus and bradykinesia → contralateral subthalamic nucleus lesion

• Stocking/glove distribution of sensory loss + LMN findings → peripheral nerve lesion

• Trouble rising from the seated position + Elevated CK → polymyositis/dermatomyositis

  • Pathophys? Problem at the level of the muscle

• Fatigable muscle weakness → neuromuscular junction issue (e.g. MG)

• 4 signs of a basilar skull fracture. 

  • Bruising behind mastoid (“Battle's sign”)

  • Raccoon eyes

  • CSF rhinorrhea

  • CSF otorrhea

• peripheral neuropathy vs. radiculopathy

  • One nerve involved, stocking-glove distribution → peripheral neuropathy

  • Multiple nerves involved, dermatomal distribution → radiculopathy

• Weakness in shoulder abduction/deltoid paralysis. → axillary nerve lesion

  • Cause? 

    • Anterior shoulder dislocation

    • Surgical neck fracture

• Weakness in foot eversion and dorsiflexion → peroneal nerve lesion

  • DroPED → peroneal nerve lesion/eversion/dorsiflexion

• Weakness in foot inversion and plantarflexion → tibial nerve lesion

  • TIP → tibial/inversion/plantarflexion

• Weakness in wrist extension. → radial nerve lesion 

  • Cause? Midshalf fracture of humerus

  • Reflex supplied by radial nerve? triceps

• Problems with thumb abduction with sensory losses on the ventrolateral 3.5 digits. → medial nerve compression in CTS

  • Pathophys? Compression of medial nerve by flexor retinaculum 

  • Risk factors?

    • Pregnancy

    • RA

    • Hypothyroidism

• Problems with hip flexion → femoral nerve

  • F for Femoral and Flexion

• Problems with hip adduction. → obturator nerve

  • Also cannot internally rotation

  • Lots of AIR between thighs (AIR = adduction/internal rotation)

• Weakness with finger spread and problems with flexion at the MCPs and extension at the IP joints  → ulnar nerve lesion

  • Pathophys? Ulnar nerve supplies the dorsal interossei, whose function is finger aBduction (spreading) + MCP flexion + IP joint extension

• Nerve roots controlling the following reflexes:

  • Biceps → C5/6

  • Triceps → C7/8 (radial nerve)

  • Knee extension/patellar tendon reflex → L3/4

  • plantarflexion/ankle jerk reflex → S1/2

• Nerve roots associated with the following levels:

  • Nipple line → T4

  • Xiphoid process → T7

  • Umbilicus → T10

• Paresthesias in pt being treated for TB? INH toxicity

  • Prevention? Vit B6 (pyridoxine)

• Pt with hx of poorly treated Crohn’s disease now with paresthesias + loss of proprioception + lower extremity hyperreflexia → B12 deficiency

  • Pathophys:

    • Crohn’s destroys the terminal ileum, where B12-IF is absorbed

    • B12 deficiency → subacute combined degeneration of spinal cord

      • Destroy dorsal columns → loss of proprioception, vibration, and fine touch

      • Destroy lateral corticospinal tract → UMN signs (spasticity, hyperreflexia)

    • Dx? Macrocytic anemia on CBC + high MMA + high homocysteine

    • Schilling’s test: inject B12 then give radiolabeled oral B12, everything is saturated so oral B12 should go to urine → if not, it indicates pernicious anemia or terminal ileum disease.

      • Then give radiolabeled oral B12 + IF.

      • If you see B12 in the urine now, the problem is pernicious anemia.

      • If not, it’s probably a terminal ileum absorption issue

        • D-xylose test will be abnormal

    • Tx? Supplementation

• Ataxia and hemolytic anemia in a pt with hx abetalipoproteinemia. MRIb shows cerebellar atrophy. → Vit E deficiency 

  • Pathophys? deficiency in microsomal triglyceride transfer protein which is necessary for creating beta-lipoproteins (ApoB48 & ApoB100), which transport fats, cholesterol, and fat-soluble vitamins from intestines to bloodstream

  • Peripheral blood smear finding? Acanthocytosis 

• Mamillary body infarcts in a chronic alcoholic. → Wernecke’s syndrome

  • Pathophys? Thiamine (B1) deficiency

• Dermatitis and dementia in a patient with a long history of carcinoid syndrome. → pellagra → 2/2 niacin deficiency

  • Pathophys? All of the tryptophan is shunted towards serotonin production, so niacin cannot be produced

  • Dx of carcinoid? Urine 5-HIAA (serotonin metabolite) 

  • Other causes of pellagra?

    • Hartnup disease

      • Pathophys? Defect in neutral amino acid transporter

• Child with chronic abdominal pain and foot drop who over the past 6 mo has been performing poorly in school. → lead poisoning

  • Peripheral blood smear findings? ringed-sideroblast

  • Dx? Blood lead levels

    • Confirmatory test? Venous lead level

  • Tx? Lead chelator

    • EDTA

    • succimer

• Weird lines on the fingers + garlic breath. → arsenic poisoning

  • Derm findings? Mees lines (horizontal white lines) on nails 

• Child with declining grades and many staring episodes → absence seizures

  • Seizure type? Generalized seizure

  • EEG findings? 3 Hz spike-and-wave

  • Tx? Ethosuximide (T-type CCB)

• Seizures heralded by olfactory hallucinations and a rising sensation in the abdomen → temporal lobe epilepsy

  • Seizure type? Auras = simple partial seizure

• Seizures associated with sensory/motor problems → parietal/frontal lobe seizures 

• Focal post-seizure paralysis and weakness → Todd’s paralysis

  • Will go away on is own in hours

• Focal seizures = partial seizures (activity localized to 1 hemisphere)

  • Focal seizure w/o loss of awareness = simple partial seizures 

    • Examples:

      • Focal muscle rigidity

      • Bizarre sensations (e.g hearing hissing sound)

      • Autonomic sxs

  • Focal seizure with loss of awareness = complex partial seizures 

    • Can be a person who appears awake but is not aware of surroundings

    • Presentation? Automatisms (e.g. chewing, smacking lips)

• Generalized seizures (activity in both hemispheres)

  • Absence

  • Generalized tonic-clonic

  • Atonic seizures (loss of muscle tone → pt drops to ground)

    • Also consider cataplexy in narcolepsy

  • Myoclonic seizures (jerking movements)

• Brain death = loss of all brain function, including the brainstem

  • Loss of vestibulo-ocular reflex (perform caloric testing)

  • Loss of pupillary light reflex

  • Apnea test → no spontaneous respiration after CO2 is allowed to rise

    • Hypercarbia normally stimulates respiratory center in medulla

• Reflex syncope

  • Syncope with sight of blood, emotional event, pain → vasovagal syncope

    • Dx? Tilt table test

  • Syncope after tightening tie → carotid sinus hypersensitivity

  • Pt trying to pee and they pass out → micturition syncope

• Syncope w/o prodrome in pt with ASCVD risk factors → cardiogenic syncope

• Algorithm for the management of a patient in status epilepticus. 

  • 1st step: benzo (e.g. lorazepam) 

  • 2nd step: IV phenytoin/fosphenytoin

  • 3rd step: sedate with phenobarbital

• AED with the strongest risk of neural tube defects → valproic acid

  • What if the patient is already pregnant and couldn’t be controlled on other agents but is now well-controlled on valproic acid? Continue valproic acid

  • Why? Seizures are very dangerous to the fetus

• Involuntary flailing movement of 1 arm → hemiballismus

  • Pathophys? Lesion in contralateral subthalamic nucleus

• Involuntary movements of the jaw with no other neuro deficits relieved with “gentle stroking” (geste antagoniste) → focal dystonia

  • Tx? Botulinum toxin injection

    • Mechanism? Cleaves SNARE proteins → no vesicle fusion → no ACh release

• Fever, headache, stiff neck → meningitis

  • NBS? Head CT, especially if signs of increased ICP on exam

• Sudden onset severe headache and neck stiffness → SAH

  • NBS? Non-con head CT 

• Fever, headache, and FNDs → brain abscess

  • Dx? Brain MRI

  • Tx? Broad-spectrum abx + drainage by neurosurg

• Recent history of otitis media with FNDs and papilledema on fundoscopic exam → brain abscess

• IVDU with back pain + FNDs → spinal epidural abscess 

  • Dx? MRI spine

  • Tx? Broad-spectrum abx + drainage by neurosurg

• Ataxia, urinary incontinence, and dementia → normal pressure hydrocephalus

  • “Wacky, wet, and wobbly”

  • Tx? Decrease ICP with VP shunt

• Elderly patient with a MMSE of 20/30 with no past neurological history who took Benadryl last night → deliremia  

  • Pathophys? Diphenhydramine has powerful anticholinergic activity

  • Anticholinergic drugs?

    • Drugs for urge incontinence (e.g. oxybutynin, trospium, darifenacin/solifenacin, tolterodine) 

• MMSE 19/30 + difficulty speaking + inability to perform ADLs → Alzheimer’s disease

  • What do you need before formal AD dx? Brain MRI

  • Apo association? 

    • ApoE4 → higher risk 

    • ApoE2 → protective

  • Associated genetic dz? Down syndrome

    • Pathophys? Amyloid precursor protein is on chromosome 21

    • Presentation? AD in 40s

  • Pathology?

    • Senile plaques (extracellular deposits of amyloid)

    • Neurofibrillary tangles (intracellular aggregations of hyperphosphorylated tau protein)

  • Neuroanatomical association? Basal nucleus of Meynert (produces ACh)

  • Enzyme association? ChAT = choline acetyltransferase

  • Tx?

    • Three select AChE-inhibitors

      • Galantamine

      • Donepezil

      • Rivastigmine

    • Memantine (NMDA receptor antagonist)

• Parkinsonian features + visual hallucinations + syncopal episodes → Lewy body dementia

• Dementia + Choreiform movements → Huntington’s disease

  • Inheritance? AD

  • Repeat? CAG

  • Imaging? Atrophy of the caudate

  • Pathophys? Think of it as a high-dopamine disorders

  • Tx? Dopamine antagonist

    • Haloperidol

    • Tetrabenazine (VMAT inhibitor)

• Mild dementia + difficulty speaking + inappropriate behavior + Knife’s edge appearance on brain imaging → Pick’s disease = frontotemporal dementia

• “Stepwise” deterioration in cognitive function w/neuro deficits → vascular dementia

  • Presentation? 5 years ago pt started to be forgetful. 2 years ago this pt xyz.

  • RF? 

    • HLD

    • CAD

    • Hx stroke

  • Tx? Cholinesterase inhibitors used in Alzheimer’s 

• Rapidly progressive dementia in a patient that got a corneal transplant a year ago with myoclonus → Creutzfeldt-Jakob disease

  • CSF findings? Elevated 14-3-3 protein

• Mild dementia + Urinary incontinence + gait problems. → normal pressure hydrocephalus

• Inability to calculate + left to right disorientation. → dominant parietal lobe lesion (Gerstmann's syndrome)

  • Which side in most people? Left

• Neglect of one side of the body. → nondominant parietal lobe lesion

  • Which side in most people? Right

• HY AED side effects 

  • Valproate

    • Highest risk of NTD

    • hepatotoxic

  • Carbamazepine

    • Agranulocytosis

    • SIADH

    • Teratogen

  • Phenytoin

    • Gingival hyperplasia

      • Other drug? cyclosporin

    • Arrhythmias (class 1b antiarrhythmic)

    • Diplopia/visual issues

    • SJS

    • Drug-induced lupus 

      • Ab? anti-histone

    • Fetal hydantoin syndrome (microcephaly + flat nasal bridge + cleft lip/palate)

• 15 yo with jerky hand movements in the morning and occasional generalized tonic clonic seizures. → juvenile myoclonic epilepsy

• Recurrent seizures + contralateral homonymous hemianopia + problems understanding speech (or can talk but speech is not understandable) + recurrent nosebleeds + Positive FOBT → Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)

  • Presentation? Lip telangiectasias 

  • Pathophys? AVMs in multiple organs (e.g. brain, GI tract)

    • In this example, AVM in the temporal lobe

  • Imaging? Can have dystrophic calcifications

  • Inheritance? AD

  • Contrast with Peutz-Jeghers 

    • Hyperpigmented macules on lips

    • multiple nonmalignant hamartomatous polyps in GI tract

    • Increased risk of colon, pancreatic, breast, gyn cancers

• Sudden onset of redness in the eye + “bulging eyes” + “humming sound” on auscultation of the skull + visual acuity 20/100 in the affected eye → carotid cavernous fistula

  • Pathophys? Essentially an AVM

  • PE finding? Bruit on auscultation of the skull

  • Dx? MRA

  • Tx? surgery

• 12 yo M that stands from a seated position by moving his hands over his legs → Duchenne muscular dystrophy

  • Inheritance? X-linked recessive

  • Mutated protein? dystrophy

  • Pathophys? Defective cytoskeleton proteins

  • Prognosis? Death in teens to early 20s

  • Contrast with Becker muscular dystrophy

    • Inheritance? X-linked recessive

    • Pathophys? Mutated dystrophin still has some function

    • Prognosis? Live to 50s+

• Kid that is hypotonic at birth + pediatrician has trouble releasing grip from his mom during a 3 mo well child check OR 25 yo M with “severe balding” → myotonic dystrophy

  • Inheritance? AD

  • Repeat? CTG

  • Mutated gene? DMPK

• 18 yo M presents with a chief complaint of facial weakness and UE weakness that have progressively worsened over the past 18 months → facial-scapulo-humeral dystrophy

  • Presentation? Usually in teens

  • Inheritance? AD

    • Affects boys and girls!

• Polymyositis/Dermatomyositis

  • Presentation? Symmetric proximal muscle weakness

  • Pathophys? Inflammatory myopathies

  • Ab? anti-Jo1, anti-Mi2, anti-SRP

  • Derm findings in dermatomyositis?

    • Gottron's papules

    • Heliotrope rash

    • Shawl sign

  • Associated malignancy? Usually lung cancer or visceral malignancy

  • Dx? CK level

    • If elevated → MRI of muscle (no longer muscle bx)

  • Tx? Steroids

  • Which one can show up in kids? dermatomyositis

• Inclusion body myositis

  • Presentation? Asymmetric distal arm + proximal leg muscle weakness

• Toxidromes

  • Diarrhea + rhinorrhea + sweating + miosis → cholinergic

    • Causes? 

      • Nerve gases (e.g. sarin)

      • Organophosphate poisoning

    • Tx? Atropine + pralidoxime 

  • Mydriasis + constipation + flushing + hot skin + tachycardia → anticholinergic 

    • Causes? 

      • Jimson weed 

  • Pt with psych hx with arrhythmia or wide QRS → TCAs

    • Tx? Sodium bicarb

  • Hypertension (possibly MI) + mydriasis + nasal septum perforation → cocaine

    • Tx? Benzos + alpha-1 blocker (e.g. phentolamine) 

    • What drug is contraindicated? Beta blocker

  • AMS + rash near nose/mouth + nothing on Utox + type 1 RTA → Glue/Toluene

    • Pathophys? Glue sniffing causes hyperadrenergic response, which gives you the high. But this can also cause arrhythmias (often V-fib).

    • Electrolyte imbalance? Hypokalemia due to type 1 RTA

      • No H+ excretion and K+ reabsorption in alpha-intercalated cells

  • Miosis + respiratory depression (RR = 6) → opioid overdose

    • Tx? naloxone 

    • There is no tolerance to which effects of opioids?

      • Miosis

      • Constipation 

  • Normal pupils + respiratory depression → benzodiazepines (could also be barbiturate)

    • Tx? Flumazenil for benzos or Z-drugs

  • Flashbacks + visual hallucinations + synesthesias → LSD

  • Pt that is acting like the hulk + nystagmus → PCP

  • Bad oral hygiene + sympathetic activation → Methamphetamine

  • Hyperthermia + hyponatremia + affectionate behavior → Ecstasy/MDMA 

  • Someone who took drugs now with parkinsonism → MPTP-mediated destruction of substantia nigra

• Symmetric descending flaccid paralysis w/o sensory deficits → botulism 

• Ascending paralysis w/o sensory deficits → GBS

  • CSF findings? Albuminocytologic dissociation

• Seizing patient with small cell lung cancer with a Na of 115 → hyponatremia 2/2 SIADH

  • NBS? Hypertonic saline

    • Only indication on NBMEs is seizing pt with Na+ < 120

• 2 HY electrolyte imbalances that could cause seizures in the infant of a diabetic mother? 

  • Hypoglycemia

    • Pathophys? Hyperplasia of pancreatic islet cells → hypersecretion of insulin → hypoglycemia after delivery

  • Hypocalcemia

    • Associated syndrome? DiGeorge