Too much & Too Little
Jiens
Side Effects :(
Cranial Nerves
Misc
100

Match the following toxicities with their symptoms (Aluminum, copper, Manganese, methanol): 

1. Parkinsonism, toe walking with flexed elbows, psychiatric manifestations

2. necrosis/hemorrhage of putamen and toxic optic neuropathy; headache, confusion, HAGMA

3.  encephalopathy, ataxia, tremor, spinocerebellar degeneration

4. subacute combined degeneration, can be caused by zinc toxicity


Manganese: Parkinsonism, toe walking with flexed elbows, psychiatric manifestations (prolonged parenteral nutrition)

- Manganese: T1 GPi hyperintensity
- Carbon monoxide: T2 GPi hyperintensity

2. Methanol: necrosis/hemorrhage of putamen and toxic optic neuropathy; headache, confusion, HAGMA

3. Aluminum: encephalopathy, ataxia, tremor, spinocerebellar degeneration

-Exposure in factories/cooking methadone in aluminum pots

4. Copper: subacute combined degeneration, caused by zinc toxicity


100

  1. What condition presents with peripheral neuropathy, palpable, enlarged nerves, retinitis pigmentosa, ataxia, and elevated in phytanic acid

Refsum Disease

100

Inducer or inhibitor of CYP enzyme: 

Phenytoin

Diltiazem

Valproic Acid

Primidone

Fluconazole

Phenobarbital

Carbamazepine

  1. Phenytoin (inducer)

  2. Primidone (inducer)

  3. Carbamazepine (inducer)

  4. Phenobarbital (inducer)

  5. Valproate (inhibitor)

  6. Diltiazem (inhibitor)

  7. Fluconazole (inhibitor)

100

Differentiate affected nerves in cavernous sinus lesions with superior orbital fissure

  1. Cavernous Sinus: III, IV, VI, V1, sympathetic fibers

  2. Superior orbital fissure: III, IV, VI, V1, V2, sympathetic fibers

100

42 year old presents with a 5 year history of chorea, dysarthria, and has been more irritable and managing finances. Has choreiform movements of face, trunk and extremities. LFTs are mildly elevated. MRI reveals marked atrophy of the caudate nucleus and putamen

Chorea-Acanthocytosis

200

Describe the likely drug intoxication: 

  1. nystagmus, tachycardia, muscle rigidity, decreased response to pain, seizures

  2. miosis, hypotension, bradycardia, decreased respirations

  3. mydriasis, euphoria, tachycardia

  4. posterior and lateral column myelopathy

1. PCP: nystagmus, tachycardia, muscle rigidity, decreased response to pain, seizures

2. Opioids: miosis, hypotension, bradycardia, decreased respirations

3. Cocaine/amphetamines: mydriasis, euphoria, tachycardia

4. Nitrous oxide: posterior and lateral column myelopathy

200

Describe the deficient enzyme in a patient with painful small fiber neuropathy, angiokeratomas, progressive renal disease, and stroke

Alpha-galactosidase -- Fabry Disease

200

Name two seizure medications that need to be actively monitored during pregnancy due to glucuronidation

Oxcarbazepine and lamotrigine

200

What foramina do the following nerves/arteries traverse to exit the skull? 

1. V1

2. V2

3. V3

4. Greater and deep petrosal nerves

5. middle meningeal artery/vein

  1. V1: Superior orbital fissure

  2. V2: foramen rotundum

  3. V3: foramen ovale 

  4. Greater & deep petrosal nerves: foramen lacerum

  5. Middle meningeal artery/vein and meningeal branch of V3: foramen spinosum 

200

Name 2 of the 3 classic GAD-antibody neurologic syndromes: 

  1. Limbic encephalitis

  2. Cerebellar ataxia

  3. Stiff person syndrome 

300

Which toxicities explain these symptoms when paired with abdominal pain/GI symptoms: 

1. Headache, motor neuropathy (radial nerve preferentially), bluish discoloration

2. Garlic breath, neuropathy (like diabetic neuropathy)

Lead: headache, motor neuropathy (radial nerve preferentially), bluish coloration

Arsenic: garlic breath, neuropathy (like diabetic neuropathy)

300

  1. Describe the enzyme that explains the following symptoms: patient with rapid onset fatigue with exercise that improves with continued activity. There is no evidence of of increase in lactic acid after exercise compared to pre-exercise levels

Myophosphorylase deficiency - McArdle disease

300

Name the MS DMT once that is associated with the following symptoms: 

1. Flushing

2. Macular Edema

3. Category X for teratogenicity alongside hepatotoxicity

4. Neutralizing antibodies

5. Dose-related cardiotoxicity

6. Highest risk of PML

  1. Flushing (dimethyl fumarate) 

  2. Macular edema (siponimod)

  3. Category X for teratogenicity alongside hepatotoxicity (teriflunomide)

  4. Neutralizing antibodies (interferon beta)

  5. Dose-related Cardiotoxicity (mitoxantrone)

  6. Highest risk of PML (Natalizumab)

300

This cranial nerve is often the culprit in a syndrome with attacks of pain triggered by swallowing. This can also be accompanied by syncope. What is the nerve affected and how can this cause syncope?

Glossopharyngeal neuralgia -- continued pain causes syncope due to connection with carotid body

300

Describe which condition/pathology is seen in this imaging

  1. MSA: top left

  2. PSP: bottom left

  3. CJD: bottom right

  4. Septo-Optic dysplasia: middle bottom

  5. NMO: top right/middle

400

Match the following drugs with their respective withdrawal syndromes (MDMA, Cocaine, Heroin, Alcohol, SSRI): 

1.  anxiety, tachycardia, sweating, seizure, hallucinations

2. diaphoresis, piloerection, hypersalivation, mydriasis, N/V, paranoia

3. hyperthermia, diaphoresis, jaw soreness

4. depression and insomnia

5. hypersomnia, fatigue, hyperphagia, dysphoria


BONUS: which SSRI is most likely to have withdrawal symptoms and why?

1. Alcohol: anxiety, tachycardia, sweating, seizure, hallucinations

2. Heroin: diaphoresis, piloerection, hypersalivation, mydriasis, N/V, paranoia

3. MDMA: hyperthermia, diaphoresis, bruxism

4. SSRI: depression and insomnia -- Paroxetine is most common shortest half life

5. Cocaine: hypersomnia, fatigue, hyperphagia, dysphoria

400

Compare and contrast hyperkalemia and hypokalemic periodic paralysis based on the following: Gene mutation, duration of weakness, age of onset

Hyperkalemic: SCN4A, attacks last < few hours, age of onset before 10

Hypokalemic: CACNA1S, attacks last hours-days, age in second-third decade of life

400

Name the ASM associated with their side effect (if more than one applies, which has highest risk): 

1. Hyponatremia

2. PR interval prolongation

3. Gingival hyperplasia

4. Calcium phosphate kidney stones

5. Contraindicated in patient with sulfa allergy

  1. Hyponatremia (oxcarbazepine)

  2. PR interval prolongation (lacosamide)

  3. Gingival hyperplasia (phenytoin)

  4. Calcium phosphate kidney stones (topiramate)

  5. Contraindicated in patient with sulfa allergy (zonisamide)

400

Differentiate symptoms, location of lesion (including side): 

1. Right one and a half syndrome

2. Right internuclear ophthalmoplegia

3. right CN III palsy

  1. Right One and a Half syndrome

    1. R VI nucleus/PPRF and adjacent MLF

    2. R eye without lateral movement, Left eye can only ABduct

  2. Right INO

    1. Named for eye that CANNOT ADuct

    2. Right MLF, left nystagmus  looking to the left, CAN accommodate

  3. Right CN III palsy: Ptosis, mydriasis, down and out pupil, however CANNOT accommodate 
400

Place the following disorders into correct disorder categories (cell migration, cell proliferation, cell organization): 

Pachygyria, megalencephaly, Lissencephaly, polymicrogyria, heterotopia, focal cortical dysplasia, microcephaly

  1. Cell migration

    1. Lissencephaly (folding requires migration of cortical cells)

    2. Pachygyria

    3. Heterotopias

  2. Cell proliferation

    1. Microcephaly

    2. Megalencephaly

  3. Cell organization

    1. Polymicrogyria

    2. Focal cortical dysplasia

500

Compare and contrast Neuroleptic Malignant Syndrome and Serotonin Syndrome: 

NMS: Severe rigidity, CK elevated, decreased reflexes, normal pupils, AMS, hyperthermia 

Serotonin syndrome: AMS, rigidity, hyper-reflexia, autonomic hyperactivity (including hyperthermia), tremor/myoclonus, normal pupils

500

Describe the gene associated with each set of symptoms: 

1. Sensory neuropathy, low HDL, orange and enlarged tonsils, corneal clouding

2. Sensorimotor, length dependent polyneuropathy, ocular and dermal telangiectasis, immunoglobulin deficiency, imbalance

3. Repeated Isolated nerves palsies at common compression sites, conduction blocks at sites of pressure, evidence of tomacula on pathology 

1. Sensory neuropathy, low HDL, orange and enlarged tonsils, corneal clouding (ABCA1 - Tangier)

2. Sensorimotor, length dependent polyneuropathy, ocular and dermal telangiectasis, immunoglobulin deficiency, imbalance (ATM - ataxia teleangiectasia)

3. Repeated Isolated nerves palsies at common compression sites, conduction blocks at sites of pressure, evidence of tomacula on pathology (PMP22 deletion - HNPP)

500

Describe which of the following medications is help or hurt treatment of JME: 

1. Valproic Acid

2. Zonisamide

3. Gabapentin

4. Lamotrigine

5. Topiramate

6. Carbamazepine

  1. Valproic acid (yay)

  2. Zonisamide (yay)

  3. Gabapentin (ow)

  4. Lamotrigine (yay)

  5. Topiramate (yay)

  6. Carbamazepine (ow)

500

This mitochondrial condition is distinguished by ophthalmoplegia without double vision, retinitis pigmentosa, and heart conduction abnormalities. Additionally, sensorineural hearing loss, and cerebellar ataxia are common. What is this condition name and why is there no double vision with his/her ophthalmoplegia?

  1. Kearns-Sayre

  2. Chronic progressive external ophthalmoplegia (classically no double vision due to slow progression of symptoms) 

500

Describe the direct and indirect pathways of the basal ganglia: 

  1. Direct (D1 receptors): Movement

    1. Cortex excites putamen

    2. Substantia nigra also excites putamen

    3. Putamen inhibits GPi

    4. GPi disinhibits thalamus

    5. Thalamus activates cortex

  2. Indirect (D2 receptors): turns ON the thing in cortex that will cause inhibition of movement

    1. Cortex stimulates Putamen

    2. Substantia nigra also stimulates Putamen

    3. Putamen inhibits GPe

    4. GPe disinhibits STN 

    5. STN then can stimulate GPi (activating it)

    6. GPi inhibits thalamus

    7. Thalamus stimulates cortex

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