Neurology

Question 1

What medication(s) are shown to modify the disease process of ALS?

Answer 1

Riluzole

Glutamate antagonist. Prolongs tracheostomy-free survival

Question 2

ALS is associated with what findings on nerve conduction studies or electromyography?

Answer 2

Upper and/or lower motor neuron conduction delay

Normal sensory nerve conduction

Question 3

How is ALS diagnosed?

Answer 3

Clinical or electrophysiological evidence of progressive LMN and UMN degeneration

(No gold standard diagnostic test. CPK often elevated. MRI/CT is done to exclude other conditions.)

Question 4

Spastic weakness is associated with dysfunction of what nerve type?

Answer 4

Upper motor neuron

Question 5

Flaccid weakness is associated with dysfunction of what nerve type?

Answer 5

Lower motor neuron

Question 6

What is the first diagnostic test used for suspected Charcot-Marie-Tooth disease?

Answer 6

Nerve conduction studies

Biopsy and DNA testing is used, although not all CMT genes are known.

Question 7

Charcot-Marie-Tooth disease is associated with what findings on nerve conduction studies?

Answer 7

Uniform slowing of all motor and sensory nerves

Question 8

Diagnosis: neurologic disorder with high foot arch and claw toes

Answer 8

Charcot-Marie-Tooth disease

Question 9

What main medication(s) are beneficial for symptomatic control of peripheral neuropathy?

Answer 9

Gabapentin

Pregabalin

Question 10

What medication(s) improve recovery times for Bell palsy?

Answer 10

Corticosteroids

Question 11

What is the most common complication of Bell palsy?

Answer 11

Corneal abrasion/ulcer

From severe dry eye and scratching due to inability to blink. Prevent with lubricant drops and eye patch/tape.

Question 12

How is corneal ulceration prevented in the setting of Bell palsy?

Answer 12

Eyedrops, patch/tape

Question 13

Guillain-Barre Syndrome is associated with what findings on CSF studies?

Answer 13

Elevated CSF protein level

normal CSF cell counts

Question 14

Diagnosis: unilateral facial droop with weakness of upper and lower facial muscles

Answer 14

Bell Palsy

Question 15

Diagnosis: unilateral facial droop with weakness of lower facial muscles and preservation of upper facial muscles

Answer 15

CVA/TIA

Question 16

Diagnosis: elevated CSF protein with normal CSF cell counts

Answer 16

Guillain-Barre syndrome

albuminocytologic dissociation

Question 17

Diagnosis: acute ascending flaccid weakness

Answer 17

Guillain-Barre syndrome

Question 18

Diagnosis: chronic acquired progressive upper and lower motor neuron dysfunction with preserved sensation

Answer 18

ALS

Question 19

Diagnosis: hereditary progressive sensory and motor neuron dysfunction, beginning with distal extremities

Answer 19

Charcot-Marie-Tooth disease

Question 20

What is the main feared complication of Guillain-Barre syndrome?

Answer 20

Respiratory failure

(Diaphragmatic paralysis. One third of GBS patients require ICU admission for this. Additional severe complications include hypotension/arrhythmia due to autonomic instability.)

Question 21

What is the main treatment for Guillain-Barre syndrome?

Answer 21

IVIG

Plasma exchange

Question 22

True or false: steroids have a role in the treatment of Bell Palsy

Answer 22

True

speeds recovery times

Question 23

True or false: steroids have a role in the treatment of Guillain-Barre syndrome

Answer 23

False

Question 24

What is the first step in diagnosis of suspected myasthenia gravis?

Answer 24

Anti-acetylcholine receptor antibody (AChR-Ab) titer

Does not correlate with disease severity. EMG is more sensitive but more invasive.

Question 25

What is the most sensitive diagnostic test for myasthenia gravis?

Answer 25

Electromyography (EMG)

Question 26

Diagnosis: nerve study with diminishing signals when repetitive stimulation applied

Answer 26

Myasthenia gravis

Question 27

Diagnosis: nerve study with increasing signals when repetitive stimulation applied

Answer 27

Lambert-Eaton myasthenic syndrome | Small-cell lung cancer

Question 28

Diagnosis of myasthenia gravis requires additional imaging to check for what concurrent condition?

Answer 28

Thymoma | XR/CT/MRI

Question 29

Thymoma is associated with what neurologic condition?

Answer 29

Myasthenia gravis

Question 30

What is the initial medical therapy for newly-diagnosed myasthenia gravis?

Answer 30

Pyridostimgine or neostigmine | AChE inhibitor

Question 31

Treatment: acute severe weakness in a patient with known myasthenia gravis

Answer 31

IVIG or plasmapharesis | Myesthenic crisis

Question 32

In a myasthenia gravis patient with acute severe weakness, what features are concerning for cholinergic crisis?

Answer 32

Recent increase in pyridostigmine dosage Cholinergic signs: miosis, bradycardia, etc. (Cholinergic crisis is very rare compared to myesthenic crisis)

Question 33

Treatment: acute severe weakness and bradycardia in a patient with known myasthenia gravis who was known to have recently increasing pyridostigmine dosage

Answer 33

Atropine | Cholinergic crisis

Question 34

True or false: thymectomy is generally indicated for patients with thymoma and myasthenia gravis

Answer 34

True | It is also indicated in patients age 10-55 with myasthenia gravis even WITHOUT thymoma

Question 35

True or false: steroids have a role in the treatment of myasthenia gravis

Answer 35

True | used in patients that do not respond sufficiently to AChE inhibitors and thymectomy

Question 36

Diagnosis: acute weakness in patient with myasthenia gravis, transiently improved with edrophonium

Answer 36

Myasthenic crisis | Edrophonium: short-acting AChE inhibitor

Question 37

Diagnosis: acute weakness in patient with myasthenia gravis, transiently worsened with edrophonium

Answer 37

Cholinergic crisis

Question 38

What is the gold-standard diagnostic test for craniopharyngioma?

Answer 38

MRI brain W/WO | Calcified suprasellar lesion

Question 39

Diagnosis: child with chronic headache, visual field deficit, and growth failure

Answer 39

Craniopharyngioma

Question 40

A growing craniopharyngioma will compress surrounding structures, causing what secondary deficits?

Answer 40

Optic nerve dysfunction (visual field deficit) | Hypopituitarism

Question 41

What are the main categories of Alzheimer disease medications, by mechanism?

Answer 41

Cholinesterase inhibitors NMDA antagonists

Question 42

What are the main cholinesterase inhibitors used in Alzheimer disease?

Answer 42

Donepezil Rivastigmine Galantamine

Question 43

What are the main NMDA antagonists used in Alzheimer disease?

Answer 43

Memantine

Question 44

How is Alzheimer disease diagnosed?

Answer 44

Diagnosis of exclusion | First step is to rule out alternate diagnoses including B12 deficiency, hypothyroidism, syphilis, HIV, depression

Question 45

Diagnosis: progressive dementia with hallucinations and parkinsonian motor dysfunction

Answer 45

Dementia with Lewy Bodies

Question 46

What medications are used to treat dementia with Lewy Bodies?

Answer 46

For dementia/psychiatric disturbance: donepezil, galantamine, rivastigmine, memantine For motor disturbance: levodopa/carbidopa

Question 47

Diagnosis: rapidly progressive dementia with myoclonic jerks, spasticity

Answer 47

Creutzfeldt-Jakob disease

Question 48

What is the gold-standard diagnostic test for Creutzfeldt-Jakob disease?

Answer 48

Tonsil/lymph or brain biopsy

Question 49

What is the most common chronic complication of bacterial meningitis?

Answer 49

Hearing loss | Others: intellectual disability, cerebral palsy, seizures

Question 50

What is the first step in management of a patient with acute fever, headache, and nuchal rigidity?

Answer 50

Early antibiotics: ceftriaxone, vancomycin ± acyclovir | Do not delay antibiotics to perform lumbar puncture

Question 51

How would a finding of focal weakness or numbness on neurologic exam affect the management of a patient with suspected meningitis?

Answer 51

Head CT is required prior to lumbar puncture | if structural lesion is present, LP may cause herniation and death

Question 52

Diagnosis: IV drug abuser with fever, back pain and vertebral tenderness

Answer 52

Epidural abscess

Question 53

What is the gold-standard diagnostic test for epidural abscess?

Answer 53

MRI | ESR/CRP not specific

Question 54

Diagnosis: acute lower extremity weakness with saddle numbness and incontinence

Answer 54

Cauda equina syndrome

Question 55

What is the gold-standard diagnostic test for cauda equina syndrome?

Answer 55

MRI

Question 56

How is cauda equina syndrome treated?

Answer 56

Steroids + surgical decompression + treatment of underlying cause (infection or neoplasm) (Antibiotics for infectious causes. Radiation/chemo for neoplastic causes. Discectomy if related to herniated disc) (Surgical decompression ideally performed within 6 hours of onset)