Neurology Flashcards

1
Q

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

A

Riluzole

Glutamate antagonist. Prolongs tracheostomy-free survival

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2
Q

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

A

Upper and/or lower motor neuron conduction delay

Normal sensory nerve conduction

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3
Q

How is ALS diagnosed?

A

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.)

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4
Q

Spastic weakness is associated with dysfunction of what nerve type?

A

Upper motor neuron

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5
Q

Flaccid weakness is associated with dysfunction of what nerve type?

A

Lower motor neuron

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6
Q

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

A

Nerve conduction studies

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

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7
Q

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

A

Uniform slowing of all motor and sensory nerves

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8
Q

Diagnosis: neurologic disorder with high foot arch and claw toes

A

Charcot-Marie-Tooth disease

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9
Q

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

A

Gabapentin

Pregabalin

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10
Q

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

A

Corticosteroids

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11
Q

What is the most common complication of Bell palsy?

A

Corneal abrasion/ulcer

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

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12
Q

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

A

Eyedrops, patch/tape

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13
Q

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

A

Elevated CSF protein level

normal CSF cell counts

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14
Q

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

A

Bell Palsy

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15
Q

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

A

CVA/TIA

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16
Q

Diagnosis: elevated CSF protein with normal CSF cell counts

A

Guillain-Barre syndrome

albuminocytologic dissociation

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17
Q

Diagnosis: acute ascending flaccid weakness

A

Guillain-Barre syndrome

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18
Q

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

A

ALS

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19
Q

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

A

Charcot-Marie-Tooth disease

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20
Q

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

A

Respiratory failure

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

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21
Q

What is the main treatment for Guillain-Barre syndrome?

A

IVIG

Plasma exchange

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22
Q

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

A

True

speeds recovery times

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23
Q

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

A

False

24
Q

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

A

Anti-acetylcholine receptor antibody (AChR-Ab) titer

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

25
Q

What is the most sensitive diagnostic test for myasthenia gravis?

A

Electromyography (EMG)

26
Q

Diagnosis: nerve study with diminishing signals when repetitive stimulation applied

A

Myasthenia gravis

27
Q

Diagnosis: nerve study with increasing signals when repetitive stimulation applied

A

Lambert-Eaton myasthenic syndrome

Small-cell lung cancer

28
Q

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

A

Thymoma

XR/CT/MRI

29
Q

Thymoma is associated with what neurologic condition?

A

Myasthenia gravis

30
Q

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

A

Pyridostimgine or neostigmine

AChE inhibitor

31
Q

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

A

IVIG or plasmapharesis

Myesthenic crisis

32
Q

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

A

Recent increase in pyridostigmine dosage

Cholinergic signs: miosis, bradycardia, etc.

(Cholinergic crisis is very rare compared to myesthenic crisis)

33
Q

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

A

Atropine

Cholinergic crisis

34
Q

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

A

True

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

35
Q

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

A

True

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

36
Q

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

A

Myasthenic crisis

Edrophonium: short-acting AChE inhibitor

37
Q

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

A

Cholinergic crisis

38
Q

What is the gold-standard diagnostic test for craniopharyngioma?

A

MRI brain W/WO

Calcified suprasellar lesion

39
Q

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

A

Craniopharyngioma

40
Q

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

A

Optic nerve dysfunction (visual field deficit)

Hypopituitarism

41
Q

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

A

Cholinesterase inhibitors

NMDA antagonists

42
Q

What are the main cholinesterase inhibitors used in Alzheimer disease?

A

Donepezil
Rivastigmine
Galantamine

43
Q

What are the main NMDA antagonists used in Alzheimer disease?

A

Memantine

44
Q

How is Alzheimer disease diagnosed?

A

Diagnosis of exclusion

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

45
Q

Diagnosis: progressive dementia with hallucinations and parkinsonian motor dysfunction

A

Dementia with Lewy Bodies

46
Q

What medications are used to treat dementia with Lewy Bodies?

A

For dementia/psychiatric disturbance: donepezil, galantamine, rivastigmine, memantine

For motor disturbance: levodopa/carbidopa

47
Q

Diagnosis: rapidly progressive dementia with myoclonic jerks, spasticity

A

Creutzfeldt-Jakob disease

48
Q

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

A

Tonsil/lymph or brain biopsy

49
Q

What is the most common chronic complication of bacterial meningitis?

A

Hearing loss

Others: intellectual disability, cerebral palsy, seizures

50
Q

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

A

Early antibiotics: ceftriaxone, vancomycin ± acyclovir

Do not delay antibiotics to perform lumbar puncture

51
Q

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

A

Head CT is required prior to lumbar puncture

if structural lesion is present, LP may cause herniation and death

52
Q

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

A

Epidural abscess

53
Q

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

A

MRI

ESR/CRP not specific

54
Q

Diagnosis: acute lower extremity weakness with saddle numbness and incontinence

A

Cauda equina syndrome

55
Q

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

A

MRI

56
Q

How is cauda equina syndrome treated?

A

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)