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Pediatric Boards > Neurology > Flashcards

Neurology Flashcards

1
Q

Presentation of Wilson disease?

A

Younger–liver disease; older–neuro/psych symptoms (tremors, emotional problems, handwriting difficulty, depression, abnormal eye movements)

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2
Q

Lab findings in Wilson disease?

A

Low copper and low ceruloplasmin (copper deposited in tissues, not in serum)

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3
Q

Treatment of Wilson disease?

A

D-penicillamine

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4
Q

Causes of ataxia (“TIN” as in the Tin-Man)?

A

1) Toxic ingestions (ethanol, pesticides)
2) Infections (including Guillain Barre and post-infectious)
3) Neoplasm
- Also possibly metabolic disease or cerebral hemorrhage

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5
Q

Presentation of ataxia telangiectasia?

A

Ataxic gait, skin/eye telangiectasias, +/- MR, low Ig’s and T-cell dysfunction, increased malignancies (HD, leukemia)

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6
Q

Presentation of Freidreich ataxia?

A

Presents in late childhood/early adolescence, slow/clumsy gait, decreased strength, decreased reflexes, elevated plantar arch, diabetes, CHF

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7
Q

Inheritance and presentation of Huntington chorea?

A

AD; p/w chorea, hypotonia, emotional lability (juvenile more likely to present with hypertonia)

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8
Q

Do negative ASO/anti-DNase B titers rule out Syndemham chorea?

A

NO!!

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9
Q

How do simple motor tics present?

A

Eye blinking, head/face/shoulder movements

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10
Q

How do choreiform movements present?

A

Repetitive, jerking movements–cannot be suppressed

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11
Q

How long are tics present to diagnose Tourette syndrome?

A

At least 1 year

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12
Q

Comorbidities of Tourette syndrome?

A

ADHD, OCD (stimulants may unmask tics, but do not cause them)

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13
Q

Medications that can cause pseudotumor cerebri?

A

High dose vitamin A, steroids, thyroxine, lithium, some abx

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14
Q

Bacteria associated with Guillai Barre syndrome?

A

Campylobacter jejuni

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15
Q

Treatment of choice for myasthenia gravis?

A

Pyridostigmine (acetylcholinesterase inhibitor); thymectomy (if thymoma is present)

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16
Q

Difference between congenital (life-long) myasthenia gravis and transient (Mom’s antibodies) myasthenia gravis?

A

Transient does not involve the eyes; congenital does (C for congenital and conjunctiva)

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17
Q

Presentation of infantile botulism?

A

Weak cry, constipation, listlessness, hypotonia, poor feeding, descending paralysis

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18
Q

Inheritance of Duchenne muscular dystrophy?

A

X-linked recessive

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19
Q

What distinguishes Duchenne muscular dystrophy from other neuromuscular disorders?

A

Absence of both tongue fasciculations and eye muscle involvement

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20
Q

Inheritance of myotonic dystrophy?

A

AD

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21
Q

Empiric antibiotics for brain abscess?

A

Vanco, ceftriaxone, metronidazole

22
Q

Treatment of spinal epidural abscess?

A

Abx with good staph coverage and surgical decompression

23
Q

What is transverse myelitis?

A

Post-infectious demyelination of nerves and spinal cord due to inflammation

24
Q

Presentation of transverse myelitis?

A

Acute onset of weakness, hypotonia, and decreased reflexes; progression to hypertonia and hyperreflexia; bowel/bladder dysfunction

25
Q

Treatment of transverse myelitis?

A

Supportive care (most will have spontaneous recovery in a few months)

26
Q

Presentation of SMA type 1?

A

Only motor, no sensory–hypotonia, weakness, poor suck, tongue fasciculations

27
Q

Difference between infantile botulism and SMA type 1?

A

Tongue fasciculations

28
Q

What is Todd’s paralysis?

A

Post-ictal focal motor weakness

29
Q

EEG findings for absence seizures?

A

3 Hz spike and wave

30
Q

Differences between absence and complex partial seizures?

A

Absence seizures start and end abruptly (no post-ictal), and CPS may start as simple partial and end gradually with post-ictal; absence are also short (<30s)

31
Q

What diagnositic maneuver can induce absence seizure?

A

Hyperventilation

32
Q

Treatment of absence seizures?

A

Ethosuximide or valproic acid

33
Q

Treatment of generalized tonic-clonic seizures?

A

Valproic acid, phenytoin, carbamazepine, lamotrigine (watch out for SJS)

34
Q

What are myclonic seizures?

A

Single or repetitive contractsion of isolated groups of muscles–“shock-like muscle twitches”

35
Q

How to differentiate tics from seizures?

A

Tics can be suppressed

36
Q

What is the triad in infantile spasms (West syndrome)?

A

1) Infantile spasms
2) Hypsarrhythmia on EEG
3) Developmental delay

37
Q

What do infantile spasms look like?

A

Sudden flexion or extension of body (similar to Moro reflex); may occur 20-30 in a row

38
Q

What genetic syndrome is associated with infantile spasms?

A

Tuberous sclerosis (babies with “tuberous” sclerosis love their potatoes)

39
Q

Treatment of infantile spasms?

A

ACTH, steroids, benzos, focal resection?

40
Q

Treatment of simple partial seizures?

A

Carbamazepine (treatment not always necessary)

41
Q

What are Benign Rolandic seizures?

A

Self-limited focial motor seizure, often with unilateral sensory involvement

42
Q

When do Benign Rolandic seizures occur?

A

Most often at night when child is asleep; associated with migraines

43
Q

Inheritance and treatment of Benign Rolandic seizures?

A

AD; treat with carbamazepine (may not always be indicated)

44
Q

Treatment of choice for juvenile myoclonic epilepsy?

A

Valproic acid

45
Q

How long seizure-free before withdrawing meds?

A

2 years (don’t withdraw if seizure onset >12yo, neonatal seizures, or multiple meds tried before control)

46
Q

Presentation of adrenoleukodystrophy?

A

Difficulty paying attention and concentrating, clumsy (abnormal motor function), adduction of contralateral leg with patellar reflex

47
Q

What is cerebral palsy?

A

Static encephalopathy with gross and fine motor abnormalities evident early in life

48
Q

Most common risk factor for CP in premature babies?

A

Perinatal infections

49
Q

Presentation of labyrinthitis?

A

Vertigo lasting hours or days, hearing loss

50
Q

Presentation of stroke in mitochondrial disorder?

A

Elevated lactate, sensorineural hearing loss, stroke

51
Q

Order of cognitive impairment in CP?

A

Spastic quadriplegia > spastic hemiplegia > spastic diplegia (best)

Pediatric Boards (12 decks)

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