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Laughing Pedi > neuro > Flashcards

neuro Flashcards

0
Q

night terror vs nightmare

A

night terror in early part of night and child has no recollection

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1
Q

normal age for breath holding spells?
What is the pathophys
Possible tx?

A

about 1 yr: 6 mos-2yr
Always triggered
Would have tachycardia then brain hypoxia, then rare GTC
May treat with iron, but controversial

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2
Q

recurrence of febrile sz

Risk of epilepsy

A

30% after 1st
50% after 2 or more

Epilepsy: about 1-2% (doubled risk)

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3
Q

Avoid what meds in Dravet?

A

Na channelopathy, avoid Na channel blockers

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4
Q

Na channel blockers

A

Phenytoin, Lamotrigine, Carbamaz, Oxcarbamaz

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5
Q

What is Dravet?

A

SMEI

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6
Q

Tx of JME?

Prognosis

A

VPA or topamax

Need to stop living the teenage life, but otherwise good prognosis on meds

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7
Q

Topiramate side effects

A

cognitive

nephrolithiasis

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8
Q

Bad AED combo…

VPA and what?

A

Lamotrigine (NO DEPAKOTE/LAMICTAL)

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9
Q

commonest cause of CMT

A

demyelinating, duplication PMP 22 gene

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10
Q

mechanism in Friedreich ataxia

when does it show up

A

GAA trinucleotide repeat expansion

shows up in latter half of 1st decade

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11
Q

loss of ankle jerk and a babinski? What is it?

A

Friedreich ataxia (others don’t happen in kids: spinocerebellar degen and tabes dorsalis

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12
Q

prevalence of migraine in adolescents

A

15%

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13
Q

asymmetric crying faces
which side abnormal
other issues?

A

drooping side is normal, absent DAOM in 80%

check for heart prob (assoc anomaly)

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14
Q

3 mutations in RETT

A

MECP2, CDKL5, FOX

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15
Q

abnormal CBC< ataxic gait, oculocutaneous lesions?

High risk of what?

A

Ataxia telangiectasia

Risk for Hodgkins and leukemia

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16
Q

immunologic issues in ataxia telangiectasia

A

T cell dysfunction, low Ig levels

Lots of respiratory tract infx

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17
Q

which occurs in younger kids: Ataxia telangiectasia or Friedreich ataxia?

A

Younger: TA

Older FA: late childhood / early adol

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18
Q

ataxia, high arch foot, no LE reflexes, DM, cardiomyopathy/CHF

A

Friedreich Ataxia

Fried Arch: cardiac / arch issues

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19
Q

relationship between when sydenham chorea and strep occur?

A

chorea months after strep infx, after antistreptococcal ab titers have normalized

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20
Q

chorea, hypotonia / rigidity, emotional lability

A

Huntington’s chorea

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21
Q

how long must tics be present for Tourette’s dx

A

1 yr

22
Q

possible treatments for GBS ascending paralysis

A

IVIG or plasmapharesis. NOT steroids

23
Q

antibodies often present in myasthenia gravis?

A

anti smith Abs, anti-Acetylcholine receptor Abs

24
Q

congenital myasthenia gravis cause/prognosis?

A

lifelong from genetic defect of NMJ

25
Q

how to clinically distinguish between congenital MG and transient (6wk) MG?

A

transient does NOT involve eyes.

26
Q

how does botulinum toxin work?

A

blocks release of Ach from NMJ

27
Q

gene for DMD/Beckers?

A

xp21

28
Q

proportion of time in DMD that mom is a carrier?

A

2/3

29
Q

Steinart disease, also known as what?

What does it involve?

A

Myotonic Muscular Dystrophy

Involves smooth and striated muscle

30
Q

steroid treatment for spinal cord compression vs spinal trauma?

A

compression: dexamethasone
Trauma: Methylprednisolone 30mg/kg x 1 hour

31
Q

why fosphenytoin compared to phenytoin?

A

phenytoin can produced purple glove syndrome (skin necrosis)

32
Q

% of kids who get febrile szs?

A

3% by 5th bday

33
Q

pedi boards use what drug to treat benign rolandic seizures?

A

carbamazepine

34
Q

tx for JME?

A

vpA

35
Q

most common risk factor for premies developing CP

A

perinatal infx

36
Q

New drug for Wilson’s? What else for Tx?

A

Tridentine and give zinc

37
Q

Criteria for Lennox Gastaut and Rx

A
  1. Many seizure types: Atonic, Tonic, GTC, absence atypical
  2. DD / ID
  3. Spike and slow wave on EEG
    Tx: Felbamate (Aplastic anemia), Banzel
38
Q

3-5 Hz spike and wave?

Tx?

A

JME

Tx: VPA, Lamictal

39
Q

HC rule?

A

35 at birth, then 3 mos/ 9 mos rule + 5cm each

40
Q

No more than what head growth per week in first three mos?

A

0.5cm / week

41
Q

retinal lacunes, agenesis of corpus callosum, vertebral anomaly

A

Aicardi syndrome

42
Q

SMA gene and chromosome

A

SMN 2 on chromosome 5

43
Q

anchoring of cord of conus medullaris due to thickened filum terminale
Dx and what level

A

tethered cord, L2 or below

44
Q

defect in botulism

EMG finding

A
  1. no release of ACh

2. EMG incremental response to repetitive rapid stim

45
Q

neonatal vs congenital myasthenia

A

Neonatal is transient and does NOT involve opthalmoplegia

46
Q

EMG with decrement with repetitive stim

A

myasthenia

47
Q

CTG repeat on chr 19 with anticipation

A

myotonic dystrophy

48
Q

features of what:

Frontal baldness, endocrine issues, cataracts, cognitive

A

Myotonic dystrophy

49
Q

Rx for myotonic dystrophy

A

mexilitine, phenytoin, carbamazepine

50
Q

weakness pattern in nemaline myopathy

A

neck, face, proximal muscles

51
Q

marker for ataxia telangiectasia

A

high AFP

52
Q

NF1 chromosome

TS chromosomes

A

NF: 17
TS: 9., 16

53
Q

tram track calcifications on XR/CT?

A

Sturge Weber under angioma

Laughing Pedi (24 decks)

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