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Pediatrics > BRS- Neuro > Flashcards

BRS- Neuro Flashcards

1
Q

Contrast hypotonia and weakness

A
  • hypotonia: decreased resistance of movement during passive stretch
  • weakness: less force for active contraction
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2
Q

Cause of central vs peripheral hypotonia

A
  • central: upper motor neuron dysfxn

- peripheral: LMN dysfxn

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3
Q

Antenatal/neonatal signs of hypotonia

A

antenatal: decreased fetal movement, breech presentation (peripheral)
neonatal: seizures (central)

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4
Q

PE findings in both central and peripheral hypotonia

A

decreased movement
frogleg posture
muscle contractures

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5
Q

Central hypotonia workup

A
  • CT
  • serum electrolytes
  • chromosome studies
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6
Q

Peripheral hypotonia workup

A
  • CK levels
  • DNA tests
  • EMG
  • muscle biopsy
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7
Q

Four possible areas lesioned in peripheral hypotonia

A

1) spinal cord
2) peripheral nerves
3) NMJ
4) muscle

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8
Q

Three disorders affecting the NMJ/ causing hypotonia

A
  • botulism
  • myasthenia gravis
  • mag tox
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9
Q

Disorder affecting the spinal cord causing hypotonia?

A

spinal muscular atrophy

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10
Q

Disorder affecting the peripheral nerves causing hypotonia

A

familial dysautonomia

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11
Q

Two most common neuromuscular disorders?

A 
#1: DMD 
#2: SMA
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12
Q

SMA:

  • affects what cells?
  • chromosome
  • gene
  • inheritance
A
  • anterior horn cells
  • chromosome 5
  • SMN1 (survival motor neuron)
  • AR
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13
Q

Three types of SMA:

A

1: infantile/ Werdnig Hoffman (less than 6 mos)
2: intermediate 6-12 mos
3: juvenile, less than 3 years

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14
Q

3 PE findings in SMA

A
  • tongue fasiculations
  • bell shaped chest
  • normal sensation
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15
Q

Gold standard for diagnosis of SMA

A

DNA testing

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16
Q

Prognosis for SMA:

A

type 1: rare to live beyond 1st year

II-II: survive to adolescent/adult hood

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17
Q

Botulism:

  • weakness type
  • mechanism of weakness
  • time from ingestion to onset
A
  • bulbar weakness, symmetric and descending
  • prevents presynaptic release of Ach
  • 12-48 hours after ingestion of spores
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18
Q

Treatment botulism

How long until recovery?

A

IVIG
abx contraindicated
full recovery in weeks –> months

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19
Q

Congenital myotonic dystrophy:

  • inheritance pattern
  • parent affected
  • time of onset
A

-AD
-mother
-myotonia onset at age 5
(but may have neonatal feeding/ respiratory trouble)

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20
Q

Myotonic dystrophy:

  • typical facial appearance
  • typical IQ
  • three other features
A
  • temporalis/ masseter atrophy
  • IQ 50-65
  • cataracts, arrhythmias, infertility
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21
Q

Three types of hydrocephalus:

A
  • noncommunicating: obstruction
  • communicating (^ production/ low reabsorption)
  • hydrocephalus ex vacuo (atrophy)
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22
Q

Describe Chiari II malformation

A
  • lumbosacral myelomeningocele

- medullary/cerebellar displacement through foramen magnum

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23
Q

Describe Dandy Walker malformation

A

-absent cerebellar vermis + dilation of the fourth ventricle

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24
Q

Congenital aqueductal stenosis:

  • inheritance pattern
  • other assc abnormalities
A
  • X linked
  • thumb abnormalities
  • spina bifida
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25
Q

Cause of sunset sign in hydrocephalus

A

-eyes down because third ventricle enlargement causes pressure on the upward gaze center of the midbrain

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26
Q

Nerve palsy suggestive of hydrocephalus

A

-unilateral CN6 palsy

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27
Q

First step in hydrocephalus workup

A

-urgent head CT

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28
Q

Spina Bifida is failure of fusion in what part of the vertebral column?

A

posterior midline

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29
Q

Three types of neural tube defects

A
  • meningocele
  • myelomeningocele
  • SB occulta
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30
Q

What areas have the highest and lowest incidences of neural tube defects?

A

highest: Ireland
lowest: Japan

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31
Q

Meningocele PE finding

A

mass that transluminates

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32
Q

90% of lumbosacral myelomeningoceles are assc with ______.

A

Chiari II & hydrocephalus

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33
Q

PE findings with myelomeningocele:

  • above L3
  • below S3
A

above L3: paraplegia

below S3: incontinence

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34
Q

How are NTDs diagnosed prenatally?

A

80% diagnosed by increased AFP in weeks 16-18

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35
Q

Myelomeningoceles must be surgically repaired within _____.

A

24 hours of birth

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36
Q

Two most common causes of coma in children under 5?

older than 5?

A
  • young: drowning and nonaccidental trauma

- older: accidental head injury and drug OD

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37
Q

Drugs that may be assc with coma:

A

-alcohol, opiates, benzos, TCAs, atropine, lead/ mercury etc

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38
Q

Key PE components in case of coma:

A
  • head and neck

- scalp injuries, breath odor, nuchal rigidity, CSF/ Blood leakage

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39
Q

Describe decerebrate posturing

A
  • extension of arms and legs

- subcortical injury

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40
Q

Describe decorticate posturing

A
  • bilateral cortical injury

- flexion of arms and legs

41
Q

Coma with no movement suggests _____

A

severe spine or brainstem injury

42
Q

Coma with hypoventilation =

A

opiates, sedatives

43
Q

Coma with hyperventilation=

A

metabolic acidosis
neurogenic pulmonary edema
midbrain injury

44
Q

Cheyene Strokes Breathing

  • pattern
  • cause
A
  • pattern: alternating apnea and hyperpnea

- bilateral cortical injury

45
Q

Apneustic Breathing

  • pattern
  • cause
A
  • pausing at full inspiration

- pontine injury

46
Q

Ataxic/agonal breathing: cause

A

medullary injury/ impending brain death

47
Q

Cause of unilateral dilated nonreactive pupil?

bilateral?

A

uni: uncal herniation
bilateral: brainstem injury, postictal/dilating meds

48
Q

Cause of bilateral pupillary constriction?

A
  • opiates

- pontine injury

49
Q

Loss of oculocephalic reflex: cause

A

injury to brainstem

50
Q

STAT tests in comatose patients

A
  • CT
  • LP if CT is negative
  • gluc, UDS, BMP
  • EEG
51
Q

Define epilepsy

Define status

A

2+ seizures without cause

30+ minutes

52
Q

Afebrile seizures:

generalized vs partial

A

generalized: starts in both hemispheres
partial: starts in one hemisphere

53
Q

6 types of generalized seizures

A
  • tonic
  • clonic
  • tonic clonic
  • myoclonic
  • absence
  • atonic
54
Q

Two types of partial seizures

A

simple vs complex (conscious vs unconscious)

55
Q

Describe absence seizures

A

staring less than 15 seconds with no postictal states

56
Q

Workup for first time afebrile seizure

A

none

electrolytes and imaging with prior history

57
Q

Workup needed in febrile seizures

A
  • LP
  • Cultures
  • CBC
58
Q

Treatment of status

A
  • short acting benzo (lorazepam/diazepam)

- loading dose of phenobarbital or phenytoin

59
Q

Generalized epilepsy treatment:

A
  • valproic acid

- phenobarb

60
Q

Partial epilepsy treatment

A
  • carbamazepine

- phenytoin

61
Q

“Alternate” seizure treatment

A

ketogenic diet

vagal nerve stimulator

62
Q

Age range for febrile seizures

A

6 months –> 6 years

63
Q

Contrast simple and complex febrile seizures

A
  • simple less than 15 mins

- comples longer than 15 minutes

64
Q

Abortive treatment for febrile seizure

A

rectal diazepam

65
Q

Infantile Spasms/ West Syndrome:

  • most common cause
  • EEG pattern
  • treatment of choice
A
  • TS
  • hypsarrythmia
  • ACTH
66
Q

Describe appearance of infantile spasms:

A

-jack knife seizures

arm extension, head flexion

67
Q

Absence seizures:

  • inheritance pattern
  • sex
  • EEG finding
A
  • AD
  • predominantly female
  • 3 Hz generalized spike and wave pattern
68
Q

DOC absence seizures

A

ethosuximide

69
Q

Most common childhood partial epilepsy + inheritance pattern & sex

A
  • Benign rolandic epilepsy– AD

- predominantly male

70
Q

First line drug for benign temporal epilepsy + EEG pattern

A
  • spike and wave pattern in temporal region on EEG

- valproate= DOC

71
Q

Systemic causes of anemia in kiddos

A
  • anemia
  • hypoglycemia
  • depression
  • HTN
72
Q

Most common cause of headaches in children and adolescents. More common in boys or girls? Inheritance pattern?

A

migraine
AD
Common in boys before puberty, girls after

73
Q

Hormones/ Chemicals assc with migraine

A

Vasoactive intestinal Peptide
5HT
substance P

74
Q

Most common form of migraine in kiddos?

A

migraine without aura

75
Q

DOC for migraine px and abortion in kiddos

A

sumatriptan, propranolol

76
Q

Two symptoms assc with cluster headache

A
  • lacrimation

- conjunctival erythema

77
Q

Most common cause of ataxia in childhood

A

acute cerebellar ataxia (autoimmune, postinfectious)

usually in kiddos under 10

78
Q

Common infections preceding ACA

A
  • varicella
  • flu
  • EBV
  • mycoplasma
79
Q

Workup of acute ataxia in kids

A
  • neuroimaging

- normal in ACA

80
Q

Guillian Barre:

  • bug
  • weakness type
A
  • campy jejuni

- ascending weakness

81
Q

Describe demyelination in GBS

A

-demyelination of the peripheral myelin nerves/ schwann cells via cell mediated immunity

82
Q

Describe the miller fischer variant of GBS

A
  • ataxia
  • areflxia
  • ophthalmoplegia
83
Q

CSF findings in GBS

Treatment

A

albuminocytologic dissociation

IVIG

84
Q

Sydenham chorea is assc with ______ and affects _____.

A

Rheumatic fever ; basal ganglia

85
Q

PE findings in Sydenham chorea

A
  • milkmaids grip

- choreic hand

86
Q

Diagnosis of Syd C:

A
  • ASO/ADB titers

- increased signal density at caudate and putamen

87
Q

Treatment of syd C

A

Haldol
valproate
phenobarb

88
Q

How long does it take sydenhams chorea to resolve

A

months- 2 years

89
Q

When must tics be diagnosed in tourettes?

How long must they be present?

A

before 18

at least one year

90
Q

Drug of choice in tourettes

A

pimozide

91
Q

Duchenne and Becker Muscular Dystrophies:
-Inheritance Patterns
0assc gene

A
  • X linked

- dystrophin gene

92
Q

Appearance of muscle in DMD

A

damaged muscle –> replaced by lipid deposits/ fibroblasts

93
Q

When do children lose the ability to walk in DMD/BMD

A

DMD: 10
BMD: 20

94
Q

Three PE findings in DMD

A
  • Gowers sign
  • Pseudohypertrophy of calves
  • cardiac involvement
95
Q

Diagnosis of DMD

A
  • CK levels
  • Muscle biopsy
  • absent dystrophin
  • EMG= weak potentials
96
Q

Drug that increases strength in DMD

A

steroids

97
Q

Myasthenia Gravis:
cause
sex most commonly affected

A
  • AChR Abs

- girls

98
Q

MG:

  • most common presenting sign
  • classic test
A
  • bilateral ptosis

- edrophonium/ tensilon test

99
Q

Treatment of choice for MG

A

-pyridostigmine (cholinesterase inhibitor)

+/- thymectomy

Pediatrics (12 decks)

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