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PEDIATRIC BRS > NEURO > Flashcards

NEURO Flashcards

1
Q

altered level of consciousness, increased DTR’s , weak cry, decreased spent movement

A

central hypotonia

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2
Q

unaffected consciousness, decreased muscle bulk and decreased DTR’s?

A

peripheral hypotonia

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3
Q

central hypotonia management

A

head CT
serum electrolytes
FISH

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4
Q

peripheral hypotonia management

A

CK levels
DNA tests
Nerve conduction
muscle biopsy

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5
Q

autosomall recessive inheritence mutations in chromosome 5 w/ hopotonia, weakness and tongue fasciculations?

A

spinal muscular atrophy

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6
Q

werdnig hoffman disease

A

onset of SMA

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7
Q

type II sma onset

A

6-12 months

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8
Q

type III onset

A

> 3 years

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9
Q

what is affected in spinal muscular atrophy

A

degeneration and loss of anterior horn motor neurons

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10
Q

clinical features of SMA

A

weak cry, tongue fasciculations, bell shaped chest, frog leg posture

NORMAL EXTRA OULAR MOVEMENTS AND NORMAL SENSORY EXAM

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11
Q

diagnosis of werdnig hoffman?

A

DNA testing/ muscle biopsy

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12
Q

what causes death in werdnigg hoffman?

A

resp insufficiency or pneumonia

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13
Q

prevents the presynaptic release of acetyl choline?

A

botulinum toxin

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14
Q

treatment of botulinum?

A

botulism immune globulin - NOT ANTIBIOTICS

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15
Q

trinucleotide repeat disorder autosomal dominant disease on chromosome 19

MOM IS ALSO AFFECTED?!

A

congenital myotonic dystrophy

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16
Q

atrophy of masseter and temporal is muscles, stiff straight smile and inability to release grip after hand shaking

A

myotonia

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17
Q

true or false

those with congenital myotonic dystrophy will have completely normal academic abilities?

A

FALSE

all survivors have mental retardation

  • IQ of 50-65
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18
Q

enlarged ventricles caused by obstruction of CSF flow– aqueductal stenosis

A

noncommunicating hydrocephalus

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19
Q

increased production of CSF or decreased absorption of CSF

A

communicating hydrocephalus

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20
Q

term used to describe ventricular enlargement caused by brain atrophy

A

hydrocephalus ex vacuo

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21
Q

downward place meant of the cerebellum and medulla through the foramen magnum blocking CSF flow associated w/ a lumbosacral myelomeningocele?

A

arnold chiari type II

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22
Q

absent or hypoplastic vermis w/ cystic enlargement of fourth ventricle

A

dandywalker malformation

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23
Q

when should you suspect hydrocephalus?

A

large head circumference
large anterior and posterior fontanelles w/ split sutures
sunset sign

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24
Q

evaluation of hydrocephalus

A

urgent head CT scan

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25
Q

management of hydrocephalus ?

A

VP shunt although they may cause infection and obstruction

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26
Q

taratogens that cause SB include

A

valproate, phenytoin, colchicine, vincristine,azathioprine, methotrexate

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27
Q

menningocele or myelomeningocele present w/ mental retardation?

A

myelomeningocele

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28
Q

what is the lab marker for neural tube defect?

A

increased AFP

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29
Q

unilateral dilated nonreactive pupil suggests

A

uncal herniation

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30
Q

chenye stokes breathing and decortical posture suggests?

A

bilateral cortical injury

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31
Q

what do you do after ABC”s in a comatose patient?

A 
glucose
urine tox
serum elec
metabolic panel
CT scan 
lumbar punch if CT negative 
EEG
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32
Q

involuntary alteration of conciousnessness, behavior, motor activity, sensation or autonomic function caused by

A

seizures

33
Q

occurrence of two or more spontaneous seizures w/out an obvious precipitating cause

A

epilepsy

34
Q

seizure that last more than 30 minutes and the patient doesn’t regain conciousness

A

status epilepticus

35
Q

tonic clonic and absence seizures are both?

A

generalized afebrile seizures

36
Q

treatment of status epileptics

A

IV benzo’s (lorazapam, diazepam) w/ phenobarbital or phenytoin

37
Q

what must be ruled out in a febrile seizure ?

A

CSF infection

38
Q

generalized epilepsy tx

A

valproic acid or phenobarbital

39
Q

absence epilepsy tx

A

ethosuximide

40
Q

partial epilepsy tx

A

carbamazepine or phenytoin

41
Q

what are 2 alternative treatments for patients w/ poorly controlled seizures?

A

vagal nerve stimulator and ketogenic diet

42
Q

if someone has a first febrile seizure do you treat it?

A

no

43
Q

if someone has recurrent febrile seizures

A

daily anticonvulsant prophylaxis w/ valproic acid/ phenobarbital

abortive tx w/ rectal diazepam

44
Q

what is the most common cause of infantile spasms?

A

tuberous sclerosis

45
Q

brief myoclonic jerks lasting 1-2 seconds each occurring in 5-10 seizures over 3-5 minutes?

A

west syndrome

46
Q

buzz words for west syndrome

A

jack knife seizure / salaam zeizures

47
Q

how do you treat west syndrome?

A

ACTH IM injections
valproic acid
vigabatrin ( tuberous sclerosis)

48
Q

most common partial epilepsy during childhood

A

benign rolandic epilepsy

benign centreotemporal epilepsy

49
Q

when do centrotemporal epilepsy occur?

A

early morning hours when patients are asleep w/ oral buccal manifestations

50
Q

headaches caused by ICP occur when?

A

in the morning

51
Q

unilateral headaches associated w/ nausea, vomiting or visual changes

A

migraines

52
Q

migraine associated w/ vertigo, tinnitus, ataxia or dysarthria

A

basilar artery migraine

53
Q

migraine associated w/ unilateral ptosis or ranial nerve II palsy

A

opthalmoplegic migraine

54
Q

prophylactic treatment of migraines?

A

propanolol

55
Q

abortive treatment of migraines

A

sumatriptan

56
Q

dull aching headaches associated w/ muscle contraction

A

tension headaches

57
Q

are tension headaches or migraines more common in childhood?

A

migraines

58
Q

unilateral frontal or facial pain accompanied by conjunctival erythema, lacrimation and nasal congestion

A

cluster headaches

59
Q

tx for cluster headaches

A

oxygen or sumatriptan

60
Q

phrophylactic tx of tension headaches

A

ccb and valproic acid

61
Q

ataxia secondary to a presumed autoimmune / postinfectious cause

A

acute cerebellar ataxia of childhood

62
Q

what infections cause acute cerebellar ataxia of childhood?

A

varicella, influenza, EBV and mycoplasma

63
Q

what does head ct scan show in cerebellar ataxia of childhood?

A

normal ct

64
Q

demyelinating polyneuritis w/ ascendnig weakness, areflexia and normal sensation

A

guillain barre

65
Q

what is the most common associated infection agen of guillain barre

A

campylobacter jejuni

66
Q

what kind of immune response is guillain barre

A

cell mediated immune response

67
Q

what does lumbar puncture show in guillain bar?

A

albuminocytologic dissociation

68
Q

what it the biggest risk w/ guillain barre?

A

respiratory muscle paralysis

69
Q

management of guillain barre?

A

IVIG and plasmapheresis

70
Q

how do you treat syndenhams chorea?

A

haloperidol valproic acid or phenobarbital

71
Q

what is the drug of choice in turrets?

A

pimozide

72
Q

autoimmune disorder presents w/ progressive weakness and diploplia?

A

myasthenia gravis

73
Q

cause of myasthenia gravis

A

antibodies against eh acetyl choline receptor at neuromuscular junctions

74
Q

what is neonatal myasthenia caused by?

A

transient weakness in new born from transplacental transfer of maternal AchR antibodies

75
Q

clinical features of myasthenia?

A

bilateral ptosis
characteristic increasing weakness
diplopia

76
Q

diagnosis of myasthenia

A

tensilon test ?edrophonium chloride

decremental response

presence of AChR antibody titrs

77
Q

myasthenia gravis treatment of choice

A

cholinesterase inhibitors

prydostigmide bromide

78
Q

what is the first symptom in botulinum?

A

constipatio

PEDIATRIC BRS (9 decks)

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