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PEDIATRIC BRS > NEURO > Flashcards

NEURO Flashcards

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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
management of hydrocephalus ?
VP shunt although they may cause infection and obstruction
26
taratogens that cause SB include
valproate, phenytoin, colchicine, vincristine,azathioprine, methotrexate
27
menningocele or myelomeningocele present w/ mental retardation?
myelomeningocele
28
what is the lab marker for neural tube defect?
increased AFP
29
unilateral dilated nonreactive pupil suggests
uncal herniation
30
chenye stokes breathing and decortical posture suggests?
bilateral cortical injury
31
what do you do after ABC"s in a comatose patient?
``` glucose urine tox serum elec metabolic panel CT scan lumbar punch if CT negative EEG ```
32
involuntary alteration of conciousnessness, behavior, motor activity, sensation or autonomic function caused by
seizures
33
occurrence of two or more spontaneous seizures w/out an obvious precipitating cause
epilepsy
34
seizure that last more than 30 minutes and the patient doesn't regain conciousness
status epilepticus
35
tonic clonic and absence seizures are both?
generalized afebrile seizures
36
treatment of status epileptics
IV benzo's (lorazapam, diazepam) w/ phenobarbital or phenytoin
37
what must be ruled out in a febrile seizure ?
CSF infection
38
generalized epilepsy tx
valproic acid or phenobarbital
39
absence epilepsy tx
ethosuximide
40
partial epilepsy tx
carbamazepine or phenytoin
41
what are 2 alternative treatments for patients w/ poorly controlled seizures?
vagal nerve stimulator and ketogenic diet
42
if someone has a first febrile seizure do you treat it?
no
43
if someone has recurrent febrile seizures
daily anticonvulsant prophylaxis w/ valproic acid/ phenobarbital abortive tx w/ rectal diazepam
44
what is the most common cause of infantile spasms?
tuberous sclerosis
45
brief myoclonic jerks lasting 1-2 seconds each occurring in 5-10 seizures over 3-5 minutes?
west syndrome
46
buzz words for west syndrome
jack knife seizure / salaam zeizures
47
how do you treat west syndrome?
ACTH IM injections valproic acid vigabatrin ( tuberous sclerosis)
48
most common partial epilepsy during childhood
benign rolandic epilepsy | benign centreotemporal epilepsy
49
when do centrotemporal epilepsy occur?
early morning hours when patients are asleep w/ oral buccal manifestations
50
headaches caused by ICP occur when?
in the morning
51
unilateral headaches associated w/ nausea, vomiting or visual changes
migraines
52
migraine associated w/ vertigo, tinnitus, ataxia or dysarthria
basilar artery migraine
53
migraine associated w/ unilateral ptosis or ranial nerve II palsy
opthalmoplegic migraine
54
prophylactic treatment of migraines?
propanolol
55
abortive treatment of migraines
sumatriptan
56
dull aching headaches associated w/ muscle contraction
tension headaches
57
are tension headaches or migraines more common in childhood?
migraines
58
unilateral frontal or facial pain accompanied by conjunctival erythema, lacrimation and nasal congestion
cluster headaches
59
tx for cluster headaches
oxygen or sumatriptan
60
phrophylactic tx of tension headaches
ccb and valproic acid
61
ataxia secondary to a presumed autoimmune / postinfectious cause
acute cerebellar ataxia of childhood
62
what infections cause acute cerebellar ataxia of childhood?
varicella, influenza, EBV and mycoplasma
63
what does head ct scan show in cerebellar ataxia of childhood?
normal ct
64
demyelinating polyneuritis w/ ascendnig weakness, areflexia and normal sensation
guillain barre
65
what is the most common associated infection agen of guillain barre
campylobacter jejuni
66
what kind of immune response is guillain barre
cell mediated immune response
67
what does lumbar puncture show in guillain bar?
albuminocytologic dissociation
68
what it the biggest risk w/ guillain barre?
respiratory muscle paralysis
69
management of guillain barre?
IVIG and plasmapheresis
70
how do you treat syndenhams chorea?
haloperidol valproic acid or phenobarbital
71
what is the drug of choice in turrets?
pimozide
72
autoimmune disorder presents w/ progressive weakness and diploplia?
myasthenia gravis
73
cause of myasthenia gravis
antibodies against eh acetyl choline receptor at neuromuscular junctions
74
what is neonatal myasthenia caused by?
transient weakness in new born from transplacental transfer of maternal AchR antibodies
75
clinical features of myasthenia?
bilateral ptosis characteristic increasing weakness diplopia
76
diagnosis of myasthenia
tensilon test ?edrophonium chloride decremental response presence of AChR antibody titrs
77
myasthenia gravis treatment of choice
cholinesterase inhibitors | prydostigmide bromide
78
what is the first symptom in botulinum?
constipatio

PEDIATRIC BRS (9 decks)

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