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Pediatrics > BRS Neurology > Flashcards

BRS Neurology Flashcards

0
Q

central vs. peripheral hypotonia

A

central- UMN, altered level of consciousness, increased DTRs, ankle clonus
peripheral- LMN, consciousness not affected, muscle bulk and DTRs are decreased

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1
Q

weak cry, decreased spontaneous movement, frog-leg posture, muscle contractures are signs of ______

A

hypotonia

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2
Q

when you suspect central hypotonia you should get these tests

A

head CT
serum electrolytes
genetic study (Down syndrome, Prader-Willi)

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3
Q

when you suspect peripheral hypotonia, get these labs

A

serum CK
DNA test for spinal muscular atrophy
EMG and nerve conduction studies
muscle biopsy

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4
Q

weak cry, tongue fasciculations, bell shaped chest ,frog-leg posture, normal extraocular movements and normal sensory exam

A

spinal muscular atrophy- anterior horn cell degeneration (hypotonia, weakness, tongue fasciculations)

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5
Q

classification of SMA

A

type 1: infantile form, onset < 6 months, aka Werdnig-Hoffman dz
type 2: intermediate form, onset 6-12 months
type 3: juvenile form, onset > 3 years

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6
Q

______ is the second most common inherited neuromuscular disorder after Duchenne muscular dystrophy

A

SMA

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7
Q

how is SMA inherited and where is the gene

A

AR

mutation of survival motor neuron gene (SMN1) on chromosome 5

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8
Q

how to dx SMA

A

DNA testing for abnormal gene is diagnostic in > 90% of cases
muscle bx shows characteristic atrophy of muscles innervated by the degenerating axons

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9
Q

how to tx SMA

what’s the prognosis

A

supportive care: G tube, monitor for respiratory infections, PT
prognosis: type 1 does not survive past year 1, type 2 survives to adolescence, type 3 survives to adulthood

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10
Q

constipation –> weak cry and suck, loss of previously obtained motor milestones, ophthalmoplegia, hyporeflexia
-paralysis is symmetric and descending

A

infantile botulism- bulbar weakness and paralysis that develops during year 1 secondary to ingestion of clostridium botulinum spores and absorption of botulinum toxin

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11
Q

what does botulinum toxin do

A

prevent presynaptic release of ACh

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12
Q

when do sxs of infantile botulism occur in relation to spore ingestion

A

12-48 hours afterwards

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13
Q

what does EMG show with infantile botulism

A

incremental response during high-frequency stimulation

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14
Q

how to tx infantile botulism

what’s the prognosis

A

treatment is supportiv: +/- NG feeding, ventilator
-botulism immune globulin improves clinical course
-do NOT give abx, they might make it worse
prognosis is excellent!

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15
Q

as a neonate

  • polyhydramnios
  • feeding and respiratory problems as a neonate
  • facial diplegia (bilateral weakness), hypotonia, areflexia, arthrogryposis (multiple joint contractures)
  • myotonia develops later on
A

congenital myotonic dystrophy

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16
Q

in an adult, myotonic facies (atrophy of masseter and temporalis muscles), prosis, stiff straight smile, inability to release grip after handshaking

A

congenital myotonic dystrophy

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17
Q

define myotonia

A

inability to relax contracted muscle

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18
Q

inheritance of congenital myotonic dystrophy

A

AD trinucleotide repeat disorder- mostly through the affected mother
chromosome 19

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19
Q

how to dx congenital myotonic dystrophy

A

DNA testing

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20
Q

how to tx congenital myotonic dystrophy

what’s the prognosis

A

treatment is supportive
prognosis is guarded… infant mortality can be as high as 40%
all survivors are mentally retarded with average IQ of 50-65
feeding problems subside with time

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21
Q

noncommunicating vs. communicating vs. ex vacuo hydrocephalus

A
  • noncommunicating- enlarged ventricles caused by obstruction of CSF flow
  • communicating- enlarged ventricles as a result of increased CSF production or decreased CSF absorption
  • ex vacuo- not true hydrocephalus, ventricular enlargement due to brain atrophy
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22
Q

downward displacement of cerebellum and medulla through foramen magnum
what is it and what is it associated with often?

A

chiari type II malformation

often associated with lumbosacral myelomeningocele

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23
Q

absent or hypoplastic cerebellar vermis and cystic enlargement of the 4th ventricle –> block of CSF flow

A

Dandy Walker malformation

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24
Q

common cause of hydrocephalus in a premie

A

intraventricular hemorrhage

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25
Q

signs of hydrocephalus in an infant

A

bulging fontanelles and split sutures

sunset sign- looks down b/c large 3rd ventricle is pushing on the upward gaze center of the brain

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26
Q

what to do if you suspect hydrocephalus

A

urgent CT scan

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27
Q

how to tx hydrocephalus and what’s the prognosis

A

VP shunt

  • pts with aqueductal stenosis have the best cognitive outcome
  • pts with chiari type II have low-normal intelligence and language d/os
  • pts with X linked hydrocephalus have severe MR
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28
Q

any failure of bone fusion in the posterior midline of the vertebral column

A

spina bifida

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29
Q

herniation of spinal cord tissue and meninges through a bony cleft

A

myelomeningocele

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30
Q

herniation of the meninges only through a bony cleft

-usually not associated with any neural deficits

A

meningocele

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31
Q

no herniation of tissue through the vertebral cleft

A

spina bifida occulta

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32
Q

for spina bifida: ______ is protective

these drugs are teratogenic

A

folic acid

**depakote, phenytoin, colchicine, vincristine, azathioprine, methotrexate

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33
Q

hairy patch on lumbosacral area… no neuro sxs

A

SB occulta

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34
Q

fluctuant midline back mass overlying the spine at birth

mass can be transilluminated

A

meningocele

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35
Q

fluctuant midline back mass mostly in lumbosacral spine

-neurologic defects are present and depend on level of the lesion

A

myelomeningocele

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36
Q

associated anomalies and complications of myelomeningocele

A
  • hydrocephalus… many cases assoc with chiari II malformation
  • cervical hydrosyringomyelia- fluid in central cord canal and in cord itself
  • defects in neuronal migration (agenesis of corpus callosum, gyral anomalies)
  • orthopedic problems
  • GU defects
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37
Q

how to dx spina bifida

A

elevated AFP in maternal serum- 80% sensitive at 16-18 weeks
fetal US
-if after birth, PE and then xrays for SB occulta, MRI for meningocele

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38
Q

how to manage spina bifida

what’s the prognosis

A

occulta- nothing –> excellent prognosis
meningocele- surgical repair –> excellent prognosis
myelomeningocele- surgical repair within 24 hours –> most survive to teen years but many are handicapped with neuro deficits

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39
Q

MCC of coma in children < 5 years

A

nonaccidental trauma

near drowning

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40
Q

MCC come in children > 5 years

A

drug OD

accidental head injury

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41
Q

coma patient:

flaccidity or no movement suggests damage at ____ or _____

A

spinal cord or brainstem

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42
Q

decerebrate posturing (extension of arms and legs) indicate _____ injury

A

subcorticate

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43
Q

decorticate posturing (flexion of arms and extension of legs) sugests _____ injury

A

b/l cortical injury

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44
Q

hyperventilation in coma suggests ____, ____, or _____

A

metabolic acidosis
neurogenic pulmonary edema
midbrain injury

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45
Q

cheyne-stroke breathing

what is it and what does it indicate

A

alternating apneas and hyperpneas

suggests bilateral cortical injury

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46
Q

what is apneustic breathing and what does it indicate

A

pausing at full inspiration

indicates pontine damage

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47
Q

ataxic or agonal breathing

what is it and what does it indicate

A

irregular respirations with no particular pattern

indicates medullary injury and impending brain death

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48
Q

unilateral dilated and fixed pupil suggests ______

A

uncal herniation

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49
Q

bilateral fixed and dilated pupils indicate 3 things

A

dilating agent
postictal state
irreversible brainstem injury

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50
Q

bilateral constricted reactive pupils suggest _______ or ______

A

opiate ingestion or pontine injury

51
Q

if you have an injured ______, you have a negative doll’s eye (oculocephalic maneuver)

A

brainstem

52
Q

normal cold caloric results in eye deviation to the ______ (irrigated/nonirrigated side)
abnormal corneal and gag reflexes indicate ____ injury

A

irrigated

brainstem

53
Q

what to check in a comatose patient

A 
FSG
UDS, electrolytes, metabolic panel
head CT
urgent EEG
\+/- LP to look for meningoencephalitis
54
Q

epilepsy is _____ or more spontaneous seizures without an obvious precipitating cause

A

2

55
Q

______ of children who have a single seizure go on to develop epilepsy

A

fever than 1/3

56
Q

most causes of childhood seizures are identifiable (T/F)

A

F

60-70% of cases, the etiology is unknown

57
Q

_______ seizures are caused by discharge from a group of neurons in both cerebral hemispheres
what are 2 types

A

generalized
tonic clonic- has a postictal state
absence- does not have a postictal state

58
Q

______ seizure are caused by discharge from one hemisphere

what are two types

A

simple partial- consciousness is not impaired

complex partial- consciousness is decreased

59
Q

how to dx epilepsy

A

history and physical
EEG- but this is not required for epilepsy diagnosis
video EEG monitoring- good for little kids, poor historians, sleep epilepsy, etc.

60
Q

neuroimaging studies should be performed in all children with epilepsy except those with _____ and _____

A

absence seizures

benign rolandic epilepsy

61
Q

what to do with a first-time afebrile seizure in an otherwise healthy child with a normal neurologic examination

A
  • doesn’t warrant an investigation

- if they have another one, you should look at serum lytes and neuroimaging

62
Q

what must you rule out with a febrile seizure

A

CNS infection

63
Q

how to tx status epilepticus

A

IV anticonsulsants (ex. lorazepam or diazepam) followed by a loading dose of either phenobarbital or phenytoin

64
Q

drug recommendations for treatment of epilepsy
generalized
absence
partial

A

generalized- depakote or phenobarbital
absence- ethosuximide
partial- carbamazepine or phenytoin

65
Q

best prognosis for epilepsy surgery is for ______

A

temporal lobe lesions

66
Q

other tx for epilepsy (not drugs or surgery)

A

vagal nerve stimulator- side effect of hoarseness

ketogenic diet- high fat, low carb, produces a state of ketosis

67
Q

prognosis for epilepsy

A 
not a lifelong disorder
many children (70%) can be weaned off meds after 2 years of no seizures
68
Q

seizure accompanied by a fever owing to a non-CNS cause in pts 6 months to 6 years

A

febrile seizure

these are fairly common

69
Q

simple vs. complex febrile seizure

A

simple- < 15 minutes, generalized

complex- > 15 minutes, focal features, or recurs within 24 hours

70
Q

dx of febrile seizure

do you need LP, neuroimaging, or EEG?

A

H&P, normal neuro exam, exclusion of CNS infection
no LP unless you suspect meningitis
no neuro imaging or EEG unless neuro exam is abnormal

71
Q

management of febrile seizure

A

first time or occasional- no tx needed

frequent recurrent febrile seizures- daily ppx with depakote or phenobarbitol, abortive tx with rectal diazepam

72
Q

____% of pts with a febrile seizure will have a recurrence

A

30%

73
Q
  • brief myoclonic jerks, lasting 1-2 seconds each, occurring in clusters of 5-10 seizures spread over 3-5 minutes
  • sudden arm extension or head and trunk flexion (jack-knife seizures or salaam seizures)
A

infantile spasms (West syndrome)

74
Q

infantile spasm age of onset

______ is the most commonly identified cause

A

3-8 months

tuberous sclerosis

75
Q

EEG: hypsarrhythmia pattern (highly disorganized pattern of high amplitude spike and waves in both cerebral hemispheres)

A

infantile spasm (West syndrome)

76
Q

treatment and prognosis of infantile spasm (West syndrome)
what’s first and second line
what’s good for cases caused by tuberous sclerosis

A

ACTH IM injections for 4-6 weeks
depakote is second line
vigabatrin is most effective if it’s associated with tuberous sclerosis
prognosis is poor: moderate to severe MR despite therapy

77
Q

absence seizures age of onset and inheritance

A

5-9 years

AD with age-dependent penetrance

78
Q

generalized 3-Hz spike and wave discharge on EEG

A

absence seizure

79
Q

how to tx absence

what’s the prognosis

A

ethosuximide (first line), depakote

prognosis is good; usually resolve spontaneously w/o cognitive impairment

80
Q

nocturnal partial seizure with secondary generalization

  • early morning, pts are asleep with oral-buccal manifestations (moaning, grunting, pooling of saliva)
  • then generalization into tonic clonic seizures
A

benign rolandic epilepsy (benign centrotemporal epilepsy)

81
Q

____ is the most common partial epilepsy during childhood

what age is peak incidence

A

benign rolandic epilepsy

age 6-7

82
Q

how is benign rolandic epilepsy inherited

A

AD with variable penetrance

83
Q

EEG: biphasic spike and sharp wave disturbance in the mid-temporal and central regions

A

benign rolandic epilepsy

84
Q

how to tx benign rolandic epilepsy and prognosis

A

tx: depakote (first line) and carbamazepine

prognosis is great: usually spontaneous remission with no cognitive defects

85
Q

_____ are the MCC headaches in children and adolescents

A

migraines

86
Q

inheritance and pathophys of migraines

A

AD inheritance

changes in cerebral blood flow 2/2 release of 5-HT, substance P, and VIP

87
Q

migraine _____ (with/without) aura is the most common form of migraine in children

A

without

88
Q

what is a migraine equivalent

A

no headache

prolonged transient alteration of behavior that manifests as cyclic vomiting, cyclic abdominal pain, paroxysmal vertigo

89
Q

headache with unilateral ptosis or cranial nerve III palsy

headache preceded by vertigo, tinnitus, ataxia, or dysarthria

A

ophthamoplegic migraine

basilar artery migraine

90
Q

fortifications in migraines

A

type of visual aura- jagged streaks of light that take on the outline of old forts

91
Q

migraine tx
abortive
ppx

A

abortive: sumatriptan (selective 5-HT agonist)
ppx: propranolol

92
Q

you may find isometric contraction of facial muscles in this type of headache
what don’t you find

A

tension headache

you don’t find vomiting, visual changes, or paresthesias

93
Q

how to tx tension HA

A

ibuprofen, acetaminophen

94
Q

how to tx cluster HAs
abortive
ppx

A

abortive: oxygen, sumatriptan
ppx: CCB, depakote

95
Q

these two types of primary HA are rare in children

A

tension and cluster

96
Q

unsteady, wide-based stance with irregular steps and veering

A

cerebellar gait

97
Q

_______ is the most common cause of ataxia in children

what’s the age of onset

A

acute cerebellar ataxia

18 months-7years

98
Q

common infections that cause acute cerebellar ataxia

A

varicella, influenza, EBV, mycoplasma
the ataxia occurs 2-3 weeks after infection
mechanism: immune complex deposition in the cerebellum

99
Q

clinical features of acute cerebellar ataxia

A

truncal ataxia, deterioration of gait
slurred speech and nystagmus
no fever

100
Q

how to work up and treat acute cerebellar ataxia

A

H&P
do head CT to r/o life threatening causes
tx is supportive… usually takes 2-3 months to resolve. PT may be helpful

101
Q

ascending weakness, areflexia, normal sensation

what is this and what is it most likely caused by?

A

Guillain-Barré syndrome

campylobacter jejuni, which causes a prodromal gastroenteritis

102
Q

where is the area of demyelination in GBS

A

ventral spinal roots and peripheral myelinated nerves

103
Q

clinical features of GBS

A

ascending symmetric paralysis
no sensory loss but there may be leg/back pain
cranial nerve involvement (facial weakness in 40-50% of pts)

104
Q

ophthalmoplegia, ataxia, areflexia

A

Miller Fisher syndrome (variant of GBS)

105
Q

dx of GBS- LP, EMP, spinal MRI (when should you do this)

A

LP: albuminocytologic dissociation (high protein, normal cell count)
EMG: decreased nerve conduction velocity l
spinal MRI: do this in young kids where sensory exam is not reliable, used to r/o spinal compressive lesions

106
Q

how to manage GBS

what’s the prognosis

A

IVIG for 2-4 days is the preferred tx in kids
can also do plasmapheresis
complete recovery is the rule in children

107
Q
  • children appear restless- face, hands, arms have continuous, quick, and random movements
  • speech can be jerky or indistinct
  • chameleon tongue
  • choreic hand (flexed at wrist, hyperextended at MCP joint), milkmaid’s sign
  • emotional lability
  • gait and cognition are not affected
A

sydenham chorea

  • self limited autoimmune disorder associated with rheumatic fever
  • occurs 2-7 months after GABHS infection
  • cross-reacting antibodies to GABHS and basal ganglia cells
108
Q

how to dx sydenham chorea

-labs and imaging

A

elevated ASO or ADB titer (indicates strep infection)
head MRI: increased signal intensity in the caudate and putamen
SPECT: increased perfusion to the thalamus and striatum

109
Q

how to tx sydenham chorea

prognosis

A

helperidol, depakote, or phenobarbital

all pts recover within several months to 2 years

110
Q

tourette’s requires sxs for > _____

also must have have motor and phonic tics before age ______

A

1 year

18 years

111
Q

how to tx tourette’s

A

pimozide, risperidone are effective
clonidine is less effective- can cause sedation
haldol- used to be used but risk of tardive dyskinesia
hypnotherapy

112
Q

tourette’s prognosis

A

tics tend to decrease in adulthood

113
Q

X linked myopathies: _______ and ________

these are due to deletion in the _______ gene

A

Duchenne and Becker muscular dystrophy

dystrophin

114
Q

pathophys of DMD and BMD

A

lack of dystrophin –> weakness and rupture of the plasma membrane –> injury and degeneration of muscle fibers –> infiltration of lymphs into the injured area and replacement of damaged muscle fibers with fibroblasts and lipid deposits

115
Q

slow progressive weakness affecting the legs
pseudohypertrophy of calves
Gower’s sign
cardiac involvement

A

DMD or BMD

116
Q

dx of DMD or BMD

A

enlarged calf muscles in young boy with muscle weakness
high CK levels
EMG
muscle biopsy
absent or decreased dystrophin levels on Western blot
DNA testing

117
Q

EMG findings in DMD or BMD

A

small polyphasic muscle potentials with normal nerve conductions

118
Q

how to tx DMD or BMD

A

oral steroids early on can improve strength transiently

119
Q

prognosis of DMD and BMD

A

DMD: no walking by age 10 years, most die in late teens from respiratory failure
BMD: no walking by 20s, most survive to 50s

120
Q

antibodies to AChR at neuromuscular junctions

what are the two types

A

myasthenia gravis

  • neonatal: mother’s antibodies cross the placenta… resolves in a few weeks
  • juvenile: AChR antibody formation
121
Q
  • hypotonia, weakness, feeding problems in _______

- bilateral ptosis, increasing weakness later in day, diplopia, DTRs are preserved in _______

A
  • neonatal myasthenia gravis

- juvenile myasthenia gravis

122
Q

how to dx myasthenia gravis

A
  • tensilon test: IV injection of edrophonium (AChE inhibitor) transiently improves ptosis
  • decremental response to repetitive nerve stimulation
  • presence of AChR antibody titers
123
Q

tx of myasthenia gravis, both neonatal and juvenile

A

nenoatal- symptomatic; if respiration is compromised, give AChE inhibitor or IVIG
juvenile- pyridostigmine is the mainstay (AChE inhibitor), steroids if those don’t work, plasmapheresis during flares, thymectomy

124
Q

prognosis of myasthenia gravis
neonatal
juvenile

A

neonatal- resolves in 1-3 weeks

juvenile- remission of sxs occur in 60% after thymectomy

Pediatrics (14 decks)

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