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PPS - From Nelson's > Neuro > Flashcards

Neuro Flashcards

1
Q

test to measure neural tube defect while pregnant

A

AFP -> 16th to 18th week

acetylcholinesterase

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2
Q

drugs that antagonize folic acid

A 
trimethoprin
carbamazepine
phenobarbital
phenytoin
primidone
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3
Q

downward herniation of medulla and cerebellar tonsils through foramen magnum
pooling of secretions
vocal cord paralysis
stridor

A

chiari crisis

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4
Q 
encephalocele
cleft lip and palate
microcephaly
microphtalmia
abnormal genitals
polycystic kidneys
polydactyly
A

meckel gruber

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5
Q

absent cerebral convolutions

A

lissencephaly

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6
Q

presence of unilateral or bilateral clefts within cerebral hemispheres

A

schizencephaly

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7
Q

presence of cysts in brain

A

porencephaly

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8
Q

most common location porencephaly

A

near sylvian fissure

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9
Q

porencephaly assoc with

A 
maternal cocaine 
maternal ab trauma
protein C
factor V leiden
von willebrand
perinatal alloimmune thrombocytopenia
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10
Q

female
agenesis of corpus callosum
chorioretinal lacunae (punched out lesions in pigmented layer of retina)
infantile spasms

A

aicardia syndrome

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11
Q

abnormal enlargement of occipital horns

A

colpocephaly

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12
Q

colpochephaly assoc with

A

agensis of corpus callosum

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13
Q

defective formation of prosencephalon

forebrain structures affected

A

holoprosencephaly

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14
Q

sucking jaw movements with eye blinking

A

marcus gunn phenomenon

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15
Q

paralysis of abducens nerve CN 6

A

mobius syndrome

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16
Q

Limitation of abduction (outward movement) of the affected eye

A

duane retraction syndrome

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17
Q 
autosomal recessive
underdeveloped cerebellar vermis
molar tooth sign
hypotonia
ataxia
apnea
A

joubert syndrome

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18
Q 
scaphocepahly
cleft lip/palat
syndactyly of second and third toes
upturned nose
CHD
ambiguous genitals
agenesis of corpus callosum
decreased cholesterol levels
autosomal recessive
A

Smith–Lemli–Opitz syndrome

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19
Q

CSF rate

A

20ml/hr

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20
Q

amount of csf

A

50ml in infant

150ml in adult

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21
Q

Incidence 1 in 50,000 live births

Round facies, prominent epicanthic folds, low-set ears, hypertelorism, characteristic cry No specific neuropathology

A

cri du chat

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22
Q

Ptosis, scaphocephaly, inner epicanthic folds, anteverted nostrils Low birthweight, marked feeding problems

A

Smith-Lemli-Opitz

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23
Q

Small for dates, petechial rash, hepatosplenomegaly, chorioretinitis, deafness, mental retardation, seizures Central nervous system calcification and microgyria

A

CMV

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24
Q

Growth retardation, purpura, thrombocytopenia, hepatosplenomegaly, congenital heart disease, chorioretinitis, cataracts, deafness
Perivascular necrotic areas, polymicrogyria, heterotopias, subependymal cavitations

A

rubella

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25
Q

Purpura, hepatosplenomegaly, jaundice, convulsions, hydrocephalus, chorioretinitis, cerebral calcifiation

A

toxoplasmosis

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26
Q

Growth retardation, ptosis, absent philtrum and hypoplastic upper lip, congenital heart disease, feeding
problems, neuroglial heterotopia, disorganization of neurons

A

fetal alcohol

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27
Q

Growth delay, hypoplasia of distal phalanges, inner epicanthic folds, broad nasal ridge, anteverted nostrils

A

fetal hydantoin

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28
Q

premature closure saggital

A

scaphocephaly

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29
Q

most common craniosynostosis

A

scaphocephaly

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30
Q

skull that resembles cloverleaf

A

kleeblattschadel deformity

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31
Q

autosomal dominant syndrome with a highly variable
phenotype
Onset is usually in early childhood and remission is usually
in mid-childhood
It is characterized by multiple febrile seizures

A

generalized epilepsy with febrile seizures plus

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32
Q

characterized by febrile and
afebrile unilateral clonic seizures recurring every 1 or 2 mo
severe myoclonic seizure of infancy

A

Dravet syndrome

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33
Q

temporal discharges

loss of speech and verbal agnosia

A

landau kleffner epileptic aphasia syndrome

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34
Q

unilateral intractable partial seizures
epileptia partia continua
progressive hemiparesis of affected side

A

rasmussen’s encephalitis

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35
Q

adolescent
myoclonic
drops things often

A

juvenile myoclonic epilepsy

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36
Q

seen in first three months of life
mental retardation
tonic seizure
burst suppresion on EEG

A

ohtahara syndrome

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37
Q

infantile spasms that occur in clusters
developmental regression
hypsarrhythmia

2-12mo

A

west syndrome

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38
Q

2-10yrs old
developmental delay
multiple seizure types
1-2 hz spike and slow waves

A

lennox gastaut syndrome

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39
Q

treatment of landau kleffner syndrome

A

valproic acid

prednisone

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40
Q

four processes of seizures

A
  1. underlying etiology
  2. epileptogenesis (kindling)
  3. epileptic state of increased excitability
  4. seizure related neuronal injury
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41
Q

DOC focal seizure

A

oxcarbazepine

carbamazepine

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42
Q

DOC absence

A

ethosuximide

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43
Q

DOC juvenile myoclonic epilepsy

A

valproate

lamotrigine

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44
Q

DOC Lennox gastaut

A

valproate
topiramate
lamotrigine
rufinamide

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45
Q

DOC infantile spasms

A

ACTH

adrenocorticotropic hormone

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46
Q

DOC west syndrome

A

ACTH

adrenocorticotropic hormone

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47
Q

side effect of
ACTH
adrenocorticotropic hormone

A 
hypertension
electrolyte imbalance
infection
hyperglycemia
glycosuria
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48
Q

DOC Dravet

A

valproate

benzodiazepines (clonazepam)

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49
Q

DOC benign myoclonic epilepsy

A

valproate

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50
Q

side effect weight gain

A

valproate

carbamazepine

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51
Q

side effect gingival hyperplasia

A

phenytoin

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52
Q

side effect alopecia

A

valproate

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53
Q

side effect hyperactivity

A

benzodiazepines
barbiturates
valproate
gabapentin

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54
Q

drug that can cause sjs like syndrome
chinese
HLA B 1502

A

carbamazepine

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55
Q

side effect rickets

A

phenytoin
phenobarbital
primidone
carbamazepine

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56
Q

AED to avoid in IEM

A

valproate

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57
Q

how to give IVIG for west, dravet, lennox gastaut, landau kleffner

A

2g/kg over 4 consecutive days

then 1g/kg once a month for 6 months

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58
Q

when to d/c AED

A

2 years seizure free

if benign epilepsy, 6 months

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59
Q

risk factors of seizure relapse after withdrawal

A

older age
longe duration of epilepsy
>1 AED used
multiple seizure types

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60
Q

apneic
focal motor seizures
fifth day of life
EEG sharp 4-7Hz theta pointu alterant

A

fifth day fits

benign idiopathic neonatal seizures

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61
Q

cerebellar hemangioblastomas
retinal angioma
autosomal dominant
VHL gene

A

von hippel lindau disease

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62
Q

PHACE

A 
posterior fossa
hemangioma
arterial anomalies
cardiac anomalies/coarctation
eye abnormalitites
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63
Q

dental
ocular
dermatologic (vesicular-> verrucous plaques-> hyperpigmented-> hypopigmented)
cranial

A

incontinenta pigmenti

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64
Q

pathogen in acute cerebellar ataxia

A

varicella
coxsakie
echovirus

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65
Q

failure to thrive, photosensitivity, intermittent ataxia, nystagmus, and tremor
autosomal recessive
pellagra like

A

hartnup

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66
Q 
steatorrhea
fialure to thrive
acanthocytosis
ataxia
retinitis pigmentosa
decreased serum cholesterol and TG
A

abetalipoproteinemia
or
Bassen kornzweig

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67
Q 
ataxia
strabismus
telangiectasia
recurrent sinopulmonary infection
low igA igG igE
A

ataxia telangiectasia

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68
Q

malignancy assoc with ataxia telangiectasia

A

leukemia
lymphoma
hodgkin
brain tumor

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69
Q 
autosomal recessive
progressing ataxia 
explosive dysarthritic speech
pes cavus
scoliosis
decreased DTR and vibratory
involve spinocerebellar tracts , dorsal column of spine, cerebellum, medulla

vit E and co q10 slows progression

A

friedrich ataxia

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70
Q

dance like
darting tongue
milkmaid grip

A

chorea

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71
Q

chorea disappears with

A

sleep

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72
Q

chorea increases with

A

stress

73
Q

clinical hallmark of sydenham chorea

A

emotional lability
hypotonia
chorea

74
Q

tx for sydenham chorea

A

valproate
carbamazepine
dopamine receptor antagonist (haloperidol)

75
Q

tremor

feature of cerebellar disease

A

intention tremor

76
Q

tx of tremor

A 
propranolol
primidone (barbiturate)
77
Q

excess deposition of copper in liver, brain
progressive basal ganglia disease
choreoathetosis
parkinsonism

A

wilson disease

78
Q

excess iron deposition in globus pallidus and substantia nigra
“eye of the tiger” sign -> edema and necrosis of globus pallidus
autosomal recessive

A

pantothenate kinase associated neurodegeneration
or
hallervorden spatz disease

79
Q

seen at 1st mo of life, resolve by 3 yrs
torticollis
irritability
ataxia

A

benign paroxysmal torticollis of infancy

80
Q

tx for generalized dystonia

A

trihexyphenidyl

anticholinergic

81
Q

arm more involved than leg
hadn preference at early age
cerebral palsy

A

spastic hemiplegia

82
Q

commando crawl/rudder
uses arms more in crawling
periventricular leukomalacia
cerebral palsy

A

spastic diplegia

83
Q

CP from kernicterus

A

athetoid cp

84
Q
  1. recurrent strokelight episodes with hemiparesis
  2. lactic acidosis
  3. seizure
A

MELAS

mitochondrial myopathy encephalopathy lactic acidosis and strokelike episodes

85
Q 
progressive myoclonic epilepsy
mitochondrial myopathy
ataxia
nystagmus
dysarthria
elevated lactate
ragged red fibers in muscle biopsy
A

myoclonus epilepsy and ragged red fibers (MERRF)

86
Q 
feeding and swallowing problems
delayed motor and language
pyramidal signs
weakness
hypotonia
ataxia
nystagmus
A

leigh disease
or
subacute necrotizing encephalomyopathy

87
Q 
triad:
1. progressive external ophthalmoplegia
2. onset before 20yr old
3. pigmentary retinopathy
PLUS ONE:
heart block, cerebellar syndrome, CSF protein >100mg/dl

can be associ with endocrine problems

A

kearns sayre

88
Q 
congenitally infected
arrest in brain growth
dev delay
weak pyramidal tract signs
seizures
A

hiv encephalopathy

89
Q

mood or personality change
seizure
dyskinesia
sleep disturbance

A

anti NMDAR encephalitis

90
Q 
peroxisomal disorder
cerebral pachygyria
progressive loss of hearing and vision
high forehead, hypoplastic supraorbital ridges, epicanthal folds, midface hypoplasia, and a large fontanel
hepatomegaly
renal cysts
A

zellweger syndrome
or
cerebrohepatorenal syndrome

91
Q 
defect galactocerebrosides B galactosidase
excessive crying
hyperpyrexia
rigidity 
blindness 
deafness
demyelinating disease
A

krabbe

92
Q 
lorenzo's oil (glyceryl trioleate and glyceryl trieucate) 
tanning without sun exposure
ataxia
swallowing disturbance
adrenal insufficiency
inflammatory demyelinating disease
boy
A

adrenoleukodystrophy

93
Q 
x linked recessive
deficient copper
chubby cheeks, friable hairs
severe mental retardation
failure to thrive
hypotonia
generalized myotonic seizures
A

menkes disease

kinky hair disease

94
Q 
regression of language and motor
tremor
ataxic gait
repetitive hand movements
autistic
Generalized tonic clonic seizure
A

rett syndrome

95
Q 
measles
aggressive behaviour
impaired cognitive
Generalized tonic clonic seizure
choreathetosis
A

subacute sclerosing panencephalitis

96
Q

relapsing remitting course of episodes separated in time and space
demyelinating

A

multiple sclerosis

97
Q 
hemiparesis
optic neuritis
focal sensory loss
ataxia
diplopia
dysarthria
bowel/bladder dysfxn
discrete lesions on periventricular white matter on MRI
A

multiple sclerosis

98
Q

treatment of multiple sclerosis

A

MPPT

99
Q

pathogens associated with ADEM

A 
influenza
ebv
cmv
varicella
enterovirus
measles, mumps, rubella
herpes
mycoplasma pneumoniae
100
Q

post vaccination ADEM

A 
mmr
jap b
rabies
smallpox
dpt
flu
101
Q 
lethargy 
fever
headache 
vomiting
encephalopathy
visual loss
ataxia
motor/sensory deficit
bladder/bowel dysfxn

MRI: swelling and variable enhancement within white and gray matter

A

ADEM

acute disseminated encephalomyelitis

102
Q

tx ADEM

A

MPPT

then oral steroids

103
Q

leading cause of acquired brain injury

A

artreial ischemic stroke

104
Q

leading cause of term born cerebral palsy

congenital hemiplegia

A

perinatal stroke

105
Q

virchow triad

A
  1. Hypercoagulability
  2. Hemodynamic changes (stasis, turbulence)
  3. Endothelial injury/dysfunction
106
Q

tx strep meningitis

A

10-14 days
3rd gen ceph or penicillin
add Vanco if resistant

107
Q

tx n. meningitidis meningitis

A

5-7 days

penicillin

108
Q

tx hib meningitis

A

7-10 days

109
Q

repeat LP in mengitis done for

A

neonates if indicated
gram negative meningitis
B lactam resistant strep meningitis

110
Q

csf should be sterile after

A

24-48 hours after initiation of tx

111
Q

tx gram (-) meningitis

A

3 weeks

or at least 2wks after csf sterilization

112
Q

dexamethasone for __ meningitis

A

Hib

113
Q

most common sequelae in bacterial meningitis

A

sensorineural hearing loss

114
Q

chemoprophylaxis for neiserria meningitidis

A

rifampin 10mkdose q12 x 2 days

115
Q

chemoprophylaxis for hib

A

rifampin 10mkdose q12 x 4 days

116
Q

most common cause of helminthic/eosinophilic meningitis

A

rat lungworm

angiostrongylus cantonensis

117
Q

emperic tx in brain abscess -unknown cause

A

vancomycin
3rd gen cephalosporin
metronidazole

118
Q

emperic tx in brain abscess - otitis media, sinusitis, mastoiditis

A

vancomycin
3rd gen cephalosporin
metronidazole

119
Q

emperic tx in brain abscess - penetrating head injury

A

vanco

3rd gen ceph

120
Q

emperic tx in brain abscess - cyanotic heart disease

A

ampicillin sulbactam
OR
3rd gen ceph + metronidazole

121
Q

tx citrobacter brain abscess neonate

A

3rd gen ceph + aminoglycoside

122
Q

tx listeria brain abscess neonate

A

3rd gen ceph + aminoglycoside + ampicillin

123
Q

emperic tx in brain abscess - immunocompromised

A

broad spectrum

amphotericin B

124
Q

brain surgery for brain abscess not indicated if

A

<2cm
<2weeks duration
no signs of increased ICP
neurologically intact

125
Q

surgery of brain abscess indicated if

A 
>2.5cm
gas inside abscess
fungus identified
multiloculated
located at posterior fossa
126
Q

pseudotumor cerebri definition

A

ICP
>200mm H2O in infants
>250mm H2O in kids

normal CSF picture

127
Q

central cord syndrome

upper ext weakness (proximal first then distal)

A

syringomyelia

128
Q

brown sequard syndrome

A

ipsilateral weakness, ataxia, spasticity

contralateral loss of pain and temperature

129
Q

SCIWORA

A

spinal cord injury without radiographic bone abnormalities

130
Q

injury T12 - L1
loss of sphincter control (urinary/rectal)
flaccid
sensory loss

A

conus medullaris syndrome

131
Q

lower motor neuron signs in upper extremity
upper motor neuron signs in lower extremity
bladder dysfxn
loss of sensation caudally
upper extremities are weaker

A

central cord syndrome

132
Q

motor and sensory deficit dysfxn in spine
urinary retention first, then incontinence later
weakness first, then spasticity after

CSF: minimal PMN, protein mildly elevated, elevated myelin basic protein and immunoglobulin levels

A

transverse myelitis

133
Q

weakness proximal > distal

A

myopathy

134
Q

weakness distal > proximal

A

neuropathy

135
Q

intact tendon stretch reflex

A

myopathy

136
Q

decreased tendon stretch reflex

A

neuropathy

137
Q

type 1 sma

A

wernig hoffman

138
Q

most commonly sampled muscle

A

vastus lateralis

quadriceps femoris

139
Q

most commonly sampled nerve

A

sural nerve

140
Q

sensory or motor: sural nerve

A

sensory

141
Q

myotubular myopathy arrest in

A

maturation of fetal muscle in the myotubular phase 8-15weks AOG

142
Q

X-linked recessive disorder characterized by congenital cataracts, hypotonia, intellectual disability, proximal tubular acidosis, aminoaciduria, and low-molecular-weight proteinuria

A

oculocerebral syndrome

Lowe syndrome

143
Q

floppy infant prone to malignant hyperthermia (dantrolene)

A

central core myopathy

144
Q

muscle biopsy of duchenne

A

endomysial connective tissue proliferation
scattered degenerating and regenerating myofibers
mononuclear infiltrations

145
Q

muscles do not hypertrophy
contractures and wasting in scapulohumeralperoneal distribution
absent myotonia
cardiomyopathy

A

emery dreifuss muscular dystrophy

146
Q 
facial wasting 
inverted V shaped lip
generalized muscle wasting
myotonia
slow gastric empyting/peristalsis
heart block
endocrine problems (hypothy, adrenal insuff, diabetes)
low igG
cataract
A

myotonic muscular dystrophy

147
Q

diagnosis of myotonic musclular dystrophy

A

DNA analysis CTG repeat

148
Q

kids that looks like body builder

A

myotonia congenita
or
thomsen disease

149
Q

juvenile spinal muscular atrophy

A

kugelberg welander disease

150
Q

prominent scapular winging in infants

A

facioscapulohumeral muscular dystrophy
or
Landouzy - Dejerine disease

151
Q

factors that precipitate hypoK periodic paralysis

A
  1. heavy carb meal
  2. emotional stress
  3. insulin
  4. epinephrine
  5. hyperaldosteronism
  6. hyperthyroidism
  7. amphotericin B
  8. licorice
152
Q

triad:

  1. (micrognathia), low-set ears, and an abnormal curvature of the fingers called clinodactyly
  2. periodic paralysis
  3. potentially fatal cardiac ventricular ectopy (with long QT)
A

andersen tawil syndrome

153
Q 
floppy infant
administration of general anesthetic
fevere
muscle rigidity
metabolic and respiratory acidosis
serum CK elevated 35, 000
acute tubular necrosis
myoglobinuria
A

malignant hyperthermia

154
Q

small cell lung cancer
myasthenia gravis
adult

A

eaton lambert

155
Q

EMG in myasthenia gravis

A

decremental response in repeated stimulation

156
Q

clinical test for myasthenia gravis >2yrs old

A

edrophonium (short acting cholinesterase inhibitor)

157
Q

clinical test for myasthenia gravis used for <2yrs old

A 
prostigmine methylsulfate (neostigmine)
IM
158
Q

tx myasthenia gravis caused by failure to release Acetylcholine

A

ephedrine
or
diaminopyridine

159
Q

overdose of cholinesterase inhibitor

A

cholinergic crisis

160
Q

myasthenia gravis cannot tolerate

A

NMJ blocking drugs

succinylcholine and pancuronium

161
Q

cholinergic crisis

A

Salivation, Lacrimation, Urination, Defecation, Gastrointestinal Distress and Emesis

162
Q

neurodegeneration of voluntary muscles
weakness
spasticity
sensory intact

A 
Amyotrophic lateral sclerosis (ALS)
OR
motor neurone disease (MND) 
OR
 Lou Gehrig's disease
163
Q

stork leg

A

charcot marie tooth disease

164
Q

nerves affected early in charcot marie tooth disease

A

peroneal and tibial nerves

165
Q

findings in biopsy sural nerve charcot marie tooth disease

A

onion bulb formation
proliferated schwann around axons
interstitial hypertrophy neuropathy

166
Q

poison burning paresthesias

A

arsenic

167
Q

poison mononueritis complex ( motor neuropathy of peroneal, radial, median nerves)

A

lead

168
Q

toxin puffer fish similar to

A

guillan barre syndrome

169
Q 
autosomal recessive
reduced number of unmyelinated nerves (pain, temp, taste, autonomic)
fungiform papilae in tongue absent
swallowing problem
excessive sweating
tendon stretch reflex absent
bradycardia
urinary incontinence
A

familial dysautonomia

RIley Day

170
Q

achalasia
alacrima
autonomic dysfunction

A

allgrove syndrome

171
Q

landry ascending paralysis

A

guillain barre

172
Q

acute external ophthalmoplegia
ataxia
areflexia

A

miller fischer

173
Q

csf in guillain barre

A

protein 2x normal
glucose normal
normal cells

174
Q

tx for guillain barre

A

ivig 0.4g/kg/day for 5 days

175
Q

last function to recover in GBS

A

tendon reflexes

176
Q

3 clinical features GBS that predict poor outcome

A
  1. intubation
  2. cranial nerve involvement
  3. maximum disability at time of presentation
177
Q

pathogen cause bells palsy

A 
herpes
varicella
ebv
lyme
mumps
mycoplasma
178
Q

manifestation of bells

A

paretic upper and lower face
taste anterior 2/3 affected
unable to close eye
NO paresthesia

179
Q

tx bells palsy

A

prednisone 1mg/kg/dose x 1 wk then taper

PPS - From Nelson's (24 decks)

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