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Neurology Flashcards

1
Q

GA at primary neurulation

A

GA 3-4 wks

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2
Q

GA at secondary neurulation

A

GA 4-7 wks

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3
Q

D/Os of primary neurulation

A

anencephaly
encephalocele
myelomeningocele
arnold chiari

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4
Q

D/Os of secondary neurulation

A

spinal cord, lower sacral segments disorders:
spinal cysts
tethered cord
lipoma
teratoma
myelocystocele
meningocele-lipomeningocele

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5
Q

GA prosencephalic development (ventral induction)

A

2-3 months

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6
Q

D/Os of prosencephalic development

A

aprosencephaly (formation)
holoprosencephaly (cleavage)

midline:
agenesis of corpus callosum
agenesis of septum pellucidum
septooptic dysplasia

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7
Q

GA of neural and glial proliferation

A

3-4 months

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8
Q

D/Os of neural and glial proliferation

A

micrencephaly
macrencephaly

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9
Q

GA of neuronal migration

A

3-5 months

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10
Q

D/Os of neuronal migration

A

schizencephaly - abnormal clefts
lissencephaly - underdevelopment of gyri
pachygyria - abnormal large gyri
polymicrogyria

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11
Q

GA of neuronal organization

A

axonal - 3 months to birth
dendritic and synaptic - 6 mos - 1 year
synaptic rearrangement: birth - 1 year

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12
Q

D/Os of organization

A

mental deficiencies
trisomy 21
fragile x
autism
angelman
prematurity

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13
Q

GA of myelination

A

birth to years (

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14
Q

D/Os of myelination

A
  • cerebral white matter hypoplasia
  • prematurity
  • malnutrition
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15
Q

Level of lesions and reflex present

A

cervical/thoracic/until L2 - no reflex
Lumbar L3-L4 - knee jerk
L5-S1 - ankle jerk
S2-S4 - anal wink

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16
Q

Dandy walker malformation triad

A

agenesis/hypoplasia cerebellar vermis
cystic dilatation of the fourth ventricle
enlargement of posterior fossa

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17
Q

cerebral perfusion pressure =

A

MAP - ICP

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18
Q

Factors that increase CBF

A

BP
paCO2
hypoxemia
dopamine
anemia
increased fetal hgb proportion
hypoglycemia
seizures

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19
Q

Factors that decrease CBF

A

hypocarbia
paO2
increased hgb
decreased fetal proportion

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20
Q

head circumference growth

A

week 1: decrease HC
week 2 increase 0.5 cm
week 3: increase 0.7 cm
week 4 + 1 cm/week

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21
Q

scaphocephaly

A

sagital

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22
Q

dolichocephaly

A

sagital

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23
Q

frontal

A

coronal
Crouzon or Apert

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24
Q

brachycephaly

A

bilateral coronal
Carpenter

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25
Q

trigonacephaly

A

metopic

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26
Q

occipital

A

lamboid

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27
Q

focal cerebral injury symptoms

A

contralateral hemiparesis
eye deviates to side of lesion
FT UE weak
PT LE weak

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28
Q

parasagittal cerebral injury

A

weak proximal (UE >LE)

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29
Q

periventricular cerebral, bilateral injury symptoms

A

symmetric (LE)

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30
Q

spinal cord injury symptoms

A

facial sparing
flacid initially then spastic

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31
Q

Lower motor neuron injury symptoms

A

facial sparing
flaccid weakness
fasciculations

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32
Q

nerve root injury symptoms

A

focal

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33
Q

peripheral nerves

A

generalized weakness

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34
Q

NMJ disorder symptoms

A

generalized weakness/hypotonia

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35
Q

palmer appears, established and disappears

A

26, 32, 2-4 months

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36
Q

plantar appears, established and disappears

A

26, 32, 9-12 months

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37
Q

sucking appears, established and disappears

A

28, 32-34, 12 months

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38
Q

crossed extensor appears, established and disappears

A

30, 34, 2 mos

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39
Q

rooting appears, established and disappears

A

30, 34, 4 months

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40
Q

moro appears, established and disappears

A

30, 38, 2-4 mos

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41
Q

tonic-neck appears, established and disappears

A

35, 2 mos, 6 mos,

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42
Q

placing and stepping appears, established and disappears

A

35-37, term, 2-3 mos

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43
Q

rate of CSF formation

A

0.37 mL/min; remade every 5-7 hours

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44
Q

CSF pathway

A

CP > Munro > 3rd > Sylvius > 4th > Lushka/Megendie > subarachnoid > brainstem/cerebellum/spinal cord

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45
Q

hypoglycorrhachia

A

meningitis, hypoglycemia, ich

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46
Q

xanthochromic

A

ich, hyperbilirubinemia, protein

47
Q

central positive sharp waves

A

periventricular leukomalacia

48
Q

when does eeg become continuous

A

31-33 weeks

49
Q

when does eeg start having continuity and synchrony in awake and active sleep

A

34-35 weeks

50
Q

DWI

A

diffusion weight images - random brownian motion of water molecules allows for earlier identification of ischemic strokes

51
Q

stage 2 HIE outcomes

A

80% normal
20% mild moderate deficitis

52
Q

sequelae of PVL

A

spastic diplegia

53
Q

predispose to PVL

A

periventricular vascular anatomy
cerebral perfusion dependent on systemic perfusion
vulnerability of actively differentiating of myelinating periventricular glial cells
vascular or inflammatory insults leading to oligodendrocyte death and myelin deficiency

54
Q

timing of IVH

A

50% in first 24 hours
90% in first 72 hours

55
Q

location of cephalohematoma and subgaleal

A

superiosteal and beneath galae aponeurotica

56
Q

confined by suture lines

A

cephalohematoma

57
Q

erb duchenne nerve roots

A

C5-C7

58
Q

klumpke nerve roots

A

C8-T1

59
Q

disorders of anterior horn cells

A

HIE
SMA type I Werdnig Hoffman
Neurogenic arthrogryposis

60
Q

disoders of NMJ

A

transient neonatal MG
congenital MG
Diseases affecting ACh release (c.tetani, c. botulinum, hyperMg, aminoglycoside tox)

61
Q

disorders of congenital myopathy

A

myotubular myopathy

62
Q

types of muscular dystrophy

A

congenital myotonic dystrophy
duchenne Xp21 linked
congenital muscular dystrophy

63
Q

SMA1 Werdnig Hoffman genetics

A

AR chr 5

64
Q

Dx tests in SMA1

A

CPK normal
EMG: nonspecific denervation, fasciculations, fibrillations
Biopsy: atrophy of motor units
Nerve conduction: normal

65
Q

Congenital myotonic dystrophy genetics

A

AD chromosome 19, expanded CTG repeat
inherited from mother

66
Q

pathogenesis of congenital myotonic dystrophy

A

altered protein –> dysfunctional Na and K channels

67
Q

Diagnostic tests in congenital myotonic dystrophy

A

CPK normal
EMG: dive bomber
Biopsy: small and round muscle fibers with large nuclei and sparse myofibrils
Nerve conduction: normal

68
Q

Riley-Day genetics

A

AR, Ashkenazi
defective 9q31-33

69
Q

Riley-day pathogenesis

A

peripheral nerve system
reduced number of small unmyelinated nerves that carry pain, temperature, taste and mediate autonomic functions, large myelinated afference nerve fibers

70
Q

Riley-day diagnostic tests

A

pupil constriction to metacholine eye drops or pilocarpine (normal pupils don’t respond)
no flare with intradermal histamine

71
Q

mechanism of phenobarbital

A

barbiturate
increase timing of chloride channels by acting on GABA A receptors that depresses CNS

72
Q

MoA phenytoin/fosphenytoin

A

blocks voltage gated Na channels, blocking repetitive firing of APs

73
Q

MoA levetiracetam

A

binds synaptic vesicle protein SV2A reducing vesicle release

74
Q

MoA midazolam

A

increases GABA activity

75
Q

MoA lidocaine

A

blocks Na channel

76
Q

MoA topiramate

A

blocks Na channel

77
Q

MoA Bumetanide

A

inhibits NaKCl cotransporter - for temporal lobe epilepsy

78
Q

pathogenesis of vein of galen

A

persistent median prosencephalic vein of Markowski - usually regresses by week 2, drains into vein of galen

79
Q

MC types of intracranial tumors

A

teratomas > neuroepithelial (medulloblastoma, astrocytoma, choroid plexus papilloma)

80
Q

calcifications in Sturge Weber

A

ipsilateral “tramline” intracortical

81
Q

hemiparesis in sturge weber

A

contralateral to facial lesion

82
Q

genetics sturge weber

A

sporadic

83
Q

genetics tuberous sclerosis

A

AD, ch9 and 16 implicated

84
Q

Genetics NF

A

chr 17 AD

85
Q

tumors associated with NF

A

cutaneous neurofibroma, schwannoma, pheochromocytoma

86
Q

genetics mccune albright

A

sporadic

87
Q

mccune albright clinical features

A

irregular brown pigmentations
fibrous dysplasia
precocious puberty
hyperthyroid
hyperparathyroid
pituitary adenomas

88
Q

von Hippel Lindau genetics

A

AD short arm chr 3

89
Q

vHL tumors

A

hemangioblastoma, retinal angiomas, pheochromocytoma

90
Q

Rate of CP in term and 32-36 and < 28 (out of 1000 births)

A

1, 10, 40-100

91
Q

APGAR score 0-3 at 5 - 10 - 20 minutes and risk of CP

A

1 - 9- 57%

92
Q

hemiplegia vs diplegia

A

hemi is one side, diplegia is just legs

93
Q

MC type CP

A

spastic 85-90%
2/3 bilateral
pyramidal

94
Q

dyskinetic types of CP

A

dystonic and choreathetotic

95
Q

dystonic type of CP symptoms

A

reduced activity with increased tone - stiff movements

96
Q

choreoathetotic

A

increased activity and reduced tone - uncoordinated writing/jerky movements

97
Q

Classification of IQ

A

52-68 mild
36-51 moderate
20-35 severe
< 20 profound

98
Q

Hydrancephaly

A
  • absence of cerebral hemispheres
  • replaced with homogeneous echoic material
  • preservation of thalami, brainstem, and cerebellum
    a/w internal carotid artery occlusion
99
Q

neurochemical findings in menkes

A
  • partial deficiency of dopamine-beta-hydroxylase
  • compensatory increases in catecholamine in sympathetic nerves and brain (increased NE release)
  • Increased tyrosine hydroxylation
100
Q

virus associated with lissencephaly

A

cmv

101
Q

differences between middle interspheric variant, lobar, semilobar and alobar

A

MIHV - nonseparation of posterior aspects of frontal lobe and parietal lobe

lobar: nonseparation of basal aspects of frontal lobe

semilobar: nonseparation of the frontal lobes

alobar: cortical nonseparation

102
Q

MC location for stroke

A

L MCA

103
Q

MC type of PVL

A

diffuse

104
Q

hypomyelination from PVL caused by injury to oligodendrocyte at which stage?

A

pre-oliogodendrocyte

105
Q

NDI among those with recurrent seizure

A

40-60%

106
Q

time period fetus responds to sound

A

20-25 weeks

107
Q

SCM nerve

A

accessory CN 11

108
Q

Mobius

A

CN 6 and 7

109
Q

pupillary light

A

CN 3 oculomotor

110
Q

gag reflex

A

CN 9 and 10

111
Q

hyperthermia post hypoxia-ischemia neuronal cell death in

A

hippocampus

112
Q

onion bulb formation biopsy

A

dejerine sottas

113
Q

rod like on muscle biopsy

A

nemaline

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