Neurology Flashcards
(113 cards)
1
Q
GA at primary neurulation
A
GA 3-4 wks
2
Q
GA at secondary neurulation
A
GA 4-7 wks
3
Q
D/Os of primary neurulation
A
anencephaly
encephalocele
myelomeningocele
arnold chiari
4
Q
D/Os of secondary neurulation
A
spinal cord, lower sacral segments disorders:
spinal cysts
tethered cord
lipoma
teratoma
myelocystocele
meningocele-lipomeningocele
5
Q
GA prosencephalic development (ventral induction)
A
2-3 months
6
Q
D/Os of prosencephalic development
A
aprosencephaly (formation)
holoprosencephaly (cleavage)
midline:
agenesis of corpus callosum
agenesis of septum pellucidum
septooptic dysplasia
7
Q
GA of neural and glial proliferation
A
3-4 months
8
Q
D/Os of neural and glial proliferation
A
micrencephaly
macrencephaly
9
Q
GA of neuronal migration
A
3-5 months
10
Q
D/Os of neuronal migration
A
schizencephaly - abnormal clefts
lissencephaly - underdevelopment of gyri
pachygyria - abnormal large gyri
polymicrogyria
11
Q
GA of neuronal organization
A
axonal - 3 months to birth
dendritic and synaptic - 6 mos - 1 year
synaptic rearrangement: birth - 1 year
12
Q
D/Os of organization
A
mental deficiencies
trisomy 21
fragile x
autism
angelman
prematurity
13
Q
GA of myelination
A
birth to years (
14
Q
D/Os of myelination
A
- cerebral white matter hypoplasia
- prematurity
- malnutrition
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
16
Q
Dandy walker malformation triad
A
agenesis/hypoplasia cerebellar vermis
cystic dilatation of the fourth ventricle
enlargement of posterior fossa
17
Q
cerebral perfusion pressure =
A
MAP - ICP
18
Q
Factors that increase CBF
A
BP
paCO2
hypoxemia
dopamine
anemia
increased fetal hgb proportion
hypoglycemia
seizures
19
Q
Factors that decrease CBF
A
hypocarbia
paO2
increased hgb
decreased fetal proportion
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
21
Q
scaphocephaly
A
sagital
22
Q
dolichocephaly
A
sagital
23
Q
frontal
A
coronal
Crouzon or Apert
24
Q
brachycephaly
A
bilateral coronal
Carpenter
25
trigonacephaly
metopic
26
occipital
lamboid
27
focal cerebral injury symptoms
contralateral hemiparesis
eye deviates to side of lesion
FT UE weak
PT LE weak
28
parasagittal cerebral injury
weak proximal (UE >LE)
29
periventricular cerebral, bilateral injury symptoms
symmetric (LE)
30
spinal cord injury symptoms
facial sparing
flacid initially then spastic
31
Lower motor neuron injury symptoms
facial sparing
flaccid weakness
fasciculations
32
nerve root injury symptoms
focal
33
peripheral nerves
generalized weakness
34
NMJ disorder symptoms
generalized weakness/hypotonia
35
palmer appears, established and disappears
26, 32, 2-4 months
36
plantar appears, established and disappears
26, 32, 9-12 months
37
sucking appears, established and disappears
28, 32-34, 12 months
38
crossed extensor appears, established and disappears
30, 34, 2 mos
39
rooting appears, established and disappears
30, 34, 4 months
40
moro appears, established and disappears
30, 38, 2-4 mos
41
tonic-neck appears, established and disappears
35, 2 mos, 6 mos,
42
placing and stepping appears, established and disappears
35-37, term, 2-3 mos
43
rate of CSF formation
0.37 mL/min; remade every 5-7 hours
44
CSF pathway
CP > Munro > 3rd > Sylvius > 4th > Lushka/Megendie > subarachnoid > brainstem/cerebellum/spinal cord
45
hypoglycorrhachia
meningitis, hypoglycemia, ich
46
xanthochromic
ich, hyperbilirubinemia, protein
47
central positive sharp waves
periventricular leukomalacia
48
when does eeg become continuous
31-33 weeks
49
when does eeg start having continuity and synchrony in awake and active sleep
34-35 weeks
50
DWI
diffusion weight images - random brownian motion of water molecules allows for earlier identification of ischemic strokes
51
stage 2 HIE outcomes
80% normal
20% mild moderate deficitis
52
sequelae of PVL
spastic diplegia
53
predispose to PVL
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
timing of IVH
50% in first 24 hours
90% in first 72 hours
55
location of cephalohematoma and subgaleal
superiosteal and beneath galae aponeurotica
56
confined by suture lines
cephalohematoma
57
erb duchenne nerve roots
C5-C7
58
klumpke nerve roots
C8-T1
59
disorders of anterior horn cells
HIE
SMA type I Werdnig Hoffman
Neurogenic arthrogryposis
60
disoders of NMJ
transient neonatal MG
congenital MG
Diseases affecting ACh release (c.tetani, c. botulinum, hyperMg, aminoglycoside tox)
61
disorders of congenital myopathy
myotubular myopathy
62
types of muscular dystrophy
congenital myotonic dystrophy
duchenne Xp21 linked
congenital muscular dystrophy
63
SMA1 Werdnig Hoffman genetics
AR chr 5
64
Dx tests in SMA1
CPK normal
EMG: nonspecific denervation, fasciculations, fibrillations
Biopsy: atrophy of motor units
Nerve conduction: normal
65
Congenital myotonic dystrophy genetics
AD chromosome 19, expanded CTG repeat
inherited from mother
66
pathogenesis of congenital myotonic dystrophy
altered protein --> dysfunctional Na and K channels
67
Diagnostic tests in congenital myotonic dystrophy
CPK normal
EMG: dive bomber
Biopsy: small and round muscle fibers with large nuclei and sparse myofibrils
Nerve conduction: normal
68
Riley-Day genetics
AR, Ashkenazi
defective 9q31-33
69
Riley-day pathogenesis
peripheral nerve system
reduced number of small unmyelinated nerves that carry pain, temperature, taste and mediate autonomic functions, large myelinated afference nerve fibers
70
Riley-day diagnostic tests
pupil constriction to metacholine eye drops or pilocarpine (normal pupils don't respond)
no flare with intradermal histamine
71
mechanism of phenobarbital
barbiturate
increase timing of chloride channels by acting on GABA A receptors that depresses CNS
72
MoA phenytoin/fosphenytoin
blocks voltage gated Na channels, blocking repetitive firing of APs
73
MoA levetiracetam
binds synaptic vesicle protein SV2A reducing vesicle release
74
MoA midazolam
increases GABA activity
75
MoA lidocaine
blocks Na channel
76
MoA topiramate
blocks Na channel
77
MoA Bumetanide
inhibits NaKCl cotransporter - for temporal lobe epilepsy
78
pathogenesis of vein of galen
persistent median prosencephalic vein of Markowski - usually regresses by week 2, drains into vein of galen
79
MC types of intracranial tumors
teratomas > neuroepithelial (medulloblastoma, astrocytoma, choroid plexus papilloma)
80
calcifications in Sturge Weber
ipsilateral "tramline" intracortical
81
hemiparesis in sturge weber
contralateral to facial lesion
82
genetics sturge weber
sporadic
83
genetics tuberous sclerosis
AD, ch9 and 16 implicated
84
Genetics NF
chr 17 AD
85
tumors associated with NF
cutaneous neurofibroma, schwannoma, pheochromocytoma
86
genetics mccune albright
sporadic
87
mccune albright clinical features
irregular brown pigmentations
fibrous dysplasia
precocious puberty
hyperthyroid
hyperparathyroid
pituitary adenomas
88
von Hippel Lindau genetics
AD short arm chr 3
89
vHL tumors
hemangioblastoma, retinal angiomas, pheochromocytoma
90
Rate of CP in term and 32-36 and < 28 (out of 1000 births)
1, 10, 40-100
91
APGAR score 0-3 at 5 - 10 - 20 minutes and risk of CP
1 - 9- 57%
92
hemiplegia vs diplegia
hemi is one side, diplegia is just legs
93
MC type CP
spastic 85-90%
2/3 bilateral
pyramidal
94
dyskinetic types of CP
dystonic and choreathetotic
95
dystonic type of CP symptoms
reduced activity with increased tone - stiff movements
96
choreoathetotic
increased activity and reduced tone - uncoordinated writing/jerky movements
97
Classification of IQ
52-68 mild
36-51 moderate
20-35 severe
< 20 profound
98
Hydrancephaly
- absence of cerebral hemispheres
- replaced with homogeneous echoic material
- preservation of thalami, brainstem, and cerebellum
a/w internal carotid artery occlusion
99
neurochemical findings in menkes
- partial deficiency of dopamine-beta-hydroxylase
- compensatory increases in catecholamine in sympathetic nerves and brain (increased NE release)
- Increased tyrosine hydroxylation
100
virus associated with lissencephaly
cmv
101
differences between middle interspheric variant, lobar, semilobar and alobar
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
MC location for stroke
L MCA
103
MC type of PVL
diffuse
104
hypomyelination from PVL caused by injury to oligodendrocyte at which stage?
pre-oliogodendrocyte
105
NDI among those with recurrent seizure
40-60%
106
time period fetus responds to sound
20-25 weeks
107
SCM nerve
accessory CN 11
108
Mobius
CN 6 and 7
109
pupillary light
CN 3 oculomotor
110
gag reflex
CN 9 and 10
111
hyperthermia post hypoxia-ischemia neuronal cell death in
hippocampus
112
onion bulb formation biopsy
dejerine sottas
113
rod like on muscle biopsy
nemaline
