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CORE--Peds > Pediatric Neuro > Flashcards

Pediatric Neuro Flashcards

1
Q

Myelination maturity

A

2 years, frontal lobes continue to myelinate through adolescence

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2
Q

cortex development dependent on?

A

germinal matrix cell migration and organization into 6 layer cortex

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3
Q

What is polymicrogyria

A

malformation of cortex; abnormal shallow/numerous sulcation due to late neuronal migration or early cortical lamellar organization

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4
Q

Causes of polymicrogyria

A

CMV, in utero ishcemia, genetic causes

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5
Q

Most common distribution of polymicrogyria

A

bilateral perisylvian; irruglar grew white junction

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6
Q

Absent/decreased cortex

A

Lissencephaly; 4 instead of 6 layers

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7
Q

Causes of lissencephaly

A

CMV, Walker-Warburg syndrome (muscular dystrophy), etc

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8
Q

Type 1 lissencephaly

A

smooth cortex, figure 8/hour glass configuration of cerebral hemispheres

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9
Q

Type 2 lissencephaly

A

cobblestone appearance, finely undulating

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10
Q

Gray matter heterotopia

A

clusters of normal neurons in abnormal locations (periventricular nodular, subcortical, marginal glioneural)

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11
Q

corpus callosum embryology

A

genu, splenium, rostrum

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12
Q

dilation of the occipital horns/posterior lateral ventricles

A

copocephaly

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13
Q

Probst bundles

A

axons that form the corpus callosum

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14
Q

Associations with corpus callosum abnormalities

A

midline abnormalities; midline hematoma, interhemipheric cyst

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15
Q

Cleft within the cerebral hemispheres lined by gray matter

A

schizencephaly; abnormal connection between ventricles and subarachnoid space

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16
Q

Open vs closed lip schizencephaly

A

open lip: walls of cleft are divided by CSF

closed lip: walls of cleft are incompletely divided/apposed, still touching

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17
Q

schizencephaly mostly involves?

A

frontal lobes

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18
Q

Association with schizencephaly

A

septo-optic dysplasia (agenesis of septum pellucidum, optic nerve hypoplasia)

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19
Q

Ddx interruption of clef in cortext

A

schizencephaly (lined by gray matter), porencephaly, encephalomalacia, surgical resection

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20
Q

Spectrum of holoprosencephaly

A

alobar > semilobar > lobar

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21
Q

Association holoprosencephaly

A

midline maxillofacial anomalies (central incisor); azygous configuration of anterior cerebral artery; Patau syndrome of trisomy 13; Meckel Gruber

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22
Q

lack of separation of cerebral hemispheres; monoventricle with large dorsal cyst; no falx

A

alobar holoprosencephaly

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23
Q

anterior hemispheres fails to separate; some degree of separation of posterior cerebral hemispheres

A

semilobar holoprosencephaly

24
Q

rostral aspects of the frontal neocortex not separated; posterior corpus callosum is present

A

lobar holoprosencephaly

25
Complication of chiari 1
cervical syringomyelia, hydrocephalus
26
inferior displacement of cerebellar tonsils 5mm
Chiari 1
27
Manifestations of Chiari 1
exertional headache, myelopathy, brainstem compromise
28
Imaging findings Chiari 1
crowding posterior fossa, inferior herniation of cerebellar tonsils 5 mm, possible syringomyelia/hydrocephalus
29
Imaging findings chiari 2
beaking of tectum, herniation of vermis/tonsils; myelomeningocele; hydrocephalus from 4th ventricle obstruction; possible corpus callosal dysgenesis, heterotopia, sulcation abnormalities; prenatal US lemon head and banana cerebellum
30
Dandy walker complex imaging features
enlarged posterior fossa, posterior fossa cyst, inferior vermian hypoplasia, ?hydrocephalus, torcular-lambdoid inversion
31
Cause of Dandy Walker
in utero insult to developing fourth ventricle
32
What is torcular lambdoid inversion
confluence of sinuses above the lambdoid suture
33
von Recklinghausen disease
NF1, neurocutaneous disorder
34
Manifestations of NF1
cafe au lait spots, peripheral nerve sheath tumors (plexiform neurofibroma), CNS malignancy (optic nerve glioma), bony abnormalities (sphenoid wing dysplasia)
35
NF genetics
AD, defect in chromosome 17
36
MR findings of NF1 in kids
bright spots (T2 imaging) from myelin vacuolization that become less prominent
37
Cell of origin for plexiform neurofibromas
Schwann cells; commonly involve cutaneous/subcutaneous nerves
38
NF1 brain neoplasm associations
optic nerve glioma, juvenile pilocytic astrocytoma, brainstem glioma
39
Bone manifestations of NF1
sphenoid wing dysplasia (pulsatile enophtalmos/exopthalmos), posterior vertebral body scalloping, rib notching (from neurofibromas), focal gigantism, cervical kyphoscoliosis, neural foraminal enlargement, tibial bowing
40
Eye manifestations of NF1
hamartomas of iris (Lisch nodules)
41
extracranial neoplasms of NF1
Wilms tumors, Rhabdomyosarcoma, AML, leiomyosarcoma
42
NF 2 genetics
AD; chromosome 22; less common than NF1
43
MISME for NF2
multiple inherited schwannomas, meningiomas, ependymomas
44
Presentation for NF2
bilateral vestibular schwannomas
45
facial port-wine stain, ocular abnormalities, fialure of cortical venous development
Sturge Weber
46
port wine stain in what nerve region?
V1 opthalamic branch (forehead, upper eyelid)
47
vascular disorder due to regression of primitive embryologic cephalic venous plexus; leptomeningeal venous angiomatosis
Sturge Weber
48
Sturge Weber findings
chronic ischemia, cortical atrophy , calcifications
49
Presentation of sturge weber in pt
developmental delay, seizures
50
Triad: adenoma sebaceum, seizures, mental retardation
TS
51
nodular rash in the nasolabial folds
adenoma sebaceum
52
Imaging findings of TS neuro
multiple white matter cortical/subcortical tubers (hamartomas), subependymal nodules
53
Extracranial manifestations of TS
renal AML, cardiac rhabdomyoma, lung lymphangioleiomyomatosis
54
TS association with CNS malignancy
supependymal giant cell astrocytoma (SEGA)
55
Molar tooth midbrain syndrome
Joubert syndrome ; aplasia of cerebellar vemis/cerebellum; cerebello-oculo-renal syndrom
56
Presentation of Joubert
hypotonic child with developmental delay/ataxia; ocular anomalies

CORE--Peds (7 decks)

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