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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
Q

Complication of chiari 1

A

cervical syringomyelia, hydrocephalus

26
Q

inferior displacement of cerebellar tonsils 5mm

A

Chiari 1

27
Q

Manifestations of Chiari 1

A

exertional headache, myelopathy, brainstem compromise

28
Q

Imaging findings Chiari 1

A

crowding posterior fossa, inferior herniation of cerebellar tonsils 5 mm, possible syringomyelia/hydrocephalus

29
Q

Imaging findings chiari 2

A

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
Q

Dandy walker complex imaging features

A

enlarged posterior fossa, posterior fossa cyst, inferior vermian hypoplasia, ?hydrocephalus, torcular-lambdoid inversion

31
Q

Cause of Dandy Walker

A

in utero insult to developing fourth ventricle

32
Q

What is torcular lambdoid inversion

A

confluence of sinuses above the lambdoid suture

33
Q

von Recklinghausen disease

A

NF1, neurocutaneous disorder

34
Q

Manifestations of NF1

A

cafe au lait spots, peripheral nerve sheath tumors (plexiform neurofibroma), CNS malignancy (optic nerve glioma), bony abnormalities (sphenoid wing dysplasia)

35
Q

NF genetics

A

AD, defect in chromosome 17

36
Q

MR findings of NF1 in kids

A

bright spots (T2 imaging) from myelin vacuolization that become less prominent

37
Q

Cell of origin for plexiform neurofibromas

A

Schwann cells; commonly involve cutaneous/subcutaneous nerves

38
Q

NF1 brain neoplasm associations

A

optic nerve glioma, juvenile pilocytic astrocytoma, brainstem glioma

39
Q

Bone manifestations of NF1

A

sphenoid wing dysplasia (pulsatile enophtalmos/exopthalmos), posterior vertebral body scalloping, rib notching (from neurofibromas), focal gigantism, cervical kyphoscoliosis, neural foraminal enlargement, tibial bowing

40
Q

Eye manifestations of NF1

A

hamartomas of iris (Lisch nodules)

41
Q

extracranial neoplasms of NF1

A

Wilms tumors, Rhabdomyosarcoma, AML, leiomyosarcoma

42
Q

NF 2 genetics

A

AD; chromosome 22; less common than NF1

43
Q

MISME for NF2

A

multiple inherited schwannomas, meningiomas, ependymomas

44
Q

Presentation for NF2

A

bilateral vestibular schwannomas

45
Q

facial port-wine stain, ocular abnormalities, fialure of cortical venous development

A

Sturge Weber

46
Q

port wine stain in what nerve region?

A

V1 opthalamic branch (forehead, upper eyelid)

47
Q

vascular disorder due to regression of primitive embryologic cephalic venous plexus; leptomeningeal venous angiomatosis

A

Sturge Weber

48
Q

Sturge Weber findings

A

chronic ischemia, cortical atrophy , calcifications

49
Q

Presentation of sturge weber in pt

A

developmental delay, seizures

50
Q

Triad: adenoma sebaceum, seizures, mental retardation

A

TS

51
Q

nodular rash in the nasolabial folds

A

adenoma sebaceum

52
Q

Imaging findings of TS neuro

A

multiple white matter cortical/subcortical tubers (hamartomas), subependymal nodules

53
Q

Extracranial manifestations of TS

A

renal AML, cardiac rhabdomyoma, lung lymphangioleiomyomatosis

54
Q

TS association with CNS malignancy

A

supependymal giant cell astrocytoma (SEGA)

55
Q

Molar tooth midbrain syndrome

A

Joubert syndrome ; aplasia of cerebellar vemis/cerebellum; cerebello-oculo-renal syndrom

56
Q

Presentation of Joubert

A

hypotonic child with developmental delay/ataxia; ocular anomalies

CORE--Peds (7 decks)

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