Sos: development of CNS Flashcards

1
Q

brain to gut; the ultimate survival machine

A

autonomic NS

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2
Q

areas that close last along neural tube where congenital malformations happen

A

cranial and sacral region

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3
Q

neural plate to neural _____ to neural ____

A

plate to crest cells to tube

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4
Q

sensory and regulated by TGF-beta and BMPs (bone morphogenic proteins)

A

Alar plate (dorsal)

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5
Q

motor and induced by SHH

A

basal plate (ventral)

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6
Q

neural tube has same ____ as spinal cord

A

orientation

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7
Q

neural migration:

A

ventricular region to pia mater using radial glial cells and hormones

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8
Q

telencephalon and diencephalon

A

forebrain

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9
Q

mesencephalon

A

midbrain

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10
Q

pons and cerebellum

A

metencephalon

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11
Q

medulla oblongata

A

myelencephalon

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12
Q

metencephalon and myelencephalon

A

hindbrain

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13
Q

cerebrum, thalamus, hypothalamus

A

forebrain

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14
Q

brain stem

A

midbrain and hindbrain

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15
Q

comes from neural crest tissue and from mutation in C-MYC gene

A

neuroblastoma

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16
Q

causes of neuronal migration defects

A

failure of neurons to migrate
scattered neuron arrangement
late stage neuronal migration

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17
Q

abnormal migration of neurons causes abnormal ____ pattern

A

gyral

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18
Q

well studied gene associated with neuronal migration defects

A

RELN/Lis

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19
Q

With _____ mutation, actin and microtubules don’t form properly and they don’t migrate; don’t migrate to final resting place=cant be fully functional

A

RELN/Lis

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20
Q

neuronal migration defects lead to a 4 layer cortex instead of a ____ layer cortex

A

6

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21
Q

stain to look at cell bodies

A

Nissl stain

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22
Q

Smooth cortex without gyri and thick cortex with four cell layers

A

Lissencephaly

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23
Q
A

Lissencephaly

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24
Q

smooth cortex
microcephaly
mental delay/seizures

A

Lissencephaly

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25
Q
A

Lissencephaly

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26
Q
A

Lissencephaly

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27
Q

cortex has irregular grooves forming a cobblestone pattern; total disarray of cortex

A

cobblestone cortex

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28
Q

no cortical layers
irregular grooves
neurons migrate past cortex

A

cobblestone cortex

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29
Q

Broad gyri and thick cortex with few layers
tissue not compatible with learning and development

A

Pachygyria

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30
Q

broad gyri

A

Pachygyria

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31
Q

broad gyri and thick cortex

A

Pachygyria

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32
Q

Surface of the cortex appears to form microgyri

A

Polymicrogyria

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33
Q

Often found in vascular territories or watershed areas suggesting a hypoxic/ischemic event; microgyri

A

Polymicrogyria

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34
Q

normal neurons/glia found in the wrong place

A

heterotopia

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35
Q

Collections of neurons found under the ependyma of the lateral ventricles
Unilateral variants are silent and incidental findings during imaging studies
(neurons found in wrong place)

A

Periventricular (subependymal) Heterotopia

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36
Q
A

Periventricular (subependymal) heterotopia

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37
Q

neurons in wrong place (didnt migrate out)

A

periventricular (subependymal) heterotopia

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38
Q

Collections of neurons in the cerebral white matter diffusely scattered, clustered into nodules, bands or grouped in large masses

A

Subcortical neuronal (band) heterotopia

39
Q

associated with seizures

A

subcortical neuronal (band) hetertopia

40
Q

used to stain myelin

A

Luxol fast blue (LFB)

41
Q

not termed precancerous when talking about the brain

A

cortical dysplasia

42
Q

(grey matter) improperly forming
Most common form is focal ____

A

cortical dysplasia

43
Q

most common cause of epilepsy (often gray matter found in temporal lobe)

A

cortical dysplasia

44
Q
A

cortical dysplasia

45
Q

top
bottom

A

top: normal
bottom: cortical dysplasia

46
Q

Infected in utero; skull in vault doesn’t form and brain and cortex don’t form
(microcephaly)

A

Zika virus

47
Q

autism, muscle spasms, mental delay
Due to disrupted neuronal migration along with an abnormal neuronoglial differentiation

A

tuberous sclerosis syndrome

48
Q

organs involved in tuberous sclerosis syndrome

A

brain, heart, kidneys, skin

49
Q

Neocortical tubers
Subependymal nodules
Subependymal giant cell astrocytomas (SEGAs)

A

brain involvement of tuberous sclerosis syndrome

50
Q

rhabdomyomas— heart
angiomyolipomas (hamartomas)– kidney
angiofibromas —-skin

A

Tuberous sclerosis syndrome

51
Q
A

tubers
(tuberous sclerosis sydnrome)

52
Q

heart mass in children

A

tuberous sclerosis syndrome

53
Q
A

heart rhabdomyoma

54
Q

kidney angiomyolipoma (hamartoma)

A

tuberous sclerosis syndrome

55
Q

A single telencephalic ventricle with continuity of the cerebral hemisphere across the midline

A

Holoprosencephaly

56
Q

forebrain aka

A

prosencephalon

57
Q

Associated craniofacial abnormalities, most severe being cyclopia

A

holoprosencephaly

58
Q
A

holoprosencephalies

59
Q
A

alobar holoprosencephalies

60
Q

most common congenital malformation disorders

A

neural tube defects

61
Q

increased AFP in amniotic fluid

A

neural tube defects

62
Q

poorly controlled DM
valproic acid
folic acid and vitamin B12 deficiency

A

neural tube defects

63
Q

open brain and lack of skull vault

A

Anencephaly

64
Q

herniation of meninges and midbrain

A

Encephalocele

65
Q

some of vertebrae are not completely closed; asymptomatic (can sometimes feel tuff of hair there)

A

Spina bifida occulta

66
Q

protrusion of meninges and CSF
through defect in skull or spine

A

Meningocele

67
Q

open spinal cord w/ a meningeal cyst (spinal cord and meninges pushed out)

A

Myelomeningocele

68
Q
A

meningocele

69
Q

skull defect
involves meninges and CSF

A

meningocele

70
Q

skull defect involves brain parenchyma, meninges, and CSF

A

Encephalocele

71
Q

80% of cephaloceles happen in what region

A

occipital region

72
Q
A

myeloencephalocele or encephalocele

73
Q

midline of cerebellum

A

vermis

74
Q

hindbrain aka

A

posterior fossa

75
Q

condition in which brain extends through the foramen magnum

A

Chiari malformations

76
Q

minimal brain tissue extends downwards
cerebellar tonsils are displaced and
Syrinx formation

A

Type I Chiari malformation

77
Q

greater amounts of brain tissue extends downward and a myelomeningocele and hydrocephalus 90% of time

A

type II chiari malformation

78
Q

kinking of medulla and hydrocephalus

A

type II chiari malformation

79
Q

most severe and rare, includes cerebellum and brainstem extending downward

A

type III chiari malformation

80
Q

cerebellum pushed up
enlarged ventricles
hydrocephalus

A

Dandy-Walker syndrome

81
Q

cerebellum pushed up

A

Dandy-Walker syndrome

82
Q

can’t find vermis
(vermal aplasia)

A

Dandy-Walker syndrome

83
Q

Most likely due to decreased or lack of blood flow and brain development
brain still intact
almost complete loss of cortical tissue though

A

Hydranencephaly

84
Q
A

hydranencephaly

85
Q

Brain parenchyma compressed to where it is almost nonexistent/nonfunctioning

A

Hydranencephaly

86
Q

MCA stroked and all lateral part of brain necrosed
cavitary necrosis of the cerebrum, extending to the surface of the brain

A

Porencephaly

87
Q
A

Porencephaly

88
Q

all tissue looks like lissencephaly
a cleft of the cerebrum without ventricular communication

A

Schizencephaly

89
Q

blood into ventricles of fetus and causes clot and cardiac arrhythmia and death

A

germinal matrix bleeds

90
Q

Immature vessels w/o stroma
Hypoxic stress
Ischemic endothelial cells

A

germinal matrix bleeds

91
Q
A

germinal matrix bleed

92
Q

Neurological disorders that appear in infancy or early childhood and permanently affects body movement and muscle coordination but doesn’t worsen over time

A

cerebral palsy

93
Q

muscle tone that is either too stiff or too relaxed and appears before child reaches 3
ataxia

A

Cerebral palsy