CNS Development Flashcards

1
Q

Lumen of the ______ forms the ventricular system of the brain

A

neural tube

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2
Q

The prosencephalon divides into the _____ and the ______

A

telencephalon, diencephalon

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3
Q

The ________ remains undivided during development of the CNS

A

mesencephalon

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4
Q

The rhombencephalon divides into the _____ and _______

A

metencephalon, myelencephalon

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5
Q

The ______ forms the cerebral hemisphere including the basal nuclei and internal capsule

A

telencephalon

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6
Q

The diencephalon forms the ______ and _______ as well as the _____ and _____

A

thalamus, hypothalamus, neural retina, optic nerve

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7
Q

The ______ forms the midbrain

A

mesencephalon

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8
Q

The metencephalon forms the ______ and _______

A

pons, cerebellum

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9
Q

The ______ forms the medulla oblongata

A

myelencephalon

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10
Q

initial flexures of CNS development are noticeable between weeks ______

A

4-5

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11
Q

initial flexures of CNS developement include the ______ between the ______ and ________; and the _______ between the ______ and ______.

A

cephalic flexure, prosencephalon, mesencephalon

cervical flexure, rhombencephalon, spinal cord

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12
Q

This initial flexure is not maintained in the adult:

A

cervical flexure (between the rhombencephalon and spinal cord)

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13
Q

3 primary vessicles are converted to 5 secondary vessicles by __________

A

deepening of all flexures

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14
Q

The deepening of the ______, _____, ______, and ______ flexures produces the 5 secondary vessicles

A

cephalic, cervical, pontine, telencephalic

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15
Q

At 8.5 weeks gestation, the ______ becomes C-shaped after rapid growth

A

telencephalon

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16
Q

The epithelium of the wall of the neural tube is ________

A

pseudostratified epithelium

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17
Q

The _____ is the innermost cell layer surrounding the lumen of the neural tube and is the site of ______

A

ventricular zone, cell division/proliferation

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18
Q

During the G1 —> S —> G2 phase of the cell cycle, progenitor cells in the ventricular zone have _____ morphology and attach to both the ______ and _____ surfaces.

A

fusiform (spindle-like, taper at both ends); ventricular surface, pial surface

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19
Q

During the ________ phase of the cell cycle, progenitor cell nuclei migrate to the pial surface and back to the ventricular surface

A

G1 —> S —> G2

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20
Q

During the M —> G1 phase of the cell cycle, progenitor cells _____________

A

lose contact with pial surface in mitosis, but daughter cells regain contact

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21
Q

asymmetrical division during mitotic proliferation of neuroepithelial progenitors produces ______

A

blast cells

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22
Q

50% of neuroblasts _______ during development

A

undergo apoptosis

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23
Q

The marginal zone is closest to the _______ surface

A

pial (basal) surface

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24
Q

asymmetrical division of neuroepithelial progenitors is ______ to the _____ surface of the ventricular zone

A

parallel, apical

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25
Q

_____ division produces neuroblasts which eventually migrate out of the ventricular zone to become glioblasts

A

asymmetric (radial)

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26
Q

Symmetrical division of neuroepithelial progenitors is _______ to the apical surface of the ventricular zone

A

perpendicular

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27
Q

The _____ forms immediately after the ventricular zone is formed

A

marginal zone

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28
Q

The ______ contains processes of proliferating cells within the ventricular zone

A

marginal zone

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29
Q

The _____ forms as post-mitotic neuroblasts migrate

A

intermediate zone

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30
Q

The _____ forms between the ventricular zone and marginal zone

A

intermediate zone

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31
Q

The ______ is located at the interface of the ventricular zone and the intermediate zone.

A

subventricular zone

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32
Q

The subventricular zone contains _________ and in some areas it also contains ______

A

progenitor cells of macroglia, stem cells

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33
Q

Cells that remain in the ventricular zone become _______ cells which line the ventricles in the brain

A

ependymal cells

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34
Q

Neuroblasts organized by location from the apical surface to the basal surface are as follows:

A

ependymal zone, ventricular zone, subventricular zone, intermediate zone, subplate, cortical plate, marginal zone

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35
Q

radial glial fibers extend from the _________ to the _______ to guide migrating neuroblasts

A

subventricular zone, cerebral cortex

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36
Q

Neuroblast migration always begins in the _______ and the first cells to migrate detach from radial glia close to the __________ and as time goes on successive waves of migrating neuroblasts detach closer to the ______

A

ventricular zone, ventricular surface, pial surface

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37
Q

Initial waves of neuroblast migration detach at the _______, later waves pass through layers of neuroblasts to detach close to the _____

A

intermediate zone/subplate, pial surface

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38
Q

Cerebral cortex forms from the expansion of the superficial part of the _______ into the _____ and ______

A

intermediate zone, subplate, cortical plate

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39
Q

The ______ is the future cortex lamina I

A

marginal zone

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40
Q

The cortical plate is the future _______

A

cortex laminae II-VI

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41
Q

The _______ and ______ become subcortical white mater, and the ______ is also a transient structure during development with transient synaptic connections

A

subplate, intermediate zone, subplate

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42
Q

The ventricular zone becomes the _______ layer

A

ependymal layer

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43
Q

Neuroblast migration begins at __ weeks and ends at __ weeks

A

6 weeks, 34 weeks

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44
Q

Initially neurons in layers ___ through ___ are undifferentiated functionally

A

II-VI

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45
Q

Layers ____ and ____ extend axons and are output layers.

A

III, V

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46
Q

Layer ___ receives thalamocortical axons and is an input layer

A

IV

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47
Q

The convolutions (reshaping) of the cortex are caused directly by ______

A

neuron migration

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48
Q

Formation of the ______ is complete by 16 weeks

A

primary sulci (lateral, longitudinal, calcarine)

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49
Q

______ is expressed at glial end feet and affects detachment of neurons from radial glia

A

Reelin (RLN)

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50
Q

Mutations in _______ disrupt the final stage of neuronal migration through the cortical plate

A

Reelin (RLN)

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51
Q

_____ is expressed in migrating neurons and interacts with microtubules

A

doublecortin (DCX)

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52
Q

Mutations in ______ result in arrest of neuron migration in subcortical white matter before reaching the cortical plate

A

doublecortin (DCX)

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53
Q

_____ is expressed in migrating neurons and interacts with dynein

A

lissencephaly (LIS1)

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54
Q

Mutations in _______ disrupt neuron migration and lamination

A

lissencephaly (LIS1)

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55
Q

A patient has thickened cortex with a cortical subplate but no cortical plate. His cortical layers are not deliniated. What mutation does he likely have?

A

Doublecortin (DCX) mutation

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56
Q

A patient has an inverted cortical plate and cortical subplate, what mutation does he likely have?

A

Reelin (RLN) mutant

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57
Q

A patient has a defect in development of his telencephalic flexure (which forms the lateral fissure) as shown on MRI. He also has continuity of the cortex from the surface of his brain into the channel of this defect. What disorder does he likely have?

A

schizencephaly

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58
Q

A patient dies and his autopsy reveals that a defect in the cerebral hemisphere left a cleft into his lateral ventricle. What disorder did he likely have?

A

schizencephaly

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59
Q

The 2 types of heterotopic disorders of cortical development are:

A

subcortical laminar (band) heterotopia, focal cortical dysplasia

60
Q

A patient has a disorder of cortical development that causes his cortex to be excessively thick and lack lamination. The ventricles are enlarged on MRI. What disorder does he likely have and what mutation led to it?

A

subcortical laminar (band) heterotopia, DCX mutation (X-linked recessive)

61
Q

Subcortical laminar (band) heterotopia is due to a ________ mutation which is _____ (type of mutation)

A

DCX mutation, X-linked recessive

62
Q

A patient has a thick neural cortex and heterotopic aggregates of gray matter (periventricular) on MRI. What disorder of cortical development does he likely have?

A

focal cortical dysplasia

63
Q

Subcortical laminar (band) heterotopia and focal cortical dysplasia are both caused by:

A

neuroblasts arrest in subcortical white matter before reaching the cortical plate

64
Q

_______, a disorder of neuroblast migration, may be genetic or nongenetic

A

lissencephaly (genetic: LIS1 mutation; non-genetic: congenital infection with CMV as a fetus)

65
Q

This disorder of neuroblast migration is characterized by smooth cerebral cortex and enlarged lateral ventricles:

A

lissencephaly

66
Q

_______, a disorder of neuroblast migration, may be accompanied by microcephaly, dysmorphic facies, epilepsy, visual impairment, hypotonia, or intellectual disability

A

lissencephaly

67
Q

Lissencephaly is often accompanied by ________, or ________, or ________

A

Dandy-Walker, Pachygyria (few wide gyri), polymicrogyria (multiple, excessively small gyri)

68
Q

The _______ arises from the rhombic lip (alar plate structure that forms part of wall of 4th ventricle)

A

cerebellum

69
Q

The _______ expand posteromedially to meet and fuse in the midline to form the cerebellar plate

A

rhombic lips (alar plate structure that forms part of the wall of 4th ventricle)

70
Q

Fissures divide the ______ into flocculonodular lobes

A

cerebellar plate

71
Q

The ___________ zone becomes the internal germinal (granular) layer of the mature cerebellar cortex, white matter, and cerebellar nuclei

A

intermediate zone (IZ)

72
Q

Initially, the fetus has only these cortical layers in the CEREBELLUM:

A

ventricular zone and marginal zone

73
Q

The ________ contains immature neuroblasts in the CEREBELLUM which are migrating outward (toward pial surface) along radial glia

A

intermediate zone/internal germinal layer

74
Q

The immature migrating neuroblasts in the _______ zone of the CEREBELLUM become purkinje cells and golgi cells, others that do not migrate become cerebellar nuclei

A

intermediate zone

75
Q

Cells that do not migrate in the __________ zone/_________ of the CEREBELLUM become cerebellar nuclei

A

intermediate zone/internal germinal layer

76
Q

Neuroblasts in the _______ layer of the CEREBELLUM migrate inward (away from the pial surface) along radial glia

A

external germinal layer

77
Q

The ______ of the CEREBELLUM is the 2nd layer of immature neuroblasts that forms at outer edge of the marginal zone

A

external germinal layer

78
Q

The immature neuroblasts in the external germinal layer of the CEREBELLUM become _______ after they migrate

A

granule cells

79
Q

neuroblasts in the external germinal layer of the CEREBELLUM that do not migrate become ______ cells and ______ cells

A

basket cells, stellate cells

80
Q

The migrating cells of the ______ layer of the CEREBELLUM produce purkinje cells and golgi cells, while the non-migrating cells produce cerebellar nuclei

A

internal germinal layer

81
Q

The migrating cells of the _________ layer of the CEREBELLUM produce granule cells

A

external germinal layer

82
Q

The non-migrating cells of the _______ layer of the CEREBELLUM that stay near the pial surface are basket cells and stellate cells

A

external germinal layer

83
Q

The ________ is the central area of the cerebellum on axial MRI

A

vermis

84
Q

“molar tooth” sign in axial MRI is characteristic of _________ which is a disorder of cerebellar/hindbrain development

A

Joubert syndrome

85
Q

The 4 disorders of cortical development are:

A

schizencephaly, subcortical laminar heterotopia, focal cortical dysplasia, lissencephaly

86
Q

Clinical signs of _______ are hypotonia, truncal ataxia, developmental delay, oculomotor ataxia, episodic hyperpnea, and progressive worsening of this disorder

A

Joubert syndrome

87
Q

__________ is characteristic of Joubert syndrome and is characterized by irregular, jerky eye movements secondary to role of the cerebellum in smooth pursuit eye movements

A

oculomotor ataxia

88
Q

The only disorder of cerebellar development that is not genetic is:

A

Dandy-Walker malformation

89
Q

This disorder of cerebellar/hindbrain development is characterized by hypoplasia of the cerebellar vermis and cystic dilation of the fourth ventricle

A

Dandy-Walker malformation

90
Q

This disorder is characterized by a large cystic dilation of the fourth ventricle which looks like a large hypodense area in the posterior brain on both axial and sagital MRI

A

Dandy-Walker malformation

91
Q

Clinical signs of ________ are hypotonia, delayed motor development and ataxia. Intellectual impairment in majority of patients)

A

Dandy-Walker malformation

92
Q

________ is characterized by herniation of cerebellar tonsils through the foramen magnum

A

chiari malformation

93
Q

Type II chiari malformation is associated with _________ diagnosed in this time in life: _______

A

meningomyelocele, childhood

94
Q

Type I chiari malformation is associated with _________ and is diagnosed in this time in life: ______

A

being asymptomatic, adulthood

95
Q

Syringomyelia is a hyperdense spot in the middle of the spinal cord near the cerebellum which is characteristic of _________

A

chiari malformations

96
Q

All of the cells of the CNS develop from the neural tube except _______

A

microglia

97
Q

macroglia derive from _________

A

mesenchymal cells

98
Q

The 3 CNS neuron types are:

A

multipolar neurons in brain and spinal cord, LMNs, autonomic preganglionic neurons

99
Q

The 3 types of CNS (macro)glial cells are:

A

astrocytes, oligodendrocytes, ependymal cells

100
Q

______ is expressed at the apical cortical surface, while _____ is expressed at the basal (pial) surface

A

notch, numb

101
Q

_______ amplifies level of stem cell population formed by perpendicular cleavage plane and symmetrical division

A

notch

102
Q

_______ is associated with symmetrical neuronal and glial cell division

A

notch

103
Q

Daughter neuronal or glial cell exposed to high numb closer to the basal surface undergoes _________ division and its cleavage plane is _______ to the basal surface

A

asymmetrical division, parallel

104
Q

In early asymmetrical division, both Notch signaling and expression of bHLH biases neural progenitor cells towards becoming ________

A

neurons

105
Q

Early asymmetrical division produces mostly ______, while later stages produce mostly ________

A

neuroblasts, glioblasts

106
Q

Late asymmetrical division with Notch signaling but little expression of bHLH genes promotes _________ development

A

astrocyte

107
Q

The _______ separates the (posterior) alar plate from the (anterior) basal plate

A

sulcus limitans

108
Q

High BMP is present at the _______ and becomes the posterior horns in spinal cord

A

alar plate

109
Q

High SHH is expressed in the ________ which becomes the anterior and lateral horns in the spinal cord

A

basal plate

110
Q

The development of the basal plate is induced by the _______ through high levels of ______

A

notocord, SHH

111
Q

The _______ in the brainstem divides alar and basal derivatives into lateral and medial columns

A

sulcus limitans

112
Q

The lateral portion of the brainstem is derived from ________ and is called the _______ during development

A

neural crest, alar plate

113
Q

The _______ of the developing brainstem is made up of afferent functional columns (VA, SA, cranial nerve nuclei)

A

alar plate

114
Q

The _______ of the developing brainstem is made up of efferent functional columns (VE, SE, cranial nerve nuclei)

A

basal plate

115
Q

The medial portion of the developing brainstem is made up of primarily ________

A

efferent functional columns (VE, SE, cranial nerve nuclei)

116
Q

The ______ derivatives in the medulla are the gracile and cuneate nuclei, vestibular, and trigeminal, as well as the solitary nucleus and inferior olivary nuclei

A

alar plate

117
Q

The _______ develop from the rhombic lip

A

inferior olivary nuclei

118
Q

The dorsal motor nucleus of vagus, inferior salivatatory nucleus, hypoglossal, and nucleus ambiguus develop from the ________ of the medulla

A

basal plate

119
Q

The ______ derivatives in the pons are the vestibular, cochlear, principle sensory, spinal trigeminal, solitary, and pontine nuclei

A

alar plate

120
Q

The ________ develop from the rhombic lip

A

pontine nuclei

121
Q

The _______ derivatives of the pons are the superior salivatory, abducens, facial, and trigeminal nuclei

A

basal plate

122
Q

VA nuclei are more ________ in the pons and medulla than the SA nuclei

A

medial

123
Q

Midbrain _______ derivatives include the superior and inferior colliculi, red nucleus, and substantia nigra

A

alar plate

124
Q

Midbrain _______ derivatives include the EW, oculomotor, and trochlear nuclei

A

basal plate

125
Q

immature neuroblasts (specialized neuroepithelial cells) that migrate through/to the intermediate zone are ______

A

apolar

126
Q

The immature neuroblasts within the intermediate zone or at cortical plate extend ________

A

neurites

127
Q

The longest neurite in a neuroblast is its:

A

axon

128
Q

__________ and ________ contain dense actin filaments and their central core contains _______ and _________

A

lamellipodia, filopodia, tubulin, microtubules

129
Q

central core of axon growth cones is made up of _______ and _______

A

tubulin, microtubules

130
Q

The purpose of _______, ________ and ________ of growing axons is to explore and determine direction of growth of the axon

A

lamellipodia, filopodia, central core

131
Q

Binding of ________ to __________ prevents axons of retinal ganglion cells of the temporal retina from crossing at the optic chiasm

A

EphB1, ephrin-B2

132
Q

Radial glia at the cross point of the optic chiasm expresses ________ which prevents RGCs of temporal retina from crossing the chiasm

A

ephrin-B2

133
Q

The _______ transpforms into a ________ as an axon reaches its target

A

growth cone, presynaptic nerve terminal

134
Q

NGF, BDNF, and FGF are ___________

A

trophic factors secreted by a target cell to an axon that promote neuron survival

135
Q

In neuromuscular synaptogenesis, a single site on a target cell is occupied by multiple axons, what stage of development are these axons in?

A

synaptogenesis

136
Q

The first two steps in neuromuscular synaptogenesis are:

A
  1. growth cone approaches newly fused myotube

2. makes morphologically unspecified but functional contact

137
Q

What type of synaptogenesis involves multiple axons converging on a single site on a target cell?

A

PNS neuromuscular junction

138
Q

synapses in the CNS and the PNS are structurally dissimilar. True or false?

A

false

139
Q

_____ provides a clinically useful measure of neurological maturation in the preterm infant

A

EEG

140
Q

In _______ synaptogenesis, initial contact between an axon and a filopodium on a developing dendrite leads to a stable dendritic spine

A

CNS

141
Q

CNS synaptogenesis involves a(n) ________ and a(n) __________ contacting each other

A

axon, filopodium of developing dendrite

142
Q

_______ and ________ do not begin myelination until near term

A

optic nerve, geniculocalcarine tract

143
Q

The _______ begins myelination at 4 months postnatally and continues into mid-adolescence

A

corpus callosum

144
Q

The ________ reflects myelination of the corticospinal tract

A

Babinski sign

145
Q

The _______ is normal in infants up to 18 months of age

A

babinski sign