CNS Development

Question 1

These 3 primary vessicles become these 5 secondary vessicles:
prosencephalon (forebrain) divides into ______ and ________.
mesencephalon (midbrain) _________
rhombencephalon (hindbrain) _________ and __________

Answer 1

telencephalon, diencephelon, remains mesencephalon, metencephalon, myelencephalon

Question 2

The adult derivatives of the telencephalon are the _____ and the _______.

Answer 2

cerebral hemisphere (including basal nuclei), internal capsule

Question 3

The adult derivatives of the diencephalon are the ______ and the _______.
The _______ and ________ are also derivatives.

Answer 3

thalamus, hypothalamus, neural retina, optic nerve

Question 4

The adult derivative of the mesencephalon is the ______.

Answer 4

midbrain

Question 5

The adult derivatives of the metencephalon are the _______ and the ________.

Answer 5

pons, cerebellum

Question 6

The adult derivative of the myelencephalon is the _______.

Answer 6

medulla oblongota

Question 7

The _______ flexure in the developing embryo is not maintained in the adult.

Answer 7

cervical

Question 8

The site of cell division and proliferation of neuroepithelial progenitors is the _________.

Answer 8

ventricular zone (VZ)

Question 9

neuroepithelial cells complete mitosis while attached to the _________ surface.

Answer 9

ventricular

Question 10

Neural tube layers (there are 5):

Answer 10

ventricular zone, marginal zone, intermediate zone, subventricular zone, ependymal zone

Question 11

cells that remain in ventricular zone become ________ cells that line the ventricles

Answer 11

ependymal

Question 12

The adult derivative of marginal zone is ______.

Answer 12

lamina 1 of cortex (marginal layer)

Question 13

cerebral cortex forms from the expansion of the superficial part of the __________ into the subplate and the cortical plate.

Answer 13

intermediate zone (IZ)

Question 14

The adult derivative of the cortical plate is the __________

Answer 14

cortex laminae II-IV

Question 15

subplate and intermediate zone (IZ) become the _________ in the adult

Answer 15

subcortical white matter

Question 16

cortical layer III and V neurons are _________ and extend ______.

Layer ____ receives thalamocortical axons and is an input layer.

Answer 16

output layers, axons, IV

Question 17

The formation of most gyri occurs by week ___ and are produced by peak periods of ____________.

Answer 17

20, neuroblast migration

Question 18

mutations in _________ disrupt final stage of neuronal migration through the cortical subplate.

Answer 18

Reelin (RLN)

Question 19

________ is expressed in migrating neurons and mutation results in arrest of migration in subcortical white matter before reaching the cortical plate.

Answer 19

Doublecortin (DCX)

Question 20

__________ is expressed in migrating neurons and interacts with dynein. Mutations result in disruption to migration and lamination (integration into cortical lamina).

Answer 20

Lissencephaly (LIS1)

Question 21

Reelin (RLN) mutation results in ________

Answer 21

cortical plate develops beneath subplate

Question 22

schizencephaly results in defect in development of ________ flexure which forms lateral fissure.

Answer 22

telencephalic

Question 23

schizencephaly is characterized by _________________.

Answer 23

continuity of cortex from surface of brain into channel of defect

Question 24

subcortical laminar (band) heterotopia is caused by a mutation in \_\_\_\_\_\_ and this mutation is inherited in a \_\_\_\_\_\_\_\_\_ manner.
Results in enlarged \_\_\_\_\_\_\_\_ and lack of appropriate lamination.

Answer 24

DCX, X-linked recessive, ventricles

Question 25

__________ is characterized by thick cortex and periventricular aggregates of gray matter.
It is caused by neuroblast arrest in subcortical white matter before reaching cortical plate.

Answer 25

focal cortical dysplasia

Question 26

____________ is a disorder of neuroblast migration and may be genetic or congenital (CMV during fetal life).
It is characterized by a smooth cerebral cortex and enlarged lateral ventricles.

Answer 26

lisencephaly

Question 27

The cerebellum arises from the _________ and expands posteromedially to form the _____________.

Answer 27

rhombic lip, cerebellar plate

Question 28

Intermediate zone in cerebellum contains immature neuroblasts which become ______ cells and _______ cells.
Others that do not migrate become ____________.

Answer 28

purkinje, golgi, cerebellar nuclei

Question 29

Cells that migrate through external germinal layer of the cerebellum become ________ cells.
Cells that do not migrate become ______ and _________ cells.

Answer 29

granule, basket, stellate

Question 30

___________ syndrome: molar tooth sign (looks like a molar in the middle of an axial MRI of the brain).
Deficits include hypotonia, truncal ataxia, irregular jerky eye movements, episodic hyperpnea.

Answer 30

Joubert

Question 31

____________ malformation (not genetic) is characterized by hypoplasia of cerebellar vermis and cystic dilation of 4th ventricle (large black area where cerebellum should be on sagital MRI.

Answer 31

Dandy-Walker

Question 32

___________ malformation: herniation of cerebellum through foramen magnum.
characterized by syringomyelia (white area in spinal cord on sagital MRI)

Answer 32

chiari

Question 33

Type II chiari malformation associated with ___________ and is diagnosed in _________ time of life.

Answer 33

meningomyelocele, childhood

Question 34

_____________ malformation may be asymptomatic and is diagnosed in adulthood.

Answer 34

Type I chiari malformation

Question 35

microglia include _____, ______, and ________ neurons.

They are all derived from __________ cells.

Answer 35

multipolar neurons in brain and spinal cord, LMNs, autonomic preganglonic neurons, mesenchymal

Question 36

All CNS cells derived from neural tube except ________.

Answer 36

microglia