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Development of Cerebellum/Cerebrum and Congenital Disorders
Medical School Devo
40
Other
Graduate
09/26/2011

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Cards

Term
From where does the cerebellum arise?
Definition
Alar plate of the metencephalon
Term
What is the part of the brain that is the site for sensorimotor integration?
Definition
Cerebellum
Term
What part of the brain contains ~50% of brain neurons?
Definition
Cerebellum
Term
What is the last brain region to develop neurons?
Definition
Cerebellum
Term
What is the name for the midline structure of the cerebellum?
Definition
Vermis
Term
What disease is associated with a missing vermis giving rise to an enlarged fourth ventricle?
Definition
Dandy-Walker syndrome
Term
Neuroblasts in cerebellar development come from what area and move through what to form the EGL?
Definition
Neuroblasts are derived from the fourth ventricle and move through the rhombic lips
Term
What is the External Germinal layer? What type of cells is it composed of?
Definition
The external germinal layer is a transient layer of proliferative neuroblasts that give rise to the neurulation of the cerebellum
Term
What signalling molecule drives the EGL neuroblast proliferation?
Definition
Sonic Hedgehog
Term
What happens once the EGL is done proliferating?
Definition
The now post-mitotic cells travel inward and create the inner granule layer. The cells leave behind a trail of axons (Molecular layer) as they migrate
Term
What is the molecular layer of the cerebellum?
Definition
Axons of the internal granular layer created as the external granular layer migrated inward
Term
What is the purkinje layer of the cerebellum?
Definition
Layer between the inner granular layer and molecular layer composed of purkinje cells
Term
What derives from abnormal activation of Shh signalling pathway in EGL Neuroblasts?
Definition
Medullablastoma (most common malignant brain tumor)
Term
The cerebral hemispheres derive from what brain region?
Definition
Telencephalon (one of two divisions of the Prosencephalon; the other division is the diencephalon)

The telencephalon also gives rise to the cerebral cortex and subcortical white matter
Term
What is the Lamina Terminalis?
Definition
The anterior limit of the neural tube
Term
In cerebellar brain development, how many distinct layers form?
Definition
Up too 6 (gray matter)

Order of waves it not the histological designation for the layers
Term
In cerebellar neuronal development, where are the progenitor cells?
Definition
Ventricular Zone
Term
How do the waves of generated neurons make it to the cortical plate?
Definition
By traveling along radial glia
Term
The ventricular zone is eventually replaced by what?
Definition
The ependyma and a subventricular zone
Term
What area is the source for adult neuronal stem cells?
Definition
Subventricular zone
Term
What are the name of the cerebellar neural cells that migrate tangentially as opposed to radially?
Definition
Cajal-Retzius neurons
Term
Radially migration derived neural cells have what function?
Definition
Exicitatory
Term
Cajal-Retzius cells have what function?
Definition
Inhibitory
Term
Failure of Cajal-Retzius cells to migrate can manifest as what?
Definition
Seizure disorders
Term
Where do Cajal-Retzius cells originate?
Definition
Ventricular zone of lateral ganglionic eminence
Term
What accounts for brain growth in the third trimester?
Definition
increase in gyration and brain volume from forming connections and growth of glial cells, neuronal growth at this point it complete
Term
Why is myelin "white matter"?
Definition
High lipid-protein ratio
Term
What area of the brain myelinates late?
Definition
Frontal lobe, do not gain excecutive functions until second decade
Term
What is lissencephaly?
Definition
cerebellar cortex disorder that prevents neuronal migration in various degrees of severity.

Arises from a mutation in doublecortin
Term
What condition is characterized by enlarged gyri?
Definition
Pachygeria
Term
What condition is characterized by too many undersized gyri?
Definition
Polymicrogyria
Term
What is schizencephaly?
Definition
congential cleft from pial surface into ventrical.

Infant cerebral infact leading to ischemia
Term
What is porencephaly?
Definition
Any fluid filled cavity in fetal or neonatal brain

Cerebral infarct or prenatal infection suspected
Term
What is the name of the disease associated with the failure of select waves of neurons from migrating?
Definition
Cortical dysplasias, specifically heterotopias

typically presents with seizure disorder
Term
What is the leading teratogen in the western hemisphere?
Definition
Alcohol
Term
Which part of the brain is most sensitive to deleterious effects from Fetal alcohol syndrome?
Definition
Hippocampus
Term
What are the more severe effects of FAS?
Definition
Microencephaly leading to microcephaly
learning disabilities
attention deficits
hyperactivity
memory impairment
intellectual impairment
poor school performance
Term
What is the last region of the cerebral cortex to populate its neurons?
Definition
Hippocampus
Term
What are the top teratogens?
Definition
anti-epileptic meds, lithium, methotrexate, ilicit drugs (NTDS)

retinoic acid (inhibits regionalization)

ionizing radiation (inhibits neuroblast proliferation)

ethanol (1st trimester [FAS], 2/3rd trimester [ARND])

lead (lead encephalopathy, blood-brain barrier breakdown)
Term
How does Down's Syndrome affect brain development?
Definition
post migratory neuronal differentiation is not as pronounced, making fewer synaptic connections

reduced neural stem cell number and reduced neuronal production
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