Embryology and abnormalities Flashcards

1
Q

what induces the ectoderm to form neural plate and tube

A

notochord

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2
Q

what are the three germ layers

A

ectoderm, mesoderm, endoderm

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3
Q

primary neurulation, secondary neurulation

A

1-neural plate>folds and groove>tube. 2- forms spinal segments below S2.

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4
Q

types of spina bifida in increasing order of severity

A

oculta, lipoma and lipocele, cystica (meningocele, meningomyelocele), rachischisis

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5
Q

what defects do you see with meningomyelocele

A

sensory and motor in bowel, bladder, lower limb

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6
Q

tethered cord syndrome: problem with 1* or 2* neurulation? what happens? Results in?

A

Seconday. fibrous bands pull down on cord. Arnold-Chiari and hydrocephalus

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7
Q

Types of exencephaly

A

encephalocele (w/ or w/o brain tissue). Anencephaly (most common cause of stillbirths)

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8
Q

Name the primary brain vesicles

A

prosencephalon (forebrain), mesencephalon (midbrain), rhombencephalon (hindbrain)

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9
Q

name secondary brain vesicles and what comes from them

A

telencephalon (cereb hemis), diencephalon (thal,hypothal), mesencephalon (midbrain), metencephalon (pons, cerebellum), myelencephalon (medulla)

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10
Q

folding of the midbrain in the ventral direction. consequences

A

cephalic flexure. vertical orientation of cord and brianstem, horizontal orientation of forebrain

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11
Q

two defects in ventral induction

A

holoprosencephaly, agenesis of the corpus callosum

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12
Q

three layers of neural tube

A

neuroepithelial layer (all cells of cns except microglia), mantle layer (gray matter), marginal layer (white matter)

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13
Q

two parts of mantle layer and what divides them

A

basal (somotomotor [ant] and visceromotor [lateral]) and alar plates (sensory [post]). Sulcus limitans

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14
Q

what do the roof and floor plates of enural tube form?

A

commissures where axons cross from one side to the other

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15
Q

when does myelination occur

A

4th gestational month to end of second year

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16
Q

in ventricles: what happens to basal and alar plates? roof plate?

A

form medial motor and lateral sensory areas divided by sulcus limitans. Roof plate gets covered by pia to form tela choroidea which invaginates to form choroid plexus

17
Q

four steps necessary to form cerebral hemispheres

A

proliferation, migration, organization, myelination

18
Q

describe proliferation and migration and organization stages

A

neuroblasts and glioblasts. Neurblasts migrate along radial glia, pass cells of earlier birth, form 6 layers of cortex

19
Q

defects in proliferation phase

A

microcephaly, macrocephaly, tuberous sclerosis, focal cortical dysplasia

20
Q

defects in neuronal migration

A

seizures (abnormal conduction), lissencephaly, schizencephaly, focal dysgenesis, heterotopias

21
Q

when does neurulation begin

22
Q

what affects 2/3 of all NTDs

A

folate metabolism. All women of child bearing age should take it

23
Q

Relationship of radial glial cells and neurons in cortex

A

same stem cells gives rise to glial then neurons. Radial cells move from ventricular zone to SVZ then back and forth escorting new cells

24
Q

diastomyelia

A

notochord is split by adhesion b/w endo and ectoderm

25
what genes are mutated in classical lissencephaly. What do they affect
LIS1, DCX. Microtubule formation
26
subcortical band herterotopia: what? what mutation? what gradient?
bands of grey matter b/w cortex and lat. ventricles. DCX mutation (A>P gradient)
27
males with dcx get___. females get___. What inheritance? what protein does it produce
lissencephaly, subcortical band heterotopia. X-linked! Doublecortin
28
problems of organization phase
polymicrogyria. Predilection for perisylvian areas.
29
implication of malformation of cortical development (MCD)
epilepsy, learning disability, cerebral palsy, developmental delay
30
Three ways primary neurulation can go awry
diastomyelia, premature disjunction of neural tube from ectoderm (lipomas), nondisjunction (myelomeningocele)