Malformations/developmental Diseases of the CNS

Question 1

very common premature pediatric neuropathology lesion

Answer 1

hypoxic-ischemic lesions

Question 2

when does implantation of embryo occur?

Answer 2

7 days

Question 3

when does neural tube formation occur?

Answer 3

22-28 days (4th week)

Question 4

when does organogenesis occur?

Answer 4

4-8 weeks

Question 5

when does migration of neuroblasts occur?

Answer 5

8 weeks-birth

Question 6

when does myelination occur?

Answer 6

20 weeks on

Question 7

order of formation: hindbrain neuropore, posterior neuropore or anterior neuropore?

Answer 7

posterior neuropore, hindbrain neuropore, anterior neuropore

Question 8

lab evidence that neural tube has a problem

Answer 8

elevated alpha-fetal protein level in amniotic fluid (also use ultrasound to see it)

Question 9

where is alpha-fetal protein produced?

Answer 9

liver of the fetus

Question 10

Arnold-chiari malformation is a result of failure to close

Answer 10

anterior neuropore

Question 11

things that are absent in anencephaly

Answer 11

absent brain and calvarium, absent descending tracts, atrophic anterior pituitary

Question 12

most common neural tube defect is that of the

Answer 12

posterior neuropore: meningocele, myelomeningoeele

Question 13

myelomeningocele characteristics

Answer 13

absence of vertebral arches; herniation of meninges and spinal cord through the cystic lesion; most common in lumbosacral region

Question 14

Arnold-chiari main problem

Answer 14

flattening of the posterior fossa and a wide foramen magnum

Question 15

disorders of organogenesis/ which is very common?

Answer 15

holoprosencephaly, olfactory aplasia (very common), agenesis of the corpus callosum, dandy-walker malformation

Question 16

what is the main finding in Dandy-Walker malformation?

Answer 16

absence of the cerebellar vermis and a cyst in its place that connects to the 4th ventricle

Question 17

early destructive lesion of the brain

Answer 17

schizencephaly (porencephaly)

Question 18

late destructive lesions result in

Answer 18

cerebral palsy due to hypoxic hemorrhage complications, which usually block CSF flow in the cerebral aqueduct

Question 19

damage in the white matter due to hypoxic-ischemic injury hemorrhage in the germinal matrix, choroid plexus, parenchymal, or intradural spaces

Answer 19

periventricular leukomalacia