Nervous system embryology and malformations Flashcards

1
Q

Does the nervous system develop from ectoderm, mesoderm or endoderm?

A

ectoderm

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2
Q

What week does the CNS appear?

A

3

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3
Q

What is the neural plate?

A

Thickening of ectoderm anterior to the primitive node

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4
Q

What are neural folds?

A

Edges of the neural plate thicken and move upwards to form these

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5
Q

How is the neural tube formed?

A

Neural folds migrating towards eachother and fusing in the midline

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6
Q

Is the neural tube initially open or closed?

A

open

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7
Q

When does the anterior and posterior neuropore close?

A
anterior = day 25 
posterior = day 27
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8
Q

What can failure of the neural tube closing lead to? Give 3 examples

A

death

NTD = spina bifida, anencephay, encephalocoele

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9
Q

Which neural tube defect is incompatible with life?

A

anencephaly

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10
Q

Is anencephaly more common in males or females?

A

females by 4 times

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11
Q

What happens in anencephaly?

A

anterior neuropore fails to close and the skull fails to form leading to brain tissue degeneration

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12
Q

Craniorachischisis

A

Entire neural tube fails to close

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13
Q

What is encephalocoele and what causes it?

A

Herniation of cerebral tissue through a defect in the skull due to failure of rostral tube closure

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14
Q

Where is encephalocoele most common?

A

occipital region

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15
Q

What causes spina bifida?

A

Defective closure of the caudal neural tube

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16
Q

What is affected in spina bifida?

A

tissue overlying spinal cord

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17
Q

What is spina bifida?

A

non-fusion of vertebral arches

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18
Q

Spina bifida occulta - what causes it?

A

failure of embryonic halves of vertebral arches to grow normally and fuse

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19
Q

What vertebral level does spina bifida occulta occur and what is a sign?

A

L5/6

dimple with patch of hair

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20
Q

What is spina bifida cystica?

A

Protrusion of spinal cord and/or meninges through vertebral arch defect

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21
Q

Spina bifida with meningocele - what is protruding??

A

CSF and meninges

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22
Q

spina bifida with meningiomyelocle - what else is included in the protrusion?

A

nerve roots/spinal cord - neurological defecit

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23
Q

What is spina bifida with meningiomyelocle associated with?

A

hydrocephalus

24
Q

What is myeloschisis?

A

Rare form and most severe of spina bifida

spinal cord open in affected area as neural tube fails to close

25
Q

4 prevention methods of spina bifida/NTD

A

folic acid supplements
ultrasound
amniocentesis
blood test

26
Q

What are you looking for in the blood in prenatal diagnosis of NTDs and why?

A

AFP - alphafetoprotein

from foetus liver which means an open communication

27
Q

Risk factors for NTDs

A

genetic predisposition
Nutrition - too much vitamin A, too little folate
Environmental eg sodium valproate, hyperthermia

28
Q

Name the 3 primary brain vesicles

A

Prosencephalon
mesencephalon
rhombencephalon

29
Q

Name the 5 secondary brain vesicles

A
Telencephalon 
diencephalon 
mesencephalon 
metencephalon 
myencephalon
30
Q

When does the cephalic flexure form and where is it?

A

end of 3rd week

between midbrain and hindbrain

31
Q

When does the cervical flexure form and where is it?

A

End of 4th week

between hindbrain and spinal cord

32
Q

When does the pontine flexure form and where is it?

A

5th week

in hindbrain

33
Q

Name the 3 flexures of the vesicles

A

cephalic
cervical
pontine

34
Q

What does the telencephalon form?

A

cerebral hemispheres
hippocampus
basal ganglia

35
Q

What does the diencephalon form?

A

thalamus
hypothalamus
pituitary gland
pineal

36
Q

What does the mesencephalon form?

A

superior and inferior colliculi

midbrain

37
Q

What does the metencephalon form?

A

cerebellum

pons

38
Q

What does the myencephalon form?

A

medulla

39
Q

What does the caudal neural tube form?

A

spinal cord

40
Q

What does the lumen of the neural tube form?

A

ventricular system

41
Q

When does CSF begin to form?

A

week 5

42
Q

What is hydrocephalus and what is the main cause?

A

Accumulation of CSF resulting in enlarged brain and cranium

usually due to blocked aqueduct so lateral and 3rd ventricle cannot drain into 4th ventricle to be absorbed

43
Q

Causes of hydrocephalus

A

genetics
prenatal viral infection
intraventricular haemorrhage
spina bifida cystica

44
Q

The neural tube is initially a single layer of…..cells

A

neuroepithelial

45
Q

What do the neuroepithelial cells go on to form?

A

Neurons, astrocytes, oligodendrocytes, ependymal cells - CNS

46
Q

What is microglia formed by?

A

mesenchymal cells - mesodermal in origin

47
Q

What do neural crest cells go on to form?

A

cells of PNS, autonomic NS and non neuronal cells eg melanocytes, adrenal medulla, meninges, facial bones and cartilage

48
Q

How does the spinal cord position change?

A

initially entire length of vertebral column but then dura and vertebral column grow more rapidly

49
Q

What is lissencephaly?

A

smooth brain - no sulci or gyri

50
Q

What do people with lissencephaly normally die of?

A

problems with swallowing and aspiration

51
Q

Polymicrogyria - what is it and what are the symptoms?

A

excessive number of small gyri

variable - mental retardation, seizures, motor defecits

52
Q

What is microcephaly and give a recent common cause of this

A

small brain

Zika virus

53
Q

What is porencephaly and what does it usually follow?

A

CSF filled cysts or cavities

postnatal stroke or infection

54
Q

What is schizencephaly?

A

Large slits or clefts

55
Q

What is diastematomyelia and what are the symptoms/signs?

A

split cord longitudinally

vertebral anomalies, scoliosis, hairy patch, loss of sensation

56
Q

Causes of intellectual impairment

A

Formation of brain cells affected due to maternal alcohol, genetic, radiation, infection, birth trauma, postnatal insults eg meningitis