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Neuro > CNS Development > Flashcards

CNS Development Flashcards

1
Q

What is the purpose of gastrulation?

A
  • establishes the basic vertebrate body plan
  • first establishes neuroepithelium
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2
Q

What is the starting process of neurulation?

A

Formation of a neural plate derived from ectoderm

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3
Q

What structure regulates the proper formation of the neural tube?

A

Notochord

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4
Q

What is primary neurulation?

A

Formation of the neural folds ending up in the closure that leads to the closure of the neural tube. It creates the brain and most of the spinal cord.

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5
Q

What is secondary neurulation?

A

It occurs distally to S2 and the filum terminale and it is a cylinder of primitive neural tissue that becomes canalized by within. It creates the lowest portion of the spinal cord.

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6
Q

What lineage do arachnoid and pia mater derive from?

A

Neural crest

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7
Q

What lineage does the dura mater arise from?

A

Mesenchymal origin

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8
Q

What secondary vesicles does the forebrain/prosencephalon form?

A

Telencephalon and diencephalon

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9
Q

What secondary vesicles does the midbrain/mesencephalon form?

A

Mesencephalon

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10
Q

What secondary vesicles does the hindbrain/rhombencephalon form?

A

Metencephalon, myelencephalon

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11
Q

What walls and cavities are derived from the telencephalon?

A

Cerebral hemispheres (walls) and lateral ventricles (cavities)

derivatives of the forebrain

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12
Q

What walls and cavities are derived from the diencephalon?

A

Walls: thalami

Cavities: third ventricle

derivatives of the forebrain

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13
Q

What walls and cavities are derived from the mesencephalon?

A

Walls: midbrain

Cavities: aqueduct

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14
Q

What walls and cavities are derived from the metencephalon?

A

Walls: pons and cerebellum

Cavities: upper part of fourth ventricle

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15
Q

What walls and cavities are derived from the myelencephalon?

A

Walls: medulla

Cavities: lower part of fourth ventricle

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16
Q

What are the first neurons that appear?

A

Motor neurons in the ventral horn of the cervical spinal cord

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17
Q

What is the make up of the wall of the neural tube?

A

Pseudostratified neuroepithelial cells (called the ventricular zone)

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18
Q

Describe proliferation of the neuroepithelium in early embryonic development?

A

Initially proliferation is symmetric and then switches to asymmetric after week 5 that gives rise to radial glia

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19
Q

In what order are cortical layers derived during embryogenesis?

A

Inside-out (layer 6 first, layers 2-3 last)

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20
Q

What is the timeline of gliogenesis?

A

It follows neurogenesis and peaks around birth

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21
Q

What lineage do ogliodendrocytes arise from in gliogenesis? Astrocytes?

A

Oligodendrocytes: dorsal and ventral telencephalon

Astrocytes: dorsal telencephalon

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22
Q

What is synaptogenesis?

A

It is a process to form the first synapses, which are present in the cervical spinal cord.

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23
Q

When does the corticospinal tract become fully myelinated? Corpus callosum? Ipsilateral frontotemporal association bundle?

A

CST: 2 years

CC: late adolescence

IFAB: 32 years

24
Q

What is the general order of CNS development?

A

gastrulation –> neurulation –> proliferation –> gliogenesis –> synaptogenesis –> myelination

25
Q

Gastrulation occurs at which post-conceptional week?

A

3 weeks

26
Q

Neurulation refers to which embryonic process?

A

Formation of the neural tube

27
Q

Where does the neural tube close first?

A

Cranial end

28
Q

Name 3 defects of neural tube closure?

A

Anecephaly, spina bifida, craniorahischisis

29
Q

When are the 6 cortical layers identified?

A

18 weeks post conception

30
Q

Inhibitory neurons derive from radial cells that express which factors?

A

Nkx2.1 and Gsx2

31
Q

Excitatory neurons derive from radial cells which express what factor?

A

Pax6

32
Q

Which primary vesicle gives rise to the cerebral hemispheres?

A

Prosencephalon

33
Q

What is the most common developmental disorder of the human forebrain?

A

Holoproscencephaly

34
Q

Name disorders of migration?

A

Lissencephaly, heterotopias

35
Q

Name one disorder of synaptogenesis.

A

MECP-2 related disorder (Rett’s syndrome)

36
Q

What are examples of neural tube defects/spinal dysraphisms?

A
  • anecephaly
  • myelomeningocele (chiari malformations and hydrocephalus)
  • encephalocele
  • occult spina bifida
37
Q

What are examples of disorders of prosencephalic development/midline defects?

A
  • holoprosencephaly
  • agenesis of corpus callosum
  • septo-optic dysplasia
38
Q

What are some examples of disorders of neuronal migration?

A
  • lissencephaly
  • heterotopias
39
Q

What are some examples of disorders of cortical organization/postmigrational defects?

A
  • polymicrogyria
  • schizencephaly
40
Q

What are some examples of disorders of neuronal proliferation/differentiation?

A
  • hemimegalencephaly
  • focal cortical dysplasia
41
Q

What are some examples of disorders of hindbrain development?

A
  • dandy walker malformation
  • joubert syndrome
42
Q

What is the current recommendation for folic acid supplementation to prevent neural tube defects?

a) at least one month prior to conception
b) all women of childbearing age should consistently take folic acid, all the time
c) if folate started anytime between conception and 12 weeks of pregnancy, defects will be prevented
d) dietary folate is sufficient for prevention of NTDs

A

b) all women of childbearing age should consistently take folic acid, all the time

43
Q

A woman at 34 weeks gestation presents for prenatal consult with fetal brain MRI showing complete agenesis of the corpus callosum, with no other structural brain anomalies detected. You are the fetal neurologist counseling her on her baby’s expected neurodevelopmental outcome. What do you counsel on the basis of this MRI?

a) the baby has a 75% chance of having hemiplegic cerebral palsy
b) the baby has a 50% chance of presenting with epilepsy before age 10
c) the baby is likely to have grossly normal intelligence
d) the infant is expected to be blind

A

c) the baby is likely to have grossly normal intelligence

44
Q

What malformation of cortical development is a common cause of focal epilepsy in a normal developing child, often presenting remote from the infantile period?

a) schizencephaly
b) focal cortical dysplasia
c) tuberous sclerosis
d) semilobar holoprosencephaly

A

b) focal cortical dysplasia

45
Q

What is the “pathognomonic finding” of the MRI of the class of hindbrain malformations known as Joubert syndrome?

a) cobblestone lissencephaly
b) hindbrain hamartoma
c) colobomata
d) molar tooth sign

A

d) molar tooth sign

46
Q

An infant is admitted to the NICU after postnatal imaging confirms septo-optic dysplasia complex, with bilaterally hypoplastic optic nerves, an absent septum pellucidum and a thin corpus callosum. What screening tests should be ordered? Select all that apply.

a) glucose monitoring screening
b) endocrine function tests
c) whole genome sequencing
d) pituitary biopsy

A

a) glucose monitoring screening
b) endocrine function tests

47
Q

What type of hydrocephalus is most commonly associated with meylomeningocele and chiari II malformation?

a) normal pressure hydrocephalus
b) obstructive hydrocephalus
c) communicating hydrocephalus
d) ventriculomegaly

A

b) obstructive hydrocephalus

48
Q

What is anecephaly?

A

Failure of closure of the anterior/rostral neuropore –> absent forebrain

49
Q

What is encephalocele?

A

Failure of anterior neuropore closure –> herniation of intracranial contents

50
Q

What is myelomeningocele?

A

Herniation of spinal cord and meningeal tissue through a midline defect

51
Q

What is a chiari II malformation?

A

A hindbrain malformation associated with myelomeningocele

52
Q

What is holoprosencephaly?

A

Defect in prosencephalon formation –> midline defects

53
Q

What is lissencephaly?

A

A disease due to faulty neuroblast migration resulting in decreased layers of cortex

54
Q

What is polymicrogyria?

A

Excessive gyration and abnormal cortical organization and layering

55
Q

What is the dandy walker malformation?

A

Most common cerebellar malformation with a constellation of symptoms

56
Q

What is Joubert syndrome?

A

A disease that has a pathognomonic “molar tooth” sign with elongated cerebellar peduncles and vermis hypoplasia

57
Q
A

Neuro (12 decks)

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