Brain Development in Paediatrics Flashcards

1
Q

When does human brain development commence?

A

3rd week post conception

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2
Q

Which intrauterine factors may negatively impact the development of the nervous system?

A
  1. Maternal health and disease
  2. Insults (physical or infection)
  3. Placental or nutritional issues
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3
Q

Which extrauterine factors may negatively impact the development of the nervous system?

A
  1. Nutrition or environmental toxins
  2. Emotional and social factors
  3. Exposure to alcohol or drugs
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4
Q

How long does the embryonic period last?

A

Conception until gestational week 8

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5
Q

How many layers is the embryo comprised of during the embryonic period and what are they called?

A

2

Epiblast and hypoblast

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6
Q

The primitive streak is a transient structure that marks what?

A

The start of gastrulation

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7
Q

What happens in gastrulation?

A

The formation of the trilaminar embryonic disc, which is comprised of the three germ layers (ectoderm, mesoderm and endoderm)

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8
Q

What is the role of the primitive node?

A

Determines migration of cells into cleft where cells form 3 layers

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9
Q

Which adult tissues form from the ectoderm?

A
  1. Skin
  2. Nails
  3. Hair
  4. Neural tissue
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10
Q

Which adult tissues form from the mesoderm?

A
  1. Muscle
  2. Bone
  3. Cartilage
  4. Vascular system
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11
Q

Which adult tissues form from the endoderm?

A
  1. Gut
  2. Respiratory
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12
Q

Between which days of development is the neural tube expected to develop?

A

20-27

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13
Q

Which cells is the neural crest derived?

A

Neural progenitor cells

(from neural plate)

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14
Q

Failure of the neural groove, and then tube, to close results in what?

A

Neural tube defects

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15
Q

Which cells line the inside of the neural tube and what is this zone called?

A

Neural progenitor cells

Ventricular zone

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16
Q

The hollow centre of the neural tube will develop into what?

A
  1. Ventricular system
  2. Central channel of spinal cord
17
Q

Which aspect of the neural tube will become the brain?

A

Anterior/rostral tube

18
Q

Which aspect of the neural tube becomes the spinal cord?

A

Caudal tube

19
Q

What are the three primary brain vesicles in a three to four week embryo?

A
  1. Prosencephalon (forebrain)
  2. Mesencephalon (midbrain)
  3. Rhombencephalon (hindbrain)
20
Q

How many secondary brain vesicles develop in a five week embryo?

A

5

21
Q

Which two signalling molecules influece the development of the neocortex?

A
  1. EMX2
  2. PAX6
22
Q

A high PAX6 concentration and low EMX2 concentration induce progenitor cells to develop into what?

A

Motor neurones

23
Q

A high EMX2 concentration and low PAX6 concentration induce progenitor cells to develop into what?

A

Visual cortical neurones

24
Q

What is required in the foetal period in order to accomodate the proliferating neural cell populations?

A

Development of gyri and sulci

25
Q

How are neurones produced during the foetal period?

A

Neuronal progenitor cells (in ventricular zone) divide initially symmetrically to produce two neural progenitor cells

Asymmetrical division follows producing one progenitor cell and one neurone

26
Q

When neurones are produced, where do they migrate?

A

Developing neocortex

27
Q

In terms of brain development what are two of the main processes which occur postnatally?

A
  1. Continued proliferation and migration
  2. Myelination
28
Q

In which sequential manner does myelination occur in the brain postnatally?

A
  1. Bottom to top
  2. Front to back
29
Q

For how long does myelination occur postnatally?

A
  • Mainly in first 2 years
  • Continues into 20s
30
Q

Physical maturation of the brain in adolescent years is associated with what?

A

Rising gonadal hormone concentrations

31
Q

What is holoprosencephaly?

A

A cephalic disorder in which the prosencephalon (the forebrain of the embryo) fails to develop into two hemispheres

32
Q

In terms of myelin, what is the PLP1 gene responsible for?

A

Encodes the most predominant transmembrane proteolipid protein in myelin

Responsible for compactation, stabilisation and maintenance of myelin sheaths, development of oligodendrocytes and axonal survival

33
Q

Where is the PLP1 gene located?

A

X chromosome

34
Q

What is the most severe condition associated with a PLP-1 mutation?

A

Pelizaeus-Merzbacher disease

35
Q

When does Pelizaeus-Merzbacher disease present and how?

A

Infancy or early childhood

  1. Nystagmus
  2. Hypotonia
  3. Cognitive impairment
  4. Progresses to severe spasticity and ataxia
  5. Short life span