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Neurology-Week 1 > Neuro Embryology & Development > Flashcards

Neuro Embryology & Development Flashcards

1
Q

Why is folic acid important if you are planning for or think you are pregnant?

A

Because the nervous system starts to form at Week 3, usually before a pregnancy test turns up positive (to avoid folic acid deficiency and neural defects)

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2
Q

The Nervous system begins formation usually around week _____

A

3 (as soon as the 3 germ layers have formed via gastrulation)

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3
Q

Formation of the 3 germ layers (Ectoderm, Mesoderm and Endoderm)

A

Gastrulation

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4
Q

Portion of the Mesoderm; will give rise to the “somites” or body segments: examples are skeletal muscle, bone, cartilage, CT and blood vessels

A

Paraxial mesoderm

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5
Q

Portion of the Mesoderm; will give rise to the genitourinary systems

A

Intermediate mesoderm

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6
Q

Portion of the Mesoderm; will give rise to the anterolateral body wall, smooth muscle and CTs of the viscera, heart and blood vessels

A

Lateral mesoderm

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7
Q

Process of forming the neural tube and neural crest

A

Neurulation

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8
Q

Difference between the neural “fold” and neural “crest” during

A

neurulation

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9
Q

Neurulation begins around week ____ and ends at week _____ with closure of the Cranial and Caudal Neuropores

A

3; 4

*extra importance to folic acid supplementation

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10
Q

The end of Neurulation is marked by the closure of what structures

A

Cranial and Caudal Neuropores

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11
Q

The neural tube will provide the (CNS/PNS)

A

CNS

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12
Q

The neural crest will provide the (CNS/PNS)

A

PNS (ganglia and melanocytes)

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13
Q

The neural tube (wall/lumen) is composed of neuroepithelium and will become neurons and glia

A

Wall

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14
Q

The neural tube (wall/lumen) will become the ventricles (brain) filled with CSF and the central canal (spinal cord)

A

Lumen

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15
Q

What are the 3 layers of the Neural Tube

A

Ventricular (inner): ependyma (ventricle lining)
Intermediate: gray matter (cell bodies)
Marginal (outer): white matter (axons)

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16
Q

The (Ventricular/Intermediate/Marginal) layer of the neural tube is the innermost layer and will form the ependyma (glia cell lining of the ventricles)

A

Ventricular

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17
Q

The (Ventricular/Intermediate/Marginal) layer of the neural tube will form the gray matter (cell bodies) and will subdivide into the Alar and Basal plates (dorsal and ventral regions)

A

Intermediate

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18
Q

The (Ventricular/Intermediate/Marginal) layer of the neural tube is the outermost layer and will form the white matter (axons)

A

Marginal

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19
Q

In the spinal cord, the (white/gray) matter is deep

A

Gray

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20
Q

In the brain, the (white/gray) matter is deep

A

White

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21
Q

The neural tube induces vertebrae formation from what somite portion

A

Sclerotome

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22
Q

The Intermediate layer of the spinal cord will subdivide into the _________ and ___________ plates that will give rise to the Dorsal and Ventral horns of the gray matter in the spinal cord

A

Alar (posterior) and Basal (anterior)

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23
Q

Why do the caudal spinal nerves not exit at the same level as they do the spinal cord (have to travel down the column and then exit)

A

Originally, the spinal cord and vertebral column are same length, but then the vertebrae grow faster and thus stretch the caudal spinal nerves downward (forming the Cauda Equina)

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24
Q

The cranial tip of the Neural tube eventually forms 2 constrictions, forming what three portions

A

Forebrain (prosencephalon)
Midbrain (mesencephalon)
Hindbrain (rhombencephalon)

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25
Q

Fancy name for the Forebrain

A

Prosencephalon

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26
Q

Fancy name for the Midbrain

A

Mesencephalon

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27
Q

Fancy name for the Hindbrain

A

Rhombencephalon

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28
Q

The Forebrain (prosencephalon) will further subdivide into what 2 regions

A

Telencephalon

Diencephalon

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29
Q

The Hindbrain (rhombencephalon) will further subdivide into what 2 regions

A

Metencephalon

Myelencephalon

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30
Q

What is the order of the 5 portions of the neural tube during development (the -cephalons)

A 
Telencephalon
Diencephalon
Mesencephalon
Metencephalon
Myelencephalon

*T is on top, and it’s in alphabetical order from there

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31
Q

The Telencephalon gives rise to what brain structure(s)

A

Cerebrum

Lateral Ventricles

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32
Q

The Diencephalon gives rise to what brain structure(s)

A

Diencephalon (shocker) (thalamus and hypothalamus)

3rd Ventricle

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33
Q

The Mesencephalon gives rise to what brain structure(s)

A

Midbrain

Aquaduct

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34
Q

The Metencephalon gives rise to what brain structure(s)

A

Pons
Cerebellum
Upper part of 4th Ventricle

35
Q

The Myelencephalon gives rise to what brain structure(s)

A

Medulla (oblongata) (always think of the movie Water Boy)

Lower part of 4th Ventricle

36
Q

In the BRAINSTEM, the lumen (4th ventricle) widens and pushes the alar plate (medial/lateral) and subsequently the basal plate (medial/lateral)

A

Alar (sensory)–> lateral

Basal (motor)–> medial

37
Q

During cerebrum development, explain how the Intermediate layer changes and migrates

A

Some neurons cluster within the Marginal (outer) layer (forming nuclei)
Some neurons migrate beyond to form a NEW outermost layer of gray matter (cortex)

*migrating neurons are guided by astrocytes

38
Q

Migrating neurons of the Intermediate layer (forming the nuclei and outer gray matter) are guided to their destination by what glial cells

A

Astrocytes

39
Q

Why do many structures within the brain adopt a C shape (lateral ventricles, caudate nucleus, corpus callosum)

A

Growth of the brain does so to fit in the skull, bending to corm a C shape in the process

40
Q

Once the neural tube forms, it induces the surrounding _______ and _______ to develop for protection

A

bone and skin

*abnormalities result in neural tube defects

41
Q

Main risk factor for Neural Tube Defects

A

Maternal folic acid (B9) deficiency

42
Q

Vitamin B9

A

Folic acid

43
Q

Diagnosis of Neural Tube Defects (before birth)

A

Inc. a-fetoprotein (maternal blood)

Ultrasound

44
Q

When a portion of the neural tube fails to develop or close properly, causing defects in the spinal cord and in the bones of the spine; can range from mild to severe, depending on the type of defect, size, location and complications

A

Spina Bifida

45
Q

Type of Spinal Neural Tube Defect; due to a unfused vertebral arch (bony defect); typically mild, can see a tuft of hair; no functional impairments

A

Spina Bifida Occulta (“hidden”)

46
Q

Type of Spinal Neural Tube Defect; the meninges protrude through the opening causing a lump or sac on the back, BUT the cord still remains within the vertebrae; often no functional impairments

A

Meningocele (Spina Bifida Cystica)

47
Q

Type of Spinal Neural Tube Defect; MOST COMMON; both the meninges AND the spinal cord protrude through the back, BUT still enclosed in a sac; paralysis below the lesion, bowel/urinary dysfunction and possible hydrocephalus

A

Myelomeningocele (Spina Bifida Cystica) (myelo= spinal cord, cele = sac)

48
Q

Type of Spinal Neural Tube Defect; due to complete failure of closure of the caudal neuropore; nerves are open to external environment; no function below, often anencephaly

A

Rachischisis

49
Q

Most common site for Spinal Neural Tube Defects

A

Lumbosacral region

50
Q

Functional impacts of the various Spinal Neural Tube Defects

A

SB Occulta: none
Meningocele: often none
Myelomeningocele: paralysis below, bowel/urinary impacts, possible hydrocephalus
Rachischsis: no function below, often anencephaly

51
Q

Special name for Cranial Neural Tube Defects

A

Cranium Bifidum

52
Q

Cranial Neural Tube Defects usually are (soft/bony) and are located where?

A

Bony; occipital bone

53
Q

Cranial Neural Tube Defect; inadequate development of the upper end of the neural tube; herniated sac consists of only meninges.

A

Cranial Meningocele (B)

54
Q

Cranial Neural Tube Defect; inadequate development of the upper end of the neural tube; herniated sac consists of both meninges AND brain contents

A

Meningoencephalocele (or Encephalocele) (C)

55
Q

Cranial Neural Tube Defect; absence of a major portion of the brain, skull, and scalp; results from a neural tube defect that occurs when the rostral end of the neural tube fails to close; usually born unconscious, deaf, blind, etc. and usually are either stillborn or die soon after birth

A

Anencephaly (Meroanencephaly)

56
Q

Neural Tube Defect; herniation of the meninges from the vertebral canal, but normal spinal cord; still closed from the environment; no neurologic deficits

A

Meningocele

57
Q

Neural Tube Defect; herniation of BOTH the meninges and the spinal cord from the vertebral canal; still closed from the environment; associated with neurologic deficits

A

Myelomeningocele

58
Q

Neural Tube Defect; entire failure of the neural tube to close; open to the environment

A

Craniorachisisis Totalis

59
Q

Herniation of brain tissue through a skull defect

A

Encephalocele

60
Q

Bottom structures of the Cerebellum, located medially and caudally; above the foramen magnum; tend to herniate with increased ICP

A

Tonsils

61
Q

Midline portion of the Cerebellum

A

Vermis

62
Q

Herniation of part of a Cerebellar tonsil through the Foramen Magnum in the absence of an intracranial mass lesion or hydrocephalus (CSF can’t escape, so builds up in head); strongly associated with Syringomyelia (inc. pressure pushes into spinal cord??)

A

Chiari Type I

63
Q

Chiari Type I is associated with…

A

Syringomyelia

64
Q

LESS common, but MORE severe herniation of BOTH the cerebellum and downward displacement of the brain stem through the foramen magnum; can also see “beak like” deformity of mesencephalon (above cerebellum); almost ALWAYS associated with lumbosacral myelomeningocele and hydrocephalus

A

Chairi Type II

65
Q

Difference between Chiari Type I and II

A

Type I: part of cerebellar tonsil herniates

Type II: herniation of BOTH cerebellum and brain stem

66
Q

Congenital Brain Malformation; combination of Vermal agenesis, dilated 4th ventricle, enlarged posterior fossa and hydrocephalus

A

Dandy Walker Syndrome

67
Q

Most common Forebrain anomaly; failure of the separation of the Forebrain (prosencephalon) into 2 hemispheres; associated with mutations in HPE gene; associated with midline facial defects from hypotelorism (eyes too close) to cyclopia

A

Holoprosencephaly

68
Q

Agenesis of the Corpus Callosum is associated with what syndromes

A

Dandy-Walker syndrome
Chairi II
Fetal-Alcohol syndrome

69
Q

Neuronal migration defect; absence of gyri and sulci

A

Agyria (Lissencephaly)

70
Q

Neuronal migration defect; reduced number of gyri and sulci

A

Pachygyria

71
Q

Abnormailty of cortical development; TOO MANY gyri that are small and have abnormal cortical lamination; can be caused by genetics, hypoxia or CMV; associated with maple syrup urine disease

A

Polymicrogyria

72
Q

Heterotopic neurons in cerebral white matter (normal tissue in abnormal location); scattered or clustered into nodules; associated with peroxisomal, mitochondrial or chromosomal disorders

A

Neuronal Heterotopias

73
Q

Malformation of the Spinal Cord; tubular cavitation of the spinal cord; frequently associated with Chiari type I; usually presents in 2nd and 3rd decade of life (wasting, weakness and loss of hand/forearm muscles)

A

Syringomyelia

74
Q

Class of metabolic disorders that primarily affect the White matter

A

Leukodystrophies (Krabbe’s disease, Metachromatic, Adreno-, etc.)

75
Q

Examples of Leukodystrophies (3 total)

A

Krabbe’s disease
Metachromatic leukodystrophy
Adrenoleukodystrophy

76
Q

Type of Leukodystrophy; AR inheritance; lysosomal storage disease due to deficiency of Galactocerebroside-B-Galactosidase; leads to accumulation of Galactocerebroside and destroys myelin (LOSS OF WHITE MATTER)

A

Krabbe’s disease

77
Q

Krabbe’s disease will result in a loss of (white/grey) matter

A

White

*accumulation of Galactocerebrosidase destroys myelin

78
Q

Enzyme deficient in Krabbe’s disease

A

Galactocerebroside-B-Galactosidase

*excess galactocerebroside destroys myelin

79
Q

Metabolic disorder affecting GREY matter; AR inheritance; infantile Gangliosidosis; cherry red spot on retina; brain can be overly small or large (due to gyral atrophy)

A

Tay-Sachs disease

80
Q

Metabolic disorder affecting GREY matter; AR inheritance; usually due to Sphingomyelinase deficiency (Group 1); cherry red spot on maculla

A

Niemann-Pick disease

81
Q

Metabolic disorder affecting GREY matter; AR inheritance; deficiency of gluocerebrosidase; due to loss of myelin; “crumpled tissue paper” cells in spleen, lymph nodes, bone marrow and liver

A

Gaucher’s disease

82
Q

Metabolic disorder affecting GREY matter; AR inheritance; affects storage of gangliosides in neurons; coarse facial features, hepatosplenomegaly and corneal clouding; associated with Dermatan Sulfate and deficiency in a-L-iduronidase

A

Hurler’s Syndrome

83
Q

Metabolic disorder affecting GREY matter; X-Linked inheritance; affects storage of gangliosides in neurons; coarse facial features, hepatosplenomegaly and corneal clouding; associated with Dermatan Sulfate and deficiency in Iduronate sulfatase

A

Hunter’s Syndrome

84
Q

Difference between inheritance and enzyme for Hurler’s and Hunter’s syndromes (Mucopolysaccharidoses)

A

Hurlers: a-L-iduronidase; AR inheritance
Hunters: Iduronate sulfatase; X-Linked inheritance

Neurology-Week 1 (9 decks)

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