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Neurology Board Review > Embryology > Flashcards

Embryology Flashcards

1
Q

Where is neuronal tissues derived from?

A

Ectoderm

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2
Q

Notochord is formed from the ___ layer.

A

Mesoderm

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3
Q

What does the notochord do?

A

Induces the overlying neuroectoderm to differentiate into the neural plate

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4
Q

When does the neuroectoderm differentiate into the neural plate?

A

18 days gestation

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5
Q

What does the notochord become?

A

Intervertebral discs

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6
Q

When does the the neural plate invaginates to form the neural tube?

A

3rd-5th weeks of gestation

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7
Q

Neurulation in two phases

A

Primary neurulation (dorsal induction)
Secondary neurulation (caudal induction)

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8
Q

Defects of primary or secondary neurulation lead to what issues?

A

Cortical and spinal cord malformations

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9
Q

What does the neural tube develop into?

A

Brain
Brainstem
Spinal cord
Preganglionic sympathetic neurons

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10
Q

What factors can cause neural tube defects (especially spina bifida)?

A

Folate/B12 deficiency
Valproic acid
Carbamazepine
Abnormal genetics

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11
Q

What becomes motor neurons of the cranial nerve and anterior horn gray matter?

A

Basal plate (ventral) of the neural tube
Week 10

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12
Q

What becomes posterior horn gray matter and sensory neurons?

A

Alar plate (dorsal) of neural tube
Week 10

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13
Q

What leads to the growth of the PNS?

A

Neural crest cells

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14
Q

What do neural crest cells become?

A

Schwann cells
Neuroglial cells
Parasympathetic and postganglionic sympathetic fibers
Melanocytes
Pia and arachnoid
Thyroid
Teeth

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15
Q

Three primary vesicles of the developing brain
Week 4

A

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

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16
Q

What does the prosencephalon develop into?

A

Telencephalon
Diencephalon

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17
Q

What does the rhombencephalon develop into?

A

Metencephalon
Myelencephalon

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18
Q

Five secondary vesicles
Week 5

A

Telencephalon
Diencephalon
Mesencephalon
Metencephalon
Myelencephalon

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19
Q

Telencephalon develops into the ___.

A

Cerebral hemispheres
Basal ganglia
Hippocampus
Amygdala

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20
Q

Diencephalon develops into the ___.

A

Thalamus
Hypothalamus
Retina

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21
Q

Mesencephalon develops into the ___.

A

Midbrain

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22
Q

Metencephalon develops into the ___.

A

Pons
Cerebellum

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23
Q

Myencephalon develops into the ___.

A

Medulla

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24
Q

What are the 6 layers of the neocortex?

A

Molecular layer (most superficial)
External granular layer
External pyramidal layer
Internal granular layer
Internal pyramidal layer
Multiform layer (deepest layer)

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25
Q

When does in-utero myelination start?

A

14 weeks

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26
Q

When does the majority of in-utero myelination occur?

A

3rd trimester

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27
Q

When do subcortical U fibers myelinate?

A

8 months
Do not finish until the second decade of life

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28
Q

When is peripheral myelination completed?

A

3-5 years

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29
Q

Which myelinates first?
Motor or sensory roots?

A

Motor

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30
Q

What is a dermal sinus tract?

A

Tract of squamous epithelium that travels from the skin to the dura of the spinal cord

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31
Q

Where are dermal sinus tracts most often found?

A

Midline and in the lumbar/lumbosacral region

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32
Q

What tumors are associated with dermoid sinuses?

A

Dermoid or epidermoid tumors

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33
Q

What causes dermal sinuses?

A

Failure of differentiation between cutaneous ectoderm and neuroectoderm in the 4th week of development

34
Q

What is the presentation of a congenital dermal sinus?

A

Recurrent meningitis (bacterial portal)
Tethered cord
Cord compression

35
Q

What is the treatment for a dermal sinus?

A

Complete excision

36
Q

What is craniorachischisis?

A

Complete failure of neural tube formation leading to both anencephaly and extensive spina bifida

37
Q

A failure of anterior neural tube closure

A

Anencephaly

38
Q

Defect of cranial mesoderm development which leads to a skull defect with protrusion of both meninges and brain

A

Encephalocele

39
Q

What disease in an encephalocele associated with?

A

Meckel-Gruber syndrome.

40
Q

A pathological opening of the vertebral column in which meninges protrudes through the skeletal defect without neurologic tissue outside the spinal cord

A

Meningocele

41
Q

Incomplete closure of the caudal neural tube
Meninges and spinal cord are both herniated through the defect in a covered membrane

A

Myelomeningocele

42
Q

Myelomeningocele without spinal contents covered by a membrane is called:

A

Myeloschisis

43
Q

Incomplete closure of the vertebrae with an overlying tuft of hair or dimple
Generally asymptomatic

A

Spina bifida occulta

44
Q

Defects of spinal cord development
Imaging show longitudinal duplication/splitting and tethering of the spinal cord

A

Diastematomyelia

45
Q

Chiari I

A

Downward displacement of the cerebellar tonsils at least 5 mm beyond the foramen magnum

46
Q

Chiari II

A

Displacement of the cerebellar tonsils as well as the brainstem

47
Q

Patients with Chiari II can also have a ____ and at risk for ____.

A

Myelomeningocele
Hydrocephalous

48
Q

Chiari malformation associated with syringomyelia:

A

I and II

49
Q

Symptoms of a syringomyelia:

A

Loss of pain in a “cape-like” distribution
Arm weakness

50
Q

Cause of symptoms with a syringomyelia:

A

Damage of the crossing spinothalamic fibers
Damaged anterior horn cells

51
Q

Cortical malformations: Disorders of cell proliferation/apoptosis

A

Focal cortical dysplasia with balloon cells
Megalencephalic
Microcephaly

52
Q

Cortical malformations:
Disorders of cell migration

A

Lissencephaly
Pachygyria
Heterotopia

53
Q

A complete absence of cortical sulci results in a smooth, cerebral cortex

A

Lissencephaly

54
Q

Most common genetic defect causing lissencephaly

A

LIS-1 or XLIS genes

55
Q

Thickened gyri with lose of normal interdigitation of gray matter

A

Pachygyria

56
Q

Ectopic position of neurons, common epilepsy focus

A

Heterotopia

57
Q

What gene is associated with periventricular Nodular Heterotopia?

A

X-linked dominant FLNA (filamin A) mutations

58
Q

Cortical malformations:
Disorders of cortical organization

A

Polymicrogyria
Schizencephaly

59
Q

What disease often occurs with polymicrogyria?

A

Zellweger’s syndrome

60
Q

Caused by disruption of the cortical mantle leading to a cleft in the cortex that extends from the lateral ventricle

A

Schizencephaly

61
Q

Failure of the prosencephalon to develop midline structures

A

Holoprosencephaly

62
Q

Possible structural findings in holoprosencephaly

A

Midline facial anomalies (cyclopia)
Absence of the olfactory system
Agenesis of the corpus callosum
Fused thalamus
Atypical ventricular structure

63
Q

Most common genetic cause of holoprosencephaly

A

Trisomy 13

64
Q

What is the structural presentation of septo-optic dysplasia (DeMorsier syndrome)?

A

Secondary symptoms to optic nerve hypoplasia
Absence of olfactory bulbs and tracts
Pituitary gland dysfunction
Absence of the septum pellucidum

65
Q

Genetic disorder that presents with cerebellar dysfunction, hyperpnea, and intellectual delay
Agenesis/underdevelopment of the vermis

A

Joubert syndrome

66
Q

“Molar tooth” sign

A

Joubert syndrome

67
Q

Agenesis of the cerebellar vermis, cystic dilation of the fourth ventricle, and elevation of the tentorium

A

Dandy-Walker malformation

68
Q

Arachnoid cyst is due to what abnormal formation?

A

Abnormal formation of leptomeninges

69
Q

In what instance can an arachnoid cysts appear “box shaped”?

A

If it’s located in the sylvian fissure

70
Q

What is a porencephalic cyst due to?

A

Early intrauterine vascular injury or infection

71
Q

What is the difference between a porencephalic cyst and schizencephaly, structurally?

A

Porencephalic cyst is smooth-walled and not lined by cortex

72
Q

Large central cyst with a lack of cortical tissue in anterior circulation-supplied areas

A

Hydranencephaly

73
Q

What structures tend to be preserved in hydranencephaly?

A

Cerebellum
Midbrain
Basal ganglia

74
Q

What causes hydranencephaly?

A

Severe hypoxic/ischemic insult early in development

75
Q

Unilateral enlargement of cortex parenchyma with thickened or duplicated grey matter

A

Hemimegaloencephaly

76
Q

Clinical findings in hemimegalencephaly

A

Epilepsy, intellectual disability, and hemiparesis

77
Q

What is the treatment for medically refractory epilepsy in setting of hemimegalencephaly?

A

Hemispherectomy

78
Q

What aqueduct is most commonly blocked in congenital aqueductal stenosis?

A

Aqueduct of Sylvius

79
Q

What are the symptoms of aqueductal stenosis?

A

Headache, nausea, visual disturbances, altered mental status
Enlargement of head size in first year of life

80
Q

What is the treatment for aqueductal stenosis?

A

Ventriculo-peritoneal shunt or ventriculostomy

Neurology Board Review (16 decks)

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