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Neurology Board Review > Embryology > Flashcards

Embryology Flashcards

(80 cards)

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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
When does in-utero myelination start?
14 weeks
26
When does the majority of in-utero myelination occur?
3rd trimester
27
When do subcortical U fibers myelinate?
8 months Do not finish until the second decade of life
28
When is peripheral myelination completed?
3-5 years
29
Which myelinates first? Motor or sensory roots?
Motor
30
What is a dermal sinus tract?
Tract of squamous epithelium that travels from the skin to the dura of the spinal cord
31
Where are dermal sinus tracts most often found?
Midline and in the lumbar/lumbosacral region
32
What tumors are associated with dermoid sinuses?
Dermoid or epidermoid tumors
33
What causes dermal sinuses?
Failure of differentiation between cutaneous ectoderm and neuroectoderm in the 4th week of development
34
What is the presentation of a congenital dermal sinus?
Recurrent meningitis (bacterial portal) Tethered cord Cord compression
35
What is the treatment for a dermal sinus?
Complete excision
36
What is craniorachischisis?
Complete failure of neural tube formation leading to both anencephaly and extensive spina bifida
37
A failure of anterior neural tube closure
Anencephaly
38
Defect of cranial mesoderm development which leads to a skull defect with protrusion of both meninges and brain
Encephalocele
39
What disease in an encephalocele associated with?
Meckel-Gruber syndrome.
40
A pathological opening of the vertebral column in which meninges protrudes through the skeletal defect without neurologic tissue outside the spinal cord
Meningocele
41
Incomplete closure of the caudal neural tube Meninges and spinal cord are both herniated through the defect in a covered membrane
Myelomeningocele
42
Myelomeningocele without spinal contents covered by a membrane is called:
Myeloschisis
43
Incomplete closure of the vertebrae with an overlying tuft of hair or dimple Generally asymptomatic
Spina bifida occulta
44
Defects of spinal cord development Imaging show longitudinal duplication/splitting and tethering of the spinal cord
Diastematomyelia
45
Chiari I
Downward displacement of the cerebellar tonsils at least 5 mm beyond the foramen magnum
46
Chiari II
Displacement of the cerebellar tonsils as well as the brainstem
47
Patients with Chiari II can also have a ____ and at risk for ____.
Myelomeningocele Hydrocephalous
48
Chiari malformation associated with syringomyelia:
I and II
49
Symptoms of a syringomyelia:
Loss of pain in a “cape-like” distribution Arm weakness
50
Cause of symptoms with a syringomyelia:
Damage of the crossing spinothalamic fibers Damaged anterior horn cells
51
Cortical malformations: Disorders of cell proliferation/apoptosis
Focal cortical dysplasia with balloon cells Megalencephalic Microcephaly
52
Cortical malformations: Disorders of cell migration
Lissencephaly Pachygyria Heterotopia
53
A complete absence of cortical sulci results in a smooth, cerebral cortex
Lissencephaly
54
Most common genetic defect causing lissencephaly
LIS-1 or XLIS genes
55
Thickened gyri with lose of normal interdigitation of gray matter
Pachygyria
56
Ectopic position of neurons, common epilepsy focus
Heterotopia
57
What gene is associated with periventricular Nodular Heterotopia?
X-linked dominant FLNA (filamin A) mutations
58
Cortical malformations: Disorders of cortical organization
Polymicrogyria Schizencephaly
59
What disease often occurs with polymicrogyria?
Zellweger's syndrome
60
Caused by disruption of the cortical mantle leading to a cleft in the cortex that extends from the lateral ventricle
Schizencephaly
61
Failure of the prosencephalon to develop midline structures
Holoprosencephaly
62
Possible structural findings in holoprosencephaly
Midline facial anomalies (cyclopia) Absence of the olfactory system Agenesis of the corpus callosum Fused thalamus Atypical ventricular structure
63
Most common genetic cause of holoprosencephaly
Trisomy 13
64
What is the structural presentation of septo-optic dysplasia (DeMorsier syndrome)?
Secondary symptoms to optic nerve hypoplasia Absence of olfactory bulbs and tracts Pituitary gland dysfunction Absence of the septum pellucidum
65
Genetic disorder that presents with cerebellar dysfunction, hyperpnea, and intellectual delay Agenesis/underdevelopment of the vermis
Joubert syndrome
66
"Molar tooth" sign
Joubert syndrome
67
Agenesis of the cerebellar vermis, cystic dilation of the fourth ventricle, and elevation of the tentorium
Dandy-Walker malformation
68
Arachnoid cyst is due to what abnormal formation?
Abnormal formation of leptomeninges
69
In what instance can an arachnoid cysts appear "box shaped"?
If it's located in the sylvian fissure
70
What is a porencephalic cyst due to?
Early intrauterine vascular injury or infection
71
What is the difference between a porencephalic cyst and schizencephaly, structurally?
Porencephalic cyst is smooth-walled and not lined by cortex
72
Large central cyst with a lack of cortical tissue in anterior circulation-supplied areas
Hydranencephaly
73
What structures tend to be preserved in hydranencephaly?
Cerebellum Midbrain Basal ganglia
74
What causes hydranencephaly?
Severe hypoxic/ischemic insult early in development
75
Unilateral enlargement of cortex parenchyma with thickened or duplicated grey matter
Hemimegaloencephaly
76
Clinical findings in hemimegalencephaly
Epilepsy, intellectual disability, and hemiparesis
77
What is the treatment for medically refractory epilepsy in setting of hemimegalencephaly?
Hemispherectomy
78
What aqueduct is most commonly blocked in congenital aqueductal stenosis?
Aqueduct of Sylvius
79
What are the symptoms of aqueductal stenosis?
Headache, nausea, visual disturbances, altered mental status Enlargement of head size in first year of life
80
What is the treatment for aqueductal stenosis?
Ventriculo-peritoneal shunt or ventriculostomy

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