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MOHD4 Exam 4 Brain Stuff > Developmental Disorders of CNS > Flashcards

Developmental Disorders of CNS Flashcards

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1
Q

Nervous system develop from ____ germ layer.

A

ectoderm

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2
Q

Regionally thickened ectodermal germ layer =

A

neural plate

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3
Q

Gives rise to most of the PNS

A

Neural crest

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4
Q

Anencephaly =

A

Failure of closure of rostral part of neural tube

Absent cranial vault, forebrain –> incompatible w/ long-term postnatal life

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5
Q

Protrusion of brain and meninges through defect in skull.

A

Encephalocele

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6
Q

Herniation of spinal cord and meninges through defect in vertebral column.

A

Myelomeningocele

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7
Q

T/F: Myelomeningocele is incompatible w/ long-term postnatal life.

A

False.

Early surgical repair improves change of survival.

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8
Q 
Which of the following is NOT one of the 3 vesicles that form from the neural tube?
A. Mesencephalon
B. Myelencephalon
C. Prosecephalon
D. Rhombencephalon
A

B. Myelencephalon and metencephalon is a subdivision of rhombencephalon.

Prosecephalon = forebrain
Mesencephalon = midbrain
Rhombencephalon = hindbrain
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9
Q

T/F: Mesencephalon divides into diencephalon and telecephalon.

A

False. These are subdivisions of prosecephalon (forebrain).

Mesencephalon does not subdivide.

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10
Q

Telecephalon becomes ____

A

cerebral hemisphere

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11
Q

Diencephalon becomes ____

A

thalamic structures

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12
Q

Mesencephalon becomes ____

A

midbrain

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13
Q

Metencephalon becomes ____

A

pons and cerebellum

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14
Q

Myelencephalon becomes ____

A

medulla oblongata

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15
Q

Complete failure of primitive forebrain to diverticulate into two telencephalic vesicles.

A

Holoprosencephaly

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16
Q 
Which of the following is NOT a clinical presentation of Holoprosencephaly?
A. Severe mental retardation
B. Endocrine disorders
C. Seizures
D. Quadriparesis
A

B. Endocrine disorder is a presentation of Septo-optic dysplasia.

17
Q

Defect in formation of midline structures of forebrain

Absence of septum pellucidum, optic nerve hypoplasia and pituitary hypoplasia.

A

Septo-optic dysplasia

18
Q 
Which of the following is NOT a clinical presentation of Septo-optic dysplasia?
A. Blindness
B. Mental retardation
C. Endocrine disorders
D. Seizures
A

D

19
Q

Generation of neurons occurs from ___ to ___ months of gestation

A

2 - 5th

20
Q

Volume of 1 cerebral hemisphere is much greater than normal due to error in control of brain cell proliferation on the affected side.

A

Hemimegalencephaly

21
Q 
Which of the following is NOT a clinical presentation of Hemimegalencephaly?
A. Large heads
B. Seizures
C. Mental retardation
D. Quadriparesis
A

D. Hemiparesis

22
Q

Which of the following is FALSE about Lissencephaly?
A. It is the most severe neuronal migration disturbances
B. Cortical surface are broad and shallow
C. Microcephalic
D. Do not survive infancy
E. Intractable seizure, severely impaired psychomotor development

A

B. This is feature of Pachygyria. Lissencephaly is smooth and featureless.

23
Q

T/F: Infants with pachygyria survive with severe focal seizures.

A

False. Survive with severe neurological impairment.

24
Q

Broad, shallow and reduced in number as result of defective neuronal migration.

A

Pachygyria

25
Q

Generalized polymicrogyria present with ____ (2)

A

Severe mental retardation

Epilepsy

26
Q

Focal polymicrogyria present with ____ (2)

A

Focal seizures

Focal weakness

27
Q

Characterized by presence of unilateral or bilateral clefts in cerebral hemispheres.

A

Schizencephaly

28
Q 
Which of the following is NOT a clinical presentation of schizencephaly?
A. Weakness
B. Seizures
C. Epilepsy
D. Mental retardation
A

C

29
Q

Normal appearing neurons in abnormal location

A

Cortical heterotopia

30
Q

Genetic disorder in which myelin fails to form properly in both the CNS and PNS during childhood.

A

Metachromatic leukodystrophy

31
Q 
Metachromatic leukodystrophy clinical presentation include the following EXCEPT:
A. Progressive weakness
B. Blindness
C. Hypotonia
D. Seizures
E. Dementia
A

D.

MOHD4 Exam 4 Brain Stuff (11 decks)

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