FETAL NEURAL AXIS PART II (Chapter 60)

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1
Agenesis of the Corpus Callosum
* Begins to develop at 8 weeks of gestation.
* Is not complete until 18 - 19 weeks.
The cause is unclear.
* Thought to involve vascular disruption or inflammatory lesion before 12 weeks.
2
When does Agenesis of the Corpus Callosum (ACC) begin?
Begins to develop at 8 weeks of gestation.
3
When is Agenesis of the Corpus Callosum complete?
Is not complete until 18 - 19 weeks.
What is the cause of agenesis (ACC)?
4
ACC - Agenesis of the Corpus Callosum
The cause is unclear.
* Thought to involve vascular disruption or inflammatory lesion before 12 weeks.
5
What is corpus callosum?
A fibrous white matter tract that connects the cerebral hemispheres and aids in the cognition and neurological functions.
6
What does "Dysgenesis of corpus callosum" describe?
Range of complete to partial absence of callosal fibers that cross the midline, forming a connection between the two hemispheres.
7
What is Agenesis of the Corpus Callosum (ACC) also known as?
Callosal agenesis
8
When does Agenesis of the Corpus Callosum (ACC) occur?
Occurs sporadically
9
What other malformations are associated with Agenesis of the Corpus Callosum (ACC)?
May be associated with other CNS malformations:
- Hydrocephalus
- gyral anomalies
- Heterotopias
- DWM
- holoprosencephaly
10
What manners may Agenesis of the Corpus Callosum (ACC) be transmitted?
May be transmitted in autosomal-dominant or autosomal-recessive manner.
11
In Agenesis of the Corpus Callosum (ACC) what syndromes have been identified?
X-linked syndromes have been idenified.
12
What chromosomal anomalies may accompany Agenesis of the Corpus Callosum (ACC)?
- Trisomies 21
- Trisomies 13
- Trisomies 18
- Triploidy.
13
What other syndromes have been associated with Agenesis of the Corpus Callosum (ACC)?
-Aicardi
- Apert
-Opitz
- Joubert
And others.
14
Explain the prognosis of Agenesis of the Corpus Callosum (ACC).
Prognosis depends on high incidence of associated anomalies; may carry poor prognosis.
15
Agenesis of the Corpus Callosum (ACC) is an isolated event. (True or False)
False
16
Explain the symptoms or associations with Agenesis of the Corpus Callosum (ACC).
Agenesis of the Corpus Callosum (ACC) may be asymptomatic or associated with mental retardation and/or seizures.
17
Explain the sonographic features of Agenesis of the Corpus Callosum (ACC).
Sonographic features:
* Absence of corpus callosum
* Elevation and dilation of third ventricle
* Widely separated lateral ventricular frontal horns with medial indentation of medial walls
* Dilated occipital horns ( colpocephaly ), giving lateral ventricles teardrop shape
* Absence of cavum septum pellucidum
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