What Is Agenesis of the Corpus Callosum?
Agenesis of the corpus
callosum (ACC) is a rare birth defect in which the structure that connects the
two hemispheres of the brain (the corpus callosum) is partially or completely
absent. ACC can occur as an isolated condition or in combination with other
cerebral abnormalities, including Arnold-Chiari malformation, Dandy-Walker
syndrome, Andermann syndrome, schizencephaly (clefts or deep divisions in brain
tissue), and holoprosencephaly (failure of the forebrain to divide into lobes.)
Girls may have a gender-specific condition called Aicardi's syndrome, which
causes severe mental retardation, seizures, abnormalities in the vertebra of the
spine and lesions on the retina of the eye. ACC also can be associated with
malformations in other parts of the body, such as midline facial defects. The
effects of the disorder range from subtle or mild to severe, depending on
associated brain abnormalities. Intelligence may be normal with mild compromise
of skills requiring matching of visual patterns. But children with the most
severe brain malformations may have intellectual retardation, seizures,
hydrocephalus and spasticity.
Is There Any Treatment?
There
is no standard course of treatment for ACC. Treatment usually involves
management of symptoms and seizures if they occur.
What Is the
Prognosis?
Prognosis depends on the extent and severity of
malformations. ACC does not cause death in the majority of children. Mental
retardation does not worsen. Although many children with the disorder have
average intelligence and lead normal lives, neuropsychological testing reveals
subtle differences in higher cortical function compared to individuals of the
same age and education without ACC.
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