Agenesis of the corpus callosum (ACC) is a birth defect characterised by the partial absence or absence of the two hemispheres of the brain (the corpus callosum). The condition may 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.)
In girls, there is a 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.
Agenesis of the corpus callosum (ACC) is 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.
As of now, there is no specific medical treatment for callosal disorders, but individuals affected with ACC may benefit from a range of developmental therapies. You may have to consult with a variety of medical, health and educational personnel such as neurologists, neuropsychologists, occupational therapists, physical therapists, pathologists, paediatricians, geneticists, special educators and early childhood intervention specialists in this regard.
ACC does not cause death in the majority of children. Mental retardation does not worsen, but 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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