Agenesis of the corpus callosum (ACC)
- Article
- 2021-03-02
Agenesis of the corpus callosum (ACC) is one of the many disorders of the corpus callosum, the structure that connects the two hemispheres (left and right) of the brain. In ACC, the corpus callosum is completely or partially absent. It is caused by a disturbance in the migration of brain cells during fetal development. ACC can occur as an isolated condition or in conjunction with other cerebral abnormalities, including Arnold-Chiari malformation, Dandy-Walker syndrome, schizencephaly (fissures or deep areas in brain tissue), and holoprosencephaly (failure of the forebrain to divide into lobes Girls can have a gender-specific condition called Aicardi syndrome, which causes severe cognitive and developmental impairment, seizures, abnormalities in the vertebrae of the spine, and lesions on the retina of the eye. ACC can also be associated with malformations in other parts of the body, such as midline facial abnormalities. The effects of the condition range from subtle or mild to severe, depending on the associated brain abnormalities. Children with the most severe brain malformations can have intellectual disabilities, seizures, hydrocephalus and spasticity. Other disorders of the corpus callosum are dysgenesis, where the corpus callosum is malformed or incompletely developed, and hypoplasia, where the corpus callosum is thinner than normal. Individuals with these conditions have a higher risk of hearing impairment and heart defects than individuals with normal structure. Disorders of social interaction and communication in individuals with corpus callosum disease may overlap with behaviors of autism spectrum disorders.
Therapy
There is no standard treatment for ACC. Treatment usually involves the management of symptoms and attacks as they arise. Associated problems are much more manageable with early recognition and therapy, especially therapies targeting left / right coordination. Early diagnosis and interventions are currently the best treatments to improve social and developmental outcomes.
Prognosis
Prognosis depends on the extent and severity of malformations. The intellectual disability is not getting worse. Individuals with corpus callosum disease typically have a delay in reaching developmental milestones such as walking, talking or reading; challenges with social interactions; clumsiness and poor motor coordination, especially in skills requiring left and right hand and foot coordination (such as swimming, cycling and driving; and mental and social processing problems that become more apparent with age, with problems particularly evident from the age of high school school into adulthood.
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