Aicardi Syndrome

Aicardi Syndrome is a rare genetic disorder identified by the French Neurologist, Dr. Jean Aicardi in 1965. The number of identified cases of girls with Aicardi syndrome is approximately 300 - 500 worldwide. It is characterized by the partial or complete absence of the structure that links the two hemispheres of the brain, the corpus callosum.

The disorder affects only girls. Onset of Aicardi Syndrome generally begins between the ages of 3 and 5 months with infantile spasms, a type of childhood seizure. Symptoms include seizures, mental retardation and lesions on the retina of the eye that are specific to the disorder. Aicardi Syndrome may be associated with other brain defects such as a smaller than average brain and cavities or gaps in the brain filled with cerebrospinal fluid. There is no cure for Aicardi Syndrome nor is there a standard course of treatment. Treatment generally involves medical management of seizures and programs to help parents and the child cope with developmental delays.
The prognosis for girls with Aicardi Syndrome varies according to the severity of their symptoms. The known age range of affected children is from birth to the mid 20's.

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