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Aicardi Syndrome
What is Aicardi Syndrome?
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Aicardi Syndrome is a rare genetic disorder
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.
Is there any treatment?
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.
What is the prognosis?
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.
What research is being done?
The NINDS supports and conducts research on neurogenetic disorders
such as Aicardi Syndrome. The goals of this research are to locate
and understand the genes involved and to develop techniques to diagnose,
treat, prevent, and ultimately cure the disorder.
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