Aicardi syndrome

Definition of Aicardi syndrome

Aicardi syndrome is a rare inherited (genetic) disorder in which the structure that connects the two sides of the brain (corpus callosum is partly or completely missing.

Causes, incidence, and risk factors

The cause of Aicardi syndrome is unknown at this time. In some cases, experts believe it may be a result of a gene defect on the X chromosome.

Symptoms

Symptoms usually start when the child is between ages 3 and 5 months. The condition causes jerking (infantile spasms), a type of childhood seizure.

Signs and tests

Children are diagnosed with Aicardi syndrome if they meet the following criteria:

Treatment

Treatment is supportive. It involves managing seizures and any other health concerns, and using programs to help the family and child cope with delays in development.

Expectations (prognosis)

The outlook depends on how severe the symptoms are and what other health conditions are present.

Review

Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 8/26/2010

Corpus callosum of the brain

ADAM Medical Encyclopedia

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