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
