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Aicardi syndrome - Overview

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.

The disorder affects only girls.

  • Reviewed last on: 8/1/2008
  • 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.

References

Glasmacher MA, Sutton Vr, Hopkins B, Eble T, Lewis RA, Park Parsons D, et al. Phenotype and management of Aicardi syndrome: new findings from a survey of 69 children. J Child Neurol. 2007;22:176-184.

Kinsman SL, Johnston MV. Congenital Abnormalities of the Central Nervous System. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 592.

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