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This version was published on May 1, 2008
Journal of Child Neurology, Vol. 23, No. 5, 531-535 (2008)
DOI: 10.1177/0883073807309782
© 2008 SAGE Publications

New Incidence, Prevalence, and Survival of Aicardi Syndrome From 408 Cases

Barbara L. Kroner, PhD

RTI International, Rockville, Maryland, byk{at}rti.org

Liliana R. Preiss, MS

RTI International, Rockville, Maryland

Mary-Anne Ardini, BA

RTI International, Rockville, Maryland

William D. Gaillard, MD

Children's National Medical Center, Washington, DC

We sought to determine the incidence, prevalence, and life expectancy of Aicardi syndrome from 408 cases compiled from multiple international sources. Last known age ranged from less than 1 month to 42 years. The incidence rates per live births for the United States and The Netherlands were 1 per 105 000 and 1 per 93 000, respectively. The prevalence in the United States is greater than 853 cases, and the worldwide estimate is several thousand. Forty-five cases were deceased (age range, 1 month to 33 years), and the risk of death peaked at age 16. The probability of survival at 27 years of age was 0.62 (95% CI, 0.47-0.77). The risk of death by age follows other congenital neurological disorders with a wide range in severity of functional disability. The longer life expectancy found in our study hints at a higher functioning capacity in Aicardi syndrome and may inform counseling to families.

Key Words: incidence • prevalence • survival • Aicardi syndrome


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