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0883073807309782v1
23/5/531    most recent
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First published on January 8, 2008, doi:10.1177/0883073807309782

Journal of Child Neurology 2008;23:531.

A more recent version of this article appeared on May 1, 2008
 © 2008 SAGE Publications

Article

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

Barbara L. Kroner, PhD1*, Liliana R. Preiss, MS1, Mary-Anne Ardini, BA1, and William D. Gaillard, MD2

1 RTI International, Rockville, Maryland
2 the Children’s National Medical Center, Washington, DC

* To whom correspondence should be addressed. E-mail: byk{at}rti.org.


  Abstract
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.


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