New incidence, prevalence, and survival of Aicardi syndrome from 408 cases

Journal of Child Neurology
Barbara L KronerWilliam D Gaillard

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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Citations

Aug 21, 2013·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Aimen S KasasbehMatthew D Smyth
Aug 18, 2012·Clinical Neurology and Neurosurgery·Pietro SpennatoGiuseppe Cinalli
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Jan 22, 2009·Journal of the Neurological Sciences·Tai-Heng ChenHsiu-Lin Chen
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Oct 21, 2009·American Journal of Medical Genetics. Part a·Anne-Claire BursztejnChristophe Nemos
Jun 12, 2012·Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus·Gary FruhmanRichard A Lewis
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Oct 24, 2019·The Annals of Otology, Rhinology, and Laryngology·Madison V EppersonCharles M Myer
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Aug 30, 2020·Progress in Retinal and Eye Research·Samantha R De SilvaOmar A Mahroo
Jun 18, 2021·Annals of Medicine and Surgery·Saeed SaadoYazane Abdallah

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