Pediatric Neurology
Volume 27, Issue 5 , Pages 343-346 , November 2002

Aicardi syndrome: spectrum of disease and long-term prognosis in 77 females

  • Tena L Rosser, MD

      Affiliations

    • Department of Neurology and Pediatrics; Center for Neuroscience and Behavioral Medicine; Children’s National Medical Center; The George Washington University; Washington, DC, USA
    • Corresponding Author InformationCommunications should be addressed to: Dr. Rosser; Department of Neurology; Children’s National Medical Center; 111 Michigan Ave., NW; Washington, DC, 20010, USA.
    • ,
  • Maria T Acosta, MD

      Affiliations

    • Department of Neurology and Pediatrics; Center for Neuroscience and Behavioral Medicine; Children’s National Medical Center; The George Washington University; Washington, DC, USA
    • ,
  • Roger J Packer, MD

      Affiliations

    • Department of Neurology and Pediatrics; Center for Neuroscience and Behavioral Medicine; Children’s National Medical Center; The George Washington University; Washington, DC, USA

Received 5 February 2002 ,Accepted 8 May 2002.

References 

  1. Aicardi J, Lefebvre J, Lerique-Koechlin A. A new syndrome (Spasms in flexion, callosal agenesis, ocular abnormalities). Electroencephalogr Clin Neurophysiol. 1965;19:609–610
  2. Wettke-Schafer R, Kanter G. X-linked dominant inherited diseases with lethality in hemizygous males. Hum Genet. 1983;64:1–23
  3. Donnenfeld AE, Packer RJ, Zackai EH, Chee CM, Sellinger B, Emanuel BS. Clinical, cytogenic, and pedigree findings in 18 cases of Aicardi syndrome. Am J Med Genet. 1989;32:461–467
  4. Hopkins IJ, Humphrey I, Keith CG, Susman M, Webb GC, Turner EK. The Aicardi syndrome in a 47,XXY male. Aust Paediatr J. 1979;15:278–280
  5. Aggarwal KC, Aggarwal A, Prasad MS, Salhan RN, Upadhaya A. Aicardi’s syndrome in a male child (An unusual presentation). Ind Pediatr. 2000;37:542–545
  6. Curatolo P, Libutti G, Dallapiccola B. Aicardi syndrome in a male infant. J Pediatr. 1980;96:286–287
  7. Aicardi J. Aicardi syndrome in a male infant. Editorial correspondence. J Pediatr. 1980;97:1040–1041
  8. Hoag HM, Taylor SAM, Duncan AMV, Khalifa MM. Evidence that skewed X-inactivation is not needed for phenotypic expression of Aicardi syndrome. Hum Genet. 1997;100:459–464
  9. Menezes AV, MacGregor DL, Buncic JR. Aicardi syndrome (Natural history and possible predictors of severity). Pediatr Neurol. 1994;11:313–318
  10. Menezes AV, Lewis TL, Buncic JR. Role of ocular involvement in the prediction of visual development and clinical prognosis in Aicardi syndrome. Br J Ophthalmol. 1996;80:805–811
  11. Neidich JA, Nussbaum RL, Packer RJ, Emanuel BS, Puck JM. Heterogeneity of clinical severity and molecular lesions in Aicardi syndrome. J Pediatr. 1990;116:911–917
  12. Chevrie JJ, Aicardi J. The Aicardi syndrome. In:  Pedley TA,  Meldrum BS editor. Recent advances in epilepsy. vol. 3:Edinburgh: Churchill Livingstone; 1986;p. 189–210
  13. Menezes AV, Enzenauer RW, Buncic JR. Aicardi syndrome–the elusive mild case. Br J Ophthalmol. 1994;78:494–496
  14. King AM, Bowen DI, Goulding P, Doran RML. Aicardi syndrome. Br J Ophthalmol. 1998;82:457
  15. Smith CD, Ryan SJ, Hoover SL, Baumann RJ. Magnetic resonance imaging of the brain in Aicardi syndrome (Report of 20 patients). J Neuroimaging. 1996;6:214–221
  16. Taylor M, David AS. Agenesis of the corpus callosum (A United Kingdom series of 56 cases). J Neurol Neurosurg Psychiatry. 1998;64:131–134
  17. Ohtsuka Y, Oka E, Terasaki T, Ohtahara S. Aicardi syndrome (A longitudinal clinical and electrographic study). Epilepsia. 1993;34:627–634
  18. Ohtsuka Y. West syndrome and its related epileptic syndromes. Epilepsia. 1998;39:30–37
  19. Ohtahara S, Ohtsuka Y, Yamatogi Y, Oka E, Yoshinaga H, Sato M. Prenatal etiologies of West syndrome. Epilepsia. 1993;34:716–722

PII: S0887-8994(02)00450-2

Pediatric Neurology
Volume 27, Issue 5 , Pages 343-346 , November 2002

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