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Child's Nervous System
Published in: Child's Nervous System 5/2009

01-05-2009 | Brief Communication

Prenatal diagnosed cyst of the quadrigeminal cistern in Aicardi syndrome

Authors: Laura Columbano, Wolf Luedemann, Yasuko Kusaka, Shizuo Oi, Madjid Samii

Published in: Child's Nervous System | Issue 5/2009

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Abstract

Aicardi syndrome is an X-linked dominant disorder characterized by the pathognonomic association of three clinical signs: agenesis of the corpus callosum, infantile spasms, and chorioretinal lacunae. This is a severe syndrome with an estimated survival rate of 76% at 6 years and 40% at 14 years and a maximum developmental level like a 12-month-old baby in 91% of cases. We illustrate a case of Aicardi syndrome with bilateral, small chorioretinal lacunae, infantile spasms with a typical hypsarrhythmia, defects of thoracic vertebra and bilateral absence of the12th rib associated with an arachnoid cyst of the quadrigeminal cistern, and presence of the corpus callosum with a favorable outcome.
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Rosser TL, Acosta MT, Packer RJ (2002) Aicardi syndrome: spectrum of disease and longterm prognosis in 77 females. Pediatr Neurol 27:343–346PubMedCrossRef
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Metadata
Title
Prenatal diagnosed cyst of the quadrigeminal cistern in Aicardi syndrome
Authors
Laura Columbano
Wolf Luedemann
Yasuko Kusaka
Shizuo Oi
Madjid Samii
Publication date
01-05-2009
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 5/2009
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-008-0772-8

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