| Aicardi syndrome: a morphologic description with particular reference to intracytoplasmic inclusions in cortical astrocytes. | |
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MedLine Citation:
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PMID: 9025834 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Aicardi syndrome is characterized by agenesis of the corpus callosum, infantile spasms, and ocular anomalies. Very few morphologic descriptions have been made of the central nervous system of children with this syndrome. We performed a postmortem examination of the brain of a 13-year-old girl with clinically well-documented Aicardi syndrome. Gross examination revealed a small brain (745 g) with the right cerebral and cerebellar hemispheres smaller than the left. There was agenesis of the corpus callosum, and ectopic gray matter was scattered throughout the cerebral hemispheres. Both choroid plexus and arachnoid cysts were present. Microscopic examination revealed indistinct cortical layering and multiple foci of ectopic gray matter. The cerebellar sections were altered by focal atrophy with gliosis and Purkinje cell dropout. Multiple sections of cerebrum contained astrocytes with coarse, paranuclear, eosinophilic inclusions. Electron microscopy, immunohistochemistry, and special stains further defined these inclusions, which we speculate represent a degenerative process. |
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Authors:
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J J Buchino; K K Nicol; J C Parker |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Volume: 16 ISSN: 1077-1042 ISO Abbreviation: Pediatr Pathol Lab Med Publication Date: 1996 Mar-Apr |
Date Detail:
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Created Date: 1997-03-06 Completed Date: 1997-03-06 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 9518033 Medline TA: Pediatr Pathol Lab Med Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 285-91 Citation Subset: IM |
Affiliation:
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Department of Pediatrics and Pathology, School of Medicine, University of Louisville, Kentucky, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Astrocytes / pathology* Cerebral Cortex / pathology* Corpus Callosum / abnormalities*, ultrastructure Eye Abnormalities / pathology* Female Humans Inclusion Bodies / pathology* Infant, Newborn Spasms, Infantile / etiology, pathology* Syndrome |
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