Document Detail


Tuberous sclerosis in the premature infant: a report of a case with immunohistochemistry on the CNS.
MedLine Citation:
PMID:  2495874     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Numerous hard cortical nodules, a large subependymal giant-cell astrocytoma and a massive rhabdomyoma were described in a hydropic infant of 31 weeks gestation who lived only four hours. With six other documented cases, tuberous sclerosis in premature infants was reviewed in the hope to pinpoint the early lesions in the CNS and heart. Three premature infants including the present case with tuberous sclerosis presented as hydrops fetalis, and all three had a massive rhabdomyoma. Immunohistochemistry on the CNS lesions disclosed aggregates of bizarre giant cells, which concomitantly expressed epitopes for astroglial, oligodendroglial and neuronal phenotypes in the subpial, cortical, subcortical and subependymal zones. The findings strongly indicate that hamartomatous differentiation of pluripotential germinal neuroglia in the subependymal layer establishes early in the gestational stage and causes aberrant and/or arrested migration towards the cortical surfaces.
Authors:
T M Chou; S M Chou
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical neuropathology     Volume:  8     ISSN:  0722-5091     ISO Abbreviation:  Clin. Neuropathol.     Publication Date:    1989 Jan-Feb
Date Detail:
Created Date:  1989-05-26     Completed Date:  1989-05-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8214420     Medline TA:  Clin Neuropathol     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  45-52     Citation Subset:  IM    
Affiliation:
University of California San Francisco, Department of Medicine 94143.
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MeSH Terms
Descriptor/Qualifier:
Brain / pathology*
Cerebrosides / analysis*
Ependyma / pathology
Female
Galactosylceramides / analysis*
Glial Fibrillary Acidic Protein / analysis*
Humans
Immunoenzyme Techniques
Infant, Newborn
Infant, Premature, Diseases / pathology*
Intermediate Filament Proteins / analysis*
Neurofilament Proteins
Neuroglia / pathology
Neurons / pathology
Tuberous Sclerosis / pathology*
Vimentin / analysis*
Chemical
Reg. No./Substance:
0/Cerebrosides; 0/Galactosylceramides; 0/Glial Fibrillary Acidic Protein; 0/Intermediate Filament Proteins; 0/Neurofilament Proteins; 0/Vimentin; 0/galactocerebroside

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