Document Detail

Ocular histopathologic studies of neonatal and childhood adrenoleukodystrophy.
MedLine Citation:
PMID:  6295171     Owner:  NLM     Status:  MEDLINE    
Histopathologic studies of the eyes of one patient (a boy who died at 14 years of age) with childhood adrenoleukodystrophy and two patients (girls who died at 24 and 31 months of age) with neonatal adrenoleukodystrophy showed the accumulation of the characteristic bileaflet inclusions in optic nerve macrophages, retinal neurons, and macrophages and loss of ganglion cell and nerve fiber layer. Additionally, in the two cases of neonatal adrenoleukodystrophy, changes resembling early retinitis pigmentosa were found, with accumulation of characteristic inclusions in the retinal pigment epithelium and pigment-laden macrophages. One of the patients with neonatal adrenoleukodystrophy also had an anterior subcapsular cataract and cystoid macular edema.
S M Cohen; W R Green; Z C de la Cruz; F R Brown; H W Moser; M W Luckenbach; D J Dove; I H Maumenee
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  American journal of ophthalmology     Volume:  95     ISSN:  0002-9394     ISO Abbreviation:  Am. J. Ophthalmol.     Publication Date:  1983 Jan 
Date Detail:
Created Date:  1983-02-25     Completed Date:  1983-02-25     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0370500     Medline TA:  Am J Ophthalmol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  82-96     Citation Subset:  AIM; IM    
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MeSH Terms
Adrenoleukodystrophy / pathology*
Child, Preschool
Diffuse Cerebral Sclerosis of Schilder / pathology*
Inclusion Bodies / ultrastructure
Infant, Newborn
Infant, Newborn, Diseases / pathology*
Macrophages / ultrastructure
Microscopy, Electron
Nerve Fibers, Myelinated / ultrastructure
Optic Nerve / pathology*
Pigment Epithelium of Eye / ultrastructure
Retina / pathology*
Retinal Ganglion Cells / ultrastructure
Retinitis Pigmentosa / pathology
Grant Support

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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