Document Detail

Histopathological abnormalities in ocular blood vessels of CADASIL patients.
MedLine Citation:
PMID:  15289148     Owner:  NLM     Status:  MEDLINE    
PURPOSE: To assess histopathological findings in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). DESIGN: Case reports and histopathological evaluation of enucleated eyes. METHODS: Four eyes from two CADASIL patients were enucleated at autopsy and prepared for histopathological analysis using light and electron microscopy. RESULTS: Thickening of arterial walls with fibrosis, eosinophilic Periodic acid Schiff-positive basement membrane material and loss of vascular smooth muscle cells (VSMC) in the central retinal artery and its branches, the leptomeninges, the ocular adnexa, and the optic disk were observed. On electron microscopy, numerous deposits of granular, osmiophilic material in arterial walls as well as VSMC and pericyte degeneration were noted. In contrast to retinal vessels, the choroid was not affected. CONCLUSION: Our findings suggest a differential involvement of small blood vessels in CADASIL, depending on the angioarchitecture and support autopsy data of nervous tissue describing that loss of VSMCs is most pronounced in tissues depending on blood-tissue barriers.
Christos Haritoglou; Jan P Hoops; Fritz H Stefani; Parviz Mehraein; Anselm Kampik; Martin Dichgans
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of ophthalmology     Volume:  138     ISSN:  0002-9394     ISO Abbreviation:  Am. J. Ophthalmol.     Publication Date:  2004 Aug 
Date Detail:
Created Date:  2004-08-03     Completed Date:  2004-08-19     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0370500     Medline TA:  Am J Ophthalmol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  302-5     Citation Subset:  AIM; IM    
Department of Ophthalmology, Ludwig-Maximilians-University, Munich, Germany.
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MeSH Terms
Bruch Membrane / ultrastructure
Dementia, Multi-Infarct / pathology*
Eosinophils / pathology
Middle Aged
Muscle, Smooth, Vascular / pathology
Pericytes / pathology
Pigment Epithelium of Eye / ultrastructure
Retinal Artery / ultrastructure*
Retinal Diseases / pathology*

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

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