Document Detail

Spontaneous occlusion of the circle of Willis (moyamoya syndrome).
MedLine Citation:
PMID:  7166015     Owner:  NLM     Status:  MEDLINE    
Three young autopsy and four temporal artery biopsy cases with an arteriographically typical moyamoya syndrome were studied by histologic, electron microscopic, and immunofluorescence methods. In all autopsy cases the intracranial segment of the internal carotid arteries showed concentric intimal thickening with severe stenosis or obstruction of the lumen, folding of the internal elastic lamina, and shrinkage of the external diameter of the vessel. Inflammatory infiltration was absent. Abnormal networks of thin-walled vessels, interpreted as secondary collaterals, were seen at the base and on the convexity of the brain. Degenerative changes, including a ruptured coronary aneurysm in one case, were also noted in the coronary and temporal arteries. In both the intra- and extracranial arteries repeated endothelial damage was indicated by the presence of redundant subendothelial basement membrane-like material. Lipid or calcium deposits were exceptional, and no evidence for the presence of immunoglobulins or components of the complement could be obtained by immunofluorescence. These pathologic alterations, distinct from atherosclerosis, fibromuscular dysplasia, and the established types of arteritis, seem to be identical in children and adults, in spite of different clinical manifestations of the moyamoya syndrome in these age groups. We suggest that a humoral factor, associated with infections, may induce repeated endothelial damage and intimal thickening in the intracranial arteries of genetically predisposed children in analogy with recent observations made in the coronary arteries of young subjects.
M Haltia; M Iivanainen; H Majuri; M Puranen
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical neuropathology     Volume:  1     ISSN:  0722-5091     ISO Abbreviation:  Clin. Neuropathol.     Publication Date:  1982  
Date Detail:
Created Date:  1983-05-27     Completed Date:  1983-05-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8214420     Medline TA:  Clin Neuropathol     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  11-22     Citation Subset:  IM    
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MeSH Terms
Arterial Occlusive Diseases / diagnosis*
Basilar Artery / pathology
Brain / pathology
Carotid Artery, Internal / radiography
Cerebral Angiography
Cerebral Arteries / pathology,  ultrastructure
Child, Preschool
Microscopy, Electron
Middle Aged
Moyamoya Disease / diagnosis*
Temporal Arteries / pathology

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

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