Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: two new cases

Two young women developed encephalopathy, hearing loss and retinal arteriolar occlusions. Their behaviour became immature and cognitive functions were severely impaired. One of the patients underwent brain biopsy, which showed several microinfarcts in both white and grey matter and microangiopathic...

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Bibliographic Details
Published in:	Journal of the neurological sciences Vol. 86; no. 2-3; p. 239
Main Authors:	Heiskala, H, Somer, H, Kovanen, J, Poutiainen, E, Karli, H, Haltia, M
Format:	Journal Article
Language:	English
Published:	Netherlands 01-09-1988
Subjects:	Adult Arterial Occlusive Diseases - complications Arterial Occlusive Diseases - diagnosis Arterial Occlusive Diseases - diagnostic imaging Brain - pathology Cerebrovascular Circulation Cerebrovascular Disorders - complications Cerebrovascular Disorders - diagnosis Cerebrovascular Disorders - pathology Female Hearing Loss - complications Hearing Loss - diagnosis Humans Radiography Retina - blood supply
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Summary:	Two young women developed encephalopathy, hearing loss and retinal arteriolar occlusions. Their behaviour became immature and cognitive functions were severely impaired. One of the patients underwent brain biopsy, which showed several microinfarcts in both white and grey matter and microangiopathic changes, with thickened arteriolar segments staining intensely for laminin and fibronectin. These findings support the concept of a new type of microangiopathy involving the brain, inner ear and retina.
ISSN:	0022-510X
DOI:	10.1016/0022-510X(88)90102-5