Moyamoya syndrome in an adult with essential thrombocythemia
Moyamoya syndrome is a rare cerebrovascular disorder characterized by progressive occlusion of the supraclinoid internal carotid artery and proximal portions of the anterior and middle cerebral arteries resulting in an extensive network of collateralized blood vessels and producing a characteristic...
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MDPI AG
2011-04-01
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Series: | Neurology International |
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Online Access: | http://www.pagepress.org/journals/index.php/ni/article/view/2128 |
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author | Marc Lazzaro Elizabeth J. Cochran Demetrius K. Lopes Shyam Prabhakaran |
author_facet | Marc Lazzaro Elizabeth J. Cochran Demetrius K. Lopes Shyam Prabhakaran |
author_sort | Marc Lazzaro |
collection | DOAJ |
description | Moyamoya syndrome is a rare cerebrovascular disorder characterized by progressive occlusion of the supraclinoid internal carotid artery and proximal portions of the anterior and middle cerebral arteries resulting in an extensive network of collateralized blood vessels and producing a characteristic angiographic appearance. Although the pathophysiology is unclear, hematologic disorders have been associated with development of the moyamoya syndrome. A case report is presented. A 29 year-old female with a history of essential thrombocythemia developed progressive ischemic strokes. Angiography revealed characteristic moyamoya changes and pathologic examination showed intimal hyperplasia with scant collagen fibers and myxoid change. This is the first reported case of moyamoya syndrome in an adult patient with essential thrombocythemia demonstrating histological findings that suggest a shared pathophysiology with moyamoya syndrome in sickle cell anemia. |
first_indexed | 2024-04-10T18:15:01Z |
format | Article |
id | doaj.art-a93e753287e647b19bad47446d2a3c8b |
institution | Directory Open Access Journal |
issn | 2035-8385 2035-8377 |
language | English |
last_indexed | 2024-04-10T18:15:01Z |
publishDate | 2011-04-01 |
publisher | MDPI AG |
record_format | Article |
series | Neurology International |
spelling | doaj.art-a93e753287e647b19bad47446d2a3c8b2023-02-02T09:08:24ZengMDPI AGNeurology International2035-83852035-83772011-04-0131e3e310.4081/ni.2011.e31451Moyamoya syndrome in an adult with essential thrombocythemiaMarc Lazzaro0Elizabeth J. CochranDemetrius K. LopesShyam PrabhakaranDepartment of Neurology, Medical College of Wisconsin, Milwaukee, Wisconsin, USAMoyamoya syndrome is a rare cerebrovascular disorder characterized by progressive occlusion of the supraclinoid internal carotid artery and proximal portions of the anterior and middle cerebral arteries resulting in an extensive network of collateralized blood vessels and producing a characteristic angiographic appearance. Although the pathophysiology is unclear, hematologic disorders have been associated with development of the moyamoya syndrome. A case report is presented. A 29 year-old female with a history of essential thrombocythemia developed progressive ischemic strokes. Angiography revealed characteristic moyamoya changes and pathologic examination showed intimal hyperplasia with scant collagen fibers and myxoid change. This is the first reported case of moyamoya syndrome in an adult patient with essential thrombocythemia demonstrating histological findings that suggest a shared pathophysiology with moyamoya syndrome in sickle cell anemia.http://www.pagepress.org/journals/index.php/ni/article/view/2128Stroke, moyamoya, essential thrombocythemia |
spellingShingle | Marc Lazzaro Elizabeth J. Cochran Demetrius K. Lopes Shyam Prabhakaran Moyamoya syndrome in an adult with essential thrombocythemia Neurology International Stroke, moyamoya, essential thrombocythemia |
title | Moyamoya syndrome in an adult with essential thrombocythemia |
title_full | Moyamoya syndrome in an adult with essential thrombocythemia |
title_fullStr | Moyamoya syndrome in an adult with essential thrombocythemia |
title_full_unstemmed | Moyamoya syndrome in an adult with essential thrombocythemia |
title_short | Moyamoya syndrome in an adult with essential thrombocythemia |
title_sort | moyamoya syndrome in an adult with essential thrombocythemia |
topic | Stroke, moyamoya, essential thrombocythemia |
url | http://www.pagepress.org/journals/index.php/ni/article/view/2128 |
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