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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Main Authors: Marc Lazzaro, Elizabeth J. Cochran, Demetrius K. Lopes, Shyam Prabhakaran
Format: Article
Language:English
Published: MDPI AG 2011-04-01
Series:Neurology International
Subjects:
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.
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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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AT demetriusklopes moyamoyasyndromeinanadultwithessentialthrombocythemia
AT shyamprabhakaran moyamoyasyndromeinanadultwithessentialthrombocythemia