Moyamoya syndrome in an adult with essential thrombocythemia. Neurol Int 2011 Jun;3(1):e3
Date
07/26/2011Pubmed ID
21785675Pubmed Central ID
PMC3141114DOI
10.4081/ni.2011.e3Scopus ID
2-s2.0-80053066644 (requires institutional sign-in at Scopus site) 9 CitationsAbstract
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.
Author List
Lazzaro MA, Cochran EJ, Lopes DK, Prabhakaran SAuthors
Elizabeth J. Cochran MD Adjunct Professor in the Pathology and Laboratory Medicine department at Medical College of WisconsinMarc A. Lazzaro MD Associate Professor in the Neurology department at Medical College of Wisconsin
