Moyamoya syndrome in an adult with essential thrombocythemia

Marc A. Lazzaro, Elizabeth J. Cochran, Demetrius K. Lopes, Shyam Prabhakaran

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Moyamoya syndrome is a rare cerebrovascu-lar 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 angio-graphic appearance. Although the pathophysi-ology is unclear, hematologic disorders have been associated with development of the moy-amoya 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.

Original languageEnglish (US)
Pages (from-to)9-11
Number of pages3
JournalNeurology International
Volume3
Issue number1
DOIs
StatePublished - 2011

Keywords

  • Essential thrombo-cythemia
  • Moyamoya
  • Stroke

ASJC Scopus subject areas

  • Clinical Neurology

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