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Beta-thalassemia intermedia associated with moyamoya syndrome
Basak Karakurum Göksel1, Hakan Ozdogu, Tulin Yildirim
1Department of Neurology, Faculty of Medicine, Adana Teaching and Medical Research Center, Baskent University, Dadaloglu Mah., 39 Sok No. 6, Yuregir, Adana, Turkey. bkarakurum@hotmail.com
Abstract:
Moyamoya syndrome (MMS) is a progressive disorder. We report a 19-year-old boy with beta-thalassemia who presented with a left hemiparesis. Brain MRI showed old middle cerebral artery and left frontal subcortical white matter infarcts. Brain magnetic resonance angiography and digital subtraction angiography revealed occlusion of the bilateral internal carotid arteries with a rich network of basal collateral vessels. To our knowledge this is the third report of beta-thalassemia intermedia and MMS, and the first report of a patient in Turkey. It emphasizes the potential for cerebral infarct due to anemia, protein S and thrombocytosis.
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