DOI: 10.36347/sjams.2014.v02i01.0073

Pages: 345-3547

We report a case of 10 year old thalassemic boy who manifested with sudden onset of left sided hemiparesis. MR angiography revealed bilateral occlusion of internal carotid artery and innumerable dilated and tortuous arterial collaterals giving 'puff of smoke' picture suggestive of Moyamoya disease. Moyamoya disease is an uncommon arteriopathy leading to cerebral ischemia in children and its association with beta thalassemia intermedia is a rarity. This rare but grave consequence of the thromboembolic complications should be considered in these patients. The patient was managed conservatively and has recovered well.