Recent experience with Moyamoya disease in Turkey.

ABSTRACT

A series of moyamoya patients is presented. Angiographic findings, outcome of revascularization surgery and a young case with moyamoya disease and hyperphosphatemia are reported. Thirteen patients (6 males and 7 females; age range 2-50 years) were included in the study group. Findings of the patients at presentation were intracranial haemorrhage in two adult cases and sequelae of cerebral ischemia in the rest of the group. One young girl had hyperphosphataemia. Angiography showed distal internal carotid or proximal anterior and middle cerebral artery stenosis, unique collaterals, microaneurysm of the posterior lateral choroidal artery and flow-related changes in the posterior circulation. In 3 patients, encephalo-duro-arterio-synangiosis (EDAS) and burrholes were performed at surgery. Follow-up angiograms of these patients showed revascularization. Moyamoya, a rare but potentially devastating disease, must be addressed as a cause of haemorrhagic and ischaemic cerebral events.