Moyamoya disease is a rare entity consisting of bilateral stenosis or
occlusion of internal carotid arteries with abnormal collateral vessel
s at the base of the brain. We present five Turkish children with this
disease, which is more common in Japan. Focal neurologic deficits and
/or epilepsy were the common presenting symptoms in two girls and thre
e boys between the ages of 1.5 and 11 years. Multiple cerebral infarct
s were diagnosed in all of the cases by Computed Tomography (CT). Abno
rmal net-like vessels at the base of the brain were detected in three
patients. Cerebral angiography, which is necessary to confirm the diag
nosis, showed moyamoya vessels and bilateral stenosis or occlusion of
the internal carotid arteries in all cases. Although the angiographic
staging was advanced in three patients, neither clinical status nor pa
renchymal abnormalities detected with CT were different from the other
two cases.