[An adult case of Moyamoya disease presenting with transient hemichorea].

A 54-year-old woman presented choreic movements in left face and extremities for three months. Cerebral angiography revealed occlusions of bilateral internal carotid arteries (right: after the furcation of posterior communicating artery; left: after the furcation of opthalmic artery) and net-like collaterals around the basal ganglia region (Moyamoya vessels). Haloperidol rapidly resolved the choreic movements and no recurrence was observed. PET demonstrated misery perfusion at bilateral temporo-parietal cortices. Especially, right peri-sylvian region showed the most severe ischemia. MRI demonstrated no infarcts. Therefore, ischemia of the right striatum was suspected to be the cause of the left-sided hemichorea. Previously, Moyamoya disease presenting chorea was infrequently reported in young people of less than 20 years of age. This is the first report of the aged patient of Moyamoya disease presenting with hemichorea and severe misery perfusion.