Hemidystonia and hemichoreoathetosis as an initial manifestation of Moyamoya disease

Objective: To describe hemidystonia and hemichoreo-athetosis in an adult pa tient with; moyamoya disease without a previous history of cerebrovascular accident. Design: Case report. Setting: Tertiary care center. Patient: A 22-year-old woman suddenly develo ped dystonic spasms in her left hand and left foot after a severe emotional stress. The dyskinesia gradually subsided over the next 4 months. Five mon ths after the onset, she suddenly developed choreoathetoid movement in her right hand and right foot. Main Outcome and Results: The patient had both somatic and cortical sensory deficits in the right hand and right foot, Magnetic resonance imaging of t he brain showed an infarction at the right putamen and lesions involving th e right frontal lobe and the left frontotemporoparietal lobe. Magnetic reso nance cerebral angiography showed severe stenoses of both internal carotid arteries at the supraclinoid portion and numerous collateral vessels, compa tible with moyamoya disease. Single photon emission tomography of the brain showed hypoperfused areas at the right frontal and left frontotemporoparie tal lobes. The choreoathetosis of the right limbs improved markedly, along with improvement of sensory deficits. Conclusions: To our knowledge, this is the first report of an adult patient presenting with hemidystonia and hemichoreoathetosis as the initial manife stations of moyamoya disease.