A Moyamoya disease in an 8-year-old german boy is reported. Medical history revealed attacks of hemiparesis and aphasia. The cerebral angiogram showed a typical, bilateral stenosis of the internal carotid arteries, collateral vessels as well as an occlusion of the basilar artery. Etiology, clinical findings, diagnostic procedures, differential diagnosis, therapy and prognosis of this rare disease is discussed on the basis of literature.
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