[Systemic vascular change associated with moyamoya-like cerebrovascular disease and microvascular coronary artery...

A 34-year-old man with hypertension and diabetes mellitus developed dizziness and visited our institute. He had history of headache with numbness of the right hand since age 15 years and left occipital lobe infarction at age 28 years. The cerebral angiogram showed several changes peculiar to advanced stage of moyamoya disease (spontaneous occlusion of the circle of Willis), i.e. segmental stenoses or occlusions of bilateral internal carotid arteries, left vertebral artery and left posterior cerebral artery with abnormal vascular networks at the bilateral basal ganglia. He was also diagnosed to have asymptomatic ischemic heart disease. The coronary angiogram showed diffuse sclerotic lesions of left anterior descending and right coronary arteries without significant stenosis, which suggested the presence of microvascular lesion as a cause of myocardial ischemia. Coronary disease has been rarely reported as a complication of moyamoya disease, and microvascular coronary artery disease has never been described. Moyamoya disease should be regarded as a part of systemic vascular disorders, and the evaluation of extracerebral cardiovascular system is necessary to clarify pathophysiology of this disease.