뇌경색을 수반한 Moyamoya Disease 1례

Abstract

Moyamoya disease, characterized by stenosis or occlusion of vessels in and around the circle of Willis in association with a telangiectatic networks of vessels emanating from the base of brain was first observed among the Japanese by Kudo in 1956. In 1969, Suzuki & Takaku introduced the cerebrovascular moyamoya disease as a clinical entity, which means characteristic cerebral angiographic features only in Japanese. But recently it is generally accepted that the disease is not confined only to the Japanese, but is distributed world widely. A 26-year-old nonhypertensive Korean man was admitted to the Department of Neurology with intractable headache associated with left hemiparesis, slight dysarthria, mental changes and left sided Babinski sign. Laboratory showed normal CSF findings. A transfemoral carotid angiography showed occlusion of suprac linoid portion of left carotid artery and of right anterior & middle cerebral arteries, with marked enlargement of left lenticulostriate artery and left anterior choroid artery. There was also a mass of telangiectatic networks of abnormal vessels in the base of brain. In vertebral angiography, spared posterior cerebral arteries caused retrograde fillings of anterior cerebral artery territory via posterior pericallosal arteries. Brain computerized axial tomogram showed the large areas of low density in right temporoparietal areas contiguous with occipital horn of lateral ventricle, and the enlarged ventricular systems supratentorially and prominent sulci, more marked in right cerebral hemisphere. But there was no midline shift or abnormal enhancement.