Moyamoya疾病：三病例報告

Moyamoya disease is a rare type of cerebrovascular occlusive disorder in childhood. It is characterized by progressive stenosis or occlusion of the supraclinoid portion of the internal carotid arteries, associated with basal telangiectasias that give the condition its name. The so-called ”Moyamoya” is a Japanese term for a hazy appearance just like a puff of cigarette smoke drifting in the air. This disease appears bilaterally n most cases, and occurs predominantly in Japanese children, with a higher incidnce in girls. This paper presents three cases of Chinese children, one boy and two girls with the age of 4, 5 and 6 years respectively. Sudden onset of repetitive and intermittent right side hemiplegia, associated with varied degree of motor aphasia are the predominant clinical features in all three cases. They are recognized as the rare Moyamoya diseases by the confirmation of carotid angiographies. The boy had received bilateral superficial artery-middle cerebral artery bypass (STA-MCA bypass) and encephalo-myo-synangiosis with clinical improvement and was followed up regularly.