Moyamoya Disease in Taiwan

Abstract

“Moyamoya”, a word indicating a puff of smoke, names a disease showing stenosis or occlusion of the intracranial arteries at the base of skull and abnormal vascular networks (intraparenchymatous anastomosis) in the region of the basal ganglia. It was first described, on angiography, by Nomura [5] and Takeuchi [8]. A variety of synonyms have been suggested, including “cerebral juxtabasilar telengiectasis,” “cerebral arterial rete,” “rete mirabile,” and “cerebral arterial rete mirabile.” Previously considered to be confined to Japan, moyamoya disease has recently been reported sporadically all over the world [2–4, 6]. In Taiwan, this rare disease is occasionally uncovered at large neurological centers. However, the epidermiology of the disease is, as yet, unclear. In the present joint study, 26 cases were retrospectively assesed in order to delineate the general aspects of this disease in Taiwan.