Abnormal Cerebrovascular Network Related to the Internal Carotid Arteries

APANESE neurosurgeons have recently observed in patients of their own race a variety of neurological disorders which are often transient and appear most frequently in young people. The typical angiographic appearance is that of narrowing or occlusion of both internal carotid arteries at the level of the siphon (C-1) together with a network at the base of the (Fig. 1). These patients were initially reported as having a type of occlusive disease of the internal carotid artery. 1~ The entity has also been reported by other Japanese authors as occlusion of the circle of Willis, 3 telangiectasia, 1,11 or vascular malformation. ~,9 In 1964 we collected 21 case reports from the Japanese literature, added three of our own, r and concluded that the entity was unique to the Japanese. We reported the entity as hemangiomatous malformation of bilateral internal carotid arteries at the base of the brain and noted the similarity of the abnormal vasculature to the vascular network in the embryo. We noted, too, that the malformation was primarily limited to the distribution of the internal carotid arteries, s The term cerebral rete mirabile has some descriptive value although it is probably not entirely appropriate. The Western literature yielded only one similar case with bilateral involvement, and this patient was a Japanese woman. 1'~ Taveras and Wood ~ reported comparable cases in non-Japanese patients, but the occlusions were unilateral. Since our paper 6 in 1964, numerous other reports have appeared in the Japanese literature. Adding these to an additional number collected by writing all the neurosurgical clinics in Japan, we are now reporting a total of 96 cases.