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Hypoplasia and obstruction of the circle of Willis in a case of atypical cerebral hemorrhage and its relationship to Nishimoto's disease

A PARTICULAR DISEASE of the circle of Willis, characterized by its progressive obstruction and associated with different cerebral vascular malformations, has been described by Japanese authors Nishimoto and Sugiul and Kudo.2 They considered the disease unique to the population of Japan since they found no other case cited in the literature of other countries. Subsequently, however, Simon et aL3 and Busch4 described, in young Europeans, progressive obstruction of the circle of Willis associated with different cerebral vascular malformations, which they compared with Nishimoto’s disease-an entity which is as yet incompletely delineated since it has been described only clinically and angiographically and has not been checked anatomically. Recently, Smith et al.5 reported the anatomicoclinical study of a hypoplasia, involving the bilateral carotid artery and the anterior part of the circle of Willis, with occlusion phenomena due to intima proliferation and atheroma. The present paper reports on the anatomicoclinical study of a 44-year-old man, not suffering from chronic hypertension, who suddenly died from cerebral hemorrhage with an atypical location. Examination of the cerebral vessels revealed a series of lesions that raised the question of their relationship to Nishimoto’s disease.