Disease with abnormal intracranial vascular network complicated with intracerebral haematoma

SYNOPSIS A case of so-called Japanese cerebrovascular disease in association with intracerebral haematoma is presented. The patient had had no neurological symptoms before the onset, in spite of an extensive intracranial occlusive vascular process. Diagnosis was established preoperatively and massive haematoma was surgically evacuated from the left frontal lobe. The postoperative course was uneventful and residual neurological deficit was minimal. Japanese neurosurgeons have recently described a new cerebrovascular syndrome in which cerebral angiography demonstrates certain characteristic features. This syndrome comprises (1) narrowing or occlusion of both internal carotid arteries at the level of the supraclinoid segment, and (2) a fine angiomatous vascular network at the base of the brain. Since the first report of this unusual cerebro-vascular pattern, by Kudo in 1968, similar cases have been reported throughout Japan. In a symposium held during the 18th general assembly of the Japan Medical Association 1971 researchers presented a total of 376 cases of this syndrome occurring in Japan alone (Kudo, 1971). In contrast with the rapid accumulation of reported cases in Japan, only a few reports have appeared in Western medical literature. As far as we know, only 25 cases have been reported outside Japan (Weidner et al., 1965; Simon et al.