[Familial occurrence of intracranial arteriovenous malformation].
It is beyond that cerebral arteriovenous malformation (AVM) is a congenital disease based on abnormal vascular formation in the embryonic stage. Nevertheless, familial occurrence of cerebral AVMs has been previously reported only in five families throughout the world, when complications of specific diseases such as Sturge-Weber-Dimitri disease, von Hippel-Lindaeu disease, and Osler-Rendu-Weber disease were ruled out. We have recently encountered four cases of two familial AVMs. In this paper, we review nineteen cases of nine familial AVMs from literature including our four cases from two families. Case 1: A 50-year-old male was admitted in June, 1975, for clonic convulsion. Cerebral angiography showed an AVM situated in the left parietal region, which was supplied from callosomarginal and central arteries. Total excision of the AVM was performed. The patient was completely cured and discharged without abnormal neurological deficits. Case 2: A 16-year-old male was admitted in May, 1978, with about ten years history of general convulsions. This patient was the third child of Case 1. Cerebral angiography revealed an AVM situated in the right parietal region, which was supplied from callosomarginal and precentral arteries. Total resection of the AVM was performed. The patient was completely cured. Case 3: An 11-year-old boy was admitted to our hospital in a comatose state in January, 1978, and soon died. Cerebral angiography showed only presence of internal hydrocephalus. The autopsy revealed a hematoma in the right cerebellar hemisphere with bilateral intraventricular hematomas, and AVM was histologically found in the cerebellum.(ABSTRACT TRUNCATED AT 250 WORDS)
D000328:Adult; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000235:genetics*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012737:Sex Factors