家族性に発生した多発性脳血管奇形

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Two multiple vascular malformation cases of the brain were reported. Case 1, a 32-year-old male, who had had syncopal attacks at the age of 28, suddenly developed left hemiparesis and left hypesthesia. The CT scan demonstrated a right parietal intracerebral hematoma and seven small calcifications. Right carotid angiography demonstrated no abnormal findings. The hematoma was evacuated and a small mass with high vascularity, which was histologically proven to be an arteriovenous malformation was excised. A largest calcified mass in the right temporal lobe was also excised. The histopathological diagnosis was cavernous angioma. There was no previously reported case of multiple cavernous angioma, associated with a cerebral arteriovenous malformation. Case 2, a sister of Case 1 was in good health until she was 23 years old, when she suddenly developed right hemiparesis and right hypesthesia. The CT scan demonstrated a left fronto-parietal intracerebral hematoma and six small calcifications similar to those in Case 1, Left carotid angiography showed no abnormal findings except for the mass signs. The liquefied hematoma was evacuated and a small mass in the wall of the cavity was excised. The histopathological diagnosis was venous angioma. This was considered to be a case in which multiple cavernous angiomas and a venous angioma coex-ist in the same brain. Familial occurrences of cavernous angiomas are rare. Six families with this disease have already been described in the literature. Awareness of the possibility of familial involve-ment may aid in the early diagnosis of cavernous angioma and other vascular malformations.
日本脳神経外科学会の論文
1984-04-15

家族性に発生した多発性脳血管奇形

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