脳内海綿状血管腫の家族発生—兄弟例の報告

Familial Occurrence of Intracerebral Cavernous Angioma:Report of cases in brothers

I．はじめに

　脳内海綿状血管腫の家族発生例の症例報告は未だ少ない．当施設では，13年前，3歳時の弟を本疾患で手術し，最近，1歳違いの兄を同疾患で手術するという経験をしたので，文献的考察を加えて報告する．

Familial occurrence of intracerebral cavernous angioma has been rerely reported. We report two histo-logically verified cases of cavernous angioma among brothers and review relevant cases in the literature.

Case 1 is that of a 3-year-old boy who suffered from acute onset of headache, vomiting, and tonic-clonic type seizure. CT revealed a well-demarcated tumor with par-tial hemorrhage in the left frontal lobe which was strongly enhanced with contrast medium. Complete ex-cision was carried out and the patient had a satisfactory clinial course and was able to be followed up for 13 years after the surgery. Case 2 is that of a 17-year-old boy who was the elder brother of case 1 and presented with gradually increasing episodes of a pyschomotor seizure which started at the age of 16.

CT and MRI revealed a well-demarcated tumor in the left subcortical temporal lobe and an asymptomatic small calcified lesion in the left subcortical parietal lobe. The temporal tumor was totally excised and histo-logically diagnosed as cavernous angioma. The seizures gradually decreased and eventually disappeared one year after the surgery.

This report reviews 13 previously reported cases and surgical indication for asymptomatic cases.