[Familial occurrence of intracerebral cavernous angioma]

This article reported a familial occurrence of intracerebral cavernous angioma in four members of one generation diagnosed by X-ray CT, MRI or operative specimen. Case 1, a 34-year-old female, was examined just after an episode of sudden convulsive seizure. On examination, she had a cutaneous angioma without any neurological deficit. X-ray CT revealed a high density mass lesion in the left frontal lobe, and MRI demonstrated a mass lesion in the chronic stage with an old hematoma circumscribed by hypointensity ring indicating peripheral hemosiderosis. Complete excision was carried out and a diagnosis of cavernous angioma was made after histological examination. Case 2, the 37-year-old brother of Case 1, suddenly developed left hemiparesis and hypesthesia with severe headache. X-ray CT revealed a high density mass in the right parietal lobe and two other calcifications. The right parietal lesion was excised and a histopathological diagnosis of cavernous angioma with intracerebral hematoma was made. Case 3, the 49-year-old sister of Case 1, suddenly fell into a coma and was admitted immediately. X-ray CT revealed a large pontine hemorrhage. She died on the 4th day of hospitalization without operative treatment. Necropsy was not carried out. Case 4, the 39-year-old sister of Case 1, was asymptomatic, however, she was examined on the supposition of a familial occurrence of intracerebral cavernous angioma. On examination, it was found she had multiple cavernous angioma without any neurological deficit. X-ray CT revealed parietal intracerebral calcification. MRI demonstrated a mass lesion with peripheral hypointensity ring in the right parietal lobe, and another small lesion in the pons.(ABSTRACT TRUNCATED AT 250 WORDS)