[A case of familial cerebral cavernous angioma and review of Japanese cases].
We present one pedigree of familial cerebral cavernous angioma (FCCA). Case 1 was a 52-year-old male with right hemiplegia. When he was 37 years old, a left occipital lesion was excised and histologically diagnosed as cavernous angioma. MR image showed many cavernous angiomas in the right temporal lobe, the right paraventriclar white matter, the right frontal lobe, the left basal ganglia, and the left parietal lobe. Stereotactic radiosurgery was undertaken for all the lesions. Although the size of each lesion was unchanged, neither hemorrhage nor neurological deterioration were recognized after radiosurgery. Case 2 was a 24-year-old male, a son of the patient in case 1. He has manifested tonic-clonic type epilepsy since the age of 2. MR image showed cavernous angiomas in the pons, the right frontal, and the left intra-Sylvian regions, and many paraventricular cysts with rims indication of previous hemorrhages. Two de novo lesions were observed on subsequent annual MR screening. Surgical excision for the left intra-Sylvian lesion and stereotactic radiosurgery for all lesions were undertaken. Histological diagnosis was cavernous angioma. In the literature, there were 17 pedigrees and 37 cases of FCCA in Japan. The incidence of both multiple lesions and hemorrhage were less than in found in Spanish or French cases. Stereotactic radiosurgery is considered an useful treatment for FCCA, because lesions are multiple and de novo lesions occur.
Sunada
I
I
Department of Neurosurgery, Saiseikai Ibaraki Hospital, 1-1-24 Mitsukeyama, Ibaraki 567-0035, Japan.
Nakabayashi
H
H
Tsuchida
K
K
Tanaka
A
A
D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis / Q000235:genetics*; D006392:Hemangioma, Cavernous / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010375:Pedigree