Familial Cerebral Cavernous Angiomas

Abstract

Article abstract-Cavernous angiomas are well-circumscribed cerebrovascular malformations whose natural history is poorly understood. We reviewed 5,000 cranial MRI reports of studies performed between 1986 and 1993 and retrospectively evaluated the histories and imaging studies of 29 patients whose lesions were suggestive of cavernous angiomas. Patients ranged from 3 to 66 years and 27 were of Hispanic origin. The number of malformations per patient ranged from one to 30, and 24 patients had more than one lesion. In our series, the number of lesions per patient increased at a rate of one lesion per decade of age, but the mean size of the lesions was smaller with advancing decade (p < 0.05). All patients had MRI evidence of old or recent hemorrhage confined only to the malformation and adjacent brain. In only one patient was it deemed necessary to remove the malformation. Two excluded patients with typical lesions on MRI had small arteriovenous malformations found at surgery. Thus, MRI or CT cannot identify cavernous angiomas with certainty. This study supports studies that propose that cavernous angioma-like lesions, as identified by MRI or CT, are more common in Hispanics. It is possible that patients may not be born with MRI-identifiable malformations, but with advancing age the malformations become MRI-visible through malformation growth, hemorrhage, or both. The declining size by decade favors hemorrhage rather than growth. Cavernous angiomas may be more benign than previously thought.NEUROLOGY 1995;45: 492-497