FAMILIAL CEREBRAL CAVERNOUS ANGIOMATOSIS

Two patient with familial cavernous angiomatosis presenting with long lasting variable epilepsy with a poor therapeutic response and variabl e neurologic impairments are presented here. One of the numerous caver nous angiomas was resected in one case. This last patient remains asym ptomatic. Familial cerebral cavernous angiomas are often numerous and disseminated in the brain, therefore clinical manifestations are very polymorphous. Moreover the course of these lesions is variable. Theref ore MRI should be performed to every patient presenting with poorly un derstood neurological symptoms, focal or generalized epileptic seizure s or absence in order to look for potentially imputable brain lesions. A reliable genetic marker might be helpful for diagnosis of this dise ase with a variable penetrance and autosomal dominant inheritance. The n a neurosurgical treatment should be carefully discussed if lesions a re accessible and medications are poorly efficient with recurrent neur ologic impairments or epilepsy.