Probably the earliest record of recognition of cerebral sclerosis associated with retinal tumor was described by von Recklinghausen1in 1863. At this time he described having, observed a large number of sclerotic areas in the brain of a newly born child and in the same case several myomas growing from the cardiac muscle. Hartdegen2in 1881 described a child 2 years old who died in convulsions ; autopsy revealed areas of sclerosis throughout the cerebral cortex, as well as a number of small tumors projecting into the lateral ventricles. Bourneville,3in a series of publications between 1881 and 1898, drew attention to a rare form of multiple cerebral sclerosis occurring in young patients who had shown during life mental deficiency and epilepsy. This condition he called tuberous sclerosis. In his subsequent reports covering the autopsies of ten patients he confirmed the association of grayish white subcapsular tumors