Neurological manifestations of Tuberous Sclerosis of Bourneville: study of 9 observations

Abstract

The Tuberous Sclerosis of Bourneville (TSB) is a phacomatosis characterized by the formation of multi-systematic hamartomas. The symptoms are consistent with the location of these hamartomas in the nervous system, skin, heart, and the eyes. According to retrospective study of the observations of nine infants affected by the TSB, we examined the clinical and paraclinical characteristics of the disease's neurological signs. We gathered four boys and five girls. The average age of diagnosis is 22 months. The epilepsy, usually pharmacoresistant, was the disclosed sign of the disease amongst all the cases. Mental retardation has been nearly constant in this study (8/9 cases). The autistic features have been noticeable in 5 cases.