Tuberous sclerosis in neurosurgery. An analysis of 18 patients

Eighteen patients with tuberous sclerosis have been analysed, treated between 1981 and 1987. The diagnosis was made on the clinical triad of adenoma sebaceum, mental retardation and epilepsy. In 6 patients a positive family history of the heredo-familial autosomal-dominant disease was found. The neuroradiological examinations showed in 4 patients tumours with obstruction of the ventricles. All 4 patients were operated on and in 2 additional cases only shunt procedures were performed. The diagnosis, neuroradiological investigations with CT scan and MRI, and the neurosurgical management will be discussed in the light of our material and by literature.