Tuberous Sclerosis

Abstract
TUBEROUS sclerosis was first recognized as a pathological entity by Bourneville1who described the potato-like appearance of the sclerotic areas in the cerebral convolutions of a mentally defective epileptic patient. The syndrome of mental deficiency, epilepsy, and adenoma sebaceum was clearly defined by Vogt.2In general, a poor prognosis has been associated with the patients who have had a fully developed clinical picture, but in the last few decades it has become increasingly apparent that there are formes frustes of this disease, including patients with normal intelligence.3,4Patients may be encountered with any of the stigmata of the disease as isolated findings, or they may occur in various combinations. The disease is a heredofamilial one involving different tissue types in widespread areas of the body. Common sites include the brain, lung, kidney, spleen, bone, retina, and soft tissues. The indications for neurosurgical intervention in patients with this