TUBEROUS sclerosis was first recognized as a pathological entity by Bourneville1 who 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.2 In 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.3,4 Patients 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
PEROT P, WEIR B, RASMUSSEN T. Tuberous SclerosisSurgical Therapy for Seizures. Arch Neurol. 1966;15(5):498–506. doi:10.1001/archneur.1966.00470170052005
Customize your JAMA Network experience by selecting one or more topics from the list below.