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Article
June 12, 1943

FAMILIAL TUBEROUS SCLEROSIS (EPILOIA) WITHOUT ADENOMA SEBACEUM: REPORT OF TWO CASES

Author Affiliations

MEDICAL CORPS, ARMY OF THE UNITED STATES; Chief Radiologist Jewish Hospital PHILADELPHIA

From the Jewish Hospital.

JAMA. 1943;122(7):429-432. doi:10.1001/jama.1943.02840240019007
Abstract

Tuberous sclerosis (epiloia, Bourneville's disease) is considered a rare and unusual entity. For this reason its presence as a diagnostic possibility in cases in which epileptiform seizures are present is seldom entertained. Yet the literature on this subject has assumed impressive proportions. In the sixty years from the time Bourneville1 first described it in 1880 down to 1940 one hundred and twelve reports have been collected dealing with tuberous sclerosis and its related neurocutaneous syndromes (Recklinghausen's neurofibromatosis, angiomatosis cerebri or Sturge-Weber's disease, and von Hippel-Lindau's disease).

In recent years the roentgenologist has become more alert to the significance of metastatic calcifications (calcified plaques) in survey films of the skull as an identifying feature of tuberous sclerosis, and the internist has in turn been stimulated to search for its other characteristic manifestations. With the increasing use of pneumoencephalography, this type of ectodermosis is being more commonly reported, its features are

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