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Article
April 1942

HAMARTIAL NATURE OF THE TUBEROUS SCLEROSIS COMPLEX AND ITS BEARING ON THE TUMOR PROBLEMREPORT OF A CASE WITH TUMOR ANOMALY OF THE KIDNEY AND ADENOMA SEBACEUM

Author Affiliations

NEW YORK

From the Pathological Laboratory, St. Peter's Hospital, New Brunswick, N. J.

Arch Intern Med (Chic). 1942;69(4):589-623. doi:10.1001/archinte.1942.00200160040005
Abstract

Tuberous sclerosis was first described clinically by Bourneville and Brissaud (1880 et seq)1 in feeble-minded children with epilepsy and progressive mental deterioration. This interesting condition has usually been classified among the diseases of the nervous system and has been given only casual attention by general clinicians. Since the term tuberous sclerosis refers primarily to the changes in the brain, the broader term tuberous sclerosis complex is therefore employed to denote the coexistence of homologous changes in other organs, notably the heart, kidneys and skin.

The clinical diagnosis is possible if two or more of the following features can be demonstrated: mental retardation, epilepsy, adenoma sebaceum, phacoma of the retina, multiple mixed tumors (hamartomas of the kidneys and a familial history of the disease. In some of the reported cases the neurologic features were insignificant or lacking clinically and were discovered first at autopsy.2 In still other cases the

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