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September 1943

TUBEROUS SCLEROSIS

Author Affiliations

MEDICAL CORPS, ARMY OF THE UNITED STATES; CARO, MICH.

From the Department of Neuropsychiatry, Indiana University School of Medicine, and the Caro State Hospital for Epileptics, Caro, Mich., R. L. Dixon, M.D., Medical Superintendent.

Arch NeurPsych. 1943;50(3):233-257. doi:10.1001/archneurpsyc.1943.02290210011001
Abstract

A number of features of tuberous sclerosis of the brain make a study of the condition most engrossing—its relative infrequency, its clinical forms, its association with anomalies elsewhere, its roentgenographic peculiarities and its evolutionary problems. We have studied 25 cases of the disease, 3 with autopsy data, and shall discuss a few of these points.

In 1863 von Recklinghausen1 observed sclerotic areas in the brain and myomas in the heart of an infant. It was not until 1880, however, that Bourneville2 described the syndrome constituting tuberous sclerosis. He demonstrated sclerotic areas in the brain of an underdeveloped girl with epilepsy, idiocy, hemiplegia and "acne rosacea." In 1908 Vogt3 delineated the classic symptom triad of tuberous sclerosis, namely, idiocy, epilepsy and adenoma sebaceum. Since then many cases of "Bourneville's disease" have been reported and various theories advanced to explain its genesis. Sherlock4 suggested that the condition be

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