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June 1953


AMA Arch NeurPsych. 1953;69(6):669-683. doi:10.1001/archneurpsyc.1953.02320300002001

TUBEROUS sclerosis is a rare disease due to an abnormal development of the germinal layers primarily involving the ectodermal structures. Bourneville1 first described this disease in 1880. He referred to the skin eruption as "acne rosacea" and demonstrated sclerotic areas in the brain at autopsy. The commonest clinical findings are mental deficiency, convulsions, and adenoma sebaceum. This syndrome is referred to as epiloia.2

Marcus, cited by Dalsgaard-Nielsen,3 is credited as being the first, in 1924, to describe the roentgenologic changes in tuberous sclerosis. His findings were limited to the skull, and he spoke of layers of "chalk shale" and calcifications the size of a bean being found on roentgenological examination of the skull. Later, in 1935, Dalsgaard-Nielsen3 demonstrated that these calcifications were sclerotic plaques within the skull. Gottlieb and Lavine,4 in 1935; Kviem,5 in 1937; Pancoast, Pendergrass, and Schaeffer6; Hueblein, Pendergrass, and Widmann,