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December 1978

Tuberous Sclerosis

Author Affiliations

Department of Pediatrics and Neurology; Department of Pediatrics Boston City Hospital Boston University School of Medicine 818 Harrison Ave Boston, MA 02118

Am J Dis Child. 1978;132(12):1215-1216. doi:10.1001/archpedi.1978.02120370067017

True precocious puberty may be idiopathic in nature or may result from congenital or acquired CNS dysfunction.1 Tuberous sclerosis has been included among the neurogenic causes of precocious sexual development since Krabbe2 reported the first two cases with this association in 1922. Since then, several more such patients have been described.3-8 We describe a patient with tuberous sclerosis who had symptoms of gynecomastia and sexual precocity. The literature is reviewed, and the importance of considering the diagnosis of this dominantly inherited disorder in children with precocious puberty is emphasized.

Report of a Case.—This boy was the product of an uncomplicated 37-week gestation. Psychomotor development was normal. At about age 10 years, the patient was noted to have advanced sexual development and enlarged breasts. His medical history was unremarkable; he had had no seizures and no exposure to exogenous hormones or drugs. The family history showed that

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