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Incidence and prevalence of tuberous sclerosis in Rochester, Minnesota, 1950 through 1982

Department of Medical Statistics and Epidemiology (Drs. Wiederholt and Kurland) and the Section of Pediatric Neurology (Dr. Gomez), Mayo Clinic and Mayo Foundation, Rochester, MN.

The incidence of tuberous sclerosis in Rochester, MN, was 0.56 per 100,000 person-years in 1950 through 1982, and point prevalence on December 31, 1980, was 10.6 per 100,000 persons. The true incidence and prevalence may be even higher, because cases with hypomelanotic macules and no other clinical manifestations are often unrecognized. Pertinent clinical observations in the eight cases identified included familial occurrence in two; hypomelanotic macules in all eight; seizures with onset before age 2 in four, of whom two were mentally retarded; and no neurologic abnormalities in those free of seizures.

Address correspondence and reprint requests to Dr. Kurland, Mayo Clinic, Rochester, MN 55905.

Supported in part by NIH Grants AM-30582 and NS-17750, Public Health Service. Dr. Wiederholt is a visiting scientist at the Mayo Clinic from the University of California at San Diego, supported by NIH Fellowship AG-05319.

Presented in part at the Tuberous Sclerosis Research Workshop at Massachusetts Institute of Technology, Boston, MA, April, 1984; sponsored by the National Institutes of Health and the Tuberous Sclerosis Assocation of America.

Accepted for publication July 23, 1984

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