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Friedreich's disease

Survival analysis in an Italian population

^*Deceased.
Clinica Neurologies II of the University of Torino (Drs. Leone, Rosso, and Schiffer), Torino, Italy; and the Neuroepidemiology Branch, Intramural Research Program (Drs. Leone, Rocca, and Schoenberg), National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health; and the Department of Mathematics, Statistics and Computer Science (Mr. Mantel), The American University, Bethesda, MD.

This investigation is the first population-based study of survival in Friedreich's disease (FD). All cases of FD diagnosed between 1945 through 1984 among residents of a defined area of northwestern Italy were ascertained (N = 58). These patients were followed to death or to December 31, 1984 (whichever came first) to determine the patterns of survival. The 10-, 20-, and 30-year survival rates were respectively 96%, 80%, and 61%, suggesting a better prognosis than previously reported. Survival of FD patients was poorer than expected from the general population. Survival for males was poorer than for females even after adjustment for expected survival. Age of onset was not a significant prognostic factor. Survival for patients diagnosed in 1960 or later was better than for those diagnosed before 1960; however, the difference was not statistically significant.

Address correspondence and reprint requests to Dr. Leone, Clinica Neurologies II, Via Cherasco 15, 10126-Torino, Italy.

Supported by the Torino chapter of the Italian Association Against the Ataxic Syndromes.

Presented in part at the thirty-ninth annual meeting of the American Academy of Neurology, New York, NY, April 1987.

Received September 17, 1987. Accepted for publication in final form February 19, 1988.