Leone M, Rocca W A, Rosso M G, Mantel N, Schoenberg B S, Schiffer D
Clinica Neurologica II, University of Torino, Italy.
Neurology. 1988 Sep;38(9):1433-8. doi: 10.1212/wnl.38.9.1433.
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.
本研究是对弗里德赖希共济失调（FD）患者生存情况的第一项基于人群的研究。确定了1945年至1984年间在意大利西北部特定地区居民中诊断出的所有FD病例（N = 58）。对这些患者进行随访直至死亡或至1984年12月31日（以先到者为准），以确定生存模式。10年、20年和30年生存率分别为96%、80%和61%，提示预后比先前报道的要好。FD患者的生存情况比一般人群预期的要差。即使在调整预期生存情况后，男性的生存率仍低于女性。发病年龄不是一个显著的预后因素。1960年或之后诊断出的患者的生存率高于1960年之前诊断出的患者；然而，差异无统计学意义。
