Prognosis in amyotrophic lateral sclerosis - A population-based study
Prognosis in amyotrophic lateral sclerosis - A population-based study
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DOI：
10.1212/01.wnl.0000049472.47709.3b
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发表时间：
2003-03-11
期刊：
影响因子：
9.9
通讯作者：
van Belle, G
中科院分区：
文献类型：
--
作者：
del Aguila, MA;Longstreth, WT;van Belle, G
背景：ALS预后的准确信息对患者、家属和临床医生都是有用的。研究方法：在华盛顿西部的一项基于人群的ALS研究中，作者在1990年至1994年期间收集了180例ALS患者。在面对面访谈中收集了潜在预后因素的信息。患者还完成了医学结局研究简表36（SF-36）。已知所有患者截至1999年12月的生命状态。结果：中位生存期为32个月的症状发作和19个月的诊断。确诊后5年生存率为7%。年龄较大和女性与生存率低密切相关。在多变量考克斯比例风险回归模型中，与预后不良显著独立相关的因素包括年龄较大、发病时的任何延髓特征、从症状发作到诊断的时间较短、缺乏配偶和居住在金郡。递归分割确定年龄，从症状发作到诊断的时间，和婚姻状况作为生存的最强预测因子。SF-36中身体健康的良好综合评分与较长的生存期显著相关，但心理健康的评分与较低评分无关。结论：这些发现与其他以人群为基础的ALS研究一致，并证实了其恶性本质。年龄较大、女性、发病时的任何延髓特征、从症状发作到诊断的短时间、缺乏配偶和疾病严重程度是关键的预后因素。对严重程度的连续测量可能会改善预测。
Background: Accurate information on prognosis of ALS is useful to patients, families, and clinicians. Methods: In a population-based study of ALS in western Washington, the authors assembled a cohort of 180 patients with incident ALS between 1990 and 1994. Information on potential prognostic factors was collected during an in-person interview. Patients also completed the Medical Outcomes Study Short Form 36 (SF-36). Vital status through December 1999 was known for all patients. Results: Median survival was 32 months from onset of symptoms and 19 months from diagnosis. The 5-year survival after diagnosis was 7%. Older age and female sex were strongly associated with poor survival. In multivariable Cox proportional hazards regression models, factors significantly and independently associated with a worse prognosis included older age, any bulbar features at onset, shorter time from symptom onset to diagnosis, lack of a marital partner, and residence in King County. Recursive partitioning identified age, time from symptom onset to diagnosis, and marital status as the strongest predictors of survival. Good summary scores for physical health on the SF-36, but not for mental health, were significantly associated with longer survival than poor scores. Conclusions: These findings are consistent with other population-based studies of ALS and confirm its pernicious nature. Older age, female sex, any bulbar features at onset, short time from symptom onset to diagnosis, lack of a marital partner, and disease severity are key prognostic factors. Serial measurement of severity would likely improve predictions.