Early symptom progression rate is related to ALS outcome -: A prospective population-based study
Early symptom progression rate is related to ALS outcome -: A prospective population-based study
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DOI：
10.1212/wnl.59.1.99
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发表时间：
2002-07-09
期刊：
影响因子：
9.9
通讯作者：
Mutani, R
中科院分区：
文献类型：
--
作者：
Chiò, A;Mora, G;Mutani, R
目的：在一项基于人群的前瞻性调查中确定与 ALS 结局相关的因素。方法：1995 年至 1996 年间皮埃蒙特和瓦莱达奥斯塔 ALS 登记册中列出的 221 名患者（120 名男性和 101 名女性）被纳入本研究。诊断后使用标准评估表对患者进行前瞻性监测。结果：平均发病年龄为 62.8 (SD = 11.2) 岁。根据 El Escorial 诊断标准 (EEDC)，112 名患者确诊为 ALS，85 名可能患有 ALS，18 名可能患有 ALS，6 名疑似 ALS。从症状出现起的中位生存时间为 915 天（95% CI = 790 至 1065）。诊断后的中位生存时间为 580 天（95% CI = 490 至 670）。在单变量分析中，结果与年龄、发病部位、EEDC 分类和症状进展率（即诊断后 6 个月内肌力、延髓和呼吸功能的下降率）显着相关。在 Cox 多变量模型中，年龄、呼吸、延髓和下肢症状的进展率、EEDC 分类、经皮内窥镜胃造口术和利鲁唑治疗与结果显着相关。结论：早期 ALS 症状进展速度可预测疾病结果。
Objective: To define the factors related to ALS outcome in a population-based, prospective survey. Methods: The 221 patients (120 men and 101 women) listed in the Piemonte and Valle d'Aosta ALS Register between 1995 and 1996 were enrolled in the study. The patients were prospectively monitored with a standard evaluation form after diagnosis. Results: Mean age at onset was 62.8 (SD = 11.2) years. According to El Escorial diagnostic criteria (EEDC), 112 patients had definite ALS, 85 probable ALS, 18 possible ALS, and six suspected ALS. The median survival time from symptom onset was 915 days (95% CI = 790 to 1065). The median survival time from diagnosis was 580 days (95% CI = 490 to 670). In univariate analysis, outcome was significantly related to age, onset site, EEDC classification, and symptom progression rate (i.e., the rate of decline of muscle strength and bulbar and respiratory function in the 6 months after diagnosis). In the Cox multivariate model, age, progression rate of respiratory, bulbar, and lower limb symptoms, EEDC classification, percutaneous endoscopic gastrostomy, and treatment with riluzole were significantly related to outcome. Conclusions: The rate of progression of symptoms in early ALS is predictive of disease outcome.