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肌萎缩侧索硬化症的预后因素:针对同一问题的不同方法。

Prognostic factors in ALS: different approaches to the same problem.

作者信息

Vázquez Maria Cristina, Perna Abayubá, Legnani Mariana, Saona Gustavo

机构信息

Universidad de la República, Hospital de Clínicas, Departamento de Neurología, Montevideo, Uruguay.

Investigator independiente, Montevideo, Uruguay.

出版信息

Arq Neuropsiquiatr. 2025 Jun;83(6):1-7. doi: 10.1055/s-0045-1809407. Epub 2025 Jun 20.

Abstract

The natural history of amyotrophic lateral sclerosis (ALS), the prognoses, and the survival times are fields of considerable interest that are scarcely studied in South American countries.To describe the survival of a representative cohort of Uruguayan ALS patients, and to identify covariates associated with survival using different analyses.Survival was assessed using the Kaplan-Meier method. Different Cox proportional hazards functions were used to identify independent prognostic predictors since the diagnosis: classic, stratified, and truncated.We included 166 definite and probable ALS patients. The median follow-up was of 13.6 years. An analysis was performed according to the recruitment groups: prevalent, exhaustive incident, and non-exhaustive incident cases. The median survival since the diagnosis was longer in the prevalent group (33 months) than in the exhaustive incident (22 months) and non-exhaustive incident (14 months) groups. The median survival time of the entire cohort from onset to death was 37 months and 23 months from the diagnosis. Factors related to survival from diagnosis to death were: age at onset, bulbar region onset, clinical form, and progression rate.The present study described the role of clinical and demographic factors in ALS survival in the Uruguayan population and shed light on differences involving survival models and the temporal bias produced by the lack of precision in determining the onset of the disease.

摘要

肌萎缩侧索硬化症(ALS)的自然病史、预后及生存时间是南美国家几乎未进行研究的、备受关注的领域。描述乌拉圭ALS患者代表性队列的生存情况,并通过不同分析确定与生存相关的协变量。采用Kaplan-Meier方法评估生存情况。自诊断起,使用不同的Cox比例风险函数来识别独立的预后预测因素:经典、分层和截断。我们纳入了166例确诊和可能的ALS患者。中位随访时间为13.6年。根据招募组进行分析:现患、详尽发病和非详尽发病病例。自诊断起,现患组的中位生存时间(33个月)长于详尽发病组(22个月)和非详尽发病组(14个月)。整个队列从发病到死亡的中位生存时间为37个月,从诊断到死亡为23个月。从诊断到死亡与生存相关的因素有:发病年龄、延髓区域发病、临床类型和进展速度。本研究描述了临床和人口统计学因素在乌拉圭人群ALS生存中的作用,并阐明了生存模型差异以及疾病发病时间确定缺乏精确性所产生的时间偏差。

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