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良性多发性硬化症患者的长期分析:对残疾病程的新认识。

Long-term analysis of patients with benign multiple sclerosis: new insights about the disability course.

机构信息

Department of Neurology, Nancy University Hospital, Hôpital Central, service de neurologie, 29 avenue du Maréchal de Lattre de Tassigny, 54035, Nancy, France.

Université de Lorraine, EA 4360 APEMAC, 54500, Nancy, France.

出版信息

J Neurol. 2021 Oct;268(10):3817-3825. doi: 10.1007/s00415-021-10501-0. Epub 2021 Mar 31.

Abstract

OBJECTIVE

To describe the course of disability in patients with benign multiple sclerosis-i.e., with an expanded disability status scale score < 3 10 years after disease onset-for up to 30 years after disease onset. We evaluated the proportion of patients remaining in the benign state on the long term and the factor associated with this favorable outcome and determined the pattern of disability course after the loss of the benign status.

METHODS

Patients were selected from the ReLSEP, a French population-based registry. We studied the probability (Kaplan-Meier method) and predictors (multivariate Cox model) of remaining < 3 after year 10, and the course of disability after score 3 according to the duration of the benign phase in patients with ≥ 30 years of follow-up (graphs of the course of the mean expanded disability status scale scores in subgroups of patients).

RESULTS

2295/3440 patients had benign multiple sclerosis (66.7%). The probability of remaining benign at year 30 was 0.26 (95% CI 0.26-0.32). A young age at disease onset and a good recovery after the first relapse were associated with remaining benign. Graphs illustrate that those who lost their benign status between years 10 and 30 follow a two-stage course. Beyond score 3, disability accumulation is similar in all but lower disability scores at advanced age are associated with longer benign periods.

CONCLUSION

The longer a patient remains in the benign state, the lower the final EDSS at advanced age.

摘要

目的

描述发病 10 年后残疾状况扩展量表评分(EDSS)<3 的良性多发性硬化症患者的病程,最长可达发病后 30 年。我们评估了长期处于良性状态的患者比例、与这种有利结局相关的因素,并确定了失去良性状态后残疾进程的模式。

方法

患者选自法国基于人群的 ReLSEP 登记处。我们研究了发病 10 年后评分仍<3 的概率(Kaplan-Meier 法)及其预测因素(多变量 Cox 模型),以及在随访时间≥30 年的评分≥3 的患者中,良性期持续时间对残疾进程的影响(根据患者亚组的平均 EDSS 评分曲线图)。

结果

2295/3440 例患者为良性多发性硬化症(66.7%)。发病 30 年时仍保持良性的概率为 0.26(95%CI 0.26-0.32)。发病年龄较小和首次复发后恢复良好与保持良性相关。曲线图表明,在发病 10 年至 30 年之间失去良性状态的患者呈两阶段病程。评分>3 后,残疾累积在所有患者中相似,但在高龄时的较低残疾评分与较长的良性期相关。

结论

患者处于良性状态的时间越长,高龄时的最终 EDSS 越低。

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