进行性核上性麻痹的自然病史和生存预测因素。
Natural history and predictors of survival in progressive supranuclear palsy.
机构信息
Neurology Unit, Brescia Hospital, Brescia, Italy.
Neurology Unit, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
出版信息
J Neurol Sci. 2017 Nov 15;382:105-107. doi: 10.1016/j.jns.2017.09.043. Epub 2017 Sep 30.
BACKGROUND
Progressive supranuclear palsy is a neurodegenerative disorder characterized by high functional disability and rapidly progressive dependency. The predictors of survival are still unclear.
METHODS
The predictors of survival were evaluated in a group of clinically diagnosed PSP patients, focusing primarily on extensive cognitive assessment.
RESULTS
The mean survival time from symptom onset was 8.25±3.0years. Sex, age at onset, education, occupation and severity of extrapyramidal symptoms did not correlate with survival. The only factor associated with a shorter life expectancy in our cohort was the presence of dementia at diagnosis. Impairment of executive functions was the best predictor of an unfavorable outcome.
CONCLUSIONS
Our findings suggest that dementia and executive functions need to be evaluated in order to define survival probability in PSP patients.
背景
进行性核上性麻痹是一种神经退行性疾病，其特点是高功能障碍和快速进展的依赖性。生存的预测因素仍不清楚。
方法
对一组临床诊断为 PSP 的患者进行了生存预测因素的评估，主要侧重于广泛的认知评估。
结果
从症状出现到平均生存时间为 8.25±3.0 年。性别、发病年龄、教育程度、职业以及锥体外系症状的严重程度与生存无关。在我们的队列中，唯一与预期寿命较短相关的因素是诊断时存在痴呆。执行功能障碍是预后不良的最佳预测因素。
结论
我们的研究结果表明，需要评估痴呆和执行功能，以确定 PSP 患者的生存概率。
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