Department of Neurology & Institute of Neurology, Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Department of Geriatrics, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
BMC Neurol. 2020 Mar 30;20(1):114. doi: 10.1186/s12883-020-01692-6.
Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aimed to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living.
All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, sex, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. Kaplan-Meier method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization.
Fifty-nine patients fulfilling MDS-PSP criteria were enrolled in our study. Nineteen patients (32.2%) had died and 31 patients (52.5%) were institutionalized by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio.
Older AAO and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.
进行性核上性麻痹（PSP）是一种预后较差的罕见运动障碍。本回顾性研究旨在描述 PSP 的自然病史，并寻找预测生存率较短和日常生活活动下降较快的指标。
所有入组的患者均符合运动障碍协会（MDS）PSP 的临床诊断标准（MDS-PSP 标准），用于可能和可能的 PSP，中位数为 12 年。收集的数据包括年龄、性别、发病日期、发病年龄（AAO）、首次就诊和随访时报告的症状、死亡日期和入院日期。在首次就诊时收集磁共振成像。终点为死亡和入院。使用 Kaplan-Meier 方法和 Cox 比例风险模型探讨与早期死亡和入院相关的因素。
本研究共纳入 59 例符合 MDS-PSP 标准的患者。19 例（32.2%）患者死亡，31 例（52.5%）患者在随访结束时入院。与生存率较差相关的预测因素为晚发性 PSP 和 M/P 面积比降低。与更早入院相关的预测因素为 AAO 年龄较大和 M/P 面积比降低。
AAO 年龄较大和 M/P 面积比降低是 PSP 早期匮乏和入院的预测因素。神经影像学生物标志物 M/P 面积比是 PSP 预后的预测指标。
