Pereira Marta F, Buchanan Tim, Höglinger Günter U, Bogdanovic Marko, Tofaris George, Prangnell Simon, Sarangmat Nagaraja, FitzGerald James J, Antoniades Chrystalina A
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
UCB Biopharma S.R.L, Brussels, UK.
BMJ Neurol Open. 2022 Jan 21;4(1):e000214. doi: 10.1136/bmjno-2021-000214. eCollection 2022.
Progressive supranuclear palsy (PSP) is a rare neurodegenerative condition characterised by a range of motor and cognitive symptoms. Very little is known about the longitudinal change in these symptoms over time. Moreover, the effectiveness of clinical scales to detect early changes in PSP is still a matter of debate.
We aimed to determine longitudinal changes in PSP features using multiple closely spaced follow-up time points over a period of 2 years. 28 healthy control and 28 PSP participants, with average time since onset of symptoms of 1.9 years, were prospectively studied every 3 months for up to 24 months. Changes from baseline scores were calculated at each follow-up time point using multiple clinical scales to identify longitudinal progression of motor and cognitive symptoms.
The Montreal Cognitive Assessment, but not the Mini-Mental State Examination, detected cognitive decline at baseline. Both scales revealed poor longitudinal sensitivity to clinical change in global cognitive symptoms. Conversely, the Movement Disorders Society Unified Parkinson's disease Rating Scale - part III and the PSP Rating Scale (PSPRS) reliably detected motor decline less than 2 years after disease onset. The 'Gait/Midline' PSPRS subscore consistently declined over time, with the earliest change being observed 6 months after baseline assessment.
While better cognitive screening tools are still needed to monitor cognitive decline in PSP, motor decline is consistently captured by clinical rating scales. These results support the inclusion of multiple follow-up time points in longitudinal studies in the early stages of PSP.
进行性核上性麻痹（PSP）是一种罕见的神经退行性疾病，其特征为一系列运动和认知症状。对于这些症状随时间的纵向变化知之甚少。此外，临床量表检测PSP早期变化的有效性仍存在争议。
我们旨在通过在2年时间内使用多个紧密间隔的随访时间点来确定PSP特征的纵向变化。对28名健康对照者和28名PSP患者进行前瞻性研究，这些患者症状出现后的平均时间为1.9年，每3个月进行一次研究，最长持续24个月。在每个随访时间点，使用多个临床量表计算相对于基线分数的变化，以确定运动和认知症状的纵向进展。
蒙特利尔认知评估量表（Montreal Cognitive Assessment）而非简易精神状态检查表（Mini-Mental State Examination）在基线时检测到认知功能下降。两种量表对整体认知症状的临床变化均显示出较差的纵向敏感性。相反，运动障碍协会统一帕金森病评定量表第三部分（Movement Disorders Society Unified Parkinson's disease Rating Scale - part III）和PSP评定量表（PSPRS）在疾病发作后不到2年时可靠地检测到了运动功能下降。“步态/中线”PSPRS子评分随时间持续下降，最早在基线评估后6个月观察到变化。
虽然仍需要更好的认知筛查工具来监测PSP中的认知功能下降，但临床评定量表能够持续捕捉到运动功能下降。这些结果支持在PSP早期阶段的纵向研究中纳入多个随访时间点。
