INTRODUCTION We investigated the disease progression rate in patients with progressive supranuclear palsy-Richardson syndrome (PSP-RS) and PSP-parkinsonism (PSP-P) in comparison with Parkinson disease (PD) patients, using MRPI (Magnetic Resonance Parkinsonism Index), and MRPI 2.0. METHODS Fifteen PSP-RS patients (disease duration, y, mean ± SD: 2.5 ± 1.1), 16 PSP-P patients (disease duration, y, mean ± SD: 6.5 ± 3.2) and 19 PD patients (disease duration, y, mean ± SD: 3.2 ± 2.3) were enrolled. All patients underwent clinical assessment and MRI at baseline, 1-year, and 2-year follow-up. MRPI, MRPI 2.0 and clinical scores over 1 and 2-years were used to evaluate disease progression rate, and to calculate sample sizes required to power placebo-controlled trials. RESULTS All groups showed increased clinical motor scores over time whereas only PSP groups had increased MRPI and MRPI 2.0 values over T1 and T2 intervals. The percentage increase over 1 and 2-years of MRPI and MRPI 2.0 values was significantly higher in PSP groups than in PD group, and in PSP-RS than in PSP-P patients while no difference between patient groups was observed when clinical motor scores were considered. Sample size estimates showed that MRPI 2.0 performed better than MRPI and clinical scales. Treatment trials with MRPI 2.0 could be performed over 2-years both in PSP-RS and PSP-P with a sample size per treatment arm of 89 and 170 patients, respectively. CONCLUSIONS Our results demonstrate that MRPI 2.0 was more powerful than MRPI and clinical motor scales in evaluating PSP progression, and in providing the best sample size estimates for clinical trials.

中文翻译：

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磁共振帕金森氏指数用于评估进行性核上性麻痹的疾病进展率：一项为期2年的纵向研究。

简介我们使用MRPI（磁共振帕金森病指数）调查了进行性核上性麻痹-理查森综合征（PSP-RS）和PSP-帕金森病（PSP-P）与帕金森病（PD）患者的疾病进展率MRPI 2.0。方法15例PSP-RS患者（疾病持续时间，y，平均±SD：2.5±1.1），16例PSP-P患者（疾病持续时间，y，平均±SD：6.5±3.2）和19个PD患者（疾病持续时间，y，平均±SD：3.2±2.3）。所有患者均在基线，1年和2年随访时接受临床评估和MRI。使用MRPI，MRPI 2.0和1年和2年以上的临床评分来评估疾病的进展率，并计算出支持安慰剂对照试验所需的样本量。结果所有组均显示随着时间的推移临床运动评分增加，而只有PSP组在T1和T2间隔内MRPI和MRPI 2.0值增加。PSP组在1年和2年中MRPI和MRPI 2.0值的增加百分比显着高于PD组，而PSP-RS则高于PSP-P患者，而当临床运动评分为考虑过的。样本量估计表明，MRPI 2.0的表现优于MRPI和临床量表。在PSP-RS和PSP-P中，可以使用MRPI 2.0进行为期2年的治疗试验，每个治疗组的样本量分别为89和170名患者。结论我们的结果表明MRPI 2.0在评估PSP进程方面比MRPI和临床运动量表更强大，