Background

Improved knowledge of the prediagnostic phase of progressive supranuclear palsy (PSP) might provide information on when and how the disease starts, along with the opportunity to test therapies in disease stages with lesser neurodegeneration.

Objective

To explore the symptoms in years preceding the PSP diagnosis.

Methods

This is a single-center retrospective case-control study based on clinical charts review and a structured interview to PSP patients and their caregivers. Prediagnostic symptoms were defined as those present more than one year before the diagnosis. We explored 35 symptoms in the following domains: visual, dizziness, motor, mood/apathy, cognitive, behavioral, sleep, gastrointestinal/urinary and miscellaneous. Non-parametric statistics were applied, with significance set at <0.05 (FDR-corrected).

Results

We included 150 subjects: 50 PSP patients (38% females, age 75.8) and an age- and sex-matched control group of 50 Parkinson's disease (PD) and 50 subjects (CS) without neurodegenerative disease. The frequencies of visual, motor, cognitive, behaviour and dizziness domains were significantly higher in PSP vs. PD, and so were the motor, mood/apathy, cognitive, behaviour and dizziness ones in PSP vs. CS. Over 50% of prediagnostic falls, apathy and anxiety, and over 30% of gait disturbances and depression started more than three and up to ten years before the diagnosis. PSP patients had more consultations to ENT and ophthalmologists than PD patients.

Conclusion

PSP patients present a broad variety of motor and non-motor symptoms several years before the diagnosis. The definition of a prediagnostic PSP phase might be helpful to identify patients in early disease stages.

中文翻译：

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进展性核上性麻痹的诊断前运动和非运动症状：逐步PSP研究。

背景

对渐进性核上性麻痹（PSP）的诊断前阶段的了解增加，可能会提供有关疾病何时和如何开始的信息，以及在神经变性程度较小的疾病阶段测试治疗方法的机会。

目的

在PSP诊断之前的几年中探索症状。

方法

这是一项基于临床图表审查和对PSP患者及其护理人员进行的结构化访谈的单中心回顾性病例对照研究。诊断前症状定义为诊断前一年以上出现的症状。我们研究了以下领域的35种症状：视觉，头晕，运动，情绪/平静，认知，行为，睡眠，胃肠道/泌尿和其他。应用非参数统计，显着性设置为<0.05（经FDR校正）。

结果

我们纳入了150名受试者：50名PSP患者（女性占38％，年龄75.8岁）以及年龄和性别匹配的对照组，分别为50名帕金森氏病（PD）和50名无神经退行性疾病的受试者（CS）。PPD相对于PD的视觉，运动，认知，行为和头晕域的频率显着更高，PSP相对于CS的运动，情绪/平静，认知，行为和头晕的频率也更高。超过50％的诊断前跌倒，冷漠和焦虑，以及超过30％的步态障碍和抑郁症在诊断之前的三年到十年内开始出现。PPD患者向耳鼻喉科和眼科医生的咨询要多于PD患者。

结论

PSP患者在诊断前数年会出现各种各样的运动和非运动症状。PSP诊断前阶段的定义可能有助于识别疾病早期阶段的患者。