首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Clinical heterogeneity in progressive supranuclear palsy: problems of clinical diagnostic criteria of NINDS-SPSP in a retrospective study of seven Japanese autopsy cases.
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Clinical heterogeneity in progressive supranuclear palsy: problems of clinical diagnostic criteria of NINDS-SPSP in a retrospective study of seven Japanese autopsy cases.

机译:进行性核上性麻痹的临床异质性:在对7例日本尸检病例的回顾性研究中,NINDS-SPSP的临床诊断标准存在问题。

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摘要

Progressive supranuclear palsy (PSP) is known to display variable atypical clinical features. In the absence of clinical markers to diagnose PSP, neuropathological examination is the "gold standard" for diagnosis. We retrospectively investigated clinical features in seven autopsy-confirmed cases of PSP. Only three patients (42.9%) matched the clinical diagnostic criteria of PSP proposed by the National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) at the time of death. In addition, only one patient (14.3%) matched these criteria at the time of the initial symptoms. Such underdiagnosis of PSP was mainly caused by heterogeneity, variety of the timing, and presence of symptoms in exclusion criteria. The present study also demonstrated that the clinical features of PSP may change dramatically according to the disease stage. Target symptoms should be selected based on time and stage to optimize patient quality of life.
机译:进行性核上性麻痹(PSP)已知表现出各种非典型的临床特征。在缺乏诊断PSP的临床指标的情况下,神经病理学检查是诊断的“金标准”。我们回顾性调查了7例经尸检确认的PSP病例的临床特征。死亡时,只有三名患者(42.9%)符合美国国家神经疾病与中风研究所和PSP学会(NINDS-SPSP)提出的PSP临床诊断标准。此外,在最初出现症状时,只有一名患者(14.3%)符合这些标准。对PSP的这种诊断不足主要是由异质性,时机变化和排除标准中出现的症状引起的。本研究还表明,PSP的临床特征可能会根据疾病阶段而发生巨大变化。应根据时间和阶段选择目标症状,以优化患者的生活质量。

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