提示进行性核上性麻痹的临床情况-诊断性能。
Clinical Conditions "Suggestive of Progressive Supranuclear Palsy"-Diagnostic Performance.
机构信息
Department of Neurology, Technische Universität München, Munich, Germany.
German Center for Neurodegenerative Diseases (DZNE), Munich, Germany.
出版信息
Mov Disord. 2020 Dec;35(12):2301-2313. doi: 10.1002/mds.28263. Epub 2020 Sep 11.
BACKGROUND
The Movement Disorder Society diagnostic criteria for progressive supranuclear palsy introduced the diagnostic certainty level "suggestive of progressive supranuclear palsy" for clinical conditions with subtle signs, suggestive of the disease. This category aims at the early identification of patients, in whom the diagnosis may be confirmed as the disease evolves.
OBJECTIVE
To assess the diagnostic performance of the defined clinical conditions suggestive of progressive supranuclear palsy in an autopsy-confirmed cohort.
METHODS
Diagnostic performance of the criteria was analyzed based on retrospective clinical data of 204 autopsy-confirmed patients with progressive supranuclear palsy and 216 patients with other neurological diseases.
RESULTS
The conditions suggestive of progressive supranuclear palsy strongly increased the sensitivity compared to the National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy criteria. Within the first year after symptom onset, 40% of patients with definite progressive supranuclear palsy fulfilled criteria for suggestive of progressive supranuclear palsy. Two-thirds of patients suggestive of progressive supranuclear palsy evolved into probable progressive supranuclear palsy after an average of 3.6 years. Application of the criteria for suggestive of progressive supranuclear palsy reduced the average time to diagnosis from 3.8 to 2.2 years.
CONCLUSIONS
Clinical conditions suggestive of progressive supranuclear palsy allow earlier identification of patients likely to evolve into clinically possible or probable progressive supranuclear and to have underlying progressive supranuclear palsy pathology. Further work needs to establish the specificity and positive predictive value of this category in real-life clinical settings, and to develop specific biomarkers that enhance their diagnostic accuracy in early disease stages. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
背景
运动障碍协会制定的进行性核上性麻痹诊断标准引入了“疑似进行性核上性麻痹”的诊断确定性水平，适用于具有细微疾病迹象的临床情况。这一类别旨在早期识别患者，随着疾病的发展，这些患者的诊断可能会被确认为该病。
目的
在经尸检证实的患者队列中评估明确的疑似进行性核上性麻痹的临床条件的诊断性能。
方法
基于 204 例经尸检证实的进行性核上性麻痹患者和 216 例其他神经疾病患者的回顾性临床数据，分析了标准的诊断性能。
结果
与国立神经病学与卒中研究院和进行性核上性麻痹协会的标准相比，疑似进行性核上性麻痹的条件显著提高了敏感性。在症状出现后的第一年，40%的明确进行性核上性麻痹患者符合疑似进行性核上性麻痹的标准。三分之二的疑似进行性核上性麻痹患者在平均 3.6 年后演变为可能的进行性核上性麻痹。应用疑似进行性核上性麻痹的标准将平均诊断时间从 3.8 年缩短至 2.2 年。
结论
疑似进行性核上性麻痹的临床条件可更早地识别出可能演变为临床可能或可能的进行性核上性，并具有潜在的进行性核上性麻痹病理的患者。需要进一步研究以确定这一类别在现实临床环境中的特异性和阳性预测值，并开发可提高其在早期疾病阶段的诊断准确性的特异性生物标志物。
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