Department of Neurology, Technische Universität München, Munich, Germany.
German Center for Neurodegenerative Diseases, Munich, Germany.
Mov Disord. 2020 Jan;35(1):171-176. doi: 10.1002/mds.27872. Epub 2019 Sep 30.
The Movement Disorder Society criteria for progressive supranuclear palsy introduced the category "probable 4-repeat (4R)-tauopathy" for joint clinical diagnosis of progressive supranuclear palsy and corticobasal degeneration.
To validate the accuracy of these clinical criteria for "probable 4R-tauopathy" to predict underlying 4R-tauopathy pathology.
Diagnostic accuracy for 4R-tauopathies according to the established criteria was estimated retrospectively in autopsy-confirmed patients with progressive supranuclear palsy and corticobasal degeneration (grouped as 4R-tauopathies), and Parkinson's disease, multiple system atrophy, and frontotemporal lobar degeneration (grouped as non-4R-tauopathies).
We identified 250 cases with progressive supranuclear palsy (N = 195) and corticobasal degeneration (N = 55) and with and non-4R-tauopathies (N = 161). Sensitivity and specificity of "probable 4R-tauopathy" was 10% and 99% in the first year and 59% and 88% at final record.
The new diagnostic category "probable 4R-tauopathy" showed high specificity and may be suitable for the recruitment of patients with progressive supranuclear palsy and corticobasal degeneration into therapeutic trials targeting 4R-tauopathy. The low sensitivity underpins the need for diagnostic biomarkers. © 2019 International Parkinson and Movement Disorder Society.
运动障碍协会制定的进行性核上性麻痹标准引入了“可能的 4 重复（4R）-tau 病”这一类别，用于进行性核上性麻痹和皮质基底节变性的联合临床诊断。
验证这些用于“可能的 4R-tau 病”的临床标准预测潜在的 4R-tau 病病理的准确性。
根据既定标准，回顾性评估尸检证实的进行性核上性麻痹和皮质基底节变性（归为 4R-tau 病）以及帕金森病、多系统萎缩和额颞叶变性（归为非 4R-tau 病）患者中 4R-tau 病的诊断准确性。
我们确定了 250 例进行性核上性麻痹（N=195）和皮质基底节变性（N=55）患者，以及 4R-tau 病（N=161）和非 4R-tau 病（N=161）患者。“可能的 4R-tau 病”在第一年的敏感性和特异性分别为 10%和 99%，在最后一次记录时分别为 59%和 88%。
新的诊断类别“可能的 4R-tau 病”具有很高的特异性，可能适合将进行性核上性麻痹和皮质基底节变性患者招募到针对 4R-tau 病的治疗试验中。低敏感性表明需要诊断生物标志物。 © 2019 国际帕金森病和运动障碍协会。
