Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies.

Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies.
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DOI:
10.1002/mds.27872
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发表时间:
2020-01
期刊:
Movement disorders : official journal of the Movement Disorder Society
影响因子:
--
通讯作者:
Movement Disorder Society-Endorsed Progressive Supranuclear Palsy Study Group
Movement Disorder Society-Endorsed Progressive Supranuclear Palsy Study Group
中科院分区:
其他
文献类型:
--
作者:
Respondek G;Grimm MJ;Piot I;Arzberger T;Compta Y;Englund E;Ferguson LW;Gelpi E;Roeber S;Giese A;Grossman M;Irwin DJ;Meissner WG;Nilsson C;Pantelyat A;Rajput A;van Swieten JC;Troakes C;Höglinger GU;Movement Disorder Society-Endorsed Progressive Supranuclear Palsy Study Group

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运动障碍协会对进行性核上性麻痹的标准引入了“可能的4-重复(4 R)-tau蛋白病”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“可能的4 R-tau病变”的这些临床标准预测潜在4 R-tau病变的准确性。根据既定标准,在尸检确认的进行性核上性麻痹和皮质基底节变性(分组为4 R-tau蛋白病)以及帕金森病、多系统萎缩和额颞叶变性(分组为非4 R-tau蛋白病)患者中回顾性评估4 R-tau蛋白病的诊断准确性。我们确定了250例进行性核上性麻痹(N=195)、皮质基底节变性(N=55)和非4 R-tau蛋白病(N=161)。“可能的4 R-tau蛋白病”的敏感性和特异性在第一年为10%和99%,在最终记录时为59%和88%。新的诊断类别“可能的4 R-tau蛋白病”显示出高特异性,可能适合招募进行性核上性麻痹和皮质基底节变性患者进入靶向4 R-tau蛋白的治疗试验。
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-tau pathology. Diagnostic accuracy for 4R-tauopathies according to 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), corticobasal degeneration (N=55), and with 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 recruitment of patients with progressive supranuclear palsy and corticobasal degeneration into therapeutic trials targeting 4R-tau.
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