Session Information
Date: Tuesday, September 24, 2019
Session Title: Parkinsonisms and Parkinson-Plus
Session Time: 1:45pm-3:15pm
Location: Agora 3 West, Level 3
Objective: To validate the accuracy of the newly proposed Movement Disorder Society diagnostic criteria for progressive supranuclear palsy (MDS-PSP criteria, Höglinger et al., 2017) to predict an underlying 4R-tauopathy.
Background: PSP and corticobasal degeneration (CBD) are tauopathies with predominant aggregation of 4-repeat tau. Clinically, they are often difficult to differentiate, since the symptoms of PSP and CBD overlap. Since PSP and CBD share many pathological mechanisms, joint clinical recognition as 4R-tauopathies may offer unique opportunities for neurobiological investigations and therapeutic interventions. Therefore, the MDS-PSP criteria introduced a new category for “probable 4R-tauopathies”, comprising patients with a diagnosis of “probable PSP” (any phenotype), “possible PSP with speech/language dysfunction (PSP-SL)” and “possible PSP with corticobasal syndrome (CBS)”. The diagnostic accuracy of this category has not yet been validated.
Method: Autopsy-confirmed patients with a pathological diagnosis of PSP and CBD (jointly grouped as 4R-tauopathies), and PD, MSA and FTLD (jointly grouped as non-4R-tauopathies) were identified. Detailed clinical information was obtained for each case by retrospective chart review. Accuracy of the MDS-PSP criteria for 4R-tauopathies was analyzed after retrospective application to this case series.
Results: We identified 250 cases with a 4R-tauopathy (PSP: 195, CBD: 55) and 161 cases with a non-4R-tauopathy (PD: 51, MSA: 50, FTLD: 60) with detailed clinical information from multiple brain banks. In the first 2 years of disease, a diagnosis of “probable 4R-tauopathy” as defined in the MDS-PSP criteria had a sensitivity of 19% and a specificity of 99% for 4R-tauopathies. At final record, a diagnosis of “probable 4R-tauopathy” had a sensitivity of 59% and a specificity of 88%. Sensitivity for CBD was lower (38%) than for PSP (65%).
Conclusion: The new diagnostic category for “probable 4R-tauopathies”, defined by the MDS-PSP criteria, shows high specificity for 4R-tauopathies and may be suitable for joint recruitment of patients into therapeutic trials targeting 4R-tau.
References: Höglinger et al. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord. 2017;32:853-864.
To cite this abstract in AMA style:
G. Respondek, MJ. Grimm, I. Piot, T. Arzberger, Y. Compta, E. Englund, LW. Ferguson, E. Gelpi, A. Giese, S. Roeber, DJ. Irwin, WG. Meissner, C. Nilsson, A. Pantelyat, A. Rajput, JB. Rowe, JC. van Swieten, C. Troakes, GU. Höglinger. Validation of the Movement Disorder Society criteria for the diagnosis of 4R-tauopathies [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/validation-of-the-movement-disorder-society-criteria-for-the-diagnosis-of-4r-tauopathies/. Accessed November 5, 2024.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/validation-of-the-movement-disorder-society-criteria-for-the-diagnosis-of-4r-tauopathies/