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. 2021 May;36(5):1203-1215.
doi: 10.1002/mds.28470. Epub 2021 Jan 29.

A Modified Progressive Supranuclear Palsy Rating Scale

Marie-Therese Grötsch  1   2 , Gesine Respondek  1   2   3 , Carlo Colosimo  4 , Yaroslau Compta  5 , Jean Christophe Corvol  6 , Joaquim Ferreira  7 , Meret Koroni Huber  3 , Martin Klietz  3 , Lea F M Krey  3 , Johannes Levin  2   8 , Milica Jecmenica-Lukic  9 , Daniel Macías-García  10 , Wassilios G Meissner  11   12   13 , Pablo Mir  10   14 , Huw Morris  15 , Christer Nilsson  16 , James B Rowe  17 , Klaus Seppi  18 , Maria Stamelou  19   20   21 , John C van Swieten  22 , Gregor Wenning  18 , Teodoro Del Ser  23 , Lawrence I Golbe  24 , Günter U Höglinger  1   2   3 ; Describe PSP Study Group, the ProPSP Study Group, and the Movement Disorder Society-Endorsed PSP Study Group
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Free article

A Modified Progressive Supranuclear Palsy Rating Scale

Marie-Therese Grötsch et al. Mov Disord. 2021 May.
Free article

Abstract

Background: The Progressive Supranuclear Palsy Rating Scale is a prospectively validated physician-rated measure of disease severity for progressive supranuclear palsy. We hypothesized that, according to experts' opinion, individual scores of items would differ in relevance for patients' quality of life, functionality in daily living, and mortality. Thus, changes in the score may not equate to clinically meaningful changes in the patient's status.

Objective: The aim of this work was to establish a condensed modified version of the scale focusing on meaningful disease milestones.

Methods: Sixteen movement disorders experts evaluated each scale item for its capacity to capture disease milestones (0 = no, 1 = moderate, 2 = severe milestone). Items not capturing severe milestones were eliminated. Remaining items were recalibrated in proportion to milestone severity by collapsing across response categories that yielded identical milestone severity grades. Items with low sensitivity to change were eliminated, based on power calculations using longitudinal 12-month follow-up data from 86 patients with possible or probable progressive supranuclear palsy.

Results: The modified scale retained 14 items (yielding 0-2 points each). The items were rated as functionally relevant to disease milestones with comparable severity. The modified scale was sensitive to change over 6 and 12 months and of similar power for clinical trials of disease-modifying therapy as the original scale (achieving 80% power for two-sample t test to detect a 50% slowing with n = 41 and 25% slowing with n = 159 at 12 months).

Conclusions: The modified Progressive Supranuclear Palsy Rating Scale may serve as a clinimetrically sound scale to monitor disease progression in clinical trials and routine. © 2021 International Parkinson and Movement Disorder Society.

Keywords: clinical meaningfulness; progressive supranuclear palsy; rating scale; sensitivity to change.

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References

    1. Rösler TW, Tayaranian Marvian A, Brendel M, et al. Four-repeat tauopathies. Prog Neurobiol 2019;180:101644.
    1. Höglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Mov Disord 2017;32(6):853-864.
    1. Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy. Brain 2007;130(6):1552-1565.
    1. Tolosa E, Litvan I, Höglinger GU, et al. A phase 2 trial of the GSK-3 inhibitor tideglusib in progressive supranuclear palsy. Mov Disord 2014;29(4):470-478.
    1. Boxer AL, Lang AE, Grossman M, et al. Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial. Lancet Neurol 2014;13(7):676-685.

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