Category: Parkinsonism, Atypical: PSP, CBD
Objective: To develop a scale to monitor clinical deficits in patients with Progressive Supranuclear Palsy across its broad phenotypes.
Background: There is currently no undisputed, validated, clinically meaningful measure for deficits in the broad spectrum of Progressive Supranuclear Palsy phenotypes.
Method: The Progressive Supranuclear Palsy Clinical Deficits Scale (PSP-CDS) was conceptualized to cover seven clinical domains (Akinesia-rigidity, Bradyphrenia, Communication, Dysphagia, Eye movements, Finger dexterity, Gait & balance), each scored from 0 to 3 (no, mild, moderate, or severe deficits). User guidelines were developed to standardize its application. PSP-CDS scores were collected in patients fulfilling the MDS-PSP diagnostic criteria in two independent multicenter observational studies, both cross-sectionally (exploratory DescribePSP cohort; confirmatory ProPSP cohort) and longitudinally (12-months follow-up, both cohorts).
Results: Cognitive pretesting demonstrated easy scale utility. In total, 164 patients were scored (70.4±7.6 years, 62% males, 35% variant phenotypes). Mean PSP-CDS completion time was 4 minutes. The PSP-CDS total score correlated with existing scales (e.g. Progressive Supranuclear Palsy Rating Scale, R=0.88, P<0.001). Individual PSP-CDS items correlated well with similar constructs in existing scales. Internal consistency (Cronbach’s alpha 0.75), inter-rater-reliability (0.96) and test-retest-stability (0.99) were acceptable. The PSP-CDS showed significant 12-months change (baseline 8.6±3.6; follow-up 10.8±3.6; annualized difference 3.4±3.4; n=49; P<0.0001). The sample sizes required per arm for a 2-arm, 1-year follow-up therapeutic trial to detect 50% change in the PSP-CDS progression was estimated to be 65 (2-sided, 2-sample t-test).
Conclusion: The PSP-CDS is a rapidly completed, clinimetrically sound scale for clinical care and research involving PSP.
To cite this abstract in AMA style:
G. Respondek, I. Piot, K. Schweyer, M. Stamelou, P. Sckopke, T. Schenk, C. Goetz, T. Stebbins, G. Höglinger. The Progressive Supranuclear Palsy Clinical Deficits Scale [abstract]. Mov Disord. 2020; 35 (suppl 1). https://www.mdsabstracts.org/abstract/the-progressive-supranuclear-palsy-clinical-deficits-scale/. Accessed May 20, 2025.« Back to MDS Virtual Congress 2020
MDS Abstracts - https://www.mdsabstracts.org/abstract/the-progressive-supranuclear-palsy-clinical-deficits-scale/