TY - JOUR
TI - The Progressive Supranuclear Palsy Clinical Deficits Scale
AU - Piot, I.
AU - Schweyer, K.
AU - Respondek, G.
AU - Stamelou, M.
AU - Sckopke, P.
AU - Schenk, T.
AU - Goetz, C.G.
AU - Stebbins, G.T.
AU - Höglinger, G.U.
AU - DescribePSP study group
AU - ProPSP study group
AU - MDS-endorsed PSP study group
JO - Movement Disorders
PY - 2020
VL - 35
TODO - 4
SP - 650-661
PB - John Wiley and Sons Inc
SN - 0885-3185, 1531-8257
TODO - 10.1002/mds.27964
TODO - adult;  aged;  akinesia;  Article;  bradyphrenia;  clinical deficits scale;  cognition assessment;  cross-sectional study;  disease assessment;  dysphagia;  eye movement;  female;  finger;  follow up;  gait;  human;  information retrieval;  interpersonal communication;  interrater reliability;  major clinical study;  male;  observational study;  phenotype;  practice guideline;  priority journal;  progressive supranuclear palsy;  rating scale;  rigidity;  test retest reliability;  clinical trial;  disease exacerbation;  motor performance;  multicenter study;  progressive supranuclear palsy;  reproducibility, Disease Progression;  Female;  Fingers;  Humans;  Male;  Motor Skills;  Reproducibility of Results;  Supranuclear Palsy, Progressive
TODO - Background: There is currently no undisputed, validated, clinically meaningful measure for deficits in the broad spectrum of PSP phenotypes. Objective: To develop a scale to monitor clinical deficits in patients with PSP across its broad phenotypes. Methods: The Progressive Supranuclear Palsy Clinical Deficits Scale was conceptualized to cover seven clinical domains (Akinesia-rigidity, Bradyphrenia, Communication, Dysphagia, Eye movements, Finger dexterity, and Gait & balance), each scored from 0 to 3 (no, mild, moderate, or severe deficits). User guidelines were developed to standardize its application. Progressive Supranuclear Palsy Clinical Deficits Scale 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 Progressive Supranuclear Palsy Clinical Deficits Scale completion time was 4 minutes. The Progressive Supranuclear Palsy Clinical Deficits Scale total score correlated with existing scales (e.g., Progressive Supranuclear Palsy Rating Scale: R = 0.88; P < 0.001). Individual Progressive Supranuclear Palsy Clinical Deficits Scale 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-month change (baseline, 8.6 ± 3.6; follow-up: 10.8 ± 3.6; annualized difference: 3.4 ± 3.4; n = 49; P < 0.0001). Sample sizes required per arm for a two-arm, 1-year follow-up therapeutic trial to detect 50% change in Progressive Supranuclear Palsy Clinical Deficits Scale progression was estimated to be 65 (two-sided, two-sample t test). Conclusion: The Progressive Supranuclear Palsy Clinical Deficits Scale is a rapidly completed, clinimetrically sound scale for clinical care and research involving PSP. © 2020 International Parkinson and Movement Disorder Society. © 2020 International Parkinson and Movement Disorder Society
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