The Progressive Supranuclear Palsy Clinical Deficits Scale

Abstract

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

Hospitalization Rates and Comorbidities in Patients with Progressive Supranuclear Palsy in Germany from 2010 to 2017

“…In order to prioritize the most important pharmacological and also non-pharmacological treatment approaches for patients, a precise clinical evaluation is necessary that can be performed with the recently developed Progressive Supranuclear Palsy Clinical Deficits Scale [ 24 ]. The detection of individual deficits in seven clinical domains can assist in the selection of accurate inpatient treatment concepts, such as multimodal complex treatment that is offered in many hospitals throughout Germany [ 25 ].…”

Best Practices in the Clinical Management of Progressive Supranuclear Palsy and Corticobasal Syndrome: A Consensus Statement of the CurePSP Centers of Care

“…The PSP Rating Scale includes items assessing solid swallowing by history and liquid swallowing by examination (41). A recently developed PSP Clinical Deficits Scale also captures elements of dysphagia (164). Once dysphagia is suspected, typically signified by coughing while drinking liquids, a referral should be made to a speechlanguage pathologist SLP.…”

DescribePSP and ProPSP: German Multicenter Networks for Standardized Prospective Collection of Clinical Data, Imaging Data, and Biomaterials of Patients With Progressive Supranuclear Palsy

“… PSPRS, PSP Rating Scale ( 12 ); PSP-SS, PSP Staging System ( 13 ); PSP-CDS, PSP-Clinical Deficits Scale ( 15 ) . …”

“…Clinical data, imaging and biomaterials from both networks have been shared with national and international collaborators for projects that serve the primary goal of DescribePSP and ProPSP . The Progressive Supranuclear Palsy Clinical Deficits Scale (PSP-CDS), a clinical scale to monitor clinical deficits in patients with PSP across its broad phenotypes, has been developed with baseline and follow-up clinical datasets of DescribePSP (exploratory) and ProPSP (confirmatory) ( 15 ).…”