Brain Advance Access originally published online on April 2, 2007
Brain 2007 130(6):1552-1565; doi:10.1093/brain/awm032
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A clinical rating scale for progressive supranuclear palsy
1Neurology, Robert Wood Johnson Medical School and 2Biometrics, School of Public Health, University of Medicine and Dentistry of New Jersey, Brunswick, NJ, USA
Corresponding to: Dr Lawrence Golbe, Neurology, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, 97 Paterson St Room 204, New Brunswick, NJ 08840, USA E-mail: golbe{at}umdnj.edu
We devised a Progressive Supranuclear Palsy (PSP) Rating Scale comprising 28 items in six categories: daily activities (by history), behaviour, bulbar, ocular motor, limb motor and gait/midline. Scores range from 0 to 100, each item graded 0–2 (six items) or 0–4 (22 items). Inter-rater reliability is good, with intra-class correlation coefficient for the overall scale of 0.86 (95% CI 0.65–0.98). A single examiner applied the PSPRS at every visit for 162 patients. Mean rate of progression was 11.3 (±11.0) points per year. Neither onset age nor gender correlated well with rate of progression. Median actuarially corrected survival was 7.3 years. The PSPRS score was a good independent predictor of subsequent survival (P < 0.0001). For example, for patients with scores from 40 to 49, 3-year survival was 41.9% (95% CI 31.0–56.6) but 4-year survival was only 17.9% (95% CI 10.2–31.5). For those patients, likelihood or retaining some gait function was 51.7% (40.0–66.9) at 1 year but only 6.5% (1.8–23.5) at 3 years. We conclude that the PSPRS is a practical measure that is sensitive to disease progression and could be useful as a dependent variable in observational or interventional trials and as an indicator of prognosis in clinical practice.
Key Words: progressive supranuclear palsy; validity; longitudinal; Steele–Richardson–Olszewski; rating scale
Abbreviations: ADL, activities of daily living; IRR, inter-rater reliability; LD/CD, levodopa/carbidopa; MMSE, Mini-Mental Status Examination; PSP, progressive supranuclear palsy; PSP-P, progressive supranuclear palsy-parkinsonism; PSPRS, Progressive Supranuclear Palsy Rating Scale; RS, Richardson's syndrome; UPDRS, Unified Parkinson's Disability Rating Scale
Received July 1, 2006. Revised February 3, 2007. Accepted February 12, 2007.
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