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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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© The Author (2007). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

A clinical rating scale for progressive supranuclear palsy

Lawrence I. Golbe1 and Pamela A. Ohman-Strickland2

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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