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Clinical features and natural history of progressive supranuclear palsy

A clinical cohort study

From the Department of Neurology (Drs. Nath and Burn) and Department of Epidemiology and Public Health (Dr. Thomson), School of Health Sciences, University of Newcastle upon Tyne; Department of Social Medicine (Dr. Ben-Shlomo), Bristol University; and Reta Lila Weston Institute of Neurological Sciences & Sara Koe PSP Research Centre (Dr. Lees), Institute of Neurology, London, UK.

Address correspondence and reprint requests to Dr. D.J. Burn, Consultant & Senior Lecturer in Neurology, Regional Neurosciences Centre, Newcastle General Hospital, Westgate Road, Newcastle-upon-Tyne NE4 6BE, UK; e-mail: d.j.burn{at}ncl.ac.uk

Objective: To describe clinical features and identify prognostic predictors in progressive supranuclear palsy (PSP).

Methods: Record-based diagnosis according to National Institute of Neurological Disorders and Stroke–Society for Progressive Supranuclear Palsy criteria was performed in 187 cases of PSP. Clinical information was abstracted from patient records. Sixty-two patients (33%) were examined by the investigators. Forty-nine of 62 patients (79%) underwent standardized clinical assessment. Predictors of survival were examined after a mean of 6.4 years.

Results: The most common symptoms at disease onset related to mobility (69%). Of patients undergoing standardized clinical assessment, diplopia occurred in 61%, photophobia in 43%, and eyelid apraxia in 43%. Seventy-five cases (40%) died during follow-up. Older age at onset and classification as probable PSP were associated with poorer survival. Onset of falls (hazard ratio 3.28, 95% CI 1.17 to 9.13), speech problems (hazard ratio 4.74, 95% CI 1.10 to 20.4), or diplopia (hazard ratio 4.23, 95% CI 1.23 to 14.6) within 1 year and swallowing problems within 2 years (hazard ratio 3.91, 95% CI 1.39 to 11.0) were associated with a worse prognosis.

Conclusions: Mobility problems are the commonest early feature in PSP and visual symptoms are often functionally disabling. Early falls, speech and swallowing problems, diplopia, and early insertion of a percutaneous gastrostomy predict reduced survival.

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