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Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study.

Nath, U; Ben-Shlomo, Y; Thomson, RG; Lees, AJ; Burn, DJ; (2003) Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study. Neurology , 60 (6) pp. 910-916. 10.1212/01.wnl.0000052991.70149.68.

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Abstract

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.

Type: Article
Title: Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study.
Location: United States
DOI: 10.1212/01.wnl.0000052991.70149.68
Keywords: Age of Onset, Aged, Cohort Studies, Deglutition Disorders, Disease Progression, Female, Gait Disorders, Neurologic, Humans, Life Tables, Male, Middle Aged, Prognosis, Proportional Hazards Models, Speech Disorders, Supranuclear Palsy, Progressive, Survival Analysis, United Kingdom, Vision Disorders
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
URI: http://discovery.ucl.ac.uk/id/eprint/116007
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