Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study.
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.
Nath
U
U
Department of Neurology, School of Health Sciences, University of Newcastle upon Tyne, UK.
Ben-Shlomo
Y
Y
Thomson
R G
RG
Lees
A J
AJ
Burn
D J
DJ
D017668:Age of Onset; D000368:Aged; D015331:Cohort Studies; D003680:Deglutition Disorders / Q000209:etiology; D018450:Disease Progression; D005260:Female; D020233:Gait Disorders, Neurologic / Q000209:etiology; D006801:Humans; D015993:Life Tables; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D013064:Speech Disorders / Q000209:etiology; D013494:Supranuclear Palsy, Progressive / Q000150:complications / Q000401:mortality / Q000503:physiopathology; D016019:Survival Analysis; D006113:United Kingdom / Q000453:epidemiology; D014786:Vision Disorders / Q000209:etiology