The CamPaIGN study of Parkinson's disease: 10-year outlook in an incident population-based cohort
- Caroline H Williams-Gray1,
- Sarah L Mason1,
- Jonathan R Evans1,
- Thomas Foltynie2,
- Carol Brayne3,
- Trevor W Robbins4,
- Roger A Barker1
- 1John Van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK
- 2Sobell Department of Motor Neuroscience, Institute of Neurology, University College London, London, UK
- 3Department of Public Health and Primary Care, University of Cambridge, Cambridge, UK
- 4Department of Psychology, and Behavioural and Clinical Neuroscience Institute, University of Cambridge, Cambridge, UK.
- Correspondence to Dr Caroline Williams-Gray, John Van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Forvie Site, Robinson Way, Cambridge, CB2 0PY, UK.
- Received 25 February 2013
- Revised 25 April 2013
- Accepted 15 May 2013
- Published Online First 18 June 2013
Background Prognosis in Parkinson's disease (PD) remains poorly understood due to a lack of unbiased data on the natural history of treated PD. The CamPaIGN study has been the first to prospectively track disease evolution from diagnosis in an unselected population-representative incident cohort. We now report the 10-year follow-up data, focusing on three key irreversible milestones: postural instability (Hoehn and Yahr 3), dementia and death.
Methods The cohort was collected between December 2000 and 2002. Those meeting diagnostic criteria (n=142) were followed-up until 1 January 2012. Clinical, neuropsychological and genetic testing were performed. Progression to key milestones was evaluated using Kaplan–Meier and Cox regression survival analyses.
Results At 10 years, 55% had died, 68% had postural instability and 46% dementia. 23% had a good outcome at 10 years (surviving free of dementia/postural instability). Death rate was comparable with the UK population (standardised mortality ratio 1.29 (0.97–1.61)). Death certificates indicated PD was a substantial contributor in only 20%, with pneumonia being the commonest cause of death. Age, non-tremor-dominant motor phenotype and comorbidity predicted earlier postural instability. Baseline predictors of dementia were age, motor impairment, ‘posterior-cortical’ cognitive deficits and MAPT genotype.
Conclusions (1) outlook in PD is heterogeneous, with most dying or developing dementia or postural instability by 10 years from diagnosis, but a quarter still doing well, with preserved mobility and intact cognition; (2) death is not directly related to PD in the majority; (3) baseline clinical and genetic variables are predictive of outcome and may be helpful in selecting patients for clinical trials.