Background Development of autonomic failure is associated with more rapid disease course and shorter survival in patients with Parkinson’s disease and multiple system atrophy. However, autonomic symptoms have not been specifically assessed as a prognostic factor in progressive supranuclear palsy (PSP). We evaluated whether development of autonomic symptoms is associated with disease progression and survival in PSP.
Methods A retrospective review of clinical data from consecutive patients with autopsy-confirmed PSP from the Queen Square Brain Bank between January 2012 and November 2016 was performed. Time from disease onset to four autonomic symptoms (constipation, urinary symptoms, erectile dysfunction and orthostatic hypotension) were noted. Time from diagnosis to five disease milestones and survival were calculated to assess disease progression, and their risk was estimated through a Cox proportional hazards model.
Results A total of 103 PSP patients were included. Urinary symptoms and constipation were present in 81% and 71% of cases, respectively. Early development of constipation and urinary symptoms were associated with higher risk of reaching the first disease milestone (respectively, HR: 0.88; 95% CI 0.83 to 0.92; p<0.001; and HR: 0.80; 95% CI 0.75 to 0.86; p<0.001) and with a shorter survival in these patients (respectively, HR: 0.73; 95% CI 0.64 to 0.84; p<0.001; and HR: 0.88; 95% CI 0.80 to 0.96; p=0.004). On multivariate analysis, Richardson syndrome phenotype was the other variable independently associated with shorter survival.
Conclusions Earlier urinary symptoms and constipation are associated with a more rapid disease progression and reduced survival in patients with PSP.
- autonomic symptoms
- progressive supranuclear palsy
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EDP-F and TTW contributed equally.
Contributions MCBO: study concept and design, acquisition of data, analysis and interpretation of data and writing of first draft. HL: analysis and interpretation of data, critical revision of manuscript for intellectual content. JLH: acquisition and interpretation of the data and critical revision of manuscript for intellectual content. AJL: critical revision of manuscript for intellectual content. EDP-F: study concept and design, analysis and interpretation of data, study supervision and critical revision of manuscript for intellectual content. TTW: study concept and design, analysis and interpretation of data, study supervision and critical revision of manuscript for intellectual content. All authors have read and approved the final version of the manuscript.
Funding Queen Square Brain Bank is supported by the Reta Lila Weston Institute for Neurological Studies, the Progressive Supranuclear Palsy Association and the Medical Research Council UK.
Competing interests MCBO has no disclosures. HL is supported by research grant from Karin & Sten Mortstedt CBD solutions. JLH is supported by the Multiple System Atrophy Trust, Multiple System Atrophy Coalition, Sophia Fund administered by the King Baudouin Foundation, Alzheimer’s Research UK, CBD Solutions and the Michael J Fox Foundation. Queen Square Brain Bank is supported by Reta Lila Weston Institute for Neurological Studies and the Medical Research Council UK. AJL is funded by the Reta Lila Weston Institute of Neurological Studies, University College London, Institute of Neurology, and reports consultancies for Britannia Pharmaceuticals and BIAL Portela. He also receives grants and/or research support from the Frances and Renee Hock Fund, and honoraria from Britannia, Profile Pharma, UCB, Roche, Lundbeck, Teva, BIAL, Nordiclnfu Care, NeuroDerm. EDP-F has received support for attending medical conferences from UCB. TTW Warner receives research support from Brain Research Trust, Cure Huntington’s Disease Initiative, Medical Research Council and CBD solutions.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.