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Association of autonomic symptoms with disease progression and survival in progressive supranuclear palsy.
J Neurol Neurosurg Psychiatry. 2019 05; 90(5):555-561.JN

Abstract

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.

Authors+Show Affiliations

Department of Neurology, University of São Paulo Medical School, São Paulo, Brazil. Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK. Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK.Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK. Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK.Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK. Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK.Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK. Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK. Department of Molecular Neuroscience, University College London, London, UK.Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK. Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK.Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK t.warner@ucl.ac.uk. Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, UK. Department of Molecular Neuroscience, University College London, London, UK.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

30598430

Citation

Oliveira, Marcos C B., et al. "Association of Autonomic Symptoms With Disease Progression and Survival in Progressive Supranuclear Palsy." Journal of Neurology, Neurosurgery, and Psychiatry, vol. 90, no. 5, 2019, pp. 555-561.
Oliveira MCB, Ling H, Lees AJ, et al. Association of autonomic symptoms with disease progression and survival in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry. 2019;90(5):555-561.
Oliveira, M. C. B., Ling, H., Lees, A. J., Holton, J. L., De Pablo-Fernandez, E., & Warner, T. T. (2019). Association of autonomic symptoms with disease progression and survival in progressive supranuclear palsy. Journal of Neurology, Neurosurgery, and Psychiatry, 90(5), 555-561. https://doi.org/10.1136/jnnp-2018-319374
Oliveira MCB, et al. Association of Autonomic Symptoms With Disease Progression and Survival in Progressive Supranuclear Palsy. J Neurol Neurosurg Psychiatry. 2019;90(5):555-561. PubMed PMID: 30598430.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Association of autonomic symptoms with disease progression and survival in progressive supranuclear palsy. AU - Oliveira,Marcos C B, AU - Ling,Helen, AU - Lees,Andrew J, AU - Holton,Janice L, AU - De Pablo-Fernandez,Eduardo, AU - Warner,Thomas T, Y1 - 2018/12/31/ PY - 2018/08/01/received PY - 2018/11/27/revised PY - 2018/12/01/accepted PY - 2019/1/2/pubmed PY - 2020/3/20/medline PY - 2019/1/2/entrez KW - autonomic symptoms KW - constipation KW - progressive supranuclear palsy KW - survival KW - urinary SP - 555 EP - 561 JF - Journal of neurology, neurosurgery, and psychiatry JO - J Neurol Neurosurg Psychiatry VL - 90 IS - 5 N2 - 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. SN - 1468-330X UR - https://www.unboundmedicine.com/medline/citation/30598430/Association_of_autonomic_symptoms_with_disease_progression_and_survival_in_progressive_supranuclear_palsy_ DB - PRIME DP - Unbound Medicine ER -