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. 2017 Jul 1;74(7):839-846.
doi: 10.1001/jamaneurol.2017.0603.

Survival and Causes of Death Among People With Clinically Diagnosed Synucleinopathies With Parkinsonism: A Population-Based Study

Rodolfo Savica  1 , Brandon R Grossardt  2 , James H Bower  3 , J Eric Ahlskog  3 , Bradley F Boeve  3 , Jonathan Graff-Radford  3 , Walter A Rocca  1 , Michelle M Mielke  1
Affiliations

Survival and Causes of Death Among People With Clinically Diagnosed Synucleinopathies With Parkinsonism: A Population-Based Study

Rodolfo Savica et al. JAMA Neurol. .

Abstract

Importance: To our knowledge, a comprehensive study of the survival and causes of death of persons with synucleinopathies compared with the general population has not been conducted. Understanding the long-term outcomes of these conditions may inform patients and caregivers of the expected disease duration and may help with care planning.

Objective: To compare survival rates and causes of death among patients with incident, clinically diagnosed synucleinopathies and age- and sex-matched referent participants.

Design, setting, and participants: This population-based study used the Rochester Epidemiology Project medical records-linkage system to identify all residents in Olmsted County, Minnesota, who received a diagnostic code of parkinsonism from 1991 through 2010. A movement-disorders specialist reviewed the medical records of each individual to confirm the presence of parkinsonism and determine the type of synucleinopathy. For each confirmed patient, an age- and sex-matched Olmsted County resident without parkinsonism was also identified.

Main outcomes and measures: We determined the age- and sex-adjusted risk of death for each type of synucleinopathy, the median time from diagnosis to death, and the causes of death.

Results: Of the 461 patients with synucleinopathies, 279 (60.5%) were men, and of the 452 referent participants, 272 (60.2%) were men. From 1991 through 2010, 461 individuals received a diagnosis of a synucleinopathy (309 [67%] of Parkinson disease, 81 [17.6%] of dementia with Lewy bodies, 55 [11.9%] of Parkinson disease dementia, and 16 [3.5%] of multiple system atrophy with parkinsonism). During follow-up, 68.6% (n = 316) of the patients with synucleinopathies and 48.7% (n = 220) of the referent participants died. Patients with any synucleinopathy died a median of 2 years earlier than referent participants. Patients with multiple system atrophy with parkinsonism (hazard ratio, 10.51; 95% CI, 2.92-37.82) had the highest risk of death compared with referent participants, followed by those with dementia with Lewy bodies (hazard ratio, 3.94; 95% CI, 2.61-5.94), Parkinson disease with dementia (hazard ratio, 3.86; 95% CI, 2.36-6.30), and Parkinson disease (hazard ratio, 1.75; 95% CI, 1.39-2.21). Neurodegenerative disease was the most frequent cause of death listed on the death certificate for patients, and cardiovascular disease was the most frequent cause of death among referent participants.

Conclusions and relevance: Individuals with multiple system atrophy with parkinsonism, dementia with Lewy bodies, and Parkinson disease dementia have increased mortality compared with the general population. The mortality among persons with Parkinson disease is only moderately increased compared with the general population.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Bower receives funding from the Parkinson’s Disease Foundation, Inc. Dr Ahlskog receives royalties from Oxford University Press for 3 recently published books. Dr Boeve receives funding from the National Institute on Aging, the National Institute of Neurological Disorders and Stroke, Roivant Sciences, C2N Diagnostics, and Axovant Sciences. Dr Rocca receives funding from the National Institutes of Health. Dr Mielke receives funding from the National Institutes of Health, the Michael J. Fox Foundation, and unrestricted research grants from Biogen and Lundbeck. She has received consulting fees from Lysosomal Therapeutics, Inc, and Eli Lilly. No other disclosures are reported.

Figures

Figure 1.
Figure 1.. Kaplan-Meier Survival Curves Among Patients With Synucleinopathies and Referent Participants
Figure 2.
Figure 2.. Kaplan-Meier Survival Curves By Type of Synucleinopathy
MSA-p indicates multiple system atrophy.
See this image and copyright information in PMC

Comment in

References

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