Survival and causes of death among people with clinically diagnosed Synucleinopathies with parkinsonism: A population-based study

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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. Amovement-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.

Original languageEnglish (US)
Pages (from-to)839-846
Number of pages8
JournalJAMA Neurology
Volume74
Issue number7
DOIs
StatePublished - Jul 1 2017

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Parkinsonian Disorders
Cause of Death
Survival
Parkinson Disease
Multiple System Atrophy
Population
Dementia
Lewy Body Disease
Medical Record Linkage
Death Certificates
Mortality
Neurodegenerative Diseases
Caregivers
Medical Records
Epidemiology
Cardiovascular Diseases
Survival Rate

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

@article{7a755f189b634ef89bfd895ae1daf7f2,
title = "Survival and causes of death among people with clinically diagnosed Synucleinopathies with parkinsonism: A population-based study",
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. Amovement-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.",
author = "Rodolfo Savica and Grossardt, {Brandon R.} and Bower, {James Howard} and Ahlskog, {J. Eric} and Boeve, {Bradley F} and Jonathan Graff-Radford and Rocca, {Walter A} and Mielke, {Michelle M}",
year = "2017",
month = "7",
day = "1",
doi = "10.1001/jamaneurol.2017.0603",
language = "English (US)",
volume = "74",
pages = "839--846",
journal = "JAMA Neurology",
issn = "2168-6149",
publisher = "American Medical Association",
number = "7",

}

TY - JOUR

T1 - Survival and causes of death among people with clinically diagnosed Synucleinopathies with parkinsonism

T2 - A population-based study

AU - Savica, Rodolfo

AU - Grossardt, Brandon R.

AU - Bower, James Howard

AU - Ahlskog, J. Eric

AU - Boeve, Bradley F

AU - Graff-Radford, Jonathan

AU - Rocca, Walter A

AU - Mielke, Michelle M

PY - 2017/7/1

Y1 - 2017/7/1

N2 - 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. Amovement-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.

AB - 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. Amovement-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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