Autonomic Dysfunction in α-Synucleinopathies
The α-synucleinopathies are a group of neurodegenerative diseases characterized by abnormal accumulation of insoluble α-synuclein in neurons and glial cells, comprising Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). Although varying in prevalence, s...
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doaj-0677aaf3477b4b0288de332d30d0c9552020-11-25T00:46:03ZengFrontiers Media S.A.Frontiers in Neurology1664-22952019-04-011010.3389/fneur.2019.00363442376Autonomic Dysfunction in α-SynucleinopathiesJosé Javier Mendoza-Velásquez0José Javier Mendoza-Velásquez1Juan Francisco Flores-Vázquez2Juan Francisco Flores-Vázquez3Evalinda Barrón-Velázquez4Ana Luisa Sosa-Ortiz5Ben-Min Woo Illigens6Ben-Min Woo Illigens7Timo Siepmann8Timo Siepmann9Division of Health Care Sciences, Center for Clinical Research and Management Education, Dresden International University, Dresden, GermanyDepartment of Psychiatry and Mental Health, School of Medicine, National Autonomous University of Mexico, Mexico City, MexicoDementia Laboratory, National Institute of Neurology and Neurosurgery, Ciudad de Mexico, MexicoFaculty of Medical Sciences, University of Groningen, Groningen, NetherlandsDepartment of Psychiatry and Mental Health, School of Medicine, National Autonomous University of Mexico, Mexico City, MexicoDementia Laboratory, National Institute of Neurology and Neurosurgery, Ciudad de Mexico, MexicoDivision of Health Care Sciences, Center for Clinical Research and Management Education, Dresden International University, Dresden, GermanyDepartment of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United StatesDivision of Health Care Sciences, Center for Clinical Research and Management Education, Dresden International University, Dresden, GermanyDepartment of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyThe α-synucleinopathies are a group of neurodegenerative diseases characterized by abnormal accumulation of insoluble α-synuclein in neurons and glial cells, comprising Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). Although varying in prevalence, symptom patterns, and severity among disorders, all α-synucleinopathies have in common autonomic nervous system dysfunctions, which reduce quality of life. Frequent symptoms among α-synucleinopathies include constipation, urinary and sexual dysfunction, and cardiovascular autonomic symptoms such as orthostatic hypotension, supine hypertension, and reduced heart rate variability. Symptoms due to autonomic dysfunction can appear before motor symptom onset, particularly in MSA and PD, hence, detection and quantitative analysis of these symptoms can enable early diagnosis and initiation of treatment, as well as identification of at-risk populations. While patients with PD, DLB, and MSA show both central and peripheral nervous system involvement of α-synuclein pathology, pure autonomic failure (PAF) is a condition characterized by generalized dysregulation of the autonomic nervous system with neuronal cytoplasmic α-synuclein inclusions in the peripheral autonomic small nerve fibers. Patients with PAF often present with orthostatic hypotension, reduced heart rate variability, anhydrosis, erectile dysfunction, and constipation, without motor or cognitive impairment. These patients also have an increased risk of developing an α-synucleinopathy with central involvement, such as PD, DLB, or MSA in later life, possibly indicating a pathophysiological disease continuum. Pathophysiological aspects, as well as developments in diagnosing and treating dysautonomic symptoms in patients with α-synucleinopathies are discussed in this review.https://www.frontiersin.org/article/10.3389/fneur.2019.00363/fullautonomic dysfunctionα-synucleinopathiesParkinson diseasedementia with Lewy bodiesmultiple system atrophypure autonomic failure |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
José Javier Mendoza-Velásquez José Javier Mendoza-Velásquez Juan Francisco Flores-Vázquez Juan Francisco Flores-Vázquez Evalinda Barrón-Velázquez Ana Luisa Sosa-Ortiz Ben-Min Woo Illigens Ben-Min Woo Illigens Timo Siepmann Timo Siepmann |
spellingShingle |
José Javier Mendoza-Velásquez José Javier Mendoza-Velásquez Juan Francisco Flores-Vázquez Juan Francisco Flores-Vázquez Evalinda Barrón-Velázquez Ana Luisa Sosa-Ortiz Ben-Min Woo Illigens Ben-Min Woo Illigens Timo Siepmann Timo Siepmann Autonomic Dysfunction in α-Synucleinopathies Frontiers in Neurology autonomic dysfunction α-synucleinopathies Parkinson disease dementia with Lewy bodies multiple system atrophy pure autonomic failure |
author_facet |
José Javier Mendoza-Velásquez José Javier Mendoza-Velásquez Juan Francisco Flores-Vázquez Juan Francisco Flores-Vázquez Evalinda Barrón-Velázquez Ana Luisa Sosa-Ortiz Ben-Min Woo Illigens Ben-Min Woo Illigens Timo Siepmann Timo Siepmann |
author_sort |
José Javier Mendoza-Velásquez |
title |
Autonomic Dysfunction in α-Synucleinopathies |
title_short |
Autonomic Dysfunction in α-Synucleinopathies |
title_full |
Autonomic Dysfunction in α-Synucleinopathies |
title_fullStr |
Autonomic Dysfunction in α-Synucleinopathies |
title_full_unstemmed |
Autonomic Dysfunction in α-Synucleinopathies |
title_sort |
autonomic dysfunction in α-synucleinopathies |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Neurology |
issn |
1664-2295 |
publishDate |
2019-04-01 |
description |
The α-synucleinopathies are a group of neurodegenerative diseases characterized by abnormal accumulation of insoluble α-synuclein in neurons and glial cells, comprising Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). Although varying in prevalence, symptom patterns, and severity among disorders, all α-synucleinopathies have in common autonomic nervous system dysfunctions, which reduce quality of life. Frequent symptoms among α-synucleinopathies include constipation, urinary and sexual dysfunction, and cardiovascular autonomic symptoms such as orthostatic hypotension, supine hypertension, and reduced heart rate variability. Symptoms due to autonomic dysfunction can appear before motor symptom onset, particularly in MSA and PD, hence, detection and quantitative analysis of these symptoms can enable early diagnosis and initiation of treatment, as well as identification of at-risk populations. While patients with PD, DLB, and MSA show both central and peripheral nervous system involvement of α-synuclein pathology, pure autonomic failure (PAF) is a condition characterized by generalized dysregulation of the autonomic nervous system with neuronal cytoplasmic α-synuclein inclusions in the peripheral autonomic small nerve fibers. Patients with PAF often present with orthostatic hypotension, reduced heart rate variability, anhydrosis, erectile dysfunction, and constipation, without motor or cognitive impairment. These patients also have an increased risk of developing an α-synucleinopathy with central involvement, such as PD, DLB, or MSA in later life, possibly indicating a pathophysiological disease continuum. Pathophysiological aspects, as well as developments in diagnosing and treating dysautonomic symptoms in patients with α-synucleinopathies are discussed in this review. |
topic |
autonomic dysfunction α-synucleinopathies Parkinson disease dementia with Lewy bodies multiple system atrophy pure autonomic failure |
url |
https://www.frontiersin.org/article/10.3389/fneur.2019.00363/full |
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