Neuropathology of autonomic dysfunction in synucleinopathies

Elizabeth Coon, Jeremy K. Cutsforth-Gregory, Eduardo E. Benarroch

Research output: Contribution to journalReview article

34 Citations (Scopus)

Abstract

The synucleinopathies—Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure—result from distinct patterns of abnormal α-synuclein aggregation throughout the nervous system. Autonomic dysfunction in these disorders results from variable involvement of the central and peripheral autonomic networks. The major pathologic hallmark of Parkinson's disease and dementia with Lewy bodies is Lewy bodies and Lewy neurites; of multiple system atrophy, oligodendroglial cytoplasmic inclusions; and of pure autonomic failure, peripheral neuronal cytoplasmic inclusions. Clinical manifestations include orthostatic hypotension, thermoregulatory dysfunction, gastrointestinal dysmotility, and urogenital dysfunction with neurogenic bladder and sexual dysfunction. Strong evidence supports isolated idiopathic rapid eye movement sleep disorder as a significant risk factor for the eventual development of synucleinopathies with autonomic and/or motor involvement. In contrast, some neurologically normal elderly individuals have Lewy-related pathology. Future work may reveal protective or vulnerability factors that allow some patients to harbor Lewy pathology without overt autonomic dysfunction.

Original languageEnglish (US)
Pages (from-to)349-358
Number of pages10
JournalMovement Disorders
Volume33
Issue number3
DOIs
StatePublished - Mar 1 2018

Fingerprint

Multiple System Atrophy
Lewy Body Disease
Inclusion Bodies
Pure Autonomic Failure
Synucleins
Pathology
Ocular Motility Disorders
Lewy Bodies
Neurogenic Urinary Bladder
Orthostatic Hypotension
REM Sleep
Neurites
Nervous System
Parkinson Disease
Neuropathology
Sleep Wake Disorders

Keywords

  • alpha-synuclein
  • autonomic
  • Lewy body
  • multiple system atrophy
  • Parkinson's disease

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Neuropathology of autonomic dysfunction in synucleinopathies. / Coon, Elizabeth; Cutsforth-Gregory, Jeremy K.; Benarroch, Eduardo E.

In: Movement Disorders, Vol. 33, No. 3, 01.03.2018, p. 349-358.

Research output: Contribution to journalReview article

Coon, E, Cutsforth-Gregory, JK & Benarroch, EE 2018, 'Neuropathology of autonomic dysfunction in synucleinopathies', Movement Disorders, vol. 33, no. 3, pp. 349-358. https://doi.org/10.1002/mds.27186
Coon, Elizabeth ; Cutsforth-Gregory, Jeremy K. ; Benarroch, Eduardo E. / Neuropathology of autonomic dysfunction in synucleinopathies. In: Movement Disorders. 2018 ; Vol. 33, No. 3. pp. 349-358.
@article{d91388e7f09645918654c1e5c67dbadb,
title = "Neuropathology of autonomic dysfunction in synucleinopathies",
abstract = "The synucleinopathies—Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure—result from distinct patterns of abnormal α-synuclein aggregation throughout the nervous system. Autonomic dysfunction in these disorders results from variable involvement of the central and peripheral autonomic networks. The major pathologic hallmark of Parkinson's disease and dementia with Lewy bodies is Lewy bodies and Lewy neurites; of multiple system atrophy, oligodendroglial cytoplasmic inclusions; and of pure autonomic failure, peripheral neuronal cytoplasmic inclusions. Clinical manifestations include orthostatic hypotension, thermoregulatory dysfunction, gastrointestinal dysmotility, and urogenital dysfunction with neurogenic bladder and sexual dysfunction. Strong evidence supports isolated idiopathic rapid eye movement sleep disorder as a significant risk factor for the eventual development of synucleinopathies with autonomic and/or motor involvement. In contrast, some neurologically normal elderly individuals have Lewy-related pathology. Future work may reveal protective or vulnerability factors that allow some patients to harbor Lewy pathology without overt autonomic dysfunction.",
keywords = "alpha-synuclein, autonomic, Lewy body, multiple system atrophy, Parkinson's disease",
author = "Elizabeth Coon and Cutsforth-Gregory, {Jeremy K.} and Benarroch, {Eduardo E.}",
year = "2018",
month = "3",
day = "1",
doi = "10.1002/mds.27186",
language = "English (US)",
volume = "33",
pages = "349--358",
journal = "Movement Disorders",
issn = "0885-3185",
publisher = "John Wiley and Sons Inc.",
number = "3",

}

TY - JOUR

T1 - Neuropathology of autonomic dysfunction in synucleinopathies

AU - Coon, Elizabeth

AU - Cutsforth-Gregory, Jeremy K.

AU - Benarroch, Eduardo E.

PY - 2018/3/1

Y1 - 2018/3/1

N2 - The synucleinopathies—Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure—result from distinct patterns of abnormal α-synuclein aggregation throughout the nervous system. Autonomic dysfunction in these disorders results from variable involvement of the central and peripheral autonomic networks. The major pathologic hallmark of Parkinson's disease and dementia with Lewy bodies is Lewy bodies and Lewy neurites; of multiple system atrophy, oligodendroglial cytoplasmic inclusions; and of pure autonomic failure, peripheral neuronal cytoplasmic inclusions. Clinical manifestations include orthostatic hypotension, thermoregulatory dysfunction, gastrointestinal dysmotility, and urogenital dysfunction with neurogenic bladder and sexual dysfunction. Strong evidence supports isolated idiopathic rapid eye movement sleep disorder as a significant risk factor for the eventual development of synucleinopathies with autonomic and/or motor involvement. In contrast, some neurologically normal elderly individuals have Lewy-related pathology. Future work may reveal protective or vulnerability factors that allow some patients to harbor Lewy pathology without overt autonomic dysfunction.

AB - The synucleinopathies—Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure—result from distinct patterns of abnormal α-synuclein aggregation throughout the nervous system. Autonomic dysfunction in these disorders results from variable involvement of the central and peripheral autonomic networks. The major pathologic hallmark of Parkinson's disease and dementia with Lewy bodies is Lewy bodies and Lewy neurites; of multiple system atrophy, oligodendroglial cytoplasmic inclusions; and of pure autonomic failure, peripheral neuronal cytoplasmic inclusions. Clinical manifestations include orthostatic hypotension, thermoregulatory dysfunction, gastrointestinal dysmotility, and urogenital dysfunction with neurogenic bladder and sexual dysfunction. Strong evidence supports isolated idiopathic rapid eye movement sleep disorder as a significant risk factor for the eventual development of synucleinopathies with autonomic and/or motor involvement. In contrast, some neurologically normal elderly individuals have Lewy-related pathology. Future work may reveal protective or vulnerability factors that allow some patients to harbor Lewy pathology without overt autonomic dysfunction.

KW - alpha-synuclein

KW - autonomic

KW - Lewy body

KW - multiple system atrophy

KW - Parkinson's disease

UR - http://www.scopus.com/inward/record.url?scp=85039991527&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85039991527&partnerID=8YFLogxK

U2 - 10.1002/mds.27186

DO - 10.1002/mds.27186

M3 - Review article

C2 - 29297596

AN - SCOPUS:85039991527

VL - 33

SP - 349

EP - 358

JO - Movement Disorders

JF - Movement Disorders

SN - 0885-3185

IS - 3

ER -