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Autonomic Nervous System01:22

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Quantitative Autonomic Testing
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Autonomic dysfunction in genetic forms of synucleinopathies.

Viorica Chelban1,2, Ekawat Vichayanrat3, Lucia Schottlaende1,4

  • 1Department of Molecular Neuroscience, UCL Institute of Neurology, London, United Kingdom, and National Hospital for Neurology and Neurosurgery, London, United Kingdom.

Movement Disorders : Official Journal of the Movement Disorder Society
|March 7, 2018
PubMed
Summary

Genetic links to alpha-synuclein cause synucleinopathies, often leading to autonomic dysfunction. Early diagnosis and treatment of dysautonomia are crucial for improving quality of life and predicting outcomes in these neurodegenerative diseases.

Keywords:
SNCAalpha-synucleinautonomic dysfunctiondysautonomiagenetic parkinsonism

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Area of Science:

  • Neuroscience
  • Genetics
  • Autonomic Neurology

Background:

  • Alpha-synuclein gene links have identified synucleinopathies, a group of neurodegenerative diseases.
  • Autonomic dysfunction, including cardiac sympathetic denervation, is observed in familial synucleinopathies with Lewy bodies.
  • Dysautonomia is associated with various synucleinopathies like Parkinson's disease (PD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB).

Purpose of the Study:

  • To review and compare autonomic dysfunction in genetic versus sporadic synucleinopathies.
  • To provide an updated overview of current understanding in the field of synucleinopathies and dysautonomia.

Main Methods:

  • Review of literature on genetic and sporadic synucleinopathies.
  • Comparison of autonomic profiles in different forms of synucleinopathies.
  • Analysis of the correlation between alpha-synuclein accumulation and autonomic system degeneration.

Main Results:

  • Autonomic dysfunction is a common feature in synucleinopathies linked to alpha-synuclein accumulation.
  • Familial parkinsonism without Lewy bodies (e.g., PARK2) shows normal autonomic function.
  • Degeneration of central and peripheral autonomic systems correlates with alpha-synuclein inclusions.

Conclusions:

  • Autonomic dysfunction significantly impacts quality of life and can serve as a prognostic factor in synucleinopathies.
  • Prompt diagnosis and treatment of dysautonomia are recommended.
  • Autonomic dysfunction may offer future diagnostic markers and research avenues for synucleinopathies.