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Pure Autonomic Failure.

Elizabeth A Coon1, Wolfgang Singer1, Phillip A Low1

  • 1Department of Neurology, Mayo Clinic, Rochester, MN.

Mayo Clinic Proceedings
|September 14, 2019
PubMed
Summary
This summary is machine-generated.

Pure autonomic failure (PAF) is a neurodegenerative disorder affecting the autonomic nervous system, causing orthostatic hypotension. It involves peripheral alpha-synuclein deposits and can progress to central nervous system synucleinopathies.

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Area of Science:

  • Neurology
  • Neurodegenerative Diseases
  • Autonomic Nervous System Disorders

Background:

  • Pure autonomic failure (PAF), also known as Bradbury-Eggleston syndrome, is a neurodegenerative disorder.
  • It primarily affects the autonomic nervous system, leading to symptoms like orthostatic hypotension and syncope.
  • Dysfunction can also impact genitourinary, bowel, and thermoregulatory systems.

Purpose of the Study:

  • To describe the clinical and pathological characteristics of Pure Autonomic Failure.
  • To highlight the diagnostic challenges and multidisciplinary treatment needs for PAF patients.
  • To discuss the progression of PAF and its relationship to other synucleinopathies.

Main Methods:

  • Clinical case descriptions and pathological findings in Pure Autonomic Failure.
  • Review of literature on Bradbury-Eggleston syndrome and autonomic dysfunction.
  • Analysis of alpha-synuclein deposition in affected tissues.

Main Results:

  • Pure Autonomic Failure presents with orthostatic hypotension and syncope, typically in midlife or later.
  • Pathological hallmark is peripheral alpha-synuclein deposition.
  • Patients may exhibit multi-systemic autonomic dysfunction requiring specialized care.

Conclusions:

  • Pure Autonomic Failure is a distinct neurodegenerative disorder of the autonomic nervous system.
  • Early recognition and multidisciplinary management are crucial for patient care.
  • Understanding PAF's pathology is key to differentiating it from and managing its progression into other synucleinopathies.