• Mayo Clinic proceedings · Oct 2019

    Review

    Pure Autonomic Failure.

    • Elizabeth A Coon, Wolfgang Singer, and Phillip A Low.
    • Department of Neurology, Mayo Clinic, Rochester, MN. Electronic address: coon.elizabeth@mayo.edu.
    • Mayo Clin. Proc. 2019 Oct 1; 94 (10): 208720982087-2098.

    AbstractPure autonomic failure (PAF) is a neurodegenerative disorder of the autonomic nervous system clinically characterized by orthostatic hypotension. The disorder has also been known as Bradbury-Eggleston syndrome, named for the authors of the 1925 seminal description. Patients typically present in midlife or later with orthostatic hypotension or syncope. Autonomic failure may also manifest as genitourinary, bowel, and thermoregulatory dysfunction. With widespread involvement, patients may present to a variety of different specialties and require multidisciplinary treatment approaches. Pathologically, PAF is characterized by predominantly peripheral deposition of α-synuclein. However, patients with PAF may progress into other synucleinopathies with central nervous system involvement.Copyright © 2019 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

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