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Review
. 2019 Oct;94(10):2087-2098.
doi: 10.1016/j.mayocp.2019.03.009. Epub 2019 Sep 9.

Pure Autonomic Failure

Elizabeth A Coon  1 , Wolfgang Singer  2 , Phillip A Low  2
Affiliations
Review

Pure Autonomic Failure

Elizabeth A Coon et al. Mayo Clin Proc. 2019 Oct.

Abstract

Pure 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.

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Figures

FIGURE 1.
FIGURE 1.
Multiple organ involvement in pure autonomic failure. Autonomic manifestations in pure autonomic failure influence a variety of different organ systems, which underscores the variety of clinical areas where patients may initially seek consultation and require coordination of care during their disease. REM = rapid eye movement.
See this image and copyright information in PMC

References

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