TY - JOUR
T1 - Pure Autonomic Failure
AU - Coon, Elizabeth A.
AU - Singer, Wolfgang
AU - Low, Phillip A.
N1 - Funding Information:
Funding Sources: Supported in part by the National Institutes of Health (P01NS44233, U54NS065736, K23NS075141, R01 FD004789, R01 NS092625) and Mayo CCaTS (UL1TR000135), and Cure PSP Foundation. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the National Institutes of Health.
Publisher Copyright:
© 2019 Mayo Foundation for Medical Education and Research
PY - 2019/10
Y1 - 2019/10
N2 - 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.
AB - 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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U2 - 10.1016/j.mayocp.2019.03.009
DO - 10.1016/j.mayocp.2019.03.009
M3 - Review article
C2 - 31515103
AN - SCOPUS:85071876285
SN - 0025-6196
VL - 94
SP - 2087
EP - 2098
JO - Mayo Clinic Proceedings
JF - Mayo Clinic Proceedings
IS - 10
ER -