title: Pure Autonomic Failure.
abstract: Pure autonomic failure (PAF) is a rare sporadic neurodegenerative autonomic disorder characterized by slowly progressive pan autonomic failure without other features of neurologic dysfunctions. The main clinical symptoms result from neurogenic orthostatic hypotension and urinary and gastrointestinal autonomic dysfunctions. Autonomic failure in PAF is caused by neuronal degeneration of pre- and postganglionic sympathetic and parasympathetic neurons in the thoracic spinal cord and paravertebral autonomic ganglia. The presence of Lewy bodies and α-synuclein deposits in these neural structures suggests that PAF is one of Lewy body synucleinopathies, examples of which include multiple system atrophy, Parkinson disease, and Lewy body disease. There is currently no specific treatment to stop progression in PAF. Management of autonomic symptoms is the mainstay of treatment and includes management of orthostatic hypotension and supine hypertension. The prognosis for survival of PAF is better than for the other synucleinopathies.
authors.affiliation: Department of Neurological Sciences, University of Nebraska Medical Center, 988435 Nebraska Medical Center, Omaha, NE, 68198-8435, USA. pthaiset@unmc.edu.
authors.forename: Pariwat
authors.initials: P
authors.lastname: Thaisetthawatkul
mesh_terms: D018450:Disease Progression; D006801:Humans; D020961:Lewy Body Disease; D011379:Prognosis; D054970:Pure Autonomic Failure / Q000175:diagnosis / Q000378:metabolism / Q000503:physiopathology; D051844:alpha-Synuclein / Q000378:metabolism
chemical_list: D051844:alpha-Synuclein
keywords: Lewy body; Neurogenic orthostatic hypotension; Pure autonomic failure; Synucleinopathy; α-synuclein