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Current Neurology and Neuroscience Reports
Published in: Current Neurology and Neuroscience Reports 8/2016

01-08-2016 | Autonomic Dysfunction (LH Weimer, Section Editor)

Pure Autonomic Failure

Author: Pariwat Thaisetthawatkul

Published in: Current Neurology and Neuroscience Reports | Issue 8/2016

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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.
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Metadata
Title
Pure Autonomic Failure
Author
Pariwat Thaisetthawatkul
Publication date
01-08-2016
Publisher
Springer US
Published in
Current Neurology and Neuroscience Reports / Issue 8/2016
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-016-0673-2

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