Critical review of pure autonomic failure raises awareness for early signs of Parkinson’s disease, Lewy body dementia, and Multiple System Atrophy
Pure autonomic failure (PAF) is both, a rare condition and a pathogenetic mystery, first described by Bradbury and Eggleston in 1925, as a syndrome characterized by orthostatic hypotension, fixed pulse rate, anhidrosis, erectile dysfunction, and constipation without motor features. The pathology of PAF is characterized by degeneration of peripheral autonomic neurons along with Alpha-synuclein-positive, Lewy body-like inclusions in sympathetic ganglia and widespread a-synuclein deposits in autonomic neurons, establishing PAF as a restricted, nonmotor form of the synucleinopathies.
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