An overview on pure autonomic failure

A Pavy-Le Traon; A Foubert-Samier; M Fabbri

doi:10.1016/j.neurol.2023.11.003

An overview on pure autonomic failure

Rev Neurol (Paris). 2024 Jan-Feb;180(1-2):94-100. doi: 10.1016/j.neurol.2023.11.003. Epub 2023 Dec 20.

Authors

A Pavy-Le Traon¹, A Foubert-Samier², M Fabbri³

Affiliations

¹ Neurology department, French reference center for Multiple System Atrophy, CHU de Toulouse, Toulouse, France; I2MC Institute-Inserm U1297, Toulouse, France.
² Bordeaux University, Inserm, Bordeaux Population Health Research Center, UMR1219, Bordeaux, France; Neurodegenerative Diseases Neurology Department, CHU de Bordeaux, IMNc, CRMR AMS, Bordeaux, France; Bordeaux University, CNRS, IMN, UMR 5293, Bordeaux, France.
³ Neurology department, French reference center for Multiple System Atrophy, CHU de Toulouse, Toulouse, France; Department of Clinical Pharmacology and Neurosciences, Toulouse Parkinson Expert Centre, Toulouse NeuroToul Center of Excellence in Neurodegeneration (COEN), French NS-Park/F-CRIN Network, University of Toulouse 3, CHU of Toulouse, Inserm, Toulouse, France.

PMID: 38129276
DOI: 10.1016/j.neurol.2023.11.003

Abstract

Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension (OH). It is a rare, sporadic disease of adults. Although OH is the primary symptom, the autonomic dysfunction may be more generalised, leading to genitourinary and intestinal dysfunction and sweating disorders. Autonomic symptoms in PAF may be similar to those observed in other autonomic neuropathies that need to be ruled out. PAF belongs to the group of α synucleinopathies and is characterised by predominant peripheral deposition of α-synuclein in autonomic ganglia and nerves. However, in a significant number of cases, PAF may convert into another synucleinopathy with central nervous system involvement with varying prognosis: Parkinson's disease (PD), multiple system atrophy (MSA), or dementia with Lewy bodies (DLB). The clinical features, the main differential diagnoses, the risk factors for "phenoconversion" to another synucleinopathy as well as an overview of treatment will be discussed.

Keywords: Conversion; Orthostatic hypotension; Pure autonomic failure; Sympathetic; Synucleinopathy.

Publication types

Review

MeSH terms

Adult
Autonomic Nervous System Diseases* / diagnosis
Autonomic Nervous System Diseases* / etiology
Humans
Lewy Body Disease* / diagnosis
Multiple System Atrophy* / complications
Multiple System Atrophy* / diagnosis
Multiple System Atrophy* / therapy
Parkinson Disease* / complications
Parkinson Disease* / diagnosis
Parkinson Disease* / epidemiology
Pure Autonomic Failure* / complications
Pure Autonomic Failure* / diagnosis
Pure Autonomic Failure* / therapy
Synucleinopathies*