Synucleinopathies

Elizabeth A. Coon, MD; Wolfgang Singer, MD Autonomic Disorders p. 72-92 February 2020, Vol.26, No.1 doi: 10.1212/CON.0000000000000819
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PURPOSE OF REVIEW This article reviews the α-synucleinopathies pure autonomic failure, multiple system atrophy, dementia with Lewy bodies, and Parkinson disease with respect to autonomic failure.

RECENT FINDINGS The pattern and severity of autonomic involvement in the synucleinopathies is related to differences in cellular deposition and neuronal populations affected by α-synuclein aggregation, which influences the degree and manifestation of autonomic failure. Clinical and laboratory autonomic features distinguish the different synucleinopathies based on pattern and severity. These features also determine which patients are at risk for evolution from pure autonomic failure to the synucleinopathies with prominent motor involvement, such as multiple system atrophy, dementia with Lewy bodies, or Parkinson disease.

SUMMARY Autonomic failure is a key feature of the synucleinopathies, with varying type and degree of dysfunction from predominantly peripheral involvement in the Lewy body disorders to central involvement in multiple system atrophy.

Address correspondence to Dr Wolfgang Singer, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, singer.wolfgang@mayo.edu.

RELATIONSHIP DISCLOSURE: Dr Coon reports no disclosure. Dr Singer serves on the editorial board of Autonomic Neuroscience: Basic & Clinical, as an associate editor for Clinical Autonomic Research, as a consultant for Biohaven Pharmaceuticals, and on an advisory board for Lundbeck. Dr Singer receives research/grant support from Dysautonomia International, the US Food and Drug Administration (R01 FD4789), and the National Institutes of Health (R01 NS092625, U54 NS65736).

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Coon and Singer discuss the unlabeled/investigational use of pyridostigmine for orthostatic hypotension and clonazepam and melatonin for dream enactment behavior.

© 2020 American Academy of Neurology.