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Review
. 2021 Jan;63(1):10-21.
doi: 10.1002/mus.27048. Epub 2020 Sep 14.

Autonomic neuropathies

Divpreet Kaur  1 , Harmanpreet Tiwana  2 , Amro Stino  3 , Paola Sandroni  4
Affiliations
Free article
Review

Autonomic neuropathies

Divpreet Kaur et al. Muscle Nerve. 2021 Jan.
Free article

Abstract

Autonomic neuropathies represent a complex group of disorders that preferentially target autonomic fibers and can be classified as either acute/subacute or chronic in onset. Acute-onset autonomic neuropathies manifest with such conditions as paraneoplastic syndromes, Guillain-Barre syndrome, Sjögren syndrome, infection, or toxins/chemotherapy. When the presentation is acute, immune-mediated, and without a secondary cause, autoimmune autonomic ganglionopathy is likely, and should be considered for immunotherapy. Of the chronic-onset forms, diabetes is the most widespread and disabling, with autonomic impairment portending increased mortality and cardiac wall remodeling risk. Acquired light chain (AL) and transthyretin (TTR) amyloidosis represent two other key etiologies, with TTR amyloidosis now amenable to newly-approved gene-modifying therapies. The COMPASS-31 questionnaire is a validated outcome measure that can be used to monitor autonomic severity and track treatment response. Symptomatic treatments targeting orthostatic hypotension, among other symptoms, should be individualized and complement disease-modifying therapy, when possible.

Keywords: autonomic neuropathy; diagnosis; evaluation; review; treatment.

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References

REFERENCES

    1. Iodice V, Sandroni P. Autonomic neuropathies. Continuum (Minneap Minn). 2014;20(5 Peripheral Nervous System Disorders):1373-1397.
    1. Low PA, Vernino S, Suarez G. Autonomic dysfunction in peripheral nerve disease. Muscle Nerve. 2003;27(6):646-661.
    1. Freeman R. Autonomic peripheral neuropathy. Continuum (Minneap Minn). 2020;26(1):58-71.
    1. Low PA, Tomalia VA, Park KJ. Autonomic function tests: some clinical applications. J Clin Neurol. 2013;9(1):1-8.
    1. Suarez GA, Fealey RD, Camilleri M, Low PA. Idiopathic autonomic neuropathy: clinical, neurophysiologic, and follow-up studies on 27 patients. Neurology. 1994;44(9):1675-1682.

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