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Journal Review: Autonomic neuropathies
Overview
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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 par-aneoplastic syndromes, Guillain-Barre syndrome, Sjögren syndrome, infection, or toxins/chemotherapy. When the presentation is acute, immune-mediated, and with-out 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.


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CREDIT DESIGNATION
The AANEM designates this enduring material for a maximum of 1 AMA PRA Category 1 Credits TM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity. Credit expires 3/4/2023.

DISCLOSURE INFORMATION
No one involved in the planning of this CME activity had any relevant financial relationships to disclose. Any conflicts have been resolved by the Journal in accordance with the Muscle & Nerve editorial process.

FORMAT
PDF
Author
Divpreet Kaur, MD; Harmanpreet Tiwana, MD; Amro Stino, MD; Paola Sandroni, MD.
Summary
Availability: Retired
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