Autonomic neuropathies are peripheral nerve disorders with disproportionate involvement of autonomic fibers.
(See also Overview of the Autonomic Nervous System.)
The best known autonomic neuropathies are those accompanying peripheral neuropathy due to diabetes, amyloidosis, or autoimmune disorders.
Autoimmune autonomic ganglionopathy is an autoimmune disorder that often develops after a viral infection; onset may be subacute.
Autonomic impairment may be a late manifestation in neuropathy due to alcohol use disorder.
Other causes can include toxins, drugs, and paraneoplastic syndromes.
Symptoms and Signs of Autonomic Neuropathies
Common symptoms of autonomic neuropathies include orthostatic hypotension, neurogenic bladder, erectile dysfunction, gastroparesis, and intractable constipation.
When somatic fibers are involved, sensory loss in a stocking-and-glove distribution and distal weakness may occur.
Diagnosis of Autonomic Neuropathies
History and physical examination
Sometimes laboratory evaluation
Diagnosis of autonomic neuropathy is based on demonstration of autonomic failure and, when possible, identification of a specific cause of neuropathy (eg, diabetes, amyloidosis).
Autoimmune autonomic ganglionopathy may be suspected after a viral infection.
Tests to detect serum ganglionic nicotinic acetylcholine receptor antibody alpha-3 (anti-ganglionic AChR [α3-AChR ] antibody) may be done. This antibody is present in about half of patients with autoimmune autonomic ganglionopathy and is occasionally present in patients with other autonomic neuropathies (1, 2).
Diagnosis references
1. Lamotte G, Sandroni P. Updates on the Diagnosis and Treatment of Peripheral Autonomic Neuropathies. Curr Neurol Neurosci Rep 2022;22(12):823-837. doi:10.1007/s11910-022-01240-4
2. Vernino S. Autoimmune Autonomic Disorders. Continuum (Minneap Minn) 2020;26(1):44-57. doi:10.1212/CON.0000000000000812
Treatment of Autonomic Neuropathies
Treatment of underlying disorders
Sometimes immunotherapy, plasma exchange, or IV immune globulin
Underlying disorders are treated, as are symptoms. If orthostatic hypotension is present, non-pharmacologic therapies are recommended such as hydration, increase in sodium intake, compression garments, and exercise. If severe, pharmacologic therapies for orthostatic hypotension can be used. If pain is prominent, topical therapies or neuropathic pain medications may be prescribed.
Autoimmune autonomic ganglioneuropathy may respond to immunotherapy (1); plasma exchange or IV immune globulin (2) can be used for refractory cases.
Treatment references
1. Kaur D, Tiwana H, Stino A, Sandroni P. Autonomic neuropathies. Muscle Nerve 2021;63(1):10-21. doi:10.1002/mus.27048
2. Tavee J, Brannagan TH 3rd, Lenihan MW, et al. Updated consensus statement: Intravenous immunoglobulin in the treatment of neuromuscular disorders report of the AANEM ad hoc committee. Muscle Nerve 2023;68(4):356-374. doi:10.1002/mus.27922
Key Points
Diagnose based on identifying symptoms due to autonomic failure (eg, orthostatic hypotension, neurogenic bladder, erectile dysfunction, gastroparesis, intractable constipation) and, when possible, identification of a cause of neuropathy.
Treat underlying disorders if identified; try immunotherapy if autoimmune autonomic ganglionopathy is diagnosed or, if symptoms are severe, plasma exchange or IV gamma-globulin.
