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  • Updated 05.30.2023
  • Released 02.06.2002
  • Expires For CME 05.30.2026

Acute autonomic neuropathies



The authors review the clinical presentation, diagnosis, pathogenesis, and management of acute autonomic neuropathies. Research indicates that many patients with acute autonomic neuropathy test positive for elevated ganglionic acetylcholine receptor autoantibodies and present with a syndrome complex, including the sicca syndrome, orthostatic hypotension, lower gastrointestinal dysautonomia, and neurogenic bladder. Mouse models in which this antibody is induced also develop autonomic neuropathy. Other causes of acute autonomic neuropathy, including Guillain-Barré syndrome, which has been shown to be associated with Zika virus infection, and paraneoplastic syndrome, are described. COVID-19 may be associated with dysautonomia, but its association with Guillain- Barré syndrome is unclear, and a large epidemiological study does not support an association. Many patients with autoimmune autonomic neuropathy respond rapidly and well to immune-modulating therapy, including intravenous immunoglobulin. Givosiran is approved by the U.S. Food and Drug Administration for the treatment of acute hepatic porphyrias; however, there are considerable adverse effects associated with this medication that can be reduced by using a personalized medicine approach. The management and treatment of these disorders is discussed, including information on the management of infant botulism.

Key points

• All acute autonomic neuropathies share common clinical features that may include orthostatic hypotension, dry eyes and mouth, cold feet and hands with color or trophic changes in the skin, reduced or accentuated sweating, anorexia or early satiety referable to gastroparesis, erectile or ejaculatory failure, and defects in micturition stream or volume, but selective syndromes are known.

• Autoantibodies to nicotine ganglionic acetylcholine receptors likely have a pathogenic role in autoimmune autonomic neuropathy.

• The presence of dysautonomia is associated with a higher incidence of mortality from Guillain-Barré syndrome.

• Assessment of a patient with a suspected autonomic neuropathy includes a detailed history, blood pressure and heart rate measurement with the patient supine and after standing for 1 minute, and laboratory tests of the autonomic nervous system (eg, tests of cardiovagal function, tests of sympathetic function, and testing for gastroparesis).

• Some autonomic neuropathies are amenable to treatment of the underlying disease, and autoimmune autonomic neuropathies may be responsive to intravenous gammaglobulin or other immunosuppressive treatments.

Historical note and terminology

Acute autonomic neuropathy is an inclusive term used to describe diseases resulting from distinct etiologies but that have in common pathology of the peripheral autonomic nervous system. Acute intermittent porphyria has been implicated in King George III of England's neuropsychiatric illness.

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ISSN: 2831-9125