Autonomic dysfunction in peripheral nerve disease

Muscle Nerve. 2003 Jun;27(6):646-61. doi: 10.1002/mus.10333.


Autonomic neuropathies are inherited or acquired neuropathies in which autonomic nerve fibers are selectively or disproportionately affected. Generally, sympathetic and parasympathetic fibers are both affected but there are exceptions. Acquired cases can be autoimmune; due to diabetes, amyloidosis, drugs, or toxins; or idiopathic. Autoimmune autonomic neuropathy is often subacute, sometimes associated with a neoplasm, and associated with high titers of antibody to ganglionic nicotinic acetylcholine receptor in about half of the severe cases. The molecular basis of inherited autonomic neuropathies is better known, including recent identification of the loci and genes of hereditary sensory and autonomic neuropathies types I, III, and IV. The inherited amyloid neuropathies are due to mutations of three proteins: transthyretin, apolipoprotein A1, and gelsolin. Non-invasive autonomic testing complements clinical and electrophysiological characterization of the autonomic neuropathies.

Publication types

  • Review

MeSH terms

  • Amyloid Neuropathies / pathology
  • Amyloid Neuropathies / physiopathology
  • Autoimmune Diseases of the Nervous System / immunology
  • Autoimmune Diseases of the Nervous System / pathology
  • Autoimmune Diseases of the Nervous System / therapy
  • Autonomic Nervous System / immunology*
  • Autonomic Nervous System / pathology
  • Autonomic Nervous System / physiopathology
  • Autonomic Nervous System Diseases / immunology*
  • Autonomic Nervous System Diseases / pathology
  • Autonomic Nervous System Diseases / therapy
  • Diabetic Neuropathies / pathology
  • Diabetic Neuropathies / physiopathology
  • Humans
  • Neurotoxins / adverse effects
  • Peripheral Nervous System Diseases / immunology*
  • Peripheral Nervous System Diseases / pathology
  • Peripheral Nervous System Diseases / therapy


  • Neurotoxins