Neuropathy in the brain-in-the-gut.

* The enteric nervous system has sensory neurons, interneurons and motor neurons and functions as a brain-in-the-gut. * Smooth muscles of the digestive tract are autogenic in the absence of neural control. * Enteric inhibitory motor neurons control excitability of the autogenic musculature. * The neuropathic form of chronic intestinal pseudo-obstruction is a form of disinhibitory motor disease linked with neuropathic degeneration in the enteric nervous system. * Patients with inflammatory degenerative neuropathy may progress from irritable bowel syndrome (IBS)-like symptoms to chronic pseudo-obstruction. * Detection of anti-enteric neuronal antibodies may be a useful diagnostic test for early stages of inflammatory degenerative neuropathy in patients with symptoms of a functional gastrointestinal disorder. Awareness is increasing that autoimmune attack targeted to neuronal elements of the enteric nervous system may underlie irritable bowel-like symptoms that progress to chronic pseudo-obstruction. The inflammatory neuropathy disrupts the integrative functions of the brain-in-the-gut, including reduction in the population of inhibitory motor neurons to the musculature. Extreme loss of inhibitory motor neurons is manifest as disinhibitory motor disease characterized by achalasia in smooth muscle sphincters and hyperactive, disorganized contractile behaviour of intestinal circular muscle which results in pseudo-obstruction. Detection of anti-enteric neuronal antibodies in the serum of patients with early symptoms of a functional gastrointestinal motility disorder may prove to be a useful diagnostic test for inflammatory enteric neuropathy.