Enteric ganglionitis

Refers to an inflammatory neuropathy caused by paraneoplastic (including lung small-cell carcinoma, thymoma, and breast cancer), infectious (i.e., Chagas’ disease), or neurological (i.e., encephalomyeloneuropathy) means; connective tissue disorders (i.e., scleroderma); and inflammatory bowel diseases (i.e., ulcerative colitis and Crohn’s disease), although some cases are idiopathic.

Enteric ganglionitis can occur throughout the alimentary tract and is associated with degeneration and functional impairment of enteric neurons. These alterations ultimately result in impaired gut motility and transit. Depending on the segment of the gastrointestinal tract that is involved, the clinical manifestations of enteric ganglionitis include esophageal and lower esophageal sphincter (LES) dysmotility, gastroparesis and intractable vomiting in the stomach, intestinal pseudo-obstruction, and megacolon in the intestine. Recent data suggest that enteric ganglionitis in the small intestine can be identified in patients with severe irritable bowel syndrome (IBS).

Treatment:

• Once the diagnosis of an inflammatory neuropathy is established, usually steroids such as prednisolone at doses of 60–100 mg/day, methylprednisolone (up to 600 mg/day), or beclomethasone (200 µg three times daily), alone or combined with other immunosuppressive therapy (azathioprine or cyclophosphamide), can dramatically improve the clinical picture.

See: ENS for background on the enteric nervous system of the gut

Source: Inflammatory neuropathies of the Enteric Nervous System by: De Giorgio et al., 2004