Chonic gastrointestinal pseudoobstruction

Clinical features

This paraneoplastic syndrome presents with weight loss, persistent constipation, and abdominal distension due to damage of the neurons of the enteric plexuses. Some patients may develop symptoms or have postmortem findings of sensory neuronopathy and more widespread neurological involvement (encephalomyelitis). The most common associated tumor is small-cell lung carcinoma. Patients may present with dysphagia, nausea, and vomiting due to esophageal dysmotility or gastroparesis, or more frequently with severe constipation. Radiologic studies show small bowel, colonic, or gastric dilatation (Figure) and esophageal manometry may disclose spasms or achalasia.

Figure: Barium enema showing severe dilatation of colon

Associated antibodies

Most of the patients with chronic gastrointestinal pseudoobstruction present anti-Hu antibodies. Other antibodies less frequently associated are anti-CV2(CRMP5).

Treatment
As in other paraneoplastic neurological syndromes associated with neuronal damage, chronic gastrointestinal pseudoobstruction rarely improves. The best chance to at least stabilize the syndrome is to induce a complete response of the tumor. Immunotherapy rarely is effective.

Selected references
1. Chinn JS and Schuffler MD. Paraneoplastic visceral neuropathy as a cause of severe gastrointestinal motor dysfunction. Gastroenterology 1988;95:1279-86.
2. Lee HR, Lennon VA, Camilleri M, et al. Paraneoplastic gastrointestinal motor dysfunction: clinical and laboratory characteristics. Am J Gastroenterol 2001;96:373-9.

3. Graus F, Keime-Guibert F, Reñé R, et al. Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain 2001;124:1138-48.
4. Lennon VA, Sas DF, Busk MF, et al. Enteric neuronal antibodies in pseudoobstruction with small-cell lung carcinoma. Gastroenterology 1991;100:137-42.

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