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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 |
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Most of the patients with chronic gastrointestinal pseudoobstruction
present anti-Hu antibodies. Other antibodies
less frequently associated are anti-CV2(CRMP5).
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.
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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Updated 2009-09-15
