[A case report of chronic intestinal pseudo-obstruction with autoimmune autonomic ganglionopathy suspected from seropositive results for anti-ganglionic acetylcholine receptor antibody]

Nihon Shokakibyo Gakkai Zasshi. 2015 Jan;112(1):62-9. doi: 10.11405/nisshoshi.112.62.
[Article in Japanese]

Abstract

A 37-year-old woman who had previously been diagnosed with idiopathic chronic intestinal pseudo-obstruction (CIPO) at another hospital was admitted to our institution with severe abdominal pain. She had a history of several abdominal surgeries to treat ileus at the previous hospital, and contrast-enhanced computed tomography on admission revealed subileus without any apparent causes of obstruction. Total parenteral nutrition, a gastrointestinal prokinetic agent, and opiates reduced persistent pain;however, breakthrough pain continued. A neurologist at our hospital suggested autoimmune autonomic ganglionopathy (AAG) as a potential cause of CIPO. The patient was diagnosed with suspected AAG on the basis of seropositive results for anti-ganglionic acetylcholine receptor antibody. Intravenous immunoglobulin administration and plasma exchange were performed in combination with immunosuppressive drugs;however, her symptoms barely improved. Although percutaneous endoscopic gastrostomy and enterostomy were subsequently performed to reduce internal intestinal pressure, her pain relief was insufficient.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Autoantibodies / blood*
  • Autoantibodies / immunology
  • Autoimmune Diseases of the Nervous System / complications*
  • Autoimmune Diseases of the Nervous System / immunology
  • Autoimmune Diseases of the Nervous System / metabolism
  • Female
  • Genetic Diseases, X-Linked / diagnosis*
  • Genetic Diseases, X-Linked / etiology
  • Genetic Diseases, X-Linked / immunology*
  • Humans
  • Intestinal Pseudo-Obstruction / congenital*
  • Intestinal Pseudo-Obstruction / diagnosis
  • Intestinal Pseudo-Obstruction / etiology
  • Intestinal Pseudo-Obstruction / immunology
  • Magnetic Resonance Imaging
  • Multimodal Imaging
  • Receptors, Cholinergic / immunology*
  • Tomography, X-Ray Computed

Substances

  • Autoantibodies
  • Receptors, Cholinergic

Supplementary concepts

  • Congenital idiopathic intestinal pseudoobstruction