Spontaneous Restoration of Nutrition Autonomy in a Case of Intestinal Failure Secondary to a Gastrointestinal Neuromuscular Disease

Nutr Clin Pract. 2019 Dec;34(6):935-939. doi: 10.1002/ncp.10312. Epub 2019 May 10.

Abstract

Polyglucosan inclusion body myopathy (PIBM) is a recently described gastrointestinal neuromuscular disease. Given its rarity, very little is known of its natural history and prognosis. In this case report, we present the first case of PIBM and resultant intestinal failure in which, following 7 years of home parenteral nutrition, gastrointestinal symptoms subsided and nutrition autonomy was restored.

Keywords: enteric dysmotility; gastrointestinal neuromuscular disease; parenteral nutrition; polyglucosan body myopathy.

Publication types

  • Case Reports

MeSH terms

  • Gastrointestinal Motility
  • Glucans / metabolism*
  • Glycogen Storage Disease / therapy*
  • Humans
  • Inclusion Bodies / pathology
  • Intestinal Diseases / etiology
  • Intestinal Diseases / pathology*
  • Intestine, Small / pathology
  • Male
  • Middle Aged
  • Nervous System Diseases / therapy*
  • Nutritional Status*
  • Parenteral Nutrition, Home / methods*
  • Prognosis
  • Recovery of Function
  • Treatment Outcome

Substances

  • Glucans
  • polyglucosan

Supplementary concepts

  • Polyglucosan Body Disease, Adult Form