Megacolon in Myotonic Dystrophy Caused by a Degenerative Neuropathy of the Myenteric Plexus

Mark M. Yoshida, Shoba Krishnamurthy, David A. Wattchow, John B. Furness, Michael D. Schuffler

Research output: Contribution to journalArticlepeer-review

52 Citations (Scopus)


A 32-yr-old man with myotonic dystrophy had a left hemicolectomy performed because of a megacolon. The colonic mucosa, smooth muscle, and connective tissue appeared normal by hematoxylin and eosin and trichrome stains and transmission electron microscopy. In contrast, the myenteric plexus had markedly fewer neurons than normal on the hematoxylin and eosin stains. Silver staining of the plexus revealed degeneration and decreased numbers of argyrophilic neurons, which were smaller and had fewer processes and a more uneven staining quality than controls. Many axons were fragmented, and increased numbers of glial cell nuclei were present in the plexus. Degenerative changes in the neurons were present in a patchy distribution on transmission electron microscopy. Immunohistochemistry revealed a decrease of the substance Pand enkephalin-immunoreactive fibers in the muscularis externa. This suggests that colonic motor dysfunction associated with myotonic dystrophy may be caused by a visceral neuropathy that involves the substance P- and enkephalin-immunoreactive fibers of the smooth muscle.

Original languageEnglish
Pages (from-to)820-827
Number of pages8
Issue number3
Publication statusPublished - Sep 1988
Externally publishedYes


  • neuropeptide Y
  • NPY
  • SP
  • substance P
  • vasoactive intestinal peptide
  • VIP


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