Abstract
We encountered three cases of chronic functional colonic obstruction caused by intramural ganglion cell death. Morphologic and pharmacological studies were performed using resected specimens. The patients included a 59-year-old man, a 72-year-old woman, and a 28-year-old man. Barium enema studies revealed segmental stenosis in their left colon. A mecholyl test was positive in all three cases and was useful in diagnosing this disorder. Histopathologic and cytometric examinations disclosed both degeneration and the disappearance of intramural ganglion cells. The number of muscarinic acetylcholine receptors was observed to increase in the muscle layers of the stenotic portion. In addition, the muscle of the affected region showed hypersensitivity to the muscarinic agonist (oxotremorine). These results seem to suggest that this disease is caused by a noncongenital injury to the intramural ganglion cells while the resulting stenosis is considered to reflect the degeneration of the ganglion cells. The etiology of ganglion cell death still remains to be clarified; however, we propose that patients with this disorder may represent a subset of patients with sporadic visceral neuropathy.
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Okamoto E, Okada A, Kuwata K (1974) Histochemical and neurohistological studies on the innervation of the rectum in Hirschsprung's disease (in Japanese). In: Fujiwara M, Tanaka C (eds) Amine fluorescent histochemistry. Igakusyoin, Tokyo, pp 129–135
Yamamura HI, Snyder SH (1974) Muscarinic cholinergic receptor binding in the longitudinal muscle of the guinea ileum with [3H]quinuclidinyl benzilate. Mol Pharmacol 10:861–867
Lowry OH, Rosebrough NJ, Farr AL, Randall RJ (1951) Protein measurement with the folin phenol reagent. J Biol Chem 193:265–273
Ueki S, Okamoto E, Kuwata K, Toyasaka A, Nagai K, Uchida S, Yoshida H (1983) Increase in muscarinic acetylcholine receptors in mouse intestine by hexamethonium treatment. Life Sci 32:2431–2437
Scatchard G (1949) The attractions of proteins for small molecules and ions. Ann NY Acad Sci 51:660–672
Cannon WB (1939) A law of denervation. Am J Med Sci 198:737–750
Ueki S, Okamoto E, Kuwata K, Toyosaka A, Nagai K (1985) Qualitative and quantitative analysis of muscarinic acetylcholine receptors in the piebald lethal model of Hirschsprung's disease. Gastroenterology 88:1934–1941
Inoue T, Okasora T, Okamoto E (1991) Effect on muscarinic acetylcholine receptors after experimental neuronal ablation in the rat colon (in Japanese). Jpn J Pharmacol 27:167–168
Schuffler MD, Rohrmann CA, Chaffee RG (1981) Chronic intestinal pseudo-obstruction. Medicine 60:173–196
Ogilvie H (1948) Large intestine colic due to sympathetic deprivation: a new clinical syndrome. Br Med J 2:671–673
Philippart AI, Reed JO, Georgeson KE (1985) Neonatal small left colon syndrome: intramural not intra luminal obstruction. J Pediatr Surg 10:733–740
Sanai T, Okuda S, Onoyama K (1990) Germanium dioxide induced nephropathy. Nephron 54:53–60
Matsui T, Iida M, Iwashita (1985) A case of chronic intestinal pseudo-obstruction due to the degeneration and disappearance of colonic intramural ganglion cell (in Japanese). I to Cho (Stomach and Intestine) 20:1131–1139
Amano S, Tsukamoto N, Sakurai A, Koroda M, Kitagawa T, Suzuki H (1988) Two cases of segmental oligoganglionosis (in Japanese). In: Okamoto E (ed) Pseudo-Hirschsprung's disease. Shyobunsha, Osaka, pp 124–126
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Niwamoto, H., Okamoto, E., Toyosaka, A. et al. Sporadic visceral neuropathy. Surg Today 25, 763–770 (1995). https://doi.org/10.1007/BF00311449
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DOI: https://doi.org/10.1007/BF00311449