Abstract
To investigate pharyngeal and esophageal motor function in myotonic dystrophy (MD), and its relationship to esophageal symptoms, we used low-compliance, high-fidelity esophageal manometry and videofluorography to evaluate 14 consecutive MD patients. Patients exhibited a consistent, typical motor pattern, involving a marked reduction in resting tone of both the upper and lower esophageal sphincters, and a reduction in contraction pressure in the pharynx and throughout the esophagus. Radiology showed hypotonic pharynx with stasis and a hypo- or amotile, often dilated, esophagus. These findings were nonspecific, however, being present in patients both with and without dysphagia, which suggests that MD patients have valid compensatory mechanisms. Dysphagia only correlated to the pharyngeal impairment at manometry. Furthermore, the results of our study suggest that not only the proximal, striated part of the gullet, but also the distal part (in which smooth muscle dominates) is involved in the disease. The latter leads to the impairment of the LES resting tone and competence, highlighting the risk of gastroesophageal reflux disease in these patients.
Similar content being viewed by others
References
Hughes DTD, Swann JC, Gleeson JA, Lee FL: Abnormalities in swallowing associated with dystrophia myotonica. Brain 88:1037–1042, 1965
Harvey JC, Sherbourne DH, Siegel CI: Smooth muscle involvement in myotonic dystrophy. Am J Med 39:81–90, 1965
Siegel CL, Hendrix TR, Collins Harvey J: The swallowing disorder in myotonia dystrophica. Gastroenterology 50:541–549, 1966
Swick HM, Werlin SL, Dodds WJ, Hogan WJ: Pharyngoesophageal motor function in patients with myotonic dystrophy. Ann Neurol 10:454–457, 1981
Eckardt VF, Nix W, Kraus W, Kohl J: Esophageal motor function in patients with muscular dystrophy. Gastroenterology 90:628–635, 1986
Mathieu J, DeBraekeeler M, Prévost C, Boily C: Myotonic dystrophy: Clinical assessment of muscular disability in an isolated population with presumed homogeneous mutation. Neurology 42:203–208, 1992
De Meester TR, Johnson LF: The evaluation of objective measurements of gastroesophageal reflux and their contribution to patient management. Surg Clin North Am 56:39–53, 1976
Zaninotto G, Di Mario F, Costantini M, Rugge M, Baffa R, Dal Santo PL, Germanà B, Naccarato R, Ancona E: Oesophagitis and pH of the refluxate: experimental and clinical study. Br J Surg 79:161–164, 1992
Costantini M, Bremner RM, Crookes PF, DeMeester TR: The slow motorized pull-through: An improved technique to evaluate the lower esophageal sphincter. Gastroenterology 102:53, 1992
Bombeck CT, Vas O, DeSalvo J, Donahue PE, Nyhus LM: Computerized axial manometry of the esophagus: A new method for the assessment of antireflux operation. Ann Surg 206:465–472, 1987
Cook IJ, Gabb M, Panagopoulos V, Jamieson GG, Dodds, WJ, Dent J, Shearman DJ: Pharyngeal (Zenker's) diverticulum is a disorder of upper esophageal sphincter opening. Gastroenterology 103:1229–1235, 1992
Knuff TE, Benjamin SB, Castell DO: Pharyngoesophageal (Zenker's) diverticulum: A reappraisal. Gastroenterology 82:734–737, 1982
DeMeester TR, Wang CI, Wernly JA, et al: Technique, indications and clinical use of 24-hour oesophageal pH monitoring. J Thorac Cardiovasc Surg 79:656–667, 1980
Rosner B: Fundamentals of Biostatistics, 3rd ed. PWS-Kent Publishing, Boston, 1990
Harper PS, Rüdel R: Myotonic dystrophy.In Myology, Basic and Clinical, 2nd ed. AG Engel, C Franzini-Armstrong (eds). McGraw-Hill, New York, 1994, pp 1192–1219
Ergun GA: Esophageal abnormalities in systemic diseases.In The Esophagus. DO Castell (ed). Little, Brown, Boston 1992, pp 367–382
Ludatscher RM, Kerner H, Amikam S, Gellei B: Myotonia dystrophica with heart involvement: An electron microscopic study of skeletal, cardiac, and smooth muscle. J Clin Pathol 31:1057–1064, 1978
Costantini M, Zaninotto G, Anselmino M, Boccù C, Nicoletti L, Bagolin F, Ancona E: Manometric evaluation of the lower esophageal sphincter in gastroesophageal reflux disease: A modern approach. Br J Surg 80:S62, 1993
Author information
Authors and Affiliations
Additional information
This work has been supported, in part, by grant 94.02522.CT04 from the Italian National Research Council.
Rights and permissions
About this article
Cite this article
Costantini, M., Zaninotto, G., Anselmino, M. et al. Esophageal motor function in patients with myotonic dystrophy. Digest Dis Sci 41, 2032–2038 (1996). https://doi.org/10.1007/BF02093607
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02093607