title: [Digestive system manifestations in Steinert's disease. Analysis of 19 cases of which 10 with digestive symptoms].
abstract: We studied digestive manifestations occurring during follow-up of 19 cases of myotonic dystrophy. GI symptoms occurred in 10 patients (53%), mainly dysphagia (n = 7). In patients undergoing investigations (n = 7), their digestive troubles were probably or definitively related to the neurological disease in 6 cases. Upper GI endoscopy was normal in 5 dysphagic patients. The oesophageal manometry was abnormal in 3 of 4 patients. Complications developed in 3 cases (16%). One patient presented two episodes of spontaneous pneumoperitoneum. A second one developed a chronic colonic pseudo-obstruction with megacolon treated by subtotal colectomy. Manometric studies revealed oesophageal aperistalsis, low amplitude of gastro-jejunal contractions but persistence of migrating motor complexes and a normal recto-anal reflex. A third patient developed an acute pseudo-obstruction with ischemic colitis. In conclusion, myotonic dystrophy frequently disturbs digestive tract motility and may cause complications such as intestinal pseudo-obstruction.
authors.affiliation: Service de gastroenterologie, Cliniques St-Luc, Université Catholique de Louvain, Bruxelles, Belgique.
authors.forename: X
authors.initials: X
authors.lastname: De Koninck
authors.forename: R
authors.initials: R
authors.lastname: Fiasse
authors.forename: P
authors.initials: P
authors.lastname: Jonard
authors.forename: J
authors.initials: J
authors.lastname: Demelenne
authors.forename: J
authors.initials: J
authors.lastname: Pringot
authors.forename: C
authors.initials: C
authors.lastname: Dive
mesh_terms: D000328:Adult; D001466:Barium Sulfate; D003109:Colonic Diseases, Functional / Q000503:physiopathology; D003680:Deglutition Disorders / Q000503:physiopathology; D004066:Digestive System Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications* / Q000503:physiopathology; D010528:Peristalsis; D011859:Radiography
chemical_list: D001466:Barium Sulfate
vernacular_title: Les manifestations digestives de la maladie de Steinert. Analyse de 19 cas dont 10 avec symptômes digestifs.