Les manifestations digestives de la maladie de Steinert. Analyse de 19 cas dont 10 avec symptômes digestifs.

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