De Koninck X, Fiasse R, Jonard P, Demelenne J, Pringot J, Dive C
Service de gastroenterologie, Cliniques St-Luc, Université Catholique de Louvain, Bruxelles, Belgique.
Acta Gastroenterol Belg. 1990 Jan-Feb;53(1):3-15.
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.
我们研究了19例强直性肌营养不良患者随访期间出现的消化系统表现。10例患者（53%）出现胃肠道症状，主要为吞咽困难（n = 7）。在接受检查的患者（n = 7）中，6例患者的消化问题可能或肯定与神经系统疾病有关。5例吞咽困难患者的上消化道内镜检查正常。4例患者中有3例食管测压异常。3例（16%）出现并发症。1例患者出现两次自发性气腹。第二例患者发生慢性结肠假性梗阻并伴有巨结肠，接受了结肠次全切除术治疗。测压研究显示食管无蠕动、胃空肠收缩幅度低，但移行性运动复合波持续存在且直肠肛门反射正常。第三例患者发生急性假性梗阻并伴有缺血性结肠炎。总之，强直性肌营养不良常干扰消化道运动，并可能导致诸如肠道假性梗阻等并发症。
