Linoli G, Ceccatelli P, Malentacchi G M
Unità Operativa di Neurologia, Ospedale di S. Maria sopra i Ponti (USL 23), Arezzo.
Pathologica. 1992 Jul-Aug;84(1092):523-30.
In Myotonic Dystrophy symptoms of gastrointestinal dysfunction may dominate the clinical picture or they may be manifested long before the musculoskeletal disturbances become apparent. A case of Myotonic Dystrophy in a 26-year-old woman with dysphagia as the symptom of clinical presentation is reported. Manometric investigations demonstrated motor dysfunctions in pharynx and in proximal, middle and distal portions of the esophageal body, indicating both striated and smooth muscle involvement. The pathophysiology of the esophageal motility disorders in Myotonic Dystrophy and the related therapeutic problems are briefly discussed.
在强直性肌营养不良中，胃肠功能障碍症状可能在临床表现中占主导地位，或者在肌肉骨骼紊乱明显出现之前很久就已表现出来。本文报道了一例26岁女性强直性肌营养不良患者，以吞咽困难作为临床表现症状。测压检查显示咽部以及食管体近端、中段和远端存在运动功能障碍，提示横纹肌和平滑肌均受累。本文简要讨论了强直性肌营养不良中食管动力障碍的病理生理学及相关治疗问题。
