Willig T N, Paulus J, Lacau Saint Guily J, Béon C, Navarro J
AFM, Hôpital Tenon, Paris, France.
Arch Phys Med Rehabil. 1994 Nov;75(11):1175-81. doi: 10.1016/0003-9993(94)90001-9.
Feeding problems in patients with neuromuscular diseases are frequently underestimated and poorly analyzed. To gain a better understanding of the most common complaints, we surveyed 451 patients and received 409 responses representing seven disorders. Difficulties in the pre-oral phase of swallowing were encountered primarily in Duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophy (LGMD), facio-scapulo-humoral muscular dystrophy (FSHMD), and spinal muscular atrophy (SMA). A limitation in the ability to open the mouth was also noted in SMA. Some features are characteristic of certain diseases such as macroglossia in DMD and dryness of the mouth in dermatomyositis and polymyositis (DMPM) and myasthenia gravis (MG). Posterior swallowing time is especially affected in MG, dermatomyositis and polymyositis, LGMD, and SMA. Overall prevalence of feeding disability in five disorders (SMA, myotonic dystrophy [MD], DMPM, FSHMD, MG) was 34.9%. A better understanding of the swallowing problems associated with these disorders may help in choosing treatment possibilities, technical aids, adaptation of the consistency of foods, swallowing rehabilitation, and nutritional support by the non-oral route.
神经肌肉疾病患者的喂养问题常常被低估且分析不足。为了更好地了解最常见的问题,我们对451名患者进行了调查,收到了代表七种疾病的409份回复。吞咽的口前期困难主要出现在杜氏肌营养不良症(DMD)、肢带型肌营养不良症(LGMD)、面肩肱型肌营养不良症(FSHMD)和脊髓性肌萎缩症(SMA)中。SMA患者还存在张口能力受限的情况。某些特征是特定疾病所特有的,如DMD中的巨舌症、皮肌炎和多肌炎(DMPM)以及重症肌无力(MG)中的口干。吞咽后期时间在MG、皮肌炎和多肌炎、LGMD和SMA中尤其受到影响。五种疾病(SMA、强直性肌营养不良症[MD]、DMPM、FSHMD、MG)中喂养障碍的总体患病率为34.9%。更好地了解与这些疾病相关的吞咽问题可能有助于选择治疗方案、技术辅助工具、调整食物质地、吞咽康复以及非经口途径的营养支持。