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贝克型肌营养不良症与杜氏肌营养不良症吞咽功能障碍的比较。

A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

机构信息

a Department of Rehabilitation Medicine , National Higashisaitama Hospital , Saitama , Japan.

b Department of Rehabilitation Medicine , Keio University School of Medicine , Tokyo , Japan.

出版信息

Disabil Rehabil. 2018 Jun;40(12):1421-1425. doi: 10.1080/09638288.2017.1298680. Epub 2017 Mar 13.

Abstract

PURPOSE

Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD.

MATERIALS AND METHODS

The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia.

RESULTS

Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p < 0.001). In the BMD group, total VDS score significantly correlated with Swinyard stage (r = 0.78, p < 0.001), but not with age or lung function.

CONCLUSION

Patients with BMD have swallowing problems similar to those observed in patients with DMD when matched according to physical functional status. These patients should be evaluated and followed-up for the duration of their disease. Implications for rehabiliation Dysphagia is one of the most critical problems in patients with progressive neuromuscular disease but dysphagia in patients with Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

摘要

目的

报道了 Becker 肌营养不良症(BMD)吞咽功能障碍的特征,并与 Duchenne 肌营养不良症(DMD)进行了比较。

材料与方法

研究对象为 18 例 BMD 患者和 18 例 DMD 患者。所有患者在吞咽 5mL 液体时均接受了视频荧光检查。使用渗透-吸入量表(P-A 量表)和视频荧光吞咽量表(VDS)评估吞咽困难。

结果

BMD 和 DMD 组的 Swinyard 功能能力阶段无显著差异。两组间的误吸率、P-A 量表评分和总 VDS 评分无差异,但 BMD 组的声带抬高 VDS 项目评分低于 DMD 组(中位数评分分别为 4.5 和 9,p<0.001)。BMD 组的总 VDS 评分与 Swinyard 分期显著相关(r=0.78,p<0.001),但与年龄或肺功能无关。

结论

当根据身体功能状态进行匹配时,BMD 患者的吞咽问题与 DMD 患者相似。这些患者在疾病期间应接受评估和随访。

对于康复的影响

吞咽困难是进行性神经肌肉疾病患者最关键的问题之一,但 Becker 肌营养不良症(BMD)患者的吞咽困难情况并不清楚。18 例 BMD 患者和 18 例 Duchenne 肌营养不良症患者接受了视频荧光检查。BMD 患者的吞咽问题与 DMD 患者相似。

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