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Papers 【 Display / hide 】

• Long-term follow-up of dysphagia in adult patients with Duchenne muscular dystrophy

  Yamada Y., Kawakami M., Wada A., Fukui S., Haruyama K., Otsuka T., Liu M.

  European Journal of Paediatric Neurology （European Journal of Paediatric Neurology)  22 （ 5 ） 786 - 790 2018.09

  ISSN  10903798

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  Background: Dysphagia is a well-known dysfunction in patients with Duchenne muscular dystrophy (DMD), but long-term follow-up of this symptom has not been reported. The aims of this study were to investigate swallowing status, prospectively study the prognosis of swallowing function in adult patients with DMD over the course of 6 years, and to identify clinical factors related to prognosis. Methods: Participants were 81 patients with DMD. Swallowing status was assessed using the Neuromuscular Disease Swallowing Status Scale (NdSSS) every 2 years for 6 years. Results: NdSSS was related to age, functional ability assessed using the Swinyard staging and the status of ventilator dependence. Median NdSSS changed from 6 to 4, 4 and 3 over the 4 time points. Number of patients with tube feeding increased by 5–10% every 2 years. Initial NdSSS was a predictor of future swallowing status. Conclusion: Swallowing status declined over time in patients with adult DMD, with initial NdSSS as a predictor.

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

  Yamada Yuka

  Disabil Rehabil. （Disability and Rehabilitation)  40 （ 12 ） 1421 - 1425 2017.03

  Research paper (scientific journal), Joint Work,  ISSN  09638288

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  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 and to compare it with that in DMD.
  Materials and methods: Participants were 18 patients with BMD and 18 patients with DMD. They were examined with videofluorography using 5 ml liquid. 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 score 4.5 and 9, respectively, p < 0.001). In the BMD group, total VDS score correlated significantly with Swinyard stage (r = 0.78, p < 0.001), but not with age or lung function parameters.
  Conclusion: Patients with BMD have swallowing problems similar to those observed in patients with DMD. These patients should be evaluated and followed up for the duration of their disease.

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