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Assessment of dysarthria with Frenchay dysarthria assessment (FDA-2) in patients with Duchenne muscular dystrophy

Hijikata N., Kawakami M., Wada A., Ikezawa M., Kaji K., Chiba Y., Ito M., Fujino E., Otsuka T., Liu M.

Disability and Rehabilitation (Disability and Rehabilitation) 2020

ISSN 09638288

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Purpose: The purpose of this study was to test the psychometric properties of the Japanese version of Frenchay Dysarthria Assessment (FDA-2) and to use this tool to describe the features of speech in patients with Duchenne muscular dystrophy (DMD). Materials and methods: The Japanese version of FDA-2 was culturally adapted, and reliability and validity were examined in 22 and 50 patients, respectively. The Japanese version of FDA-2 was administered to 51 patients with DMD. Multiple regression analysis was performed to identify factors related to FDA-2 scores. Results: Inter-/intra-rater reliabilities (ICCs) and internal consistency (Cronbach’s α) for total scores were 0.76, 0.97, and 0.94 respectively. For construct validity, two-way ANOVA showed a significant interaction between the disorders and FDA-2 sections (p < 0.05). In DMD patients, the item of tongue at rest was most severely affected, reflecting tongue hypertrophy. Multiple regression analysis identified age, swallowing status, and ventilator use as significantly related. Conclusions: The results showed that the Japanese version of FDA-2 has satisfactory reliability and validity. The present study demonstrated the features of dysarthria and related factors in patients with DMD.Implications for rehabilitation In Duchenne muscular dystrophy (DMD), an absent or defective dystrophin protein causes progressive weakness of respiratory and oropharyngeal muscles, both of which are crucial contributors to speech production. This study shows that the Japanese version of FDA-2 has satisfactory reliability and validity compared to original version. The Japanese version of FDA-2 characterizes dysarthria in patients with DMD in this cohort.
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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 Y., Kawakami M., Wada A., Otsuka T., Muraoka K., Liu M.

Disability and Rehabilitation (Disability and Rehabilitation) 40 ( 12 ) 1421 - 1425 2018.06

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 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.
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Development of a new scale for dysphagia in patients with progressive neuromuscular diseases: the Neuromuscular Disease Swallowing Status Scale (NdSSS)

Wada Ayako

Journal of NEUROLOGY (Springer Berlin Heidelberg) 262 ( 10 ) 2225 - 2231 2015.10

Research paper (scientific journal), Joint Work, Accepted
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