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Respiratory Feature in Myotonic Dystrophy

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Myotonic Dystrophy
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Abstract

Respiratory involvement is common in myotonic dystrophy (DM), and respiratory failure is the leading cause of death in DM. DM is associated with a slowly progressive impairment of lung function. Respiratory function is serially assessed by measuring the vital capacity, oxyhemoglobin saturation, peak cough flow, and end-tidal CO2 level. Sleep-disordered breathing (SDB) is also common in DM, including obstructive and/or central sleep apnea and nocturnal hypoventilation. In DM, excessive daytime sleepiness (EDS) is not necessarily attributable to SDB, and it might be caused by a dysfunction in central sleep regulation. The respiratory insufficiency is caused by the combination of muscle impairment and altered central ventilatory control. For the treatment of respiratory failure, noninvasive ventilation (NIV) is recommended, but it might be hampered by cognitive impairment, altered personality, and unawareness of the disease. Improving compliance of the NIV is essential for enhancing the effectiveness of treatment for these patients. In addition, there are various factors that exacerbate respiratory failure, including ineffective airway clearance, infection, obesity, and perioperative pulmonary complication. Multidisciplinary care should be provided for the successful management of these respiratory complications.

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Acknowledgments

I wish to thank Dr. Fumihiko Yasuma for critically reading the manuscript. This work was partly supported by grants from the Ministry of Health, Labour and Welfare of Japan (H28-Nanchitou(Nan)-Ippan-030) and Japan Agency for Medical Research and Development (AMED) (17ek0109259).

Disclosures: Satoshi Kuru declares no conflicts of interest.

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Kuru, S. (2018). Respiratory Feature in Myotonic Dystrophy. In: Takahashi, M., Matsumura, T. (eds) Myotonic Dystrophy. Springer, Singapore. https://doi.org/10.1007/978-981-13-0508-5_8

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  • DOI: https://doi.org/10.1007/978-981-13-0508-5_8

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