| 제목 |
Polysomnography in Myotonic Muscular Dystrophy versus Duchenne Muscular Dystrophy |
| 소속 |
CHA Bundang Medical Center, CHA University School of Medicine, Department of Rehabilitation Medicine1, CHA University, Rehabilitation and Regeneration Research Center2, Gangnam Severance Hospital, Yonsei University College of Medicine , Department of Rehabilitation Medicine and Pulmonary Rehabilitation Center3, Yonsei University College of Medicine, Rehabilitation Institute of Neuromuscular Disease4 |
| 저자 |
Mi Ri Suh1,2*, Won Ah Choi3,4, Seong-Woong Kang3,4† |
Introduction
Sleep-related disorders, such as obstructive sleep apnea associated with sleep hypoxia and hypercapnia, rapid eye movement sleep dysregulation, and diurnal somnolence are commonly reported in patients with myopathies. We aimed to analyze polysomnography data and ventilation monitor of myotonic muscular dystrophy (MMD), and compared them with that of duchenne muscular dystrophy (DMD) to compare the different characteristics of apnea patterns in different myopathies at similar ventilatory status.
Materials and Methods
We retrospectively collected and analyzed polysomnographic data and overnight ventilation monitoring parameters among the patients with MMD and DMD, who were admitted for evaluation of respiratory failure from January, 2012 until August, 2017 and underwent ventilation monitoring and polysomnography at the same time. Ventilation parameters such as overnight partial pressure of transcutaneous carbon dioxide (tcpCO2) and oxygen saturation (SaO2), and polysomnographic parameters such as total apnea-hypopnea index (A-HI), apnea index, hypopnea index, obstructive apnea index, central apnea index, and mixed apnea index were collected. Mean values of each parameter were compared using independent t-test and spearman correlation test was performed to find the relationship between the different parameters.
Results
Twenty-five patients with MMD and 41 patients with DMD were included in the study. All of the patients with MMD were with sleep apnea, where nearly half of them (12/25, 48.0%) were with severe type of sleep apnea. On the other hand although 34 patients with DMD (34/41, 82.9%) were with sleep apnea, only 1 was with severe type of sleep apnea. Also, all the polysomnographic parameters showed significant difference between the two groups. The average SaO2 and the lowest SaO2 during the night were significantly lower in MMD (p=0.011 and p=0.001, respectively), while mean tcpCO2 and maximal tcpCO2 during the night were similar among the two groups. In patients with DMD, A-HI showed negative correlation with average SaO2 (r=-0.450, p=0.001) and the lowest SaO2 (r=-0.405, p=0.004), while it showed negative correlation with average SaO2 (r=-0.453, p=0.012) and positive correlation with mean tcpCO2 (r=0.386, p=0.035) in patients with MMD. In patients with MMD, A-HI also showed strong positive correlation with obstructive apnea index (r=0.704, p=0.002) and central apnea index (r=0.526, p<0.001) while it only showed weak positive correlation with obstructive apnea index (r=0.457, p=0.002).
Conclusion
Among the patients with MMD and DMD, severity of apnea was much more severe and proportion of central apnea was higher in patients with MMD at similar tcpCO2 level.
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Table 1. Polysomnographic parameters and overnight ventilation monitor in DM1 and DMD. data are shown as mean±standard deviation. †p value < .05. *Shows severity of apnea according to apnea-hypopnea index – none, fewer than 5 events/h; mild, 5-14 events/h; moderate, 15-29 events/h and severe, 30 or more events per hour. Abbreviation : DM1, myotonic muscular dystrophy type1; DMD, duchenne muscular dystrophy; tcpCO2, partial pressure of transcutaneous carbon dioxide; SaO2, oxygen saturation
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