Original Article By: Lindsey Shapiro, PhD
Summarized By: Neurobit
In a French study published in Neuromuscular Disorders, researchers from Sorbonne University evaluated the respiratory function and sleep patterns of 24 children with myotonic dystrophy type 1 (DM1) over a median follow-up of 3.75 years. DM1 is a neuromuscular disease caused by mutations in the DMPK gene that lead to progressive muscle weakness and wasting, affecting voluntary movement and breathing. The study found that nearly half of the children with DM1 showed signs of poorer nighttime respiration, reflected by rising carbon dioxide (CO2) levels, which did not significantly correlate with sleep apnea or disrupted sleep. Lung function and nighttime CO2 levels remained stable over time in the children.
Sleep-related problems, including nighttime awakenings, excessive daytime sleepiness, and susceptibility to fatigue, were found to affect about 30% of children with DM1, possibly due to poor breathing at night. However, data on respiratory and sleep problems in children with DM1 and their treatment are lacking, as most sleep disorder studies have focused on adult patients. The researchers recommended early lung function tests to monitor respiratory health in DM1 patients.
The study found that 10 of the children with DM1 had sleep apnea, which was obstructive in most cases, and was mild in two children, moderate in six, and severe in two. Non-invasive ventilation (NIV) was indicated for nine children, but seven patients did not accept or stopped this treatment. The findings offer insights into disease evolution that could improve patients' respiratory follow-up and management, and the researchers called for future research to better understand the origins of respiratory and sleep disorders in DM1 patients.
Cheminelle, M., Nougues, M. C., Isapof, A., Aubertin, G., Corvol, H., Beydon, N., & Taytard, J. (2023). Respiratory function and sleep in children with myotonic dystrophy type 1. Neuromuscular Disorders, 33(3), 263-269. https://doi.org/10.1016/j.nmd.2023.01.008
Shapiro, L. (2023, February 14). Breathing changes during sleep noted in children with DM1 | Carbon... Muscular Dystrophy News. Retrieved February 15, 2023, from https://musculardystrophynews.com/news/breathing-changes-sleep-children-myotonic-dystrophy-type-1/