Sleep Disorders and Fatigue in Patients with Different Forms of Myotonic Dystrophy Type 1

Objectives . To characterize sleep disorders in children and adults with different forms of myotonic dystrophy type 1 (DM1) and to evaluate their impact on cognitive functions, excessive daytime sleepiness (EDS), and fatigue. To determine the relationship between EDS, fatigue, and sleep disorders an...

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Bibliographic Details
Published in:Neuroscience and behavioral physiology 2024, Vol.54 (1), p.35-40
Main Authors: Erokhina, E. K., Melnik, E. A., Lebedeva, D. D., Shamtieva, K. V., Peters, T. V., Pavlikova, E. P., Gepard, V. V., Vlodavets, D. V.
Format: Article
Language:English
Subjects:
Apnea
Behavioral Sciences
Biomedical and Life Sciences
Biomedicine
Central nervous system
Children
Clinical Presentation and Treatment of Nervous and Mental Diseases
Cognitive ability
Diabetes mellitus (non-insulin dependent)
Fatigue
Hypoxemia
Muscles
Myotonic dystrophy
Neurobiology
Neurosciences
Obesity
Quality of life
Sleep and wakefulness
Sleep apnea
Sleep disorders
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Summary:Objectives . To characterize sleep disorders in children and adults with different forms of myotonic dystrophy type 1 (DM1) and to evaluate their impact on cognitive functions, excessive daytime sleepiness (EDS), and fatigue. To determine the relationship between EDS, fatigue, and sleep disorders and patients’ quality of life. Materials and methods . The study included 48 adults and nine children with confirmed DM1. Patients underwent assessment of clinical and history data, neurological and cognitive status, extent of EDS and fatigue, and quality of life using international scales and questionnaires. Polysomnography was performed to identify sleep disorders. Results . Obstructive sleep apnea syndrome (OSAS) was found in 78% of children and 79.2% of adults. The severity of OSAS in adults, in contrast to children, was influenced by obesity ( p < 0.001) and the extent of muscle weakness ( p = 0.033), especially of the neck muscles ( p = 0.018). Increases in the duration of stage I sleep ( p = 0.008) and the microactivation index ( p = 0.005) were detected in patients with OSAS and nocturnal hypoxemia. EDS and fatigue were present in 31 (64.6%) and 34 (70.8%) adults, respectively, and in nine (18.8%) at disease onset. Greater severity of muscle symptoms, anxiety, and depression contributed to increased fatigue in adults and the presence of obesity and type 2 diabetes mellitus contributed to EDS. Increased fatigue had a greater impact on quality of life than EDS and sleep disturbances. Conclusions . OSAS occupies the leading position among the spectrum of sleep disorders in all age groups and its development is facilitated by the presence of muscle weakness and obesity. Cognitive and emotional disturbances do not result from sleep apnea, but rather develop as a result of primary damage to the central nervous system. The presence of increased fatigue reduces patients’ quality of life.
ISSN:0097-0549
1573-899X
DOI:10.1007/s11055-024-01565-2