Study objectives: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary, and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset myotonic dystrophy type 1. Methods: Sixty-three patients with adult-onset DM1 were subjected to EDS-sleep assessments (polysomnography, Multiple Sleep Latency Test, Epworth Sleepiness Scale). Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, and quality of life. Results: 33% and 48% of patients had EDS based, respectively, on the Epworth Sleepiness Scale and the Multiple Sleep Latency Test, with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset rapid eye movement periods on Multiple Sleep Latency Test. Patients having EDS by Multiple Sleep Latency Test had a shorter disease duration (P < .05), higher total sleep time and sleep efficiency and lower wake after sleep onset on polysomnography. Patients with self-reported EDS reported significantly higher fatigue score compared with patients without EDS (P < .05). No other difference was found in demographic, clinical, and respiratory features. Conclusions: EDS test results are contradictory, making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families. Citation: Sansone VA, Proserpio P, Mauro L, et al. Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. J Clin Sleep Med. 2021;17(12):2383-2391.

Sansone, V.a., Proserpio, P., Mauro, L., Biostat, A.l., Frezza, E., Lanza, A., et al. (2021). Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. JOURNAL OF CLINICAL SLEEP MEDICINE, 17(12), 2383-2391 [10.5664/jcsm.9438].

Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1

Rogliani, Paola;Izzi, Francesca;Liguori, Claudio;Placidi, Fabio;Massa, Roberto
2021

Abstract

Study objectives: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary, and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset myotonic dystrophy type 1. Methods: Sixty-three patients with adult-onset DM1 were subjected to EDS-sleep assessments (polysomnography, Multiple Sleep Latency Test, Epworth Sleepiness Scale). Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, and quality of life. Results: 33% and 48% of patients had EDS based, respectively, on the Epworth Sleepiness Scale and the Multiple Sleep Latency Test, with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset rapid eye movement periods on Multiple Sleep Latency Test. Patients having EDS by Multiple Sleep Latency Test had a shorter disease duration (P < .05), higher total sleep time and sleep efficiency and lower wake after sleep onset on polysomnography. Patients with self-reported EDS reported significantly higher fatigue score compared with patients without EDS (P < .05). No other difference was found in demographic, clinical, and respiratory features. Conclusions: EDS test results are contradictory, making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families. Citation: Sansone VA, Proserpio P, Mauro L, et al. Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. J Clin Sleep Med. 2021;17(12):2383-2391.
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/26
English
Epworth Sleepiness Scale
Multiple Sleep Latency Test
excessive daytime sleepiness
myotonic dystrophy type 1
polysomnography
Adult
Humans
Polysomnography
Quality of Life
Self Report
Disorders of Excessive Somnolence
Myotonic Dystrophy
Sansone, V.a., Proserpio, P., Mauro, L., Biostat, A.l., Frezza, E., Lanza, A., et al. (2021). Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. JOURNAL OF CLINICAL SLEEP MEDICINE, 17(12), 2383-2391 [10.5664/jcsm.9438].
Sansone, Va; Proserpio, P; Mauro, L; Biostat, Al; Frezza, E; Lanza, A; Rogliani, P; Pezzuto, G; Falcier, E; Aggradi, Cf; Pirola, A; Rao, F; Roma, E; Galluzzi, C; Spanetta, M; Cattaneo, F; Rubino, A; Agostoni, Ec; Amico, F; Zanolini, A; Izzi, F; Greco, G; Romigi, A; Liguori, C; Nobili, L; Placidi, F; Massa, R
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/301059
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