Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1
- Creators
- Sansone V. A.
- Proserpio P.
- Mauro L.
- Biostat A. L.
- Frezza E.
- Lanza A.
- Rogliani P.
- Pezzuto G.
- Falcier E.
- Aggradi C. F.
- Pirola A.
- Rao F.
- Roma E.
- Galluzzi C.
- Spanetta M.
- Cattaneo F.
- Rubino A.
- Agostoni E. C.
- Amico F.
- Zanolini A.
- Izzi F.
- Greco G.
- Romigi A.
- Liguori C.
- Nobili L.
- Placidi F.
- Massa R.
- Others:
- Sansone, V. A.
- Proserpio, P.
- Mauro, L.
- Biostat, A. L.
- Frezza, E.
- Lanza, A.
- Rogliani, P.
- Pezzuto, G.
- Falcier, E.
- Aggradi, C. F.
- Pirola, A.
- Rao, F.
- Roma, E.
- Galluzzi, C.
- Spanetta, M.
- Cattaneo, F.
- Rubino, A.
- Agostoni, E. C.
- Amico, F.
- Zanolini, A.
- Izzi, F.
- Greco, G.
- Romigi, A.
- Liguori, C.
- Nobili, L.
- Placidi, F.
- Massa, R.
Description
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.
Additional details
- URL
- http://hdl.handle.net/11567/1076366
- URN
- urn:oai:iris.unige.it:11567/1076366
- Origin repository
- UNIGE