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

Valeria A Sansone, Paola Proserpio, Luca Mauro, Andrea Lizio Biost, Erica Frezza, Andrea Lanza, Paola Rogliani, Gabriella Pezzuto, Elisa Falcier, Carola Ferrari Aggradi, Alice Pirola, Fabrizio Rao, Elisabetta Roma, Claudia Galluzzi, Matteo Spanetta, Federica Cattaneo, Annalisa Rubino, Elio Clemente Agostoni, Federica Amico, Alice ZanoliniFrancesca Izzi, Giulia Greco, Andrea Romigi, Claudio Liguori, Lino Nobili, Fabio Placidi, Roberto Massa

Research output: Contribution to journalArticlepeer-review

Abstract

STUDY OBJECTIVES: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (DM1) 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 DM1.

METHODS: 63 adult-onset DM1 patients were subjected to EDS-sleep assessments (polysomnography, PSG; Multiple Sleep Latency Test, MSLT; Epworth Sleepiness Scale, ESS. Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, quality of life.

RESULTS: 33% and 48% of patients had EDS based respectively on the ESS and the MSLT with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset REM periods on MSLT. Patients having EDS by MSLT had a shorter disease duration (p<0.05), higher total sleep time, sleep efficiency and lower WASO (Wake After Sleep Onset) on PSG. Patients with self-reported EDS reported significantly higher fatigue score compared to patients without EDS (p<0.05). 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.

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