Daytime Sleepiness and REM Sleep Characteristics in Myotonic Dystrophy: a Case-control Study

Study Objectives: Excessive daytime sleepiness (EDS) and high daytime REM sleep pressure are important sleep features of myotonic dystrophy (DM1). Small and uncontrolled studies have focused on EDS phenotype; none have focused on nocturnal REM sleep characteristics in DM1. Our objectives were to compare polysomnographic and multiple sleep latency test (MSLT) parameters, and both tonic and phasic components of REM sleep between DM1 and controls.

Design And Patients: Forty consecutive DM1 patients and 40 sex- and age-matched controls were included. All subjects underwent overnight polysomnography followed by a MSLT.

Results: About 80% of DM1 patients complained of EDS through clinical interview: 31.4% had Epworth scores > 10, and 12.5% had objective sleepiness (latency < 8 min). Higher apnea and central apnea indexes, and a greater proportion of subjects with severe apnea/hypopnea syndrome were found in DM1. The number of SOREMP differed between DM1 and controls, one and two SOREMPs being present in 47.5% and 32.5%, and one control had one SOREMP. Higher percentages of slow wave sleep and REM sleep were found in DM1. DM1 patients had significantly more PLMW, PLMS in both NREM and REM sleep, and PLMS-associated microarousals. Higher REM density was found in DM1 with similar tendencies for either REM sleep without atonia or phasic EMG activity.

Conclusions: This is the first case-control sleep study in DM1 to demonstrate higher frequency of daytime sleepiness and abnormalities in REM sleep regulation, with an increased daytime and nighttime REM sleep propensity, REM density, and PLMS. These data suggest a primary central sleep regulation dysfunction in DM1.