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.
|Number of pages||9|
|Journal||Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine|
|Publication status||Published - Dec 1 2021|