Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Dec 1;17(12):2383-2391.
doi: 10.5664/jcsm.9438.

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

Valeria A Sansone  1   2 , Paola Proserpio  3 , Luca Mauro  2 , Andrea Lizio Biostat  2 , Erica Frezza  4 , Andrea Lanza  3 , Paola Rogliani  5 , Gabriella Pezzuto  5 , Elisa Falcier  2 , Carola Ferrari Aggradi  2 , Alice Pirola  2 , Fabrizio Rao  2 , Elisabetta Roma  2 , Claudia Galluzzi  4 , Matteo Spanetta  4 , Federica Cattaneo  3 , Annalisa Rubino  3 , Elio Clemente Agostoni  3 , Federica Amico  3 , Alice Zanolini  2 , Francesca Izzi  4 , Giulia Greco  4 , Andrea Romigi  6 , Claudio Liguori  4 , Lino Nobili  7   8 , Fabio Placidi  4 , Roberto Massa  4
Affiliations

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

Valeria A Sansone et al. J Clin Sleep Med. .

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.

Keywords: Epworth Sleepiness Scale; Multiple Sleep Latency Test; excessive daytime sleepiness; myotonic dystrophy type 1; polysomnography.

PubMed Disclaimer

Conflict of interest statement

All authors have seen and approved the manuscript. The study was funded by a Telethon-UILDM (Italian Muscular Dystrophy Association) grant (GUP15004) given to V.A. Sansone. The authors report no conflicts of interest.

Figures

Figure 1
Figure 1. Concordance between MSLT and ESS in the assessment of EDS.
EDS = excessive daytime sleepiness, ESS = Epworth Sleepiness Scale, MSLT = Multiple Sleep Latency Test.
See this image and copyright information in PMC

Comment in

References

    1. Harper HG . Myotonic Dystrophy. Philadelphia: : WB Saunders; ; 2001. .
    1. Vanacore N, Rastelli E, Antonini G, et al. . An age-standardized prevalence estimate and a sex and age distribution of myotonic dystrophy types 1 and 2 in the Rome Province, Italy . Neuroepidemiology. 2016. ; 46 ( 3 ): 191 – 197 . - PubMed
    1. Johnson NE . Myotonic muscular dystrophies . Continuum (Minneap Minn). 2019. ; 25 ( 6 ): 1682 – 1695 . - PubMed
    1. Yu H, Laberge L, Jaussent I, et al. . Daytime sleepiness and REM sleep characteristics in myotonic dystrophy: a case-control study . Sleep. 2011. ; 34 ( 2 ): 165 – 170 . - PMC - PubMed
    1. Laberge L, Gagnon C, Jean S, Mathieu J . Fatigue and daytime sleepiness rating scales in myotonic dystrophy: a study of reliability . J Neurol Neurosurg Psychiatry. 2005. ; 76 ( 10 ): 1403 – 1405 . - PMC - PubMed

Publication types