Fatigue and daytime sleepiness in patients with myotonic dystrophy type 1: To lump or split?

Laberge, Luc; Dauvilliers, Yves; Bégin, Paul; Richer, Louis; Jean, Stéphane; Mathieu, Jean

doi:10.1016/j.nmd.2009.03.007

« Previous Next »Neuromuscular Disorders
Volume 19, Issue 6 , Pages 397-402, June 2009

Fatigue and daytime sleepiness in patients with myotonic dystrophy type 1: To lump or split?

Luc Laberge
- ÉCOBES, Cégep de Jonquière, Jonquière, Qc, Canada
- Département des sciences de l’éducation et de psychologie, Université du Québec à Chicoutimi, 555, boul. de l’Université, Chicoutimi, Qc, Canada G7H 2B1
- Centre hospitalier affilié universitaire régional (CAUR) de Chicoutimi, Centre de Santé et de Services Sociaux de Chicoutimi, Chicoutimi, Qc, Canada
- Corresponding author. Address: Département des sciences de l’éducation et de psychologie, Université du Québec à Chicoutimi, 555, boul. de l’Université, Chicoutimi, Qc, Canada G7H 2B1. Tel.: +1 418 547 2191x489; fax: +1 418 542 2853.
Yves Dauvilliers
- Service de Neurologie, CHU Montpellier, INSERM, U888, Université de Montpellier 1, France
Paul Bégin
- Centre hospitalier affilié universitaire régional (CAUR) de Chicoutimi, Centre de Santé et de Services Sociaux de Chicoutimi, Chicoutimi, Qc, Canada
Louis Richer
- Département des sciences de l’éducation et de psychologie, Université du Québec à Chicoutimi, 555, boul. de l’Université, Chicoutimi, Qc, Canada G7H 2B1
Stéphane Jean
- Clinique des maladies neuromusculaires, Centre de Santé et de Services Sociaux de Jonquière, Jonquière, Qc, Canada
Jean Mathieu
- Clinique des maladies neuromusculaires, Centre de Santé et de Services Sociaux de Jonquière, Jonquière, Qc, Canada

Received 26 February 2008; received in revised form 18 February 2009; accepted 23 March 2009.

Abstract

We assessed the relationship and clinical correlates of fatigue and Excessive Daytime Sleepiness (EDS) in 200 myotonic dystrophy type 1 (DM1) patients by means of questionnaire and neuropsychological evaluation. Fatigue levels were higher in patients with EDS and daytime sleepiness levels higher in patients with excessive fatigue. However, EDS without fatigue was rarely observed. Also, DM1 patients with fatigue (with or without EDS) showed greater muscular impairment, CTG repeats, abnormalities regarding personality, depressive symptoms and lower health-related quality of life (HRQoL) than patients without these symptoms. These findings do not readily support the contention that fatigue and EDS constitute distinct clinical manifestations in DM1. Clinicians should systematically evaluate both symptoms since fatigue and EDS have a greater impact on HRQoL than fatigue alone. However, specific rating scales for fatigue in DM1 have yet to be devised.

Keywords: Myotonic dystrophy, Fatigue, Hypersomnia, Personality, Depressive symptoms, IQ, Health-related quality of life (HRQoL)