TY - JOUR
T1 - Restless legs syndrome and daytime sleepiness are prominent in myotonic dystrophy type 2
AU - Lam, Erek M.
AU - Shepard, Paul W.
AU - Louis, Erik K.St
AU - Dueffert, Lucas G.
AU - Slocumb, Nancy
AU - McCarter, Stuart J.
AU - Silber, Michael H.
AU - Boeve, Bradley F.
AU - Olson, Eric J.
AU - Somers, Virend K.
AU - Milone, Margherita
PY - 2013/7/9
Y1 - 2013/7/9
N2 - Objectives: Although sleep disturbances are common in myotonic dystrophy type 1 (DM1), sleep disturbances in myotonic dystrophy type 2 (DM2) have not been well-characterized. We aimed to determine the frequency of sleep disturbances in DM2. Methods: We conducted a case-control study of 54 genetically confirmed DM2 subjects and 104 medical controls without DM1 or DM2, and surveyed common sleep disturbances, including symptoms of probable restless legs syndrome (RLS), excessive daytime sleepiness (EDS), sleep quality, fatigue, obstructive sleep apnea (OSA), probable REM sleep behavior disorder (pRBD), and pain. Thirty patients with DM2 and 43 controls responded to the survey. Group comparisons with parametric statistical tests and multiple linear and logistic regression analyses were conducted for the dependent variables of EDS and poor sleep quality. Results: The mean ages of patients with DM2 and controls were 63.8 and 64.5 years, respectively. Significant sleep disturbances in patients with DM2 compared to controls included probable RLS (60.0% vs 14.0%, p < 0.0001), EDS (p < 0.001), sleep quality (p 5 0.02), and fatigue (p < 0.0001). EDS and fatigue symptoms were independently associated with DM2 diagnosis (p < 0.01) after controlling for age, sex, RLS, and pain scores. There were no group differences in OSA (p 5 0.87) or pRBD (p 5 0.12) scores. Conclusions: RLS, EDS, and fatigue are frequent sleep disturbances in patients with DM2, while OSA and pRBDsymptoms are not. EDSwas independently associatedwithDM2 diagnosis, suggesting possible primary CNS hypersomnia mechanisms. Further studies utilizing objective sleep measures are needed to better characterize sleep comorbidities in DM2.
AB - Objectives: Although sleep disturbances are common in myotonic dystrophy type 1 (DM1), sleep disturbances in myotonic dystrophy type 2 (DM2) have not been well-characterized. We aimed to determine the frequency of sleep disturbances in DM2. Methods: We conducted a case-control study of 54 genetically confirmed DM2 subjects and 104 medical controls without DM1 or DM2, and surveyed common sleep disturbances, including symptoms of probable restless legs syndrome (RLS), excessive daytime sleepiness (EDS), sleep quality, fatigue, obstructive sleep apnea (OSA), probable REM sleep behavior disorder (pRBD), and pain. Thirty patients with DM2 and 43 controls responded to the survey. Group comparisons with parametric statistical tests and multiple linear and logistic regression analyses were conducted for the dependent variables of EDS and poor sleep quality. Results: The mean ages of patients with DM2 and controls were 63.8 and 64.5 years, respectively. Significant sleep disturbances in patients with DM2 compared to controls included probable RLS (60.0% vs 14.0%, p < 0.0001), EDS (p < 0.001), sleep quality (p 5 0.02), and fatigue (p < 0.0001). EDS and fatigue symptoms were independently associated with DM2 diagnosis (p < 0.01) after controlling for age, sex, RLS, and pain scores. There were no group differences in OSA (p 5 0.87) or pRBD (p 5 0.12) scores. Conclusions: RLS, EDS, and fatigue are frequent sleep disturbances in patients with DM2, while OSA and pRBDsymptoms are not. EDSwas independently associatedwithDM2 diagnosis, suggesting possible primary CNS hypersomnia mechanisms. Further studies utilizing objective sleep measures are needed to better characterize sleep comorbidities in DM2.
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U2 - 10.1212/WNL.0b013e31829a340f
DO - 10.1212/WNL.0b013e31829a340f
M3 - Article
C2 - 23749798
AN - SCOPUS:84881302437
SN - 0028-3878
VL - 81
SP - 157
EP - 164
JO - Neurology
JF - Neurology
IS - 2
ER -