Evolution of Sleep Complaints in Myotonic Dystrophy Type 1: A 9-Year Longitudinal Study.

The objective was to characterize the progression of sleep complaints in 115 dystrophy type 1 (DM1) patients who filled out a sleep questionnaire twice at a 9-year interval. Daytime napping (22.1% vs. 34.5%, p < 0.05), early awakenings (11.4% vs 21.1%, p < 0.05), nonrestorative sleep (39.5% vs 51.8%, p < 0.05), stimulant use (7.0% vs 19.3%, p < 0.01), breathing cessation (10.7% vs 23.2%, p < 0.01), and nighttime urination (42.5% vs 54.9%, p < 0.05) increased between Time 1 and Time 2. Sleep-related complaints are prominent and augment rapidly in DM1 patients. Physicians need to better identify and treat them to help alleviate the burden they impose on patients and their caregivers.

Évolution des troubles du sommeil dans la dystrophie myotonique de type 1 : une étude longitudinale de 9 ans.L'objectif était de caractériser l'évolution des plaintes liées au sommeil chez 115 patients atteints de dystrophie myotonique de type 1 (DM1) ayant rempli un questionnaire sur le sommeil à deux reprises à 9 ans d'intervalle. La prévalence des siestes (22,1 % vs 34,5 %, p < 0,05), des réveils matinaux précoces (11,4 % vs 21,1 %, p < 0,05), du sommeil non réparateur (39,5 % vs 51,8 %, p < 0,05), de la consommation de stimulants (7,0 % vs 19,3 %, p < 0,01), des arrêts respiratoires (10,7 % vs 23,2 %, p < 0,01) et des mictions nocturnes (42,5 % vs 54,9 %, p < 0,05) a augmenté entre le temps 1 et le temps 2. Les plaintes liées au sommeil sont fréquentes et augmentent rapidement dans la DM1. Les médecins doivent mieux les identifier et les traiter pour aider à alléger le fardeau qu'ils imposent aux patients et à leurs aidants.

A Blue-Enriched Light Intervention Counteracts the Alertness Decrement Among Mine Workers on Extended 12-Hour Night Shift Periods.

OBJECTIVE: The aim of the study is to assess whether a blue-enriched light intervention improves nocturnal alertness and daytime sleep of night workers. METHODS: Thirteen miners performing 12-hour night shifts for 12 consecutive nights were exposed to a baseline and a blue-enriched light condition. All subjects wore an actigraph and completed a Psychomotor Vigilance Task at the beginning and at the end of each shift. Data were analyzed with linear mixed models. RESULTS: In the blue-enriched light condition, the daily increase in median reaction time (RT), mean RT, slowest 10% of RT, and fastest 10% of RT was lower than that observed in the baseline condition between day 1 and 12 ( P ≤ 0.05). CONCLUSIONS: The addition of blue-enriched light during a long period of extended night shifts counteracts most of the daily decline in nocturnal alertness observed in the standard lighting condition, irrespectively of sleep duration and sleep efficiency.

Impact of a 12-week Strength Training Program on Fatigue, Daytime Sleepiness, and Apathy in Men with Myotonic Dystrophy Type 1.

BACKGROUND: Myotonic dystrophy type 1 (DM1) is a multisystemic neuromuscular disorder causing a plea of impairments, of which fatigue and apathy are some of the most frequent non-muscular symptoms. No curative treatment exists to date, and patients only have access to limited effective care, which are intended to decrease the burden of specific symptoms in daily life. OBJECTIVE: This study aimed to assess whether a 12-week strength training program has an impact on fatigue/daytime sleepiness, apathy, and disease bruden in men with DM1. METHODS: Eleven participants completed the Fatigue and Daytime Sleepiness Scale (FDSS) and the Myotonic Dystrophy Health Index (MDHI) at baseline, at 6 and 12 weeks, and at 6 and 9 months. Also, the Apathy Evaluation Scale (AES) was filled out at baseline, at 12 weeks, and at 6 and 9 months. RESULTS: Results show significant effects of the training program both on apathy and fatigue/daytime sleepiness, effects that are respectively greater at three and six months after the end of the program than at its very end. However, no difference was observed regarding the overall disease burden. CONCLUSION: These findings are promising for patients with DM1 considering that few non-pharmacological treatments are available.

Predictors of participation restriction over a 9-year period in adults with myotonic dystrophy type 1.

PURPOSE: For slowly progressive neuromuscular disease, prognostic approach and long-term monitoring of participation is a crucial part of rehabilitation services. To improve the prognostic approach, professionals must identify individuals at risk of having higher participation restriction. This study aimed to identify personal and environmental predictors of participation restriction over nine years in adults with myotonic dystrophy type 1 (DM1). METHODS: A secondary analysis of a longitudinal design comparing baseline with a follow-up nine years later was used with a multidimensional assessment of participation and personal and environmental factors. Based on theoretical models, multiple linear regressions were used. RESULTS: One hundred and fourteen adults with DM1 were included in the study (63.2% women; 78.9% adult onset; mean (SD) age of 43.5 (10.4) years). When age, sex, phenotype, and education were controlled for, participation restriction was predicted by a longer time to stand and walk, lower grip strength, higher body mass index, absence of perceived impact of myotonia in daily living, use of adapted transportation from community services, and perception of obstacle in physical environment (p < 0.001, adjusted R2 = 0.50). CONCLUSIONS: The majority of predictors of participation restriction can be advantageously modified by rehabilitation and environmental changes, such as politics targeting community services provision or physical environment and services accessibility.Implications for rehabilitationPredictors could better inform rehabilitation professional to recognize individuals at risk of higher participation restriction over time and to target specific interventions based on a prognostic approach.Rehabilitation professionals could inform the people living with myotonic dystrophy type 1 and their relatives of the multifactorial nature of occurrence of participation restriction to diminish the "fatality" associated with a genetic progressive disorder.Predictors allow professionals to assess and intervene in the management of specific factors depending on the rehabilitation goal.Identifying individual with myotonic dystrophy with higher risk of participation restriction could help implement a long-term community based rehabilitation intervention plan targeting both personal and environmental factors.

Responsiveness of Daytime Sleepiness and Fatigue Scales in Myotonic Dystrophy Type 1.

Daytime sleepiness and fatigue are prominent symptoms of myotonic dystrophy type 1 (DM1) that can be amenable to treatment in the context of randomized controlled trials. No study has yet documented whether self-reported measures of daytime sleepiness and fatigue can detect change over time and the meaning of this change. The aim was to explore indicators of responsiveness to change and interpretability for the Daytime Sleepiness Scale and the Fatigue Severity Scale in 115 DM1 prospectively followed patients. Results suggest that these two self-reported questionnaires are sufficiently sensitive to detect changes beyond expected measurement error over time in this population.

Timely use of in-car dim blue light and blue blockers in the morning does not improve circadian adaptation of fast rotating shift workers.

Circadian adaptation to night work usually does not occur in naturalistic conditions, largely due to exposure to low levels of light during the night and light in the morning on the way home. This leads to circadian misalignment, which has documented deleterious effects on sleep and functioning during waking hours. Chronic circadian misalignment is also being increasingly associated with long-term health comorbidities. As the circadian system is mostly sensitive to short wavelengths (i.e., blue light) and less sensitive to long wavelengths (i.e., red light), shaping light exposure in a "wavelength-wise" manner has been proposed to promote partial adaptation to night shifts, and, therefore, alleviate circadian rhythms disruption. This report presents results from two cross-over designed studies that aimed to investigate the effects of three different light conditions on circadian phase, sleepiness, and alertness of police patrol officers on a rotating shift schedule. The first study took place during summer (n = 15) and the second study (n = 25) during winter/early spring. In both studies, all participants went through three conditions composed of four consecutive night shifts: 1) in-car dim blue light exposure during the night shift and wearing of blue-blocking glasses (BBG) in the morning after 05:00 h; 2) in-car red light exposure during the night shift and wearing of BBG in the morning after 05:00 h; 3) a control condition with no intervention. To assess circadian phase position, salivary melatonin was collected hourly the night before and the night after each condition. Sleep was monitored by wrist actigraphy. Also, a 10-min Psychomotor Vigilance-Task was administered at the beginning and end of each night shift and the Karolinska Sleepiness Scale was completed every 2 h during each night shift. In the summer study, no difference was found in alertness and sleepiness between conditions. Participants though exhibited greater (≈3 h) phase delay after four consecutive night shifts in the control condition (in which morning light exposure was expected to prevent phase delay) than after the blue and red conditions (≈2 h) (in which wearing BBG were expected to promote phase delay). In the second study performed during the winter/early spring, a comparable ≈2 h phase delay was found in each of the three conditions, with no difference in alertness and sleepiness between conditions. In conclusion, participants in both studies exhibited modest phase delay across the four night shifts, even during the control conditions. Still, re-entrainment was not fast enough to produce partial circadian adaptation after four night shifts. A greater number of consecutive night shifts may be necessary to produce enough circadian alignment to elicit benefits on sleepiness and alertness in workers driving a motorized vehicle during night shifts. In-car dim blue light exposure combined with the wearing of BBG in the morning did not show the expected benefits on circadian adaptation, sleepiness, and alertness in our studies. Higher levels of light may be warranted when implementing light intervention in a motorized vehicle setting.

Good Sleep Quality and Progressive Increments in Vigilance During Extended Night Shifts: A 14-Day Actigraphic Study in Underground Miners.

OBJECTIVE: Assess the change in sleep and vigilance of underground miners during long periods of extended shifts. METHODS: Seventy miners worked 14 consecutive 12-hour day and/or night shifts. Also, they wore an actigraph and completed a visual analog scale for vigilance four times per shift. Linear regression models with mixed effects were used. RESULTS: Sleep efficiency was higher during day shifts than during night shifts (86,5 vs 85.5, P < 0.05) but sleep duration did not differ (6:34 vs 6:44, n.s.). Mean vigilance level at Time 3 (02h00) was significantly lower than that at Time 1 (19h00) during the first 10 night shifts whereas mean vigilance level at Time 4 (05h30) remained significantly lower for the 14 night shifts. CONCLUSIONS: Underground miners exhibit good sleep quality despite evidence of limited circadian adaptation in terms of nighttime vigilance.

Predicting daytime sleepiness and fatigue: a 9-year prospective study in myotonic dystrophy type 1.

OBJECTIVE: Daytime sleepiness and fatigue are prominent symptoms of myotonic dystrophy type I (DM1) that exact a heavy toll on patients' quality of life, but information is scarce on their predictive factors. This study aimed to determine factors that may influence levels of daytime sleepiness and fatigue in a large cohort of DM1 patients followed for 9 years. METHODS: This study included 115 patients with DM1 at baseline (Time 1, T1) and at Time 2 (T2) who were questioned for daytime sleepiness, fatigue, history of depression, psychological distress, pain, hypothyroidism, and sleep habits. Also, their muscular impairment and intellectual quotient were evaluated. Regression models were used to identify correlates of daytime sleepiness and fatigue while controlling for time effect. RESULTS: Both daytime sleepiness and fatigue increased between T1 and T2, but their rate of change are higher when CTG repeat number is higher (p < 0.05). Also, higher psychological distress level is associated with higher daytime sleepiness and fatigue levels both at T1 and T2 (p < 0.01). Moreover, patients with a history of depression report higher daytime sleepiness levels both at T1 and T2 (p < 0.05). In addition, patients with higher fatigue levels both at T1 and T2 have more severe muscular impairment (p < 0.01) and report a longer habitual sleep duration (p < 0.05). Finally, a higher BMI and a history of hypothyroidism predict higher daytime sleepiness levels at T2 (p < 0.05). CONCLUSION: This study identified potentially modifiable risk factors of future daytime sleepiness and fatigue in DM1 patients, including BMI, psychological distress, hypothyroidism, and sleep habits.