**6. Interventions**

DM1 leads to substantial physical impairments, which in combination with the neuropsychological effects of the condition results in severely restricted social participation. However, there is little evidence for the efficacy of rehabilitative approaches designed to improve health status. Previous studies have demonstrated that fatigue is a highly prevalent, debilitating symptom of DM1 [53, 54], and cognitive behavioral therapy reduces fatigue and increases objective activity, as well as social participation in patients with facioscapulohumeral muscular disease [55]. Therefore, Okkersen et al. [56] conducted a large randomized trial to determine whether cognitive behavioral therapy plus optional graded exercise improved the health status of patients with DM1 compared to standard care alone.

#### **6.1 Cognitive behavioral therapy for the patients with DM1**

The study by Okkersen et al. [56] was a large-scale, multicenter, single-blind, randomized trial conducted at four neuromuscular referral centers located in France, Germany, Netherlands, and the UK, which was known as Observational

**45**

**7. Conclusion**

*Cognitive Function and Quality of Life of Muscular Dystrophy*

Prolonged Trial In Myotonic Dystrophy Type 1 to Improve Quality of Life-Standards, a Target Identification Collaboration (OPTIMISTIC). Participants (N = 255) were aged 18 years and older with a confirmed genetic diagnosis of DM1, who were severely fatigued (CIS-fatigue scale, score ≧35) but able to walk independently. They were randomly assigned to either cognitive behavioral therapy plus standard care and optional graded exercise (n = 128) or standard care alone (n = 127). Cognitive behavioral therapy focused on addressing the reduced initiative in the patients, increasing physical activity, optimizing social interactions, regulating sleep–wake patterns, coping with pain, and beliefs about fatigue and DM1. Cognitive behavioral therapy was delivered over a 10-month period in 10–14 sessions based on a manual, by therapists that had extensive training. It was possible to include a graded exercise module that was individually tailored and incorporated moderate-intensity exercises such as walking, cycling, jogging, or dancing for a

The active-c score of participants in cognitive behavioral therapy increased from a mean (SD) of 61.22 (17.35) at baseline to 63.92 (17.41) at the 10th month. However, the score decreased from 63.00 (17.35) to 60.79 (18.49) in the standard care group. The mean difference between the groups was 3.27 and significant (p = 0.007). As secondary outcomes, the cognitive behavior therapy group showed significant differences in the 6-minute walk test, the fatigue and daytime sleepiness scales, CIS-fatigue, and daily activity levels. Moreover, both groups had decreased scores in the myotonic dystrophy health index and INQOL. However, there was no significant difference between the groups. Also, there were no changes or no differences between the groups on the apathy scale, Stroop-color-word interference, accelerometry for the least active 5 hours, or the Beck Depression Inventory. Based on these results, Okkersen et al. [56] emphasized that cognitive behavioral therapy could increase the capacity for activity and social participation in severely fatigued patients with DM1. This study showed that cognitive behavioral therapy could be one effective intervention for improving the health status of patients with DM1.

Cognitive impairments are observed in patients with DMD and DM1. These impairments are caused by gene mutations, especially by CNS-expressed isoforms. These impairments, however, do not encompass every aspect of their intellectual ability. Patients with DMD show deficits in sequential information processing and alterations of attention and processing speed. Moreover, patients with DM1 have weaknesses in executive function, processing speed, attention, and visuoconstructive abilities. These cognitive impairments are related to their psychosocial characteristics, social participation, and the QOL. Especially, apathy, depression, and fatigue are the key factors that deteriorate the QOL of patients with DM1. It is suggested that precisely targeted cognitive assessments and cognitive intervention

are necessary to provide them with better care and improve their QOL.

*DOI: http://dx.doi.org/10.5772/intechopen.86222*

minimum of 30 minutes, three times a week.

#### *Cognitive Function and Quality of Life of Muscular Dystrophy DOI: http://dx.doi.org/10.5772/intechopen.86222*

Prolonged Trial In Myotonic Dystrophy Type 1 to Improve Quality of Life-Standards, a Target Identification Collaboration (OPTIMISTIC). Participants (N = 255) were aged 18 years and older with a confirmed genetic diagnosis of DM1, who were severely fatigued (CIS-fatigue scale, score ≧35) but able to walk independently. They were randomly assigned to either cognitive behavioral therapy plus standard care and optional graded exercise (n = 128) or standard care alone (n = 127). Cognitive behavioral therapy focused on addressing the reduced initiative in the patients, increasing physical activity, optimizing social interactions, regulating sleep–wake patterns, coping with pain, and beliefs about fatigue and DM1. Cognitive behavioral therapy was delivered over a 10-month period in 10–14 sessions based on a manual, by therapists that had extensive training. It was possible to include a graded exercise module that was individually tailored and incorporated moderate-intensity exercises such as walking, cycling, jogging, or dancing for a minimum of 30 minutes, three times a week.

The active-c score of participants in cognitive behavioral therapy increased from a mean (SD) of 61.22 (17.35) at baseline to 63.92 (17.41) at the 10th month. However, the score decreased from 63.00 (17.35) to 60.79 (18.49) in the standard care group. The mean difference between the groups was 3.27 and significant (p = 0.007). As secondary outcomes, the cognitive behavior therapy group showed significant differences in the 6-minute walk test, the fatigue and daytime sleepiness scales, CIS-fatigue, and daily activity levels. Moreover, both groups had decreased scores in the myotonic dystrophy health index and INQOL. However, there was no significant difference between the groups. Also, there were no changes or no differences between the groups on the apathy scale, Stroop-color-word interference, accelerometry for the least active 5 hours, or the Beck Depression Inventory. Based on these results, Okkersen et al. [56] emphasized that cognitive behavioral therapy could increase the capacity for activity and social participation in severely fatigued patients with DM1. This study showed that cognitive behavioral therapy could be one effective intervention for improving the health status of patients with DM1.
