**7. Conclusion**

*Muscular Dystrophies*

QOL. Therefore, the psychological interventions for DM1 should incorporate these

In clinical practice, DM1 patients commonly showed less awareness of the disease distress and its progression. This is named anosognosia or lack of awareness, which can lead to misattributions of symptoms, delay of diagnostic procedures, and low compliance with treatment. The lack of awareness about their illness often is observed in individuals with brain diseases and neurodegenerative disorders, such as Alzheimer's diseases and acquired brain injury. In these disorders, the lack of awareness can be a direct consequence of the underlying pathological process [50]. Research on brain injuries suggests that the prefrontal cortex plays a crucial role in

Baldanzi et al. [52] conducted a study to estimate the prevalence of disease awareness in 65 adult patients with DM1. The degree of awareness was evaluated by comparing motor impairments using MIRS, patients' complaints about their symptoms, and by comparing INQOL between caregivers. The results indicated that 51.6% of patients were unaware of the disease, and the lack of awareness was most prominent in Independence (52.4%) and Social Relationship (47.6%) domains. Moreover, the lack of awareness was significantly related to failures in cognitive test performance, specifically in the domains of visuospatial memory, cognitive flexibility, and conceptualization. Baldanzi et al. suggested that gaining a better understanding of anosognosia would be useful for the medical management of patients with DM1 and for providing guidance for occupational and social 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

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

health status of patients with DM1 compared to standard care alone.

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

Minier et al. [49] conducted a systematic literature review of psychopathological features in DM1 and reported that patients with DM1 present mild psychopathological problems, such as interpersonal difficulties, lack of interest, dysphoria, concern about bodily functioning, and hypersensibility. However, they do not present more psychiatric disorders than the general population, except for personality disorders and depression. Moreover, avoidant personality disorder was the most common of

factors as potential targets for improving patients' QOL.

several personality disorders among DM1 patients.

**5.1 Lack of awareness about the illness**

maintaining awareness [51].

**6. Interventions**

**44**

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.

*Muscular Dystrophies*
