Do Psychosocial Interventions Improve Quality of Life and Wellbeing in Adults with Neuromuscular Disorders? A Systematic Review and Narrative Synthesis.

Quality of life and well-being are frequently restricted in adults with neuromuscular disorders. As such, identification of appropriate interventions is imperative. The objective of this paper was to systematically review and critically appraise quantitative studies (RCTs, controlled trials and cohort studies) of psychosocial interventions designed to improve quality of life and well-being in adults with neuromuscular disorders. A systematic review of the published and unpublished literature was conducted. Studies meeting inclusion criteria were appraised using a validated quality assessment tool and results presented in a narrative synthesis. Out of 3,136 studies identified, ten studies met criteria for inclusion within the review. Included studies comprised a range of interventions including: cognitive behavioural therapy, dignity therapy, hypnosis, expressive disclosure, gratitude lists, group psychoeducation and psychologically informed rehabilitation. Five of the interventions were for patients with Amyotrophic Lateral Sclerosis (ALS). The remainder were for patients with post-polio syndrome, muscular dystrophies and mixed disorders, such as Charcot-Marie-Tooth disease, myasthenia gravis and myotonic dystrophy. Across varied interventions and neuromuscular disorders, seven studies reported a short-term beneficial effect of intervention on quality of life and well-being. Whilst such findings are encouraging, widespread issues with the methodological quality of these studies significantly compromised the results. There is no strong evidence that psychosocial interventions improve quality of life and well-being in adults with neuromuscular disorders, due to a paucity of high quality research in this field. Multi-site, randomised controlled trials with active controls, standardised outcome measurement and longer term follow-ups are urgently required.

Mindfulness Interventions in Physical Rehabilitation: A Scoping Review.

A scoping review was conducted to describe how mindfulness is used in physical rehabilitation, identify implications for occupational therapy practice, and guide future research on clinical mindfulness interventions. A systematic search of four literature databases produced 1,524 original abstracts, of which 16 articles were included. Although only 3 Level I or II studies were identified, the literature included suggests that mindfulness interventions are helpful for patients with musculoskeletal and chronic pain disorders and demonstrate trends toward outcome improvements for patients with neurocognitive and neuromotor disorders. Only 2 studies included an occupational therapist as the primary mindfulness provider, but all mindfulness interventions in the selected studies fit within the occupational therapy scope of practice according to the American Occupational Therapy Association's Occupational Therapy Practice Framework: Domain and Process. Higher-level research is needed to evaluate the effects of mindfulness interventions in physical rehabilitation and to determine best practices for the use of mindfulness by occupational therapy practitioners.

Increased self-transcendence in patients with intractable diseases.

AIMS: Patients with intractable disease require long-term treatment and experience repeated bouts of progressive symptoms and resolutions, which cause them severe suffering. The aim of this study was to elucidate the concepts of self-transcendence and subjective well-being in patients with intractable disease. METHODS: Forty-four patients with intractable disease (men/women: 22/22) participated. The diseases of the participants were classified into five systems: (i) neural/muscle system; (ii) digestive system; (iii) immunity/blood system; (iv) visual system; and (v) bone/joint system. The controls were 1854 healthy individuals (men/women: 935/869). Participants completed the Self-Transcendence Scale (STS) and the Japanese version of the World Health Organization-Subjective Inventory. The Japanese version of the Mini-International Neuropsychiatric Interview was also used for the intractable disease group. RESULTS: Analysis of covariance found a significant increase in STS score among the intractable disease group (P < 0.001). Multiple regression analysis showed that the positive affect measured by the World Health Organization-Subjective Inventory showed the greatest effect on the STS score for the intractable disease group (ß = 0.539, P < 0.001). CONCLUSION: As a life-changing experience, an intractable disease may influence an increase in self-transcendence. The results also showed that there was a strong correlation between self-transcendence and respondents' subjective well-being. Our results suggest that patients with life-changing intractable disease can have a high level of self-transcendence, which may lead them to regain mental well-being, and increase their psychological health even in situations that cause physical and mental suffering.

Is there a role for palliative care in progressive pediatric neuromuscular diseases? The answer is "Yes!

The consequences of prolongation of survival can be oversimplified, for example, by equating technologically prolonged survival with indefinitely prolonged high quality of life. When this oversimplified view is embraced, the prognosis of ultimately fatal diseases like DMD may be viewed with unrealistic optimism and palliative care may seem irrelevant or misguided. However, we have shown that the sequelae of prolonged survival are complex. For example, NPPV does not protect prolonged survivors of progressive NMDs from potentially debilitating medical complications that can cause elevated burden of disease, high burden of care, and the potential for impaired quality of life. Also, the sequelae of prolonged survival can negatively affect a wide variety of stakeholders, including patients and their families, medical professionals, and society. It is our view that, when the implications of prolonged survival are examined carefully, their complexity is revealed, and the potential for palliative care to provide support and to relieve suffering in prolonged survivors of progressive NMDs becomes apparent. Thus, we advocate development of an integrative care model for patients with progressive NMDs, blending technological therapies with adoption of palliative strategies as patients approach end of life.

Camptocormia treated with bilateral pallidal stimulation.

The authors report the neurological, neurophysiological, and neuropsychological effects of using long-term bilateral pallidal high-frequency deep brain stimulation (DBS) in a case of disabling camptocormia. Deep brain stimulation electrodes were implanted stereotactically to target the globus pallidus internus (GPi) bilaterally. Local field potentials (FPs) were recorded using the DBS electrodes and concurrent abdominal flexor electromyography (EMG) potentials during camptocormic episodes. Videotaped assessments of the movement disorder and neuropsychological evaluations of the patient before implantation and 6 months after initiation of pallidal stimulation were recorded. There was significant functional improvement following long-term pallidal stimulation, and some improvement was noted in neuropsychological scores. A temporal correlation between the GPi FPs and EMG-recorded rectus abdominis potentials was evident. There were no treatment-related adverse effects. The authors have found that long-term pallidal stimulation was safe and offered functional benefit to a patient with this severely disabling condition. The physiological studies may help further the understanding of the pathophysiology of this rare entity.

[Neuropsychological syndromes in hereditary neuromuscular pathology]. / Nervno-psikhicheskie sindromy pri nasledstvennoi nervno-myshechnoi patologii.

Clinical and experimental psychological studies (MMPI, Eisenks's questionnaire, methods by Luria and Kraepelin, types of attitude toward disease) carried out in 157 adults and children with progressive myodystrophies and amyotrophies revealed alterations in the neuropsychic sphere in 134 patients (85%). In the structure of borderline disorders, depressive disturbances prevailed (54.5%), and the asthenic and psychopathlike symptomatology could be seen. The use of the psychopharmacological and psychotherapeutic correction promoted regression of the psychopathological symptomatology in 76% of adults and in 84% of children.

Emic accounts of a mystery illness: the Groote Eylandt syndrome.

The Aboriginal people of Groote Eylandt, in the Northern Territory of Australia, are suffering from an unusual disease complex having neurological, psychiatric and teratological features, which admits no ready explanation. The island people at various times blame it on the spirits, or accuse enemies, or take some responsibility upon themselves. In this paper, 'emic' accounts of the illness (those current among the members of the society) are described in order to compare them with 'etic' accounts of those who study the society from the outside. Since emic views regulate people's behaviours toward illness, it is suggested these views should complement and inform etic views of researchers and therapists. This principle might apply to all mysterious or poorly understood illness.