Hybrid Assistive Limb® for sporadic inclusion body myositis: A case series.

We evaluated the efficacy of rehabilitation therapy with Hybrid Assistive Limb® (HAL; hereafter HAL therapy) in three patients diagnosed with sporadic inclusion body myositis (sIBM) who were hospitalized to undergo HAL therapy. Among them, one patient participated in eight courses and the other two in two courses of HAL therapy between 2017 and 2020. We determined the mean rate of improvement in two-minute walking distance and 6 m walking speed at the time of hospital discharge. After HAL therapy, we confirmed the patients' desire to continue the use of HAL. In one patient, we observed improvements of 146.0% and 120.0% in two-minute walk and 6 m walking speed, respectively, after the first course of HAL therapy; these values are 133.7% and 130% after the eighth course of HAL therapy. These values exceeded 90% in the other two patients after the second course of HAL therapy. HAL therapy maintained both quantity and quality of ambulation and showed positive psychological effects on patient conditions because it reduces exercise load and facilitates safety. While HAL therapy might be effective in maintaining and improving ambulation in patients with sIBM, we should consider to discontinue HAL therapy as it increased risk of falling.

Exploring the efficacy of the expiratory muscle strength trainer to improve swallowing in inclusion body myositis: A pilot study.

Inclusion Body Myositis (IBM) is the most common acquired myopathy in older individuals with more than two thirds of patients experiencing impaired swallowing. There are currently no standardized exercise therapies to improve or sustain swallowing despite good evidence for exercise therapy in limb muscles. Reduced upper esophageal sphincter opening is a common abnormality associated with dysphagia in IBM. This pilot study recruited IBM patients with abnormal upper esophageal sphincter function and dysphagia into an exercise program. It was hypothesized that regular practice using the Expiratory Muscle Strength Trainer (EMST) device would improve hyolaryngeal movement by strengthening suprahyoid musculature and facilitate opening of the upper esophageal sphincter thereby improving swallowing and quality of life. Overall, IBM patients who used the EMST device demonstrated no improvement in swallowing function. Consistent with that result, there was also no change in measures of quality of life. However, further studies are needed to elucidate whether it has a preventative role in the development or progression of dysphagia in IBM as there is a suggestion that patients with a shorter duration of disease may have had some benefit. This research provides pilot data and recommendations that will guide future studies on exercise therapy and swallowing in this area.

Self-management program improves participation in patients with neuromuscular disease: A randomized controlled trial.

OBJECTIVE: To investigate the effectiveness of Energetic, a self-management group program combining aerobic training, energy conservation management, and relapse prevention to improve social participation in patients with neuromuscular disease (NMD) and chronic fatigue. METHODS: In this multicenter, assessor-blinded, 2-armed randomized controlled trial with repeated measurements, 53 patients with various types of NMD and chronic fatigue were randomly allocated to Energetic, a 4-month group intervention, or to usual care. The primary endpoint was social participation assessed with the Canadian Occupational Performance Measure (COPM) performance scale immediately postintervention. Secondary outcomes included COPM satisfaction scale, 6-Minute Walk Test (6MWT), and Checklist Individual Strength-subscale fatigue. Participants were followed for 11 months postintervention. Data were analyzed with linear models that account for repeated measurements. RESULTS: Directly after intervention, the mean group difference for COPM-performance was 1.7 (95% confidence interval [CI] 1.0-2.4; p < 0.0001) in favor of the intervention group (n = 29), adjusted for baseline, sex, diagnosis, and work status. This effect was retained at 11 months follow-up (0.9; 95% CI 0.0-1.7; p = 0.049). The COPM satisfaction scale and 6MWT improved more in the intervention group compared to usual care. After 3 and 11 months follow-up, most beneficial effects on social participation and functional endurance were retained. CONCLUSION: Energetic led to sustainable improvements in social participation and functional endurance compared to usual care in patients with NMD and chronic fatigue.Clinicaltrials.gov IDENTIFIER: NCT02208687. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that a combination of aerobic training, energy conservation management, and relapse prevention improves social participation in patients with NMD and chronic fatigue.

A 63-Year-Old Man Presents With Slowly Progressive Dyspnea on Exertion and Lower Extremity Muscle Weakness.

CASE PRESENTATION: A 63-year-old man was referred for slowly progressive dyspnea on exertion that had developed over 7 years. Dyspnea was initially only present during high-intensity physical activity, but was now present while walking rapidly on a flat surface. Symptoms were accentuated while supine and when bending forward. He reported respiratory difficulties when submerged in water and a recent onset of slight symmetric lower limb weakness that was only apparent during strenuous physical activity. He also had OSA, which was adequately controlled with continuous positive airway pressure therapy. Neurologic and rheumatologic histories were otherwise unremarkable. He denied any impact accidents or trauma to the cervical spine and prior neck or thoracic surgeries.

Blood flow restriction training in clinical musculoskeletal rehabilitation: a systematic review and meta-analysis.

BACKGROUND AND OBJECTIVE: Low-load exercise training with blood flow restriction (BFR) can increase muscle strength and may offer an effective clinical musculoskeletal (MSK) rehabilitation tool. The aim of this review was to systematically analyse the evidence regarding the effectiveness of this novel training modality in clinical MSK rehabilitation. DESIGN: This is a systematic review and meta-analysis of peer-reviewed literature examining BFR training in clinical MSK rehabilitation (Research Registry; researchregistry91). DATA SOURCES: A literature search was conducted across SPORTDiscus (EBSCO), PubMed and Science Direct databases, including the reference lists of relevant papers. Two independent reviewers extracted study characteristics and MSK and functional outcome measures. Study quality and reporting was assessed using the Tool for the assEssment of Study qualiTy and reporting in EXercise. ELIGIBILITY: Search results were limited to exercise training studies investigating BFR training in clinical MSK rehabilitation, published in a scientific peer-reviewed journal in English. RESULTS: Twenty studies were eligible, including ACL reconstruction (n=3), knee osteoarthritis (n=3), older adults at risk of sarcopenia (n=13) and patients with sporadic inclusion body myositis (n=1). Analysis of pooled data indicated low-load BFR training had a moderate effect on increasing strength (Hedges' g=0.523, 95% CI 0.263 to 0.784, p<0.001), but was less effective than heavy-load training (Hedges' g=0.674, 95% CI 0.296 to 1.052, p<0.001). CONCLUSION: Compared with low-load training, low-load BFR training is more effective, tolerable and therefore a potential clinical rehabilitation tool. There is a need for the development of an individualised approach to training prescription to minimise patient risk and increase effectiveness.

Are repeated single-limb heel raises and manual muscle testing associated with peak plantar-flexor force in people with inclusion body myositis?

BACKGROUND: Repeated heel raises have been proposed as a method of ankle plantar-flexor strength testing that circumvents the limitations of manual muscle testing (MMT). OBJECTIVE: The study objective was to examine the relationships among ankle plantar-flexion isometric maximum voluntary contraction (MVC), repeated single-limb heel raises (SLHRs), and MMT in people with myositis. DESIGN: This was a cross-sectional study with a between-group design. The ability to complete 1 SLHR determined group assignment (SLHR group, n=24; no-SLHR group, n=19). METHODS: Forty-three participants with myositis (13 women; median age=64.9 years) participated. Outcome measures included MVC, predicted MVC, Kendall MMT, and Daniels-Worthingham MMT. RESULTS: The Kendall MMT was unable to detect significant ankle plantar-flexor weakness established by quantitative methods and was unable to discriminate between participants who could and those who could not perform the SLHR task. Ankle plantar-flexion MVC was not associated with the number of heel-raise repetitions in the SLHR group (pseudo R(2)=.13). No significant relationship was observed between MVC values and MMT grades in the SLHR and no-SLHR groups. However, a moderate relationship between MVC values and MMT grades was evident in a combined-group analysis (ρ=.50-.67). LIMITATIONS: The lower half of both MMT grading scales was not represented in the study despite the profound weakness of the participants. CONCLUSIONS: Both Kendall MMT and Daniels-Worthingham MMT had limited utility in the assessment of ankle plantar-flexor strength. Repeated SLHRs should not be used as a proxy measure of ankle plantar-flexion MVC in people with myositis.

Frequency and circumstances of falls in people with inclusion body myositis: a questionnaire survey to explore falls management and physiotherapy provision.

OBJECTIVES: To survey the incidence and circumstances of falls for people with inclusion body myositis (IBM) in the UK, and to investigate the provision of physiotherapy and falls management. DESIGN: Postal questionnaire survey. SETTING: Participants completed questionnaires at home. PARTICIPANTS: Ninety-four people diagnosed with IBM were screened against the inclusion criteria. Seventy-two potential participants were sent a questionnaire, and 62 were completed and returned. Invited participants were sent an adapted Falls Event Questionnaire pertaining to falls, perceived causes of falls and the provision of physiotherapy. Questionnaires were returned anonymously. MAIN OUTCOME MEASURES: The proportions of respondents who reported a fall or a near fall, along with the frequencies of falls and near falls were calculated. Descriptive data of falls were collected pertaining to location and cause. Data analysis was performed to investigate provision of physiotherapy services. RESULTS: The response rate was 86% [62/72, mean (standard deviation) age 68 (8) years]. Falls were reported by 98% (61/62) of respondents, with 60% (37/62) falling frequently. In this study, age was not found to be an indicator of falls risk or frequency. Twenty-one percent (13/62) of respondents had not seen a physiotherapist in relation to their IBM symptoms, and of those that had, 31% (15/49) had not seen a physiotherapist until more than 12 months after IBM was diagnosed. Only 18% (11/61) of fallers reported that they had received falls management input. CONCLUSIONS: Falls are a common occurrence for people with IBM, independent of age and years since symptoms first presented, and are poorly addressed by appropriate physiotherapy management. National falls guidelines are not being followed, and referral rates to physiotherapy need to improve.

Exercise in inflammatory myopathies, including inclusion body myositis.

Exercise is an important part of treatment in patients with idiopathic inflammatory myopathies. Improved functioning, ability to perform activities of daily living, and health-related quality of life have been reported in adult polymyositis, dermatomyositis, and also recently inclusion body myositis following different exercise regimens, with no signs of increased muscle inflammation. Intensive resistance training could reduce clinical disease activity and reduce expression of genes regulating inflammation and fibrosis in chronic polymyositis and dermatomyositis. Today, exercise research in adult myositis is focused on understanding mechanisms for muscle impairment and improved muscle function in relation to exercise and verifying results from small, open studies in larger settings. There are no studies evaluating the effects of exercise over weeks or months in juvenile dermatomyositis, other than a case report; however, there is to our knowledge an ongoing effort to evaluate the safety and effects of exercise in patients with juvenile dermatomyositis.

Stance control orthosis trial in patients with inclusion body myositis.

BACKGROUND: Inclusion Body Myositis (IBM) is an inflammatory myopathy that commonly affects quadriceps strength, resulting in knee buckling and falls. Therefore, patients with IBM should be ideal candidates for stance control orthoses (SCOs). OBJECTIVES: Evaluate the effectiveness of SCO use in patients with IBM who have functional deficits due to quadriceps weakness. STUDY DESIGN: Cohort study. METHODS: Nine subjects with IBM were provided a stance control orthosis and followed for six months of home use. All patients had objective testing of their strength and gait and completed a questionnaire at baseline and six months. Gait analysis was performed both with and without the orthosis. RESULTS: Velocity and cadence were lower and step width was higher when using the orthosis. Kinematic measures were largely unchanged with orthosis use. Subjects with less knee extensor weakness had a better outcome than weaker participants. Those who spent more time wearing the orthosis also had a more positive outcome. The participants felt that the SCO was helpful in safeguarding against falls and providing stability. CONCLUSIONS: SCO use will benefit patients with IBM, but care should be taken to choose the SCO option that best suits their individual clinical presentation. CLINICAL RELEVANCE: This is the first study to examine SCO use in the IBM population. Patients with IBM have quadriceps weakness and will potentially benefit from SCO use. This study suggests that successful SCO management of patients with IBM depends on severity of weakness.

Improvement in aerobic capacity after an exercise program in sporadic inclusion body myositis.

OBJECTIVES: The study aimed to investigate the effects of a combined functional and aerobic exercise program on aerobic capacity, muscle strength, and functional mobility in a group of patients with sporadic inclusion body myositis (IBM). METHODS: Aerobic capacity, muscle strength, and functional capacity assessments were conducted on 7 participants with sporadic IBM before and after a 12-week exercise program, which included resistance exercises and aerobic stationary cycling 3 times per week on alternative days. RESULTS: Aerobic capacity of the group increased significantly by 38%, and significant strength improvements were observed in 4 of the muscle groups tested (P < 0.05). The exercise program was well tolerated, and there was no significant change in the serum creatine kinase level after the exercise period. CONCLUSIONS: An aerobic exercise program can be safely tolerated by patients with sporadic IBM and can improve aerobic capacity and muscle strength when combined with resistance training. These findings indicate that aerobic and functional muscle strengthening exercise should be considered in the management of patients with IBM.

Exercise effects in patients with adult idiopathic inflammatory myopathies.

PURPOSE OF REVIEW: To give an update on recent findings on effects of exercise in patients with adult inflammatory myopathies. RECENT FINDINGS: Although responding to treatment, a majority of patients with polymyositis and dermatomyositis develop sustained disability. The reason for this is not clear. However, a recent study further supports the hypothesis of hypoxia in muscle tissue as a contributor to muscle weakness. The percentage of type I oxygen-dependent muscle fibers increased after a 12-week submaximal home exercise program along with improved muscle endurance in patients with chronic polymyositis or dermatomyositis. Creatine supplements in addition to the same home exercise program are more beneficial than exercise alone in patients with chronic polymyositis or dermatomyositis. Patients with chronic disease tolerate intensive resistance training resulting in improved muscle strength and muscle endurance. This 7-week exercise study also reported reduced disease activity and possibly even reduced muscle inflammation. SUMMARY: These recent studies are in line with earlier ones further supporting safety and efficacy of exercise in patients with polymyositis or dermatomyositis. There is an urgent need for larger randomized controlled trials also including patients with inclusion body myositis to further increase knowledge of disease mechanisms causing disability, exercise effects, and what exercise program is most efficient in patients with different entities of idiopathic inflammatory myopathies.

Rehabilitación de un paciente con miositis por cuerpos de inclusión / Rehabilitation of patient with inclusion bodies

La miositis por cuerpos de inclusión (MCI) es una de las miopatías inflamatorias idiopáticas menos frecuente y más desconocida. Se presenta el caso de un varón de 36 años con tetraparesia secundaria a MCI trasladado a nuestro hospital para tratamiento rehabilitador consistente en: tratamiento postural, cinesiterapia, electroestimulación, ortesis, terapia ocupacional y ayudas técnicas. El enfermo era dependiente para las actividades de la vida diaria (AVD), presentaba amiotrofia, no realizaba sedestación y se le diagnosticó además de polineuropatía del enfermo crítico en miembros inferiores. Al alta, era independiente para AVD básicas e instrumentales y realizaba marcha con una ortesis antiequina y una ayuda externa contralateral. Nuestra experiencia no coincide con la creencia generalizada de que no se puede realizar cinesiterapia activa en estos pacientes, mientras existan alteraciones de los parámetros de inflamación o de las enzimas musculares

Inclusion body myositis (IBM) is one of the less frequent and most unknown idiopathic inflammatory myopathies. The case of a 36 year old male is presented. He had tetraparesis secondary to IBM and was transferred to our hospital for rehabilitation treatment consisting in: postural treatment, kinesitherapy, electrostimulation, orthesis, occupational therapy and technical help. The patient was dependent for daily life activities (DLA), had amyotrophy, did not sit up and was also diagnosed of polyneuropathy of critical patient in lower limbs. On discharge, he was independent for basic and instrumental DLA and could walk with an anti-equine orthesis and external contralateral help. Our experience does not coincide with the generalized belief that active kinesitherapy cannot be done in these patients while there are alterations of the inflammation or muscular enzyme parameters

The current status of treatment for inclusion-body myositis.

There is no established treatment that improves, arrests, or slows the progression of inclusion-body myositis (IBM). Many anti-inflammatory, immunosuppressant, or immunomodulating agents have been administered to patients with IBM but the design of clinical trials was such that it can only be concluded that none produced rapid improvement. The natural history of the disease is for stabilization or improvement in a third of patients for 6 months or more. Thus some agents that did not produce dramatic benefit may have been prematurely abandoned. However, because high-dose prednisone worsens strength while decreasing inflammation but increases amyloid accumulation, alternative targets for intervention and novel treatment strategies are needed.

Sporadic inclusion body myositis: pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction.

OBJECTIVE: To evaluate the safety and effect of a home training program on muscle function in 7 patients with sporadic inclusion body myositis. DESIGN: The patients performed exercise 5 days a week over a 12-week period. METHODS: Safety was assessed by clinical examination, repeated muscle biopsies and serum levels of creatine kinase. Muscle strength was evaluated by clinical examination, dynamic dynamometer and by a functional index in myositis. RESULTS: Strength was not significantly improved after the exercise, however none of the patients deteriorated concerning muscle function. The histopathology was unchanged and there were no signs of increased muscle inflammation or of expression of cytokines and adhesion molecules in the muscle biopsies. Creatine kinase levels were unchanged. A significant decrease was found in the areas that were positively stained for EN-4 (a marker for endothelial cells) in the muscle biopsies after training. CONCLUSION: The home exercise program was considered as not harmful to the muscles regarding muscle inflammation and function. Exercise may prevent loss of muscle strength due to disease and/or inactivity.

The benefits and limitations of a physical training program in patients with inflammatory myositis.

The clinical features of inflammatory myositis are determined by the severity and extent of muscle weakness and systemic manifestations. The benefits and limitations of physical training programs and rehabilitation strategies depend on the clinical phase of the disease and analysis of underlying impairments responsible for functional limitations in the patient. Patients with early stage disease and severe weakness will be treated differently than patients who have responded to medication and are improving. Not all patients will respond to medications; their therapy programs will have different requirements. This article reviews available data on the physiologic responses to exercise in patients with inflammatory muscle diseases. New data support more aggressive approaches to progressive strengthening exercises for patients with inflammatory myositis.