Habitual Physical Activity in Patients with Myasthenia Gravis Assessed by Accelerometry and Questionnaire.

BACKGROUND: Physical activity (PA) in patients with myasthenia gravis (MG) is considered safe and beneficial, and an active lifestyle is required to obtain the health benefits of exercise. However, as the disease leads to physical impairments an insight into the overall PA habits in this patient population is relevant but lacking. OBJECTIVE: To measure habitual physical activity in a Danish cohort of patients with MG measured by accelerometer and questionnaire, and to determine relevant predictors for PA intensities. METHODS: Habitual physical activity was assessed by; 1) the accelerometer ActiGraph in a cohort of patients recruited from our neuromuscular clinic, 2) the International Physical Activity Questionnaire (IPAQ) in a web-based survey. PA levels were compared to international recommendations. Predictors for PA (age, sex, body mass index, disease severity and duration) were included in the regression analyses. RESULTS: Habitual physical activity was measured by accelerometer for 7 days in 69 patients and by questionnaire in 691 patients. Measured by the accelerometer, 46%of the patients did not meet the international recommendations for PA at moderate/vigorous intensity and 57%were below the recommendations for steps per day. Measured by the IPAQ, 48%did not meet the recommendations. Disease severity and age were predictors for PA intensities. CONCLUSIONS: This study found that around half of the included patients did not meet the recommendations for PA. This is a concern, as it increases the risk of life-style related diseases. Disease severity and age may be taking into consideration when counseling the patients about PA.

Functional Impairments and Rehabilitation Outcomes of Patients With Immunotherapy-Induced Acute Inflammatory Demyelinating Polyradiculoneuropathy, Myasthenia Gravis, and Myositis.

ABSTRACT: Immunotherapy has led to a higher survival rate among different oncological disease groups but also associated with adverse-related events in multiple organ systems. Immunotherapy-related musculoskeletal weakness often results in a loss of cancer survivors' physical function, ultimately impacting their independence and quality of life. This is a retrospective study of 24 cancer patients who were treated with immunotherapy either alone or in conjunction with other oncological treatments. Twelve subjects (50%) were found to have acute inflammatory demyelinating polyradiculopathy/Guillain-Barré syndrome, six (25%) myositis, two (8%) myasthenia gravis, two (8%) diagnosis of myositis/myasthenia gravis, and one (4%) Guillain-Barré syndrome/myasthenia gravis combination. Physical therapy was provided in 91.7% of the cases, and physiatrist was involved in 54% of the cases. Almost half (45%) were discharged home, six (25%) to acute inpatient rehabilitation, two (8%) to subacute rehabilitation, three (12.5%) to hospice, and two (8%) died. The average length of hospital stay was 30 days, and eight patients (33%) readmitted within 3 mos. Our findings highlight the severity of functional impairments and the need for early rehabilitation interventions.

Application Prospect of Artificial Intelligence in Rehabilitation and Management of Myasthenia Gravis.

Myasthenia gravis (MG) is a chronic autoimmune disease of the nervous system, which is still incurable. In recent years, with the progress of immunosuppressive and supportive treatment, the therapeutic effect of MG in the acute stage is satisfactory, and the mortality rate has been greatly reduced. However, there is still no consensus on how to conduct long-term management of stable MG, such as guiding patients to identify relapses, practice exercise, return to work and school, etc. In the international consensus guidance for management of myasthenia gravis published by the Myasthenia Gravis Foundation of America (MGFA) in 2020, for the first time, "the role of physical training/exercise in MG" was identified as the topic of discussion. Finally, due to a lack of high-quality evidence on physical training/exercise in patients with MG, the topic was excluded after the literature review. Therefore, this paper reviewed the current status of MG rehabilitation research and the difficulties faced by stable MG patients in self-management. It is suggested that we should take advantage of artificial intelligence (AI) and leverage it to develop the data-driven decision support platforms for MG management which can be used for adverse event monitoring, disease education, chronic management, and a wide variety of data collection and analysis.

Physical training and exercise in myasthenia gravis.

Myasthenia gravis is characterized by muscle weakness and fatigue. As sustained muscle use increases the weakness, the value of physical training programs has previously been questioned. This is a review to clarify the safety and usefulness of systematic training in myasthenia gravis, based on a systematic search in available databases using the relevant key words. Ten intervention studies including 159 patients with generalized disease have been published regarding the effect of systematic physical training, three of them on respiratory muscles. Muscle strength improved, and in the majority of the studies also daily function and quality of life. The feeling of fatigue not directly related to actual muscle weakness was less influenced by physical training. Continuous training was necessary to maintain the improved function. Physical training and exercise are safe in myasthenia gravis. This can improve both muscle strength and daily function. Type and intensity of systematic training should be adapted in the individual patient. A minimum of 150 min of exercise per week is recommended for myasthenia gravis patients with mild and moderate disease.

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.

Effects of long-term respiratory muscle endurance training on respiratory and functional outcomes in patients with Myasthenia gravis.

BACKGROUND: Myasthenia gravis (MG) is characterized by reduced muscle endurance and often leads to respiratory complications. OBJECTIVE: A long-term respiratory muscle endurance training (RMET) based on normocapnic hyperpnea was introduced for the first time in MG patients. We investigated RMET effects on respiratory endurance (RE), MG symptoms, lung function and physical fitness and compared the results with a control group (CG). METHODS: The training period consisted of four weeks intensive training (IT; five 30-min training sessions per week) followed by twelve months maintenance training (MT; five 30-min training sessions over two weeks). Eighteen patients with mild to moderate MG participated as the training group (TG), six patients served as CG. RE, lung function, MG score and physical fitness were tested before and after IT and after three to twelve months of MT. RESULTS: Only 12 TG patients completed the entire training period. Thirteen months of training significantly increased RE measured as time until exhaustion (Tlim) to 412% of baseline (P < 0.001). The MG score improved from 0.67±0.09 to 0.41 ± 0.1 (p = 0.004), and the number of squats per minute as a measure of physical fitness increased in the TG to 160% of baseline (p = 0.015). While lung function did not change during the training period, we observed a modulation in the breathing pattern at rest with prolonged expiration (122% of baseline, p = 0.028). In addition, TG reported subjective improvements in MG symptoms, respiratory symptoms and physical fitness by 49%, 58% and 64%, respectively (P < 0.001). No significant changes were observed in the CG. CONCLUSION AND SIGNIFICANCE: This is the first controlled long-term RMET study in MG patients. The results demonstrated that this normocapnic hyperpnea training is feasible and beneficial for patients with mild to moderate MG and is a valuable supplement to conventional drug treatment.

The impact of physical exercise on neuromuscular function in Myasthenia gravis patients: A single-subject design study.

There is a need for tailored exercise recommendations to patients with Myasthenia gravis (MG). A few pilot studies have recently shown that physical exercise in accordance with general recommendations to healthy adults can be applied safely to patients with mild MG symptoms. How physical exercise affects muscle parameters and risk factors for lifestyle diseases in patients with MG is, however, only poorly known. We evaluated functional skeletal muscle parameters in 11 MG patients, before and after conducting a 12-week supervised physical therapy regimen of aerobic and resistance strength training. After the training program, parameters of the rectus femoris muscle improved: compound motor action potential (from 4.5 ±â€Š2.6 to 5.3 ±â€Š2.8 mV, P = .016), isometric muscle force (from 25.2 ±â€Š4.4 to 30.2 ±â€Š3.8 kg; P = .014), and ultrasound muscle thickness (from 19.6 ±â€Š5.6 to 23.0 ±â€Š3.9 mm, P = .0098) all increased. Further, physical performance based measures improved, including the 30-Second Chair Stand Test (median change +2, P = .0039) as well as the clinical MG composite score [from 3 (2-5) to 2 (0-4), P = .043]. No improvement in muscle function was observed in the biceps brachii muscle. These findings indicate that MG patients can improve their muscular functions by incorporating aerobic and resistance strength training, especially in proximal leg muscles. This is important knowledge when physical therapy is considered for this patient group, for whom no guidelines on physical exercise currently exist.

Physiotherapy immediately after thymectomy in patients with myasthenia gravis. Two cases and review of the literature.

AIM: Myasthenia gravis (MG) is a rare autoimmune disease characterized by activation of autoantibodies against acetylcholine receptors: patients with MG typically experience muscle weakness and fatigue. The aims of the present study were 1) to describe immediate postoperative physiotherapeutic interventions in two MG patients who underwent thymectomy, and 2) to discuss postoperative rehabilitative issues in MG patients. MATERIALS AND METHODS: This was a non-experimental study analyzing two subjects with MG who underwent thymectomy. Furthermore, to find evidence on postoperative rehabilitative intervention in MG patients undergoing thymectomy, four major databases were searched through August 2016. RESULTS: All subjects were able to walk on postoperative day (POD) 1 and to climb at least one flight of stairs on POD 4. Pain was more intense on POD 1 than on PODs 2 and 4. Dyspnea was worse on POD 2 than on PODs 1 and 4. Regarding the literature review, 58 papers were identified. After removal of duplicates, 51 citations remained to be screened. After the full texts were read, one paper met the inclusion criteria and was included. CONCLUSIONS: Our findings indicate that patients are willing to undergo immediate physiotherapeutic treatment in the ICU after thymectomy. It seems that physiotherapeutic intervention after thymectomy in MG-grade IIa middle-aged patients can be initiated immediately postoperatively in uncomplicated cases. KEY WORDS: Dyspnea, Myasthenia gravis, Outcome assessment, Pain measurement, Physiotherapy, Postoperative care, Rehabilitation, Thymectomy.

Benefits of Comprehensive Rehabilitation Therapy in Thymectomy for Myasthenia Gravis: A Propensity Score Matching Analysis.

OBJECTIVE: To demonstrate the effectiveness of a comprehensive program of rehabilitation therapy in patients undergoing thymectomy for myasthenia gravis (MG). DESIGN: From 2005 to 2010, 46 consecutive patients affected by MG underwent a rehabilitation program both before and after thymectomy. We matched each patient with a "control patient" who underwent thymectomy within the period 1999 to 2004 with no preoperative rehabilitation, who had the closest propensity score matching. RESULTS: All patients but 2 were able to complete the intended program. Eighteen patients (41%) experienced mild fatigue (>25 at MG quantitative score). Propensity score selected a group of 17 patients for the matching process. The group of patients who underwent the rehabilitation program showed significant preoperative improvement associated with a reduced operative risk, a decreased early postoperative morbidity, a lower rate of postoperative intensive care unit needed (12% vs 35%; P = 0.01) and a shorter hospital stay (3 vs 5 days; P = 0.04). After the expected perioperative decline, all major myasthenic outcomes demonstrated a significant faster recovery at 3 months. Complete stable remission did not reveal significant differences. CONCLUSIONS: Exercise is not necessarily a contraindication in MG, and rehabilitation can be safely performed before and after thymectomy, reducing operative risks and decreasing recovery time. TO CLAIM CME CREDITS: Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCMECME OBJECTIVES: Upon completion of this article, the reader should be able to do the following: (1) appreciate the benefits of physical therapy in individuals with myasthenia gravis; (2) describe the benefits of physical therapy on postoperative morbidity in myasthenia gravis patients who undergo thymectomy; and (3) incorporate appropriate rehabilitation into the treatment plan of patient with myasthenia gravis. LEVEL: AdvancedACCREDITATION: The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this activity for a maximum of 1.5 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Physical exercise in myasthenia gravis is safe and improves neuromuscular parameters and physical performance-based measures: A pilot study.

INTRODUCTION: Due to the shortage of exercise-related research in Myasthenia Gravis (MG), there are no consensus guidelines on physical exercise for MG patients. METHODS: In this prospective pilot study, 10 MG patients with mild disease performed supervised aerobic and resistance training twice weekly for 12 weeks. The Myasthenia Gravis Composite (MGC) score, compound motor action potential (CMAP), repetitive nerve stimulation, muscle force, physical performance-based measures, serum levels of interleukin-6, muscle enzymes, and immuno-microRNAs miR-150-5p and miR-21-5p were assessed before and after the training period. RESULTS: Physical exercise was well tolerated, and the MGC score was unchanged. Muscle resistance weights and CMAP amplitudes increased for biceps brachii and rectus femoris muscles, and physical performance-based measures improved. Muscle enzymes remained normal, whereas disease-specific microRNAs miR-150-5p and miR-21-5p were reduced after the training period. CONCLUSIONS: We propose that general recommendations regarding physical exercise can be applied safely to well-regulated MG patients. Muscle Nerve 56: 207-214, 2017.

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.

Exercise in neuromuscular disease.

In this review, the authors present an overview of the role of exercise in neuromuscular disease (NMD). The authors demonstrate that despite the different pathologies in NMDs, exercise is beneficial, whether aerobic/endurance or strength/resistive training. The authors analyze methodological flaws of existing studies and suggest improvements for future trial protocols. Finally, we discuss specialized exercise training of specific muscles, as well as new technologies adapted from other neurologic disorders, including body-weight-supported treadmill ambulation, robotic-assisted gait training, and neuromuscular electrical stimulation.

Development of the MG-DIS: an ICF-based disability assessment instrument for myasthenia gravis.

PURPOSE: To develop a preliminary version of a disease-specific, patient-reported disability assessment instrument for myasthenia gravis (MG) based on the International Classification of Functioning, Disability and Health (ICF): the MG-DIS. METHODS: Five consecutive steps were taken: literature review and selection of outcome measures; linking of measures' concepts to ICF categories and selection of those reported by 30% of the instruments; comparison of linking results with a previous selection of MG-relevant ICF categories; patient interview; development of questions based on retained ICF categories. RESULTS: Thirty-one papers containing 21 different outcome measures were found: 13 ICF categories were linked to them. Fifty-five items were retained after the comparison with the list of MG-specific categories, and were used for patient interview. Thirteen interviews were conducted before saturation of data was reached and the final list was composed of 42 categories: based upon them, 44 questions were developed. CONCLUSIONS: The preliminary version of the MG-DIS contains more information than each single MG-specific tool, in particular, for the component of environmental factors. Further research is needed to test its psychometric properties. IMPLICATIONS FOR REHABILITATION: It is important that patient-reported outcome is incorporated in MG patient's assessment. MG features can be evaluated with ICF-based methods. An MG-specific patient-reported disability assessment instrument can be used to monitor changes of functioning in patients on MG-specific treatments, and can be used in clinical trials as outcome measure.

Effects of balance strategy training in myasthenia gravis: a case study series.

INTRODUCTION: Studies of exercise in patients with myasthenia gravis (MG) are sparse. Balance strategy training (BST) multimodal exercise has proven efficacy in adults for enhancing balance and functional mobility. This prospective study aims to determine if BST improves functional ability and balance in people with MG. METHODS: Seven individuals with MG participated in a 16-session workstation intervention. Repeated measures (pre/post-intervention and 4-week follow-up) consisting of quantitative myasthenia gravis score (QMG), 6-minute walk test (6MWT), timed up and go (TUG) with dual task (TUG(manual) and TUG(cognitive)), and standing stability on foam with eyes closed (foamEC) were assessed. RESULTS: Most measurements showed sustained improvement at follow-up. QMG, TUG(cognitive), and foam EC achieved clinically significant improvements (>15%). No adverse effects were reported. CONCLUSIONS: BST was effective in improving balance and QMG scores in subjects with MG. A multimodal BST approach is thus suggested to target different aspects of balance and functional mobility.

Myasthenia gravis and sports participation.

Myasthenia gravis (MG) is an autoimmune disorder of the postsynaptic membrane that leads to severe fatigability and weakness of the proximal muscle groups. This disorder has a peak presentation age that is common in sporting populations. Due to improved treatment strategies, it would not be uncommon for sports medicine physicians to be faced with a myasthenic patient on initial diagnosis or to be consulted regarding activity recommendations. There has been a scarcity of exercise-related research in MG compared to other neuromuscular disorders. There are also no guidelines on clearance issues for active athletes with MG. This article will review the literature and case reports regarding MG in athletics and attempt to help guide recommendations for activity and exercise therapy.

Facial affect recognition in myasthenia gravis

The assessment of facial expression is an important aspect of a clinical neurological examination, both as an indicator of a mood disorder and as a sign of neurological damage. To date, although studies have been conducted on certain psychosocial aspects of myasthenia, such as quality of life and anxiety, and on neuropsychological aspects such as memory, no studies have directly assessed facial emotion recognition accuracy. The aim of this study was to assess the facial emotion recognition accuracy (fear, surprise, sadness, happiness, anger, and disgust), empathy, and reaction time of patients with myasthenia. Thirty-five patients with myasthenia and 36 healthy controls were tested for their ability to differentiate emotional facial expressions. Participants were matched with respect to age, gender, and education level. Their ability to differentiate emotional facial expressions was evaluated using the computer-based program Feel Test. The data showed that myasthenic patients scored significantly lower (p < 0.05) than healthy controls in the total Feel score, fear, surprise, and higher reaction time. The findings suggest that the ability to recognize facial affect may be reduced in individuals with myasthenia (AU)

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Disability and functional profiles of patients with myasthenia gravis measured with ICF classification.

The objective of this study is to describe functional profiles of patients with myasthenia gravis (MG), and the relationships among symptoms, activities and environmental factors (EF), by using WHO's International Classification of Functioning Disability and Health (ICF). Patients were consecutively enrolled at the Besta Institute of Milan, Italy. The ICF checklist was administered and two count-based indices were developed: 'extension', containing ICF categories rated with qualifiers 1-4, and 'severity', containing ICF categories rated with qualifiers 3-4. Spearman's correlation was performed to identify the relationships among symptoms, activities and EF, and linear regressions to identify the best predictors of performance indices in the activities and participation (A and P) domains. One hundred and one patients joined this study. Different values are reported in capacity and performance, the latter being 17.1% less limited in 'extension' and 13% in 'severity' index. Higher correlation indices are observed between A and P and body functions (BFs), than between A and P and EF. Problems in A and P are better explained by BF impairments than by facilitators within EF. In conclusion, EF plays a relevant role in improving MG patients' functioning. Nevertheless, difficulties in A and P, such as lifting objects or doing housework, are explained more by impairments in BF (e.g. in muscle endurance) than by the effect of EF (e.g. drugs and the support of family members). Methodologies and tools are needed to couple symptoms assessment with an evaluation of difficulties in executing activities and the environment's role, to plan appropriate interventions to meet MG patients' needs, especially in the labour sector, as 20% of patients gave up working before the retirement age.

The effect of respiratory muscle endurance training in patients with myasthenia gravis.

We tested the effect of a home-based respiratory muscle endurance training in patients with mild to moderate generalized myasthenia gravis (MG) on Besinger score, lung function and respiratory muscle endurance. Ten patients performed respiratory muscle endurance training in form of normocapnic hyperpnea training at 50-60% of their maximal voluntary ventilation over 4-6 weeks. MG score, lung function and respiratory endurance were assessed before and after training period. The training significantly increased respiratory endurance from 8.4+/-0.9 min to 17.1+/-1.3 min (p<0.001) and total ventilatory volume from 555+/-87 L to 1081+/-127 L (p=0.004). About 25% of this gain was lost after 3-5 months of detraining. The remaining 75% gain might result from improved neuromuscular coordination rather than muscular training. MG score and lung function, however, did not change. Patients assessed the training effects on physical fitness and respiration as positive. In conclusion, respiratory muscle endurance training can be useful for MG patients as it is enhancing respiratory muscle endurance.

Double trouble (McArdle's disease and myasthenia gravis): how can exercise help?

We report a 29-year-old patient with McArdle's disease and myasthenia gravis. She had been debilitated with McArdle's disease since childhood (with marked rhabdomyolysis) and was obese. Myasthenia gravis was diagnosed at 24 years of age. After 3 months of aerobic exercise training, her exercise capacity increased significantly and she regained the ability to live independently. We conclude that even patients with profound neuromuscular diseases may benefit from carefully prescribed exercise training.

[Effects of the thymectomy in a rehabilitation program and in quality of life in patients with myasthenia gravis]. / Valutazione degli aspetti riabilitativi e della Qualità di Vita nei pazienti affetti da miastenia gravis sottoposti a timectomia.

OBJECTIVE: The purpose of this study is to estimate the effects of a rehabilitation program on patients with myasthenia gravis (MG) subordinates to thymectomy. MATERIALS AND METHODS: Patients were evaluated at baseline (T1) and at the end of the pulmonary rehabilitation. (T2), by undergoing spirometric measurements, 6-Minutes Walking Test (6MWT), Berg Balance Scales (BBS), Rivermead Mobility Index (RMI) and Short 36 Form Health Survey version 2 (SF-36v2). The rehabilitation program included exercise to improve the respiratory functionality, the cardiovascular fitness and the global posture. OUTCOME: A single patient obtained improvement pulmonary function, unmodified in the others two patients. Mobility and balance, estimated by the RMI and BBS, effort tolerance, estimated by the 6-MWT QOL, estimated by means of SF36v2 improved, and dyspnoea (Borg scale) reduction was obtained in all of the patients. DISCUSSION: A specific rehabilitative protocol does not exist for the patients with MG subordinates to thymectomy. The AA. conclude that the training relieves dyspnoea, increases the capacity to walk, and improves health-related quality of life. CONCLUSION: Future studies will define the role of the rehabilitation for, these patients and will optimize the protocol.