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Background: Real-world data were employed to determine clinical characteristics of patients with myasthenia gravis (MG) with differing degrees of muscle weakness, as defined using the Myasthenia Gravis Foundation of America (MGFA) classification system., Methods: Data were drawn from the Adelphi MG Disease Specific Programme (DSP)™, a multinational (United States, France, Germany, Italy, Spain, United Kingdom) survey completed by physicians and their patients with MG in 2020. The association between MGFA class and impairment in activities of daily living (ADL) was tested using linear regression adjusting for sex and Charlson Comorbidity Index. Bivariate comparisons were performed for each individual item. A range of other clinical characteristics were also explored according to MGFA class., Results: Among 1232 patients, those in MGFA class I had significantly lower ADL impairment versus class II or III/IV (adjusted for sex and Charlson Comorbidity Index) (p < 0.01). However, heterogeneity occurred within each MGFA class. Bulbar symptoms (impaired speech, difficulty swallowing, and/or difficulty chewing/choking on food) were reported in some class I patients (mild in 1.1-1.9% and moderate in 0.3-1.1% of patients) and class II patients (mild in 8.5-16.4%, moderate in 4.7-7.4%, and severe in 0.3-0.9% of patients), and shortness of breath was reported in some class I (mild in 0.5% of patients) and class II patients (mild in 9.8%, moderate in 4.8%, and severe in 0.3% of patients). Conversely, in 11.2-19.2% of class III/IV patients, bulbar symptoms and shortness of breath reported were only mild in severity. In line with this finding, despite significant correlations between MGFA class and several clinical characteristics, patients across every class were at risk of experiencing myasthenic crisis or hospitalization, experiencing comorbidities including anxiety and depression, and not being in remission., Conclusions: Although MGFA class correlates with greater ADL impairment and presence of other clinical characteristics, there is variability between patients in each class in terms of symptoms experienced, overall disease burden, and the precise nature of ADL impairment., (© 2024. The Author(s).)

Introduction/aims: The impact of treatment expectations on active treatment outcomes has not been specifically investigated in neuromuscular disorders. We thus explored in myasthenia gravis (MG) the contribution of patients' pre-treatment expectations combined with an immunosuppressant drug on treatment outcomes., Methods: This pilot correlational study involved 17 patients with generalized MG, scheduled to start immunosuppressant azathioprine. At baseline, a healthcare professional administered: (i) the Stanford Expectations of Treatment Scale; (ii) a structured checklist paper form asking patients which side-effects they expected to develop after starting azathioprine, coupled with a standardized framing of statements. Quantitative Myasthenia Gravis (QMG) score and daily dose of concomitant drugs were assessed by neurologists as clinical outcomes. Clinical outcomes and side-effects were re-assessed at 3 and 6 months, and clinical outcomes were monitored at 18 months., Results: Clinically significant improvement in the QMG scores was achieved at 3 or 6 months. The level of state anxiety appeared to act as moderator of pre-treatment negative expectations (strong, positive, indicative correlation, rs = .733, p = .001). The latter were, in turn, associated with the fulfillment of side-effects that patients expected to develop with the new treatment (moderate, positive, indicative correlation, rs = .699, p = .002). No significant correlation emerged between positive and negative expectations., Discussion: Our findings show a very quick clinical response and also suggest that patients' expectations and anxiety contributed to treatment outcomes, highlighting the importance of promoting safety messages and education strategies around newly introduced treatments. Future goals include evaluating a larger cohort that includes a matched control group., (© 2024 Wiley Periodicals LLC.)

Background: The Myasthenia Gravis-Activities of Daily Living scale (MG-ADL) is an 8-item outcome measure to assess symptoms and functional limitations in myasthenia gravis (MG) patients. The MG-ADL score is an equally weighted level sum score that is used as primary outcome measures in clinical trials, in clinical practice, and as an end-point in health economic evaluation. This data analysis aims to obtain detailed knowledge of measurement properties of MG-ADL items and the MG-ADL score., Methods: Cross-sectional data from a real-world prospective study (MRW) were combined with longitudinal data from the ADAPT trial. Outcome measures included were MG-ADL, Quantitative Myasthenia Gravis score (QMG), MG 15-item Quality of Life (MG-QOL15r) and EQ-5D-5L. Patients were categorized by their Myasthenia Gravis Foundation of America (MGFA) clinical classification. The following measurement properties were assessed: distributional characteristics, inter-item correlation, convergent, known groups and construct validity and internal factor structure., Results: Correlations of items within MG-ADL dimensions were moderate, while MG-ADL correlations between comparable MG-QOL15r and QMG items were mixed. Known groups validity for the MG-ADL score was demonstrated for MGFA class. Mean MG-ADL item level scores by MGFA class demonstrated construct validity. PCA, including all four outcome measures, resulted in a nine factor solution., Discussion: Psychometric properties of individual MG-ADL items were moderate to good. This study showed that the MG-ADL adequately captures the multidimensional heterogeneous nature of MG. This is, however, accompanied by mixed psychometric performance of the MG-ADL score, which may complicate health economic modelling., Registration: MyRealWorld-MG was registered on November 25, 2019, with registration numberNCT04176211. The ADAPT randomized clinical trial is registered atClinicalTrials.gov(NCT03669588)., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Myasthenia gravis (MG) is one of the most common autoimmune diseases and can occur at any age. The study aimed to evaluate the influence of loneliness and anxiety levels on MG-specific quality of life in patients with myasthenia gravis during the COVID-19., Methods: This was a cross sectional study and the population consisted of patients with MG throughout Turkey between August 1 and October 31, 2021. The study was completed with 149 patients who met the inclusion criteria and agreed to participate in the research., Results: The mean MG-specific quality of life score was 44.15 ± 13.77, the mean UCLA loneliness scale was 56.13 ± 11.55, and the mean Beck Anxiety Inventory score was 24.63 ± 13.78. There was a moderately positive linear relationship between the participants' MG-specific quality of life (r = 0.589; p < 0.001) and the Beck Anxiety Inventory, and there was a weak negative linear relationship with the UCLA loneliness scale (r = 0.323; p < 0.001). The variables affecting the MG-specific quality of life were the following: anxiety, loneliness, employment status, income level, symptom levels, and having another chronic disease; a multiple regression model was created with these variables, and it was observed that anxiety and loneliness affected participants' MG-specific quality of life. The variables in the model explained 52.9% of the MG-specific quality of life, and the model was statistically significant (p < 0.001)., Conclusion: The study determined that the participants had intense loneliness, moderate anxiety, and a moderate MG-specific quality of life. The participants' MG-specific quality of life has decreased as their anxiety has increased, and the participants' MG-specific quality of life has increased as their loneliness has increased., (© 2023. International Society of Behavioral Medicine.)

Background: Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune-mediated neuromuscular disorder leading to muscle weakness, autonomic dysregulation and hyporeflexia. Psychosocial well-being is affected. Previously, we assessed burden of disease for Myasthenia gravis (MG). Here, we aim to elucidate burden of disease by comparing health-related quality of life (HRQoL) of patients with LEMS to the general population (genP) as well as MG patients., Methods: A questionnaire-based survey included sociodemographic and clinical data along with standardized questionnaires, e.g. the Short Form Health (SF-36). HRQoL was evaluated through matched-pairs analyses. Participants from a general health survey served as control group., Results: 46 LEMS patients matched by age and gender were compared to 92 controls from the genP and a matched cohort of 92 MG patients. LEMS participants showed lower levels of physical functioning (SF-36 mean 34.2 SD 28.6) compared to genP (mean 78.6 SD 21.1) and MG patients (mean 61.3 SD 31.8). LEMS patients showed lower mental health sub-scores compared to genP (SF-36 mean 62.7 SD 20.2, vs. 75.7 SD 15.1) and MG patients (SF-36 mean 62.7 SD 20.2, vs. 66.0 SD 18.). Depression, anxiety and fatigue were prevalent. Female gender, low income, lower activities of daily living, symptoms of depression, anxiety and fatigue were associated with a lower HRQoL in LEMS., Discussion: HRQoL is lower in patients with LEMS compared to genP and MG in a matched pair-analysis. The burden of LEMS includes economic and social aspects as well as emotional well-being. TRIAL REGISTRATION INFORMATION: drks.de: DRKS00024527, submitted: February 02, 2021, https://drks.de/search/en/trial/DRKS00024527 ., (© 2024. The Author(s).)

Background: Myasthenia gravis (MG) is a rare autoimmune disease characterised by muscle weakness, and progression from ocular (oMG) to generalised (gMG) symptoms results in a substantial negative impact on quality of life (QoL). This systematic review aimed to provide an overview of the patient burden experienced by people living with gMG., Methods: Electronic database searches (conducted March 2022), supplemented by interrogation of grey literature, were conducted to identify studies reporting patient burden outcomes in patients with gMG in Europe, the Middle East and Africa. Results were synthesised narratively due to the heterogeneity across trials., Results: In total, 39 patient burden publications (representing 38 unique studies) were identified as relevant for inclusion in the systematic review, consisting of 37 publications reporting formal patient-reported outcome measures (PROMs), and two publications describing alternative qualitative assessments of patient experience. The studies included a variety of measures including generic and disease-specific PROMs, as well as symptom-specific PROMs focusing on key comorbidities including depression, anxiety, fatigue and sleep disturbance. The findings showed some variation across studies and PROMs; however, in general there was evidence for worse QoL in patients with gMG than in healthy controls or in patients with oMG, and a trend for worsening QoL with increasing MG severity., Conclusions: This review highlights the importance of considering patient QoL when developing and assessing treatment and management plans for patients with gMG. However, the heterogeneity identified across studies illustrates the need for further representative and well-powered studies in large cohorts administering consistent, validated questionnaires., Trial Registration: The protocol for this systematic review was registered in PROSPERO: CRD42022328444., (© 2024. The Author(s).)

Introduction: Myasthenia gravis (MG) is a rare neuromuscular disorder marked by a variable combination of weakness of eye, bulbar, respiratory, axial, and limb muscles. This study compared the experience of people with MG regarding breathing, fatigue, sleep, pain/discomfort, mental health, and usual activities with the general population., Methods: The MyRealWorld-MG digital, multinational study enrolled patients with MG and collected demographics, PROMIS-Dyspnea, PROMIS-Sleep Disturbance, FACIT-Fatigue, EQ-5D-5L, Health Utilities Index (HUI-3), Hospital Anxiety and Depression Scale (HADS), MG-Activities of Daily Living (MG-ADL), and MG-Quality-of-Life (MG-QoL-15r). Comparisons with the general population were based on PROMIS population norms, published literature, or on data from a digital, multinational, observational study which enrolled a representative sample of the general population (POPUP)., Results: In MyRealWorld-MG (N = 2074), patients experienced higher intensity, frequency, and duration of PROMIS shortness of breath than a US population (p < 0.0001). Patients with MG had higher PROMIS-Sleep Disturbance scores than POPUP (53.7 vs 50.0, p < 0.0001), and 54.9% of patients had clinically severe FACIT-Fatigue scores vs 6.8% in POPUP (p < 0.0001). Among patients with MG, 69.6% and 18.5% had moderate-to-severe HADS-Anxiety and HADS-Depression compared to 20.3% and 6.9% in POPUP (p < 0.001). Statistically significant and strong associations were found between fatigue, sleep, dyspnea, usual activities, and emotions. All outcomes worsened with more severe disease., Conclusion: A considerable burden was observed in this comparison of breathing, sleep, fatigue, mental health, and usual activities between patients with MG and the general population, using data from two international studies and published population norms. Even mildly affected patients had significantly worse outcomes than the general population., (© 2023. The Author(s).)

Purpose: This study aimed to generate the health state classification system (HCS) of a condition-specific preference-based measure to capture the health-related quality of life (HRQoL) of patients with myasthenia gravis (MG) from the 15-item Myasthenia Gravis Quality of Life Scale (MG-QoL15r)., Materials and Methods: An HCS was derived from the MG-QoL15r in a large sample of 1739 patients with MG based on psychometric analysis, including factor analysis, item response theory analysis, and discussions with experts. Reliability, construct and convergent validity, and item fit of the HCS were further assessed using another sample., Results: The HCS has six dimensions: social activity, hobbies and fun activities, meeting family needs, work performance, mobility, and emotion, and it demonstrates good internal consistency reliability. The unidimensionality of the HCS was confirmed using confirmatory factor analysis. Satisfied convergent validity was supported by a significant association with the 12-item Short Form Survey (SF-12)., Conclusions: Based on a solid process of development and consultations with clinical professionals and patients, a valid MG-specific preference-based measure, MGQoL-6D, was developed. Further research will estimate the local preference weight to support the MG-related cost-utility analysis.IMPLICATION FOR REHABILITATIONA new condition-specific health state classification system (HCS) named Myasthenia gravis Quality of Life Scale (MGQoL-6D) is proposed.MGQoL-6D classifies MG health states as a combination of six dimensions with three response levels.The dimensions of the HCS are social activity, hobbies and fun activity, family needs, work performance, mobility, and emotion.The HCS and the upcoming value set of the MGQoL-6D could support the cost-effectiveness analysis of MG-related clinical and rehabilitated interventions.

Background: Patients with myasthenia gravis(MG)often experience multiple symptoms concurrently, which can have an adverse effect on their quality of life(QOL). However, a specific, systemic and reliable scale for symptom clusters in MG is lacking., Aims: To develop reliable assessment scale for symptom clusters in patients with MG., Design: A cross-sectional descriptive study., Methods: Based on the unpleasant symptom theory(TOUS), the first draft of the scale was developed through review literature, qualitative interview, and Delphi expert correspondence, the items of the scale were presented and adjusted through cognitive interviews with 12 patients. To conveniently assess the validity and reliability of the scale, a cross-sectional survey was conducted in 283 patients with MG who were recruited from Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, from June to September 2021., Results: The final symptom cluster scale for patients with MG consisted of 19 items(MGSC-19), with a content validity index ranging from 0.828 to 1.000 for each item and the content validity index was 0.980. Four common variables (ocular muscle weakness, general muscular weakness, treatment-related side effects, and psychiatric problems) were identified by exploratory factor analysis, which explained 70.187% of the total variance. The correlation coefficients between the scale dimension and the overall score ranged from 0.395 to 0.769 (all P < 0.01), while the correlation coefficients between dimensions varied from 0.324 to 0.510 (all P < 0.01). The Cronbach's alpha, retest reliability, and half reliability were 0.932, 0.845, and 0.837, respectively., Conclusion: The validity and reliability of MGSC-19 were generally good. This scale can be employed to identify the symptom clusters to help healthcare givers develop individualized symptom management measures for patients with MG., (© 2023. The Author(s).)

Introduction: Muscle weakness and easy fatigability are the clinical hallmarks of myasthenia gravis (MG). However, fatigue perception, which can be seen quite often in myasthenic patients, and its effect on the quality of life, irrespective of motor deficit, has not been elucidated yet. The aim is to evaluate the frequency of fatigue in myasthenic patients with nearly full muscle strength and the effect of fatigue on quality of life by assessing its correlation with other symptoms., Methods: Fifty-three patients with ocular or mild generalized MG in remission or minimal manifestations completed the questionnaires measuring the severity of MG and quality of life (MG Composite Scale and MG-Activities of Daily Living Profile). Both patient group and control group (53 healthy volunteers)completed the scales assessing fatigue [Fatigue Assessment Scale (FAS) and Fatigue Impact Scale (FIS)], depression [Beck Depression Inventory (BDI)] and sleep (Epworth Sleepiness Scale). Disease severity was assessed using MG Foundation of America (MGFA) and MGFA Post-Intervention Status classifications., Results: FAS, FIS physical and BDI scores were significantly higher in patients compared to the control group (p = 0.003, p = 0.001, and p = 0.003, respectively) and fatigue was associated with depression and daytime sleepiness. Inpatient group, depressive symptoms and daytime sleepiness were higher in females (p = 0.019 and p = 0.013). The mean values of FIS total and cognitive scores were higher in patients with generalized MG (p = 0.033 and p = 0.045). Fatigue scores correlated with motor signs., Discussion: Fatigue can be seen in MG independently from muscle weakness and is an important symptom worsening the quality of life., (© 2022. The Author(s) under exclusive licence to Belgian Neurological Society.)

Background: Myasthenia gravis (MG) leads to exertion-dependent muscle weakness, but also psychological and social well-being are limited. We aim to describe the burden of disease in MG including sociodemographic, economical, psychosocial as well as clinical aspects, to compare health-related quality of life (HRQoL) of patients with MG to the general population (genP) and to explore risk factors for a lower HRQoL., Methods: This case-control study was conducted with MG patients of the German Myasthenia Association. A questionnaire-based survey included sociodemographic and clinical data as well as standardized questionnaires, e.g. the Short Form Health (SF-36). HRQoL was compared to genP in a matched-pairs analysis. Participants of the German Health Interview and Examination Survey for Adults (DEGS1) served as control group., Results: In our study, 1660 MG patients participated and were compared to 2556 controls from the genP. Patients with MG showed lower levels of physical functioning (SF-36 mean 56.0, SD 30.3) compared to the genP (mean 81.8, SD 22.1, adjusted difference: 25, 95% CI 22-29) and lower mental health sub-score (SF-36 mean 67.3, SD 19.8, vs. 74.1, SD 16.7, adjusted difference: 5, 95% CI 2-8). Female gender, higher age, low income, partnership status, lower activities of daily life, symptoms of depression, anxiety and fatigue and self-perceived low social support were associated with a lower HRQoL in MG patients., Discussion: HRQoL is lower in patients with MG compared to genP. The burden of MG on patients includes economic and social aspects as well as their emotional well-being. New therapies must achieve improvements for patients in these areas., Trial Registration Information: Clinicaltrials.gov, NCT03979521, submitted: June 7, 2019, first patient enrolled: May 1, 2019, https://clinicaltrials.gov/ct2/show/NCT03979521., (© 2021. The Author(s).)

Objective: We aimed to examine the illness perception characteristics of patients with myasthenia gravis and analyze the associated factors., Methods: A general information questionnaire, Illness Perception Questionnaire, and Simplified Coping Style Questionnaire were used to survey 90 patients with myasthenia gravis. One-way analysis of variance and multivariate linear regression were used for statistical analysis., Results: The timeline (acute/chronic), consequence, and emotional representation scores of patients with myasthenia gravis were 18.31 ± 4.47, 18.58 ± 3.57, and 20.80 ± 4.56, respectively. Multivariate stepwise linear regression analysis showed that marital status, disease duration, educational level, disease type, and coping style are factors associated with illness perception in patients with myasthenia gravis., Conclusion: Patients who have myasthenia gravis have a negative illness perception. Medical professionals should provide targeted intervention measures based on the factors associated with illness perception., (© 2021 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Background: Despite improvements in the medical management of myasthenia gravis (MG) in recent years, patients continue to report poor health and wellbeing outcomes such as high levels of fatigue, reduced quality of life (QoL), walking limitation and lowered balance confidence. Physical activity has been shown to be associated with these outcomes in other populations, however, there has been limited research in adults with MG., Objective: To describe physical activity and sedentary behaviour in adults with MG and to explore associations between these behaviours and fatigue, QoL, balance confidence and walking limitation., Methods: A self-report online survey was used to assess physical activity, sedentary behaviour, fatigue, QoL, balance confidence and walking limitation in adults with MG. Multiple linear regression was used to examine associations and descriptive statistics were used to analyse participant characteristics, physical activity, and sedentary behaviour., Results: Eighty-five adults with MG were included (mean age 48±16 years). Over half of participants (n = 53, 62.4%) reported sufficient physical activity to meet public health guidelines. Participants reported an average of 9 h/day of sedentary time (mean 9.0±3.5). Physical activity and fatigue (R2 = 0.196), QoL (R2 = 0.330), walking limitation (R2 = 0.305) and balance confidence (R2 = 0.304) were significantly (p < 0.05) and positively correlated, with no associations found for sedentary behaviour. When patterns of physical activity and sedentary behaviour were combined, lower fatigue (R2 = 0.213), higher QoL (R2 = 0.364), reduced walking limitation (R2 = 0.341) and higher balance confidence (R2 = 0.279) was observed in patients who had greater physical activity levels (> 150 mins/week) and lower sedentary time (< 10 h/day)., Conclusions: Higher physical activity and lower sedentary behaviour is associated with favorable health and wellbeing outcomes in adults with MG. Further research is required to ascertain whether these behaviours may be an appropriate target intervention to improve outcomes in this population.

Objective: Generalized myasthenia gravis (gMG) is an autoimmune disease that causes disabling weakness via damage to the neuromuscular junction. In most patients, the disease is mediated by autoantibodies to the acetylcholine receptor, which activate the complement cascade. Our objective was to analyze response profiles in adult patients with anti-acetylcholine receptor antibody-positive refractory gMG treated with eculizumab-a terminal complement inhibitor-in the REGAIN study or its open-label extension (OLE)., Methods: We retrospectively analyzed Myasthenia Gravis-Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) scores recorded during REGAIN and its OLE. Early/late responses were defined as improvement in MG-ADL score (≥3 points) or QMG score (≥5 points) at ≤12 or >12 weeks, respectively, after eculizumab initiation., Results: The analysis included 98 patients. By Week 12 and conclusion of the OLE, MG-ADL response had been achieved at some point by 67.3% and 84.7% of patients, respectively, and QMG response by 56.1% and 71.4%, respectively. Response was observed over multiple consecutive assessments for most patients. At Week 130, the least-squares mean percentage changes (95% CI) from baseline in MG-ADL score were -61.9% (-69.9%, -53.9%) and -47.5% (-59.0%, -36.0%) in early and late MG-ADL responders, respectively; the least-squares mean percentage changes from baseline in QMG score were -40.8% (-48.3%, -33.4%) and -55.5% (-68.4%, -42.7%) in early and late QMG responders, respectively., Interpretation: The findings suggest that, although most patients with refractory gMG will achieve clinical response by Week 12 of eculizumab treatment, first responses can be observed with longer-term treatment., (© 2021 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Objective: Myasthenia gravis (MG) is characterized by fluctuating muscle weakness due to immune mediated damage of acetylcholine receptor (AchR). COVID-19 infection, mental stress and non-availability of drugs following lockdown may worsen myasthenic symptoms. We report the impact of COVID 19, and lockdown on the physical and mental health, and quality of sleep in a cohort of MG., Methods: Thirty-eight MG patients were telephonically interviewed 2months after the declaration of lockdown in India. The difficulty in procuring drugs, complications, and worsening in the MG Foundation of America (MGFA) stage were noted. The patients were enquired about MG Quality of Life 15 (MGQOL15), MG Activity of Daily Living (MGADL), Hospital Anxiety and Depression Scale (HADS) and Pittsburgh Sleep Quality Index (PSQI) using a prefixed questionnaire. Their pre-COVID parameters were retrieved from our earlier trial data, which was completed 4months back. The scores of the above mentioned parameters before and after COVID were compared., Results: Their median age was 45 years, and the median duration of treatment for MG was 4.5years. Eleven (28.9 %) patients were hypertensive and 3(7.9 %) diabetic. All were on prednisolone and 18(47.4 %) received azathioprine. None developed COVID, but three had other infections. Two patients needed hospitalization because of wrong medication in one and severe anxiety-insomnia in another. Following COVID19 and lockdown, MG patients had worsening in MGQOL15, MGADL, HADS and PSQI scores. Pittsburgh Sleep Quality Index score correlated with MGQOL15 and dose of acetylcholine esterase inhibitors., Conclusion: COVID-19 and lockdown were associated with anxiety, depression, poor MGQOL and sleep especially in severe MG patients., (Copyright © 2021. Published by Elsevier B.V.)

This cross-sectional study aimed to assess the impact of epilepsy, myasthenia gravis (MG), and multiple sclerosis (MS) on pregnancy and family planning decision-making in a cohort of Saudi women. Women with epilepsy, MG, and MS were recruited consecutively at the time of their follow-up visits at a neurology clinic. Data were collected using 3 standardized questionnaires, and presented using descriptive statistics. A logistic regression was performed to determine variables associated with decisions regarding abstaining from pregnancy and encouraging other women to conceive. A total of 272 (83 epilepsy, 69 MG, and 120 MS) women with a mean age of 29.9 ± 8.0 years participated. The proportion of women who abstained from or postponed pregnancy was 41.2% and 31.4%, respectively. The concerns mentioned most often were disease worsening during pregnancy, peripartum and postpartum, side effects of medications on the unborn child, and inability to care for the child. Older age was independently associated with the decision to abstain from pregnancy (odds ratio [OR] 1.14, 95% confidence interval [CI] 1.04 - 1.25). Higher knowledge levels were independently associated with encouraging other women to have children (OR 1.3, 95% CI 1.11-1.53). Over 50% of women reported that they were not counseled on issues related to pregnancy and childbirth. In conclusion, we identified a major influence of epilepsy, MG, and MS on pregnancy and family planning. Comprehensive counseling programs are needed to help women with these neurological diseases make informed family-planning decisions.

Background: Myasthenia gravis (MG), a chronic neuromuscular disorder, can adversely affect patients' health-related quality of life (HRQoL), especially in women. The study aimed to evaluate the difference in HRQoL of women and men MG patients and explore the factors that mediate the relationship between gender and HRQoL., Methods: A cross-sectional study was conducted among 1815 patients with MG in China. The revised 15-item MG quality of life scale (MG-QOL15r) was used to access patients' HRQoL in overall, physical, social and emotional domains. Socio-demographic information, diagnosis and treatment history, comorbidities, social support, active lifestyle and the MG activities of daily living scale (MG-ADL) were recorded and compared between women and men using the Student's t-test and Pearson's Chi-square test. Multivariable regression analyses were conducted to identify independent contributors to HRQoL, especially those affecting different gender., Results: On average, female patients with MG reported a lower MG-QOL15r score than the males (44.49 ± 29.10 vs 49.32 ± 29.18). The association between gender and patients' HRQoL interacted with the number of comorbidities across the overall, physical and social domains of patients. As the number of comorbidities increased, the scores of HRQoL decreased and it was faster among females than the males (p < 0.05). Moreover, unemployment, exacerbation of the disease, and active lifestyle contributed to the patients' HRQoL across all domains. Unemployment (β = - 4.99 [95%CI, - 7.80 to - 2.18], p < 0.001) and exacerbations (β = - 8.49 [95%CI, - 11.43 to - 5.54], p < 0.001) were correlated with poorer HRQoL; while an active lifestyle had a positive impact on HRQoL (β = 0.28 [95%CI, 0.16 to 0.40], p < 0.001)., Conclusions: The results indicate that the HRQoL of women MG patients was lower than that of men. The relationship between gender and HRQoL is modulated by the number of comorbidities. Thus, to improve the HRQoL of women MG patients, symptomatic treatments might not be enough, their comorbid conditions should be considered as well. Additionally, employment status, MG exacerbations, and an active lifestyle have been found as determining factors of the patients' HRQoL, which suggests future interventions should cope with these factors to improve their quality of life.

Background: Muscle weakness related to myasthenia gravis (MG) limits the daily functioning of patients. MG patients often experience subjective symptoms including psychiatric disorders, fatigue, and reduction in self-efficacy. All of which ultimately influence their life. The relationship between the subjective symptoms and health-related quality of life (HRQoL) has never been systematically explored among MG patients., Objective: This study aimed to evaluate the HRQoL of patients with MG in China, and to assess the impact of potential predictors of HRQoL., Methods: This was a cross-sectional observational study in patients with confirmed diagnosis of MG. Patients with MG were assessed using the 36-Item Short-Form Health Survey (SF-36) questionnaire, Self-Rating Depression Scale (SDS), Self-Rating Anxiety Scale (SAS), Fatigue Severity Scale (FSS), and Self-Efficacy for Managing Chronic Disease 6 items scale (SES6G). Disease severity was evaluated by two specialists at the same time., Results: Patients had significantly lower mean SF-36 scores for the categories role physical and general health (GH). The mean physical and mental composite scores were 57.76 ± 21.28 and 60.03 ± 23.75. Sex and unemployment influenced the QoL. Financial burden was negatively associated with total SF-36 scores. Compared to the control group, patients with generalized symptoms (MGFA II and III) had lower SF-36 scores, but the patients with pure ocular symptoms (MGFA I) had not significant difference, except GH. The SF-36 scores were highly correlated with the severity of the disease, the states of mood, fatigue, and self-efficacy., Conclusions: The decrease in the HRQoL of patients with MG was related not only to the gender, severity of disease, and unemployment but also to the subjective experience including depressive and anxiety disorders, fatigue, and self-efficacy. In the course of treatment, the evaluation of HRQoL should be included in the routine assessment of patients with MG. Psychosocial treatment, social support, and health education should be advocated., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Psychopathology and personality traits may influence the course of autoimmune disorders. With this prospective longitudinal cohort study, we aimed to assess personality, stress and depression in myasthenia patients who relapse and those who remain stable or improve (non-relapsers)., Method: We collected data from 155 consecutive adult patients with confirmed MG attending the Neuromuscular Clinic, Toronto General Hospital, between March 2017 and July 2018, for this study. Patients were assessed at baseline and 6 months, or at the time of MG relapse. At both visits, the patients were assessed clinically and were asked to complete self-administered questionnaires for disease severity, chronic stress and depression. Personality type was assessed at baseline only. Relapsing patients were defined as those patients with MGII score increasing by more than 5.5 points from visit 1 to visit 2., Results: Relapsers had higher baseline scores for depression (p = 0.01) and the change in disease severity correlated with the change in depression score (r = 0.2534, p = 0.0015, 95% CI: 0.098 0.3961). Higher levels of stress at baseline and neuroticism predicted higher relapse rates (p = 0.01 and p < .0001, respectively). In the linear regression model, with change of the MGII score as the dependent variable, change in depression scores (p = 0.0004) and age (p = 0.03) predicted change in disease severity., Conclusions: Since emotional factors and personality type may influence MG, attention to these factors might improve care in MG patients.

Introduction: To reduce myasthenia gravis (MG) patient risk of immunosuppressant (IS) exposure adverse events (AEs), such as infections and malignancies, and to reduce treatment burden, international guidelines recommend decreasing IS dose in stable MG patients., Methods: Online surveys were conducted of self-identified MG patients and MG physician experts about the importance of IS dose reduction for MG patients who achieve prolonged periods of disease stability., Results: Eighty-four percent of MG patients (n = 283) and 100% of physicians (n = 45) were concerned about long-term IS-associated AEs. Although both groups favored attempting IS reduction, they raised concerns including MG relapse, hospitalization, and uncertainty about the future. Presented with an estimated 12% significant relapse rate with IS dose reduction, 76% of patients would be willing to enroll in a randomized IS dose reduction trial., Discussion: Patients and physicians favor considering IS dose reduction but are also concerned about potential negative sequelae., (© 2020 Wiley Periodicals, Inc.)

Background: The revised 15-item Myasthenia Gravis (MG) Quality of Life Questionnaire (MGQoL15R) is a validated scale of quality of life in patients with MG. We aimed to study the factors causing the variability within the Arabic version of the MGQoL15R (MGQoL15R-A)., Method: A standardized questionnaire was completed by 118 patients. Correlations and hierarchical regression analyses were used to assess the contribution of sociodemographic variables, clinical factors, Patient Health Questionnaire-9 (PHQ9-A), and Generalized Anxiety Disorder-7 (GAD7-A) to the variability in the MGQoL15R-A., Results: The MGQoL15R-A was highly correlated with PHQ9-A (r = 0.76), and moderately correlated with GAD7-A (r = 0.52). Clinical factors and PHQ9-A independently explained 30.4% and 34.5% of the variability, respectively. Among the clinical factors, uncontrolled MG status, relapse within the past year, and a higher number of current MG therapies were significantly associated with a higher MGQoL15R-A score., Conclusions: MG severity and depressive symptoms (measured by PHQ9-A) can affect the MGQoL15R-A score., (© 2019 Wiley Periodicals, Inc.)

Background and Purpose: Stress is a known risk factor for the onset and modulation of disease activity in autoimmune disorders. The aim of this cross-sectional study was to determine any associations between myasthenia gravis (MG) severity and chronic stress, depression and personality type., Methods: In all, 179 consecutive adult patients with confirmed MG attending the Neuromuscular Clinic between March 2017 and December 2017 were included. At baseline, patients were assessed clinically and they completed self-administered scales for disease severity, perceived stress, depression and personality type., Results: Higher disease severity [Myasthenia Gravis Impairment Index (MGII)] showed a moderate correlation with depression score (Beck's Depression Inventory, Second Edition, r = 0.52, P < 0.001) and a lower correlation with chronic stress (Trier Inventory for Assessment of Chronic Stress, r = 0.28, P = 0.001). Chronic stress scores were different according to personality types (anova, P = 0.02). The linear regression model with MGII score as the dependent variable showed R 2 = 0.34, likelihood ratio chi-squared 74.55, with P < 0.0001. The only variables that predicted disease severity were depression scores (P < 0.0001) and female sex (P = 0.003)., Conclusions: A significant association of MG severity with depression and chronic stress was found, as well as with female gender. These findings should raise awareness that the long-term management of MG should address depression and potential stress and consider behavioural management to prevent stress-related immune imbalance., (© 2019 European Academy of Neurology.)

Objectives: This study is aimed at elucidating the prevalence of depression in patients with myasthenia gravis (MG) and examining the risk factors associated with depression., Methods: We evaluated adult patients with MG who were recruited from two tertiary hospitals in the central region (Riyadh) of Saudi Arabia. Data were collected with a two-part standardized questionnaire: the first part included data on sociodemographic and clinical features of MG including disease type and duration, therapies, prednisolone dose, time of the last relapse, previous critical care unit admissions, MG status (controlled, partially controlled, or uncontrolled), and comorbid diseases; the second part included items from the previously validated Arabic version of the Patient Health Questionnaire-9 (PHQ-9)., Results: In total, 104/150 (69.3%) patients participated (72 females) with a mean age of 38.0 ± 16.0 years. The mean PHQ-9 score was 7.02 ± 6.1. Among all the participants, 27 (26.0%) patients had depression (PHQ-9 ≥ 10). Multiple logistic regression analysis revealed that uncontrolled MG status (OR = 12.31, 95%CI = 1.13-133.8, P = 0.04) was the only factor independently associated with depression. Collectively, the prevalence of depression among patients of the primary care clinics (PCC) as reported by 5 previous studies across multiple regions of the country was 15.8%. The odds of depression among MG patients were twofold higher than those among PCC patients (OR = 2.05, 95%CI = 1.30-3.22, P = 0.002)., Conclusions: Approximately a quarter of MG patients have depression. Achieving a minimal manifestation or better MG status may decrease the depression rate in these patients., Competing Interests: The author has no conflict of interest to declare., (Copyright © 2019 Mohammed H. Alanazy.)

Background: Myasthenia gravis (MG) affects overall quality of life (QoL). The aim of the research was to evaluate QoL in patients suffering from MG in regard to epidemiological and clinical factors of the disease., Methods: The study included 70 patients. The severity of clinical manifestation was estimated using quantitative MG score and MG composite score. Patients were classified by using Myasthenia Gravis Foundation of America Classification. The Questionnaire of Life Quality Specific for Myasthenia Gravis-15 items (MGQOL15) is developed for QoL assessment in patients suffering from MG. In addition to the MGQOL15 revised version (MGQOL15r), a 36-item health survey of the Medical Outcomes Study Short Form (SF36) questionnaire, the Hamilton scales for the assessment of anxiety (HAM-A), and depression (HAM-D) were also used., Results: MGQOL15r scores are negatively correlated with SF36 scores and positively correlated with scores on HAM-A and HAM-D scales (P<0.001). Patients with longer disease duration and severe clinical manifestation often manifest anxiety, depression, and have poorer QoL (P<0.05). In relation to the presence of the anti-nAchR antibody, statistically significant differences were found in relation to the summed scores on scales for assessing QoL, as well as on a HAM-A scale (P<0.05). Physical workers and unemployed patients had worse scores in relation to retired and administrators/intellectual workers on all scales (P<0.001)., Conclusion: MGQOL15r is a life quality assessment questionnaire that could be used in routine practice in patients with MG. It is much simpler for use in these patients than SF36 and provides relevant data.

Objectives: Myasthenia gravis (MG) may be refractory to traditional therapies. Quality of life (QOL) and disease burden in patients with refractory and nonrefractory MG were compared using Myasthenia Gravis Foundation of America MG Patient Registry data., Methods: Adults aged 18 years or older with MG diagnosed ≥2 years before enrollment were included. Participants with refractory MG had received ≥2 previous and 1 current MG treatment and had MG Activities of Daily Living Scale total score ≥6 at enrollment; other participants had nonrefractory MG. MG QOL 15-item scale (MG-QOL15) scores were compared., Results: In total, 56 participants with refractory and 717 participants with nonrefractory MG enrolled. Participants with refractory MG had significantly higher mean (SD) MG-QOL15 total scores [31.4 (11.1) vs. 20.8 (15.0), P < 0.0001] and were more likely to have had exacerbations, emergency department visits, and recent hospitalizations., Conclusions: Participants with refractory MG experience worse QOL and greater clinical burden than those with nonrefractory disease.

Introduction: Muscle weakness and functional disability have not been evaluated in a population-based study of patients with myasthenia gravis (MG)., Methods: All patients with MG in a well-defined catchment area were identified in the Danish National Patient Registry. Of the 175 eligible patients, 90 participated and were studied using MG-specific scales, isometric dynamometry, and functional tests. Fifty age- and sex-matched subjects served as controls., Results: Muscle strength was reduced by 13%, 21%, and 12% for shoulder abduction, knee extension, and ankle extension, respectively (P < 0.05). Chair stand and 400-meter walking were impaired by 24% and 23%, respectively (P < 0.05). Muscle strength and functional performances were related to MG-specific scales., Discussion: MG patients have moderately reduced isometric muscle strength and impaired physical performance. Muscle weakness and functional tests relate closely to MG-specific scales, suggesting that dynamometry and functional tests can be used to monitor MG patients and as efficacy parameters in clinical trials. Muscle Nerve 59:218-223, 2019., (© 2018 Wiley Periodicals, Inc.)

Introduction: We sought to evaluate the test-retest reliability and construct validity of the 6- and 2-minute walk tests (6mWT and 2mWT, respectively) in patients with myasthenia gravis (MG)., Methods: Thirty-one patients with generalized MG were enrolled in this study. The 6mWT, 2mWT, MG-specific quality of life questionnaire Turkish version (MG-QoL15T), quantitative myasthenia gravis test (QMG), and pulmonary function tests were administered., Results: The intraclass correlation coefficients of 2mWT and 6mWT were 0.894 and 0.932, respectively. The 6mWT and 2mWT had moderate correlations with forced vital capacity, maximal inspiratory pressure, QMG score, and MG-QoL15T score (ρ for 6mWT: 0.579, 0.539, -0.572, and -0.474; ρ for 2mWT: 0.460, 0.446, -0.532, -0.457). Both tests had similar performances for predicting disease severity (area under the curve = 0.761 for 6mWT and 0.759 for 2mWT)., Discussion: The 6mWT and 2mWT have excellent test-retest reliability as well as moderate construct validity for the evaluation of functional exercise capacity patients with MG. Muscle Nerve 59:208-212, 2019., (© 2018 Wiley Periodicals, Inc.)

Introduction: We sought to translate, cross-culturally adapt, and evaluate the internal consistency and validity of the Chinese version of the 15-Item Myasthenia Gravis Quality of Life (MG-QOL15)., Methods: Translation and cross-cultural adaptation of the MG-QOL15 were performed. We used Cronbach's α to test internal consistency, one-way analysis of variance to test construct validity, and Pearson or Spearman correlations to test discriminant and concurrent validity., Results: We enrolled 168 outpatients. Internal consistency was excellent (Cronbach's α = 0.928). The MG-QOL15 discriminated MG severity as stratified by the MG Composite (MGC; P < 0.001) and Osserman class (P = 0.01). Concurrent validity was low to moderate with the subscales of the 36-item Short Form (-0.31 to ∼-0.59), MGC (r = 0.46), and Myasthenia Gravis Activities of Daily Living profile (r = 0.54)., Discussion: The Chinese MG-QOL15 showed comparable construct, discriminant and concurrent validity, and internal consistency with to the original version. Muscle Nerve 59:95-99, 2019., (© 2018 Wiley Periodicals, Inc.)

This update begins with myasthenia gravis and the roles of anti-agrin and cortactin antibodies. Regarding diagnosis, a report on repeated ice pack testing is highlighted as are several reports on the close correlation of electrodiagnostic testing with clinical features and the response to treatment. The incidence of head drop and associated clinical and ventilatory features are gleaned from a retrospective study. We also discuss a study that assessed the predominantly symmetric and conjugate ocular findings in MuSK-myasthenia gravis. Other topics that are covered include quality of life and preoperative risk. We then summarize the positive treatment trials of subcutaneous immunoglobulin and eculizumab. Turning to Lambert-Eaton Myasthenic Syndrome, we report on an epidemiologic study performed on the veteran affairs population, the results of the DAPPER study of 3, 4 diaminopyridine, and look to the future for other treatment options involving calcium gating modifiers.

Purpose: As most of patients with Myasthenia Gravis have limitations in their physical functioning, many experience changes in psychological states and often have depression. The objective of the current study was to examine the roles of communication with medical professionals, patients' loneliness, and patients' depression, in relation to their effects on the patients' quality of life., Methods: For 120 patients with MG of 18 years and older, demographic variables, along with communication with medical professionals, loneliness, depression, and quality of life were measured., Results: As a result, people suffering from MG experienced lower quality of life when their career has changed due to the illness. At the same time, depression was a significant predictor of their quality of life, both in physical and mental domains., Conclusions: The implications for clinical settings and the suggestions for future research are discussed., Competing Interests: The authors have declared that no competing interests exist.

Background: The subjective feeling of fatigue in myasthenia gravis (MG) is poorly elucidated, in part because it is often confounded with the objective sign of muscle fatigability. Another reason is the paucity of validated fatigue questionnaires in MG., Methods: We applied the 9-item Fatigue Severity Scale (FSS) and the 40-item Fatigue Impact Scale (FIS) to 73 MG patients and 230 age- and sex-matched control subjects. We ascertained levels of education, marital status, and comorbidities such as depression, sleepiness, sleep times and sleep debt. Disease severity was graded according to the Myasthenia Gravis Foundation of America (MGFA) classification., Results: All fatigue scores, with the exception of the cognitive FIS subscale, were higher in MG patients than controls. In MG, the prevalence of fatigue (defined by FSS scores ≥ 4.0) was 70%. Multiple regression analyses revealed several independent associates of fatigue, including depression (all fatigue scales), MGFA stage (FSS, physical FIS), female sex (cognitive and psychosocial FIS), and sleep debt (physical FIS)., Conclusion: Fatigue in MG is highly prevalent, mainly physical, and influenced by depressive symptoms, disease severity, female sex and sleep debt. Cognitive fatigue in MG may not be a direct disease manifestation, but secondary to depression. The FSS and FIS represent reliable and validated tools, appropriate to discern meaningful clinical aspects of fatigue in MG. Clinical recognition of the complexity of fatigue may foster individualized treatment approaches for affected MG patients.

It is believed that myasthenia gravis (MG) with antibodies to muscle-specific tyrosine kinase (MuSK) is the most severe form of the disease, especially in the first years of the disease. The aim of our study was to investigate quality of life (QoL) in a population of patients with MuSK MG compared to those with MG who have antibodies to acetylcholine receptor (AChR) in their sera. The study group consisted of 35 MuSK MG patients (28 females and 7 males), while the control group included 38 AChR MG patients matched for gender, age, and duration of the disease. SF-36 questionnaire was used to evaluate the health-related QoL. Following scales were also used: Hamilton's scales for depression and anxiety, the Multidimensional Scale of Perceived Social Support, and the Acceptance of Illness Scale. Physical domain scores of QoL were similarly affected in both MuSK and AChR groups, while mental domain and total SF-36 scores were even better in MuSK MG patients. Social support was better in the MuSK group (77.3 ± 9.3 vs. 70.6 ± 14.1, p < 0.05). SF-36 total score correlated with depression (rho = 0.54, p < 0.01), anxiety (rho = 0.49, p < 0.01), and MSPSS (rho = - 0.35, p < 0.05), and depression was an independent predictor of worse QoL. Besides therapy of weakness, psychiatric treatment and different forms of psychosocial condition should be part of regular therapeutic protocols for MG. Adequate team work of health professionals and family can provide a healthy mental environment in which a MuSK MG patient would feel more comfortable in spite of the disease.

Introduction: Patients with myasthenia gravis often experience fatigue, but its effect on quality of life (QoL) is underestimated, and fatigue is rarely measured in clinical trials., Methods: Two hundred fifty-seven myasthenic patients completed the Neuro-QoL-Fatigue and measures of disease severity and QoL. We studied the relationship between fatigue and clinical and demographic variables. Finally, we studied the responsiveness of the Neuro-QoL-Fatigue in 95 patients receiving treatments for myasthenia and estimated the minimal important difference (MID)., Results: Fatigue correlated with greater disease severity (r = 0.52-0.69, P < 0.0001) and worse QoL (r = 0.65-0.75, P < 0.0001). Patients in remission, with minimal manifestations, and pure ocular symptoms reported minimal fatigue. Regression modeling showed that, in addition to its relationship with disease severity, fatigue was worse in females, patients with generalized disease, and those with anxiety/depression. Fatigue improved after immunomodulation (P < 0.0001), and the MID was 5.3 points., Discussion: Fatigue in myasthenia correlates with disease severity, affects QoL, and can improve after treatment. Muscle Nerve 58: 197-203, 2018., (© 2018 Wiley Periodicals, Inc.)

Objective: To investigate the level and influencing factors of health-related quality of life in myasthenia gravis (MG) patients with myasthenia gravis quality of life-15 (MGQOL-15) Chinese version and to provide corresponding measures in one tertiary hospital of Sichuan Province., Methods: We collected the general data (gender, age, body mass index BMI, marital status, educational level and employee status), clinical data [Osserman type, myasthenia gravis composite (MGC), other immunopathies, disease duration, frequency of outpatient visits per month, ratio of disease cost to income each month and frequency of symptoms during the past month] and the MGQOL-15 Chinese version from 168 myasthenia gravis patients in one tertiary hospital of Sichuan Province., Results: The mean score of MGQOL-15 was 17.67±12.78. The score of the item "My occupational skills and job status have been negatively affected." was the highest, followed by "I have trouble using my eyes." and "I am frustrated by my MG." Single factor analysis showed that MG patients' QOL were different with different disease severity MGC (F=19.353, P<0.001), ratio of disease cost to income each month (F=5.831, P<0.001) and the frequency of symptoms during the past month (F=9.128,P<0.001). Multiple regression analysis showed that disease severity MGC (β=0.743,P<0.001), ration of disease cost to income each month (β=3.347,P<0.001) and the frequency of symptoms during the past month (β=2.216,P<0.003) were the main predictors of HRQOL in the MG patients., Conclusion: Our study showed that the MGQOL-15 is helpful for clinicians to evaluate MG patients' QOL regularly, investigate the influencing factors and implement corresponding interventions the so as to improve the patients' quality of life. Disease severity MGC, ratio of disease cost to income each month and the frequency of symptoms during the past month were the main predictors of MG patients' QOL. Clinicians should pay more attention to MG patients' disease severity MGC and the frequency of symptoms during the past month.

Purpose of Review: Myasthenia gravis (MG) is traditionally conceptualized as a disease with purely motor manifestations. This paper reviews the supporting evidence and pathophysiology of non-motor symptoms in MG, including pain, headache, special sense and autonomic dysfunction, sleep disturbance, and cognitive and psychosocial issues., Recent Findings: Work in this area has been limited. Recent studies have identified bodily pain and headache as common complaints in patients with MG. A growing literature also suggests that there may be an association of MG and sleep disturbance (both obstructive sleep apnea and sleep cycle dysfunction). Few studies suggest some measurable abnormalities of olfaction, gustation, audition, and autonomic function. The cognitive and psychosocial aspects of MG represent an emerging area of clinical and research interest, but large-scale data is sparse in the USA. The pathophysiology of MG is complex, and our understanding of the immunologic basis of this disease is expanding. The classic view of MG as a purely motor disorder may be incomplete. Recent work highlights non-motor symptoms that may impact patient management and quality of life.

Objective: To investigate the efficacy and safety of belimumab, a fully human immunoglobulin G1λ monoclonal antibody against B-lymphocyte stimulator, in participants with generalized myasthenia gravis (MG) who remained symptomatic despite standard of care (SoC) therapy., Methods: Eligible participants with MG were randomized 1:1 to receive IV belimumab 10 mg/kg or placebo in this phase II, placebo-controlled, multicenter, double-blind study (NCT01480596; BEL115123). Participants received SoC therapies throughout the 24-week treatment phase and 12-week follow-up period. The primary efficacy endpoint was mean change from baseline in the Quantitative Myasthenia Gravis (QMG) scale at week 24; safety assessments included the frequency and severity of adverse events (AEs) and serious AEs., Results: Forty participants were randomized (placebo n = 22; belimumab n = 18). The mean change in QMG score from baseline at week 24 was not significantly different for belimumab vs placebo ( p = 0.256). There were no statistically significant differences between treatment groups for secondary endpoints, including the MG Composite and MG-Activity of Daily Living scores. Acetylcholine receptor antibody levels decreased over time in both treatment groups. No unexpected AEs were identified and occurrence was similar in the belimumab (78%) and placebo (91%) groups. One participant receiving placebo died (severe sepsis) during the treatment phase., Conclusions: The primary endpoint was not met for belimumab in participants with generalized MG receiving SoC. There was no significant difference in mean change in the QMG score at week 24 for belimumab vs placebo. The safety profile of belimumab was consistent with previous systemic lupus erythematosus studies., Classification of Evidence: This study provides Class I evidence that for participants with generalized MG, belimumab did not significantly improve QMG score compared with placebo., (Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Myasthenia gravis (MG) is a neuromuscular autoimmune disease characterized by skeletal muscle weakness which can impact motor function and, furthermore, produce negative impact on the health-related quality of life (HRQOL)., Objective: To evaluate the predictors for HRQOL in patients with MG., Methods: Eighty patients were evaluated with the MG Foundation of America classification and the MG Composite scale. HRQOL was estimated by the MGQOL15, while anxious and depressive symptoms were evaluated with the Hospital Anxiety and Depression Scale (HAD)., Results: The mean age of patients was 41.9 years with mean illness duration of 13.5 years. Almost half of the patients (43.75%) had significant anxiety and more than a quarter (27.50%) had depressive symptoms. Factors that influenced the HRQOL in MG were skeletal muscle weakness and anxiety and depressive symptoms (p < .001 in logistic regression model)., Conclusion: Anxiety and depressive symptoms, besides motor symptoms, influence HRQOL in MG. Mental health must be a clinical focus in addition to the treatment of somatic symptoms during the course of MG., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Introduction: We sought to translate, culturally adapt, and assess the Arabic version of the 15-item myasthenia gravis quality-of-life revised scale (MGQOL15R)., Methods: We assessed reliability with Cronbach α; reproducibility with intraclass correlation coefficient (ICC); validity with Spearman correlations for myasthenia gravis (MG)-specific activities of daily living (MG-ADL), MG composite (MGC) score, and MG manual muscle test (MG-MMT) and with MGQOL15R in patients with different disease severity through the Kruskal-Wallis test; and sensitivity to change with Wilcoxon signed-rank test., Results: In 65 enrolled patients, the mean MGQOL15R score was 10.84 ± 8.11 (α = 0.94, ICC = 0.95). The correlation coefficients between MGQOL15R and MGC, MG-ADL, and MG-MMT scores were 0.75, 0.75, and 0.74, respectively (P < 0.001). MGQOL15R scores were significantly higher (worse) in patients with more severe disease at baseline and significantly lower (better) in improved patients at follow-up., Discussion: The Arabic version of MGQOL15R is valid, reliable, stable, and sensitive to changes. Muscle Nerve 57: 581-585, 2018., (© 2017 Wiley Periodicals, Inc.)

It is known that quality of life in myasthenia gravis is positively correlated with subjective sleep quality, still no data is available regarding the relationship between QOL and polysomnographically detected sleep parameters and disorders. In this study, we tried to highlighten this relationship, by performing polysomnography. Sleep-related complaints were evaluated in face-to-face interviews with 19 clinically stable MG patients and 26 healthy controls. During the interviews questionnaires assessing sleep quality, excessive daytime sleepiness, fatigue, depression, anxiety, and Turkish version of the MG-QOL 15-item scale [(MG-QOL15(T)] were administered and then an overnight polysomnography was performed. Sleep disorders, especially obstructive sleep apnea and fatigue were higher, whereas subjective sleep duration was significantly lower, in patients than controls. Excessive daytime sleepiness and poor sleep quality were not different between patients and controls. Other than percentage of sleep stage III, which was negatively correlated with MG-QOL15(T) scores, neither other sleep parameters nor sleep disorders were correlated with MG-QOL15(T) scores. MG composite, subjective sleep duration, fatigue severity and Hamilton depression rating scale scores were found to be positively correlated with MG-QOL15(T) scores. It was shown that decreasing disease severity and enhancing psychological well-being will improve patients' quality of life. We recommend that our findings should be repeated in a large prospective cohort of MG patients.

Introduction: The Myasthenia Gravis-Activities of Daily Living (MG-ADL) profile scale is a simple-to-use instrument. We aimed to validate this scale in the Korean language and compare physician- and self-assessed MG-ADL scores (pMG-ADL-K and sMG-ADL-K)., Methods: pMG-ADL-K and sMG-ADL-K and MG Composite (MGC) scores were obtained from patients. The correlation between pMG-ADL-K and MGC and the relationship between the pMG-ADL-K and sMG-ADL-K were assessed using the Cronbach α and the Spearman coefficient. By intraclass correlation coefficient (ICC), the reliability of each sub-item of pMG-ADL-K and sMG-ADL-K was evaluated., Results: We included data from 40 patients. The pMG-ADL-K score showed a strong correlation with the MGC score (rho = 0.80, P < 0.01). The Cronbach α was 0.98 between pMG-ADL-K and sMG-ADL-K, and sub-items showed good consistency (ICC 0.684-0.985, P < 0.001)., Discussion: The MG-ADL-K is a valid tool and the sMG-ADL-K shows excellent correlation with pMG-ADL-K. Both the pMG-ADL-K and sMG-ADL-K can be used to measure MG severity. Muscle Nerve 57: 419-422, 2018., (© 2017 Wiley Periodicals, Inc.)

Introduction: Assessing myasthenia gravis (MG) can be challenging, and multiple scales are available to evaluate disease severity. We evaluated the utility of a single, simple question, as part of the MG evaluation: "What percentage of normal do you feel regarding your MG, 0%-100% normal?", Methods: A retrospective chart review of patients attending the neuromuscular clinic from January 2014 to December 2015 was performed. Responses were correlated with symptoms and signs, the Quantitative Myasthenia Gravis Score (QMGS), the Myasthenia Gravis Impairment Index (MGII), and the 15-item Myasthenia Gravis Quality of Life scale (MG-QOL15)., Results: The total cohort included 169 patients. The percentage of normal correlated strongly with limb muscle weakness and MG scales, moderately with bulbar and respiratory symptoms, and weakly with ocular manifestations., Discussion: The question, "What percentage of normal do you feel regarding your MG?" is feasible and valid, and can be incorporated easily into routine clinical evaluation. Muscle Nerve 57: 240-244, 2018., (© 2017 Wiley Periodicals, Inc.)

Introduction: The 15-item Myasthenia Gravis Quality of Life (MG-QOL15) scale has been developed to assess the health-related quality of life of patients with myasthenia gravis (MG). The aim of this study was to translate the original English version into Dutch and to test the test-retest reliability and construct validity., Methods: Fifty patients with MG were included. Test-retest reliability and internal consistency were assessed using the intraclass correlation coefficient (ICC) and the Cronbach α. Construct validity was assessed by testing 5 predefined hypotheses., Results: A good test-retest reliability was confirmed with an ICC of 0.866. The Cronbach α was 0.93. The predefined hypotheses were confirmed in 80% of cases, which points to good construct validity., Discussion: The Dutch MG-QOL15 has good test-retest reliability and good construct validity. It can be used for research in a Dutch-speaking population. It is also suitable for monitoring individual patients in clinical practice. Muscle Nerve 57: 206-211, 2018., (© 2017 Wiley Periodicals, Inc.)

BackgroundFatigue in myasthenia gravis (MG) is common and difficult to manage. Unlike myasthenic weakness it is not amenable to drug therapies.ObjectiveOur primary aim was to investigate whether a combination of physical and psychological therapy would help address symptoms of fatigue in MG patients, who have stable disease but residual problematic fatigue. Our secondary aim was to quantitate fatigue by applying different scores and to ascertain which would be most relevant to apply in MG.MethodsWe recruited 10 MG patients with stable disease and who suffer from fatigue. Nine of these 10 patients participated in a 10-week program that involved physical and psychological intervention. We quantified their fatigue using the modified fatigue impact scale (MFIS), the visual analogue fatigue scale (VAFS) and the fatigue severity scale (FSS) at the start of the study, at various intervals during the program and 3 months later.ResultsDuring the program, there was a small improvement in the physical and psychosocial subscale of the MFIS. There was a significant improvement (p < 0.01) in the VAFS at the end of the program. No clear improvement was noted in FSS. Three months later, all fatigue scores declined to baseline but 50% of patients had made some life-style changes.ConclusionsThis is a small pilot study, which utilized a combined approach with physical and psychological therapy, and showed some benefit in improving fatigue in patients with MG. The improvement was small and unsustained. Because of the small patient cohort, one cannot derive any firm conclusions and a larger study is required to investigate this further.

Objective: To study responsiveness and meaningful change of the Myasthenia Gravis Impairment Index (MGII) and its relative efficiency compared to other measures., Methods: We enrolled 95 patients receiving prednisone, IV immunoglobulin (IVIg), or plasma exchange (PLEX) and 54 controls. Patients were assessed with the MGII and other measures-including the Quantitative Myasthenia Gravis Score, Myasthenia Gravis Composite, and Myasthenia Gravis Activities of Daily Living-at baseline and 3-4 weeks after treatment. Statistical markers of responsiveness included between-groups and within-group differences, and we estimated the relative efficiency of the MGII compared to other measures. Patient-meaningful change was assessed with an anchor-based method, using the patient's impression of change. We determined the minimal detectable change (MDC) and the minimal important difference (MID) at the group and individual level., Results: Treated patients had a higher change in MGII scores than controls (analysis of covariance p < 0.001). The ocular domain changed more with prednisone than with IVIg/PLEX (effect size 0.67 and 0.13, analysis of covariance p = 0.001). The generalized domain changed more with IVIg/PLEX than with prednisone (effect size 0.50 and 0.22, analysis of covariance p = 0.07). For the total MGII score, the individual MDC95 was 9.1 and the MID was 5.5 for individuals and 8.1 for groups. Relative efficiency ratios were >1 favoring the MGII., Conclusions: The MGII demonstrated responsiveness to prednisone, IVIg, and PLEX in patients with myasthenia. There is a differential response in ocular and generalized symptoms to type of therapy. The MGII has higher relative efficiency than comparison measures and is viable for use in clinical trials., (Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

The purpose of this study is to report on the validity, reliability, and sensitivity of the myasthenia gravis activities of daily living profile (MG-ADL) in a sample of Italian patients. Patients with myasthenia gravis (MG) completed a protocol that included the MG-ADL, the WHO Disability Assessment Schedule (WHODAS 2.0), the Besta Neurological Institute rating scale for myasthenia gravis, and the MG-composite. Cronbach's alpha was used to test reliability, Spearman's correlation and intra-class correlation coefficient (ICC) to test short-term test-retest, Kruskal-Wallis test to assess differences in MG-ADL between patients with different disease severity, and Wilcoxon signed-rank test to assess sensitivity to change. In total, 58 patients were enrolled: 44 were females, mean MG duration 10.5 ± 10.4 years, mean MG-ADL 3.98 ± 3.07. The MG-ADL showed good internal consistency (alpha = .774), stability (test-retest correlation = .98, ICC = .97). It was superior to the WHODAS 2.0 in differentiating patients with different MG type and severity (P < .001), it showed higher sensitivity to change (P = .001 for improved and P = .007 for worsened patients) and higher correlation with the MG-composite (RHO = .625). Our analysis shows that the Italian version of the MG-ADL is valid, reliable, stable, and sensitive to change.

Introduction: In this study we assess the Italian version of the 15-item Myasthenia Gravis Quality-of-Life questionnaire (MG-QOL15)., Methods: The validation protocol included the MG-QOL15, the 36-item Short Form (SF-36), the Besta Neurological Institute Rating Scale for Myasthenia Gravis, and the MG-Composite. We used the Cronbach α to test reliability, the Spearman correlation to test short-term test-retest, the Kruskal-Wallis test to assess differences in MG-QOL15 between patients with different disease severity, and the Wilcoxon signed-rank test to assess sensitivity to change., Results: Seventy-two patients were enrolled in the study. The mean MG-QOL15 score was 15.2 ± 12.2, with α = 0.93 and test-retest correlation = 0.93. Compared with the SF-36, the MG-QOL15 was superior in differentiating patients with different MG types (P = 0.041) and severity (P = 0.004), showed higher sensitivity to change (P = 0.003 for improved and P = 0.024 for worsened patients), and had higher correlations with the MG-Composite (rho = 0.367 vs. -0.213 and -0.154)., Conclusion: The Italian version of the MG-QOL15 is valid, reliable, stable, and sensitive to changes. Muscle Nerve 56: 716-720, 2017., (© 2016 Wiley Periodicals, Inc.)

Introduction: A phase 2 study of eculizumab for treating myasthenia gravis (MG) used the quantitative myasthenia gravis score (QMG) and myasthenia gravis activities of daily living profile (MG-ADL) to evaluate baseline disease severity and treatment response. Correlations were then analyzed between these assessments., Methods: Patients were given eculizumab or placebo during the first 16-week treatment period of the crossover study, with treatment assignments reversed for the second treatment period following a 5-week washout. QMG and MG-ADL scores at baseline and endpoint of each treatment period generated correlation coefficients for baseline status and treatment response during eculizumab therapy., Results: Correlation strength between QMG and MG-ADL scores was higher for treatment response (R = 0.726; 95% confidence interval, 0.264-0.907; P = 0.0036) than for assessing baseline disease status (R = 0.552; 95% confidence interval, -0.022-0.839; P = 0.0495)., Conclusions: MG-ADL may be more sensitive for assessing treatment response than point-in-time disease status. Muscle Nerve 56: 328-330, 2017., (© 2016 Wiley Periodicals, Inc.)