Expert consensus recommendations for improving and standardising the assessment of patients with generalised myasthenia gravis

European Journal of Neurology, Journal Year: 2024, Volume and Issue: 31(7)

Published: March 24, 2024

Regular and consistent disease assessment could provide a clearer picture of burden in generalised myasthenia gravis (gMG) improve patient care; however, the use tools practice lacks standardisation. This modified Delphi approach was taken to review current evidence on tool gMG develop expert-derived consensus recommendations for good practice.

Language: Английский

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The impact of diagnosis delay on European patients with generalised myasthenia gravis

Annals of Clinical and Translational Neurology, Journal Year: 2024, Volume and Issue: 11(9), P. 2254 - 2267

Published: Aug. 1, 2024

Abstract Objective The objective was to determine the mean duration of diagnosis delay for patients with myasthenia gravis from five European countries and explore impact >1 year delay. Methods Patients ( N = 387) Europe (France/Germany/Italy/Spain/United Kingdom) their physicians participated in Adelphi Real World Myasthenia Gravis Disease Specific Programme™. Diagnosis (time symptom onset diagnosis) calculated characteristics described experiencing ≤1 Denominators varied according outcome as missing data were not imputed. Results Mean (standard deviation) 363.1 (520.9) days, 27.1% (105 out experienced year. Among delay, respectively, 69.2% (72 104) 17.4% [45 259] had initially received a different (physician‐reported); 40.0% (42 105) 24.1% (68 282) Foundation America class III at time survey 72.4% (76 61.3% (173 fatigue (subjective physician reporting pre‐selected list symptoms); 30.5% (32 (49 anxiety 21.9% (23 13.1% (37 depression (both subjective list, Likert‐style); MG‐QoL‐15r score 14.4 (5.50) 12.6 (7.84) (self‐reported by 43 74 patients, respectively). Interpretation More than quarter These clinical profile regards severity, symptoms, comorbidities score, compared

Language: Английский

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Systematic review of the patient burden of generalised myasthenia gravis in Europe, the Middle East, and Africa

BMC Neurology, Journal Year: 2024, Volume and Issue: 24(1)

Published: Feb. 10, 2024

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

Language: Английский

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Myasthenia gravis: Molecular mechanisms and promising therapeutic strategies

Biochemical Pharmacology, Journal Year: 2023, Volume and Issue: 218, P. 115872 - 115872

Published: Oct. 21, 2023

Language: Английский

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The Burden Patients with Myasthenia Gravis Experience in Terms of Breathing, Fatigue, Sleep, Mental Health, Discomfort and Usual Activities in Comparison to the General Population

Advances in Therapy, Journal Year: 2023, Volume and Issue: 41(1), P. 271 - 291

Published: Nov. 3, 2023

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

Language: Английский

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Results of rehabilitation of myasthenia gravis patients with subclinical respiratory disturbances

Neuromuscular Diseases, Journal Year: 2025, Volume and Issue: 15(1), P. 27 - 38

Published: April 26, 2025

Background. The main cause of morbidity and mortality in myasthenia gravis (MG) patients is respiratory failure that based on subclinical disorders. Rehabilitation opportunities for such are limited by the frequent presence a subjective feeling fatigue important to distinguish from symptom disease – pathological muscle fatigability. This determined need achieve balance between obvious benefits physical activity one hand rapid increase fatigability other peculiarities selection exercises. Aim. To analyze effectiveness rehabilitation MG with disturbances, determine level MG, assess effect fatigue. Materials methods. was studied 53 using visual analog scale, “Well-being, activity, mood” questionnaire assessment scale. Those who had no therapy enhancement within 6 months before start as well during entire period were included study net rehabilitation. external respiration function after course. Before conducting sample size planning method used independed depended groups. Based results calculations group 22 generalized without signs disorders formed possibilities Results. According significant (U, p = 0.002) frequency (χ2, 0.002), poor health 0.041 according scale χ2, <0.0001 questionnaire), increased 0.005) showed. negative well-being (rs –0.78; <0.05) –0.73; established. As result vital capacity (W, 0.026) inspiratory reserve volume 0.044) achieved. Good tolerance absence effects exercise general 0.495) have been There deterioration questionnaire: 0.467), 0.396) mood 0.709). 0.368) Conclusion. Taking into account pronounced patient’s condition should be selected individually, preventing an fatigue, weakness. Soft, mild or moderate intensity exercises tolerated side effects.

Language: Английский

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Fatigue and primary sarcopenia in geriatric patients

Revista da Associação Médica Brasileira, Journal Year: 2022, Volume and Issue: 68(11), P. 1565 - 1570

Published: Nov. 1, 2022

This study aimed to investigate the frequency of fatigue in geriatric patients with primary sarcopenia and evaluate relationship between symptoms such as depression sleepiness.This case-control was conducted December 2020 August 2021 geriatrics outpatient clinic Istanbul University-Cerrahpasa. The European Working Group on Sarcopenia Older People 2 criteria were considered for diagnosis sarcopenia. Demographic data, accompanying chronic diseases, comprehensive assessments, laboratory values noted. Scales used assess all participants include Fatigue Assessment Scale, Severity Impact Scale associated Geriatric Depression Epworth Sleepiness Scale.The mean (standard deviation) age 75.3 (7.1) 51 (38 female) 73.5 (5.8) control (37 patients. There no significant difference two groups terms gender (p=0.822, p=0.171). prevalence hypertension higher, level education lower sarcopenic group than nonsarcopenic (p=0.017, p=0.013). total, cognitive, physical, social questionnaire scores significantly higher (all p<0.001). score statistically group; however, there (p=0.014, p=0.072). Multivariate analysis performed level, hypertension, questionnaires, which found be univariate logistic regression analysis. In multivariate analysis, only total determined [odds ratio 1.161, 95% confidence interval (1.084-1.242)].In sarcopenia, is mental well physical fatigue. Therefore, prevention treatment important.

Language: Английский

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Factors associated with the disease family burden of caregivers of myasthenia gravis patients in northwestern China: A cross-sectional study

Journal of Clinical Neuroscience, Journal Year: 2023, Volume and Issue: 119, P. 70 - 75

Published: Nov. 20, 2023

Patients with myasthenia gravis (MG) lose part of their working or living ability due to illness, and bring burden caregivers. The purpose this study was explore the factors related caregivers' disease family for MG patients in Northwest China.

Language: Английский

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Patient perceptions of disease burden and treatment of myasthenia gravis based on sentiment analysis of digital conversations

Scientific Reports, Journal Year: 2024, Volume and Issue: 14(1)

Published: March 27, 2024

Abstract Myasthenia gravis (MG) is a rare, autoimmune, antibody-mediated, neuromuscular disease. This study analyzed digital conversations about MG to explore unprovoked perspectives. Advanced search, data extraction, and artificial intelligence-powered algorithms were used harvest, mine, structure public domain from US Internet Protocol addresses (August 2021 August 2022). Thematic analyses examined topics, mindsets, sentiments/key drivers via natural language processing text analytics. Findings described by sex/gender treatment experience with steroids or intravenous immunoglobulin (IVIg). The 13,234 extracted message boards (51%), social media networks (22%), topical sites (21%), blogs (6%). Sex/gender was confirmed as female in 5703 male 2781 conversations, 3255 IVIg 2106 conversations. Topics focused on diagnosis (29%), living (28%), symptoms (24%), (19%). Within 3176 symptoms, eye problems facial muscle (18%), fatigue (18%) most commonly described. Negative sentiments expressed 59% of only 2% considered positive. dominated themes impact life misdiagnosis (27%), issues symptom severity (20%). Impact key driver negativity both men (27%) women (34%), dominant theme steroid-treated (29%) IVIg-treated (31%) patients. Of 1382 discussing barriers, 36% side effects, 33% lack efficacy, 21% misdiagnosis, 10% cost/insurance. Side effects formed the main barrier Capturing patient voice reveals high degree concern related burden disease, common treatments among those MG, pointing need for options that can improve quality life.

Language: Английский

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Development and validation of the scale for symptom clusters in patients with myasthenia gravis

BMC Neurology, Journal Year: 2023, Volume and Issue: 23(1)

Published: May 19, 2023

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.To develop assessment patients MG.A cross-sectional descriptive study.Based the unpleasant theory(TOUS), first draft was developed through review literature, qualitative interview, Delphi expert correspondence, items were presented adjusted cognitive interviews 12 patients. To conveniently assess validity reliability scale, survey conducted 283 who recruited from Tongji Hospital Medical College, Huazhong University Science Technology, June to September 2021.The final cluster consisted 19 items(MGSC-19), content index ranging 0.828 1.000 each item 0.980. Four common variables (ocular muscle weakness, general muscular treatment-related side effects, psychiatric problems) identified by exploratory factor analysis, explained 70.187% total variance. The correlation coefficients between dimension overall score ranged 0.395 0.769 (all P < 0.01), while dimensions varied 0.324 0.510 0.01). Cronbach's alpha, retest reliability, half 0.932, 0.845, 0.837, respectively.The MGSC-19 generally good. This be employed identify help healthcare givers individualized management measures MG.

Language: Английский

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