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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Fatigue in patients with myasthenia gravis. A systematic review of the literature

Neuromuscular Disorders, Journal Year: 2020, Volume and Issue: 30(8), P. 631 - 639

Published: July 1, 2020

Myasthenia Gravis (MG) is a chronic autoimmune disease affecting the neuromuscular junction. Although hallmark of MG muscle fatigability due to dysfunction junction (peripheral fatigue), large number patients also report symptoms central fatigue, defined as an experienced lack energy, physically and/or mentally. We systematically reviewed literature on all aspects fatigue in MG. Results were categorized 5 domains: prevalence, diagnosis, pathophysiology, treatment or impact. The prevalence patient-reported varies between 42 and 82%, which significantly higher than control subjects. Fatigue severity usually assessed with standardized questionnaires, but choice questionnaire widely studies. pathophysiology unknown, it strongly associated depressive symptoms, female gender severity. highly prevalent ocular remission, suggesting multifactorial origin. Fatigued have lower quality life. Pharmacological improvement promising results been found physical psychological training programs. symptom severe negative impact Physicians treating should be aware this symptom, may treatable

Language: Английский

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Neural dysregulation in post-COVID fatigue

Brain Communications, Journal Year: 2023, Volume and Issue: 5(3)

Published: Jan. 1, 2023

Abstract Following infection with SARS-CoV-2, a substantial minority of people develop lingering after-effects known as ‘long COVID’. Fatigue is common complaint impact on daily life, but the neural mechanisms behind post-COVID fatigue remain unclear. We recruited 37 volunteers self-reported after mild COVID and carried out battery behavioural neurophysiological tests assessing central, peripheral autonomic nervous systems. In comparison age- sex-matched without (n = 52), we show underactivity in specific cortical circuits, dysregulation function myopathic change skeletal muscle. Cluster analysis revealed no subgroupings, suggesting single entity individual variation, rather than small number distinct syndromes. Based our analysis, were also able to exclude sensory feedback circuits descending neuromodulatory control. These abnormalities objective may aid development novel approaches for disease monitoring.

Language: Английский

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Measuring Overall Severity of Myasthenia Gravis (MG): Evidence for the Added Value of the MG Symptoms PRO

Neurology and Therapy, Journal Year: 2023, Volume and Issue: 12(5), P. 1573 - 1590

Published: May 11, 2023

Accurate measurement of myasthenia gravis (MG) severity is required for appropriate clinical monitoring patients with MG and assessment the benefit new treatments in trials. Our objective was to explore how can be measured determine newly developed Symptoms Patient-Reported Outcome (PRO) instrument complements available measures severity. The conceptual coverage Quantitative (QMG), Composite (MGC), MG-Activities Daily Living (MG-ADL), PRO scrutinized against core symptoms MG: muscle weakness three groups (ocular, bulbar, respiratory), fatigability, physical fatigue. Post hoc analyses MG0002 study, a Phase 2a trial rozanolixizumab adults moderate severe generalized MG, included correlation Rasch model analyses. qualitative appraisal highlighted that only captured Data from 541 assessments (43 unique patients) were used Correlations ranged between 0.56 0.74 MG-ADL, QMG, MGC, Muscle Weakness Fatigability score, 0.20 0.71 scores focusing on independent groups. Analyses estimated meaningful continuum including all items, except ocular muscles, four instruments. QMG had broadest continuum. fatigability fatigue more characteristic low while bulbar indicated MG. reflected underpinned by MG-specific outcome measures. Only manifestations shown reflect possibly different facet With its modular nature comprehensive content, provides complementary information widely ClinicalTrials.gov identifier: NCT03052751. Myasthenia chronic disease affecting communication nerves muscles. People experience worsens after activity improves rest. affect body muscles (e.g., around eyes, limbs, face or throat). We show various summarize overall disease: people mild often report fast onset their limb fatigue, those also difficulties associated facial throat (leading difficulty swallowing speaking) respiratory (making breathing difficult). This ordering will help create accurate methods assess evaluate monitor clinic. suggest eyes eyelid drooping double vision) may represent aspect not provide as much other symptoms. However, this needs further investigation our study did include participants who eye symptom. document ability questionnaire, questionnaire completed patients, useful measuring

Language: Английский

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Improvement of fatigue in generalised myasthenia gravis with zilucoplan

Journal of Neurology, Journal Year: 2024, Volume and Issue: 271(5), P. 2758 - 2767

Published: Feb. 24, 2024

Abstract Background Fatigue is a debilitating symptom of myasthenia gravis (MG). The impact fatigue on MG can be assessed by Quality Life in Neurological Disorders (Neuro-QoL) Short Form scale. Transformation raw Neuro-QoL scores to T-scores known approach for facilitating clinical interpretation clinically meaningful and severity thresholds. Methods In the Phase 3, double-blind, placebo-controlled RAISE study (NCT04115293), adults with acetylcholine receptor autoantibody-positive generalised (MG Foundation America Disease Class II–IV) were randomised 1:1 daily subcutaneous zilucoplan 0.3 mg/kg or placebo 12 weeks. Patients completing could opt receive an ongoing, open-label extension study, RAISE-XT (NCT04225871). this post-hoc analysis, we evaluated long-term effect patients who entered RAISE-XT. We report change levels from baseline Week 60. Results Mean improved group ( n = 86) difference versus 88; least squares mean difference: − 3.61 (nominal p -value 0.0060]), these improvements continued further At 12, more 34, 47.2%) experienced ≥ 1 level 23, 28.4%; 0.017). 60, most 55, 65.5%) had mild none. Conclusion Treatment demonstrated statistical during RAISE, which sustained 60

Language: Английский

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Prevalence of depression and anxiety among myasthenia gravis (MG) patients: A systematic review and meta‐analysis

Brain and Behavior, Journal Year: 2022, Volume and Issue: 13(1)

Published: Dec. 10, 2022

Myasthenia gravis (MG) people experience adverse psychiatric outcomes, which may impact on their life and disturb daily activity. Depression anxiety are identified as significant problems that MG face. However, there is no sufficient epidemiological information about depression anxiety-based publication. Due to this limitation, the aim of study was review prevalence in patients.

Language: Английский

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Efficacy and safety of rozanolixizumab in patients with muscle-specific tyrosine kinase autoantibody-positive generalised myasthenia gravis: a subgroup analysis of the randomised, double-blind, placebo-controlled, adaptive phase III MycarinG study

Therapeutic Advances in Neurological Disorders, Journal Year: 2024, Volume and Issue: 17

Published: Jan. 1, 2024

Muscle-specific tyrosine kinase (MuSK) autoantibody-positive (Ab+) generalised myasthenia gravis (gMG) is a rare and frequently severe subtype of gMG.

Language: Английский

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Prevalence and associated factors of fatigue in autoimmune myasthenia gravis

Neuromuscular Disorders, Journal Year: 2021, Volume and Issue: 31(7), P. 612 - 621

Published: April 24, 2021

Language: Английский

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Serum Inflammatory Factors Levels and Risk of Myasthenia Gravis: A Bidirectional Mendelian Randomization Study

Molecular Neurobiology, Journal Year: 2025, Volume and Issue: unknown

Published: Feb. 11, 2025

Language: Английский

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Safety and efficacy of chronic weekly rozanolixizumab in generalized myasthenia gravis: the randomized open-label extension MG0004 study

Journal of Neurology, Journal Year: 2025, Volume and Issue: 272(4)

Published: March 19, 2025

In the Phase 3 MycarinG study (NCT03971422), six once-weekly subcutaneous infusions of rozanolixizumab significantly improved myasthenia gravis (MG)-specific outcomes versus placebo in patients with acetylcholine receptor or muscle-specific tyrosine kinase autoantibody-positive generalized MG (gMG). Following completion MycarinG, could enroll open-label extension MG0004 (NCT04124965) to receive chronic weekly rozanolixizumab. Patients were re-randomized 1:1 7 10 mg/kg for up 52 infusions. The primary endpoints occurrence treatment-emergent adverse events (TEAEs) and TEAEs leading discontinuation. After ≥6 visits/infusions switch MG0007 (NCT04650854) cyclic treatment. MG0004, 70 received (n = 35) 35). Mean treatment duration was 22.9 23.7 weeks, respectively, due rollover into MG0007. reported 60/70 (85.7%) patients; most mild/moderate. frequently headache (25/70 [35.7%]), diarrhea (13/70 [18.6%]) decreased blood immunoglobulin G (11/70 [15.7%]). There no opportunistic, serious severe infections, hypersensitivity injection-site reactions, any anaphylactic reactions albumin lipid abnormalities. Maximum mean reduction from baseline Activities Daily Living score 3.1 group 4.1 group. Chronic generally well tolerated, clinically relevant improvements across MG-specific maintained, supporting long-term use gMG. NCT04124965 (registered October 11, 2019).

Language: Английский

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