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

European Journal of Neurology, Год журнала: 2024, Номер 31(7)

Опубликована: Март 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.

Язык: Английский

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

Neuromuscular Disorders, Год журнала: 2020, Номер 30(8), С. 631 - 639

Опубликована: Июль 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

Язык: Английский

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Neural dysregulation in post-COVID fatigue

Brain Communications, Год журнала: 2023, Номер 5(3)

Опубликована: Янв. 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.

Язык: Английский

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

Brain and Behavior, Год журнала: 2022, Номер 13(1)

Опубликована: Дек. 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.

Язык: 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, Год журнала: 2023, Номер 12(5), С. 1573 - 1590

Опубликована: Май 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

Язык: Английский

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Improvement of fatigue in generalised myasthenia gravis with zilucoplan

Journal of Neurology, Год журнала: 2024, Номер 271(5), С. 2758 - 2767

Опубликована: Фев. 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

Язык: Английский

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Prevalence and associated factors of fatigue in autoimmune myasthenia gravis

Neuromuscular Disorders, Год журнала: 2021, Номер 31(7), С. 612 - 621

Опубликована: Апрель 24, 2021

Язык: Английский

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Fatigue, physical activity and associated factors in 779 patients with myasthenia gravis

Neuromuscular Disorders, Год журнала: 2021, Номер 31(8), С. 716 - 725

Опубликована: Май 27, 2021

The objective of the study was to examine association between fatigue (measured by Multidimensional Fatigue Inventory; MFI-20) and physical activity Saltin-Grimby Physical Activity Level Scale; SGPALS) in a large cohort patients (≥18 years) with myasthenia gravis (MG) including relevant disease - lifestyle-related factors. A total 1463 persons, registered at Danish National Registry Patients MG diagnosis, according International Classification Diseases, received web-based survey. 779 (53% women, mean [SD] age 60.8 [15.5]) responded. remaining persons were either non-responders (n = 390) or could not confirm diagnosis 294). most prominent MFI-20 domains general (median [inter-quartile ranges, IQR], 13 [10-16]) [IQR], [9-15]), 386 (53%) reported low levels activity. All associated (p<.01). Higher level lower fatigue. Important factors for severity Myasthenia Gravis Activities Daily Living profile), body mass index, insomnia Insomnia Severity Index) job-status, comorbidity, cohabitation.

Язык: 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, Год журнала: 2024, Номер 17

Опубликована: Янв. 1, 2024

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

Язык: Английский

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

Molecular Neurobiology, Год журнала: 2025, Номер unknown

Опубликована: Фев. 11, 2025

Язык: Английский

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