Epidemiology, diagnostics, and biomarkers of autoimmune neuromuscular junction disorders

The Lancet Neurology, Год журнала: 2022, Номер 21(2), С. 176 - 188

Опубликована: Янв. 20, 2022

Язык: Английский

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Safety and efficacy of zilucoplan in patients with generalised myasthenia gravis (RAISE): a randomised, double-blind, placebo-controlled, phase 3 study

The Lancet Neurology, Год журнала: 2023, Номер 22(5), С. 395 - 406

Опубликована: Апрель 13, 2023

Язык: Английский

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High‐Performance Hydrogel Sensors Enabled Multimodal and Accurate Human–Machine Interaction System for Active Rehabilitation

Advanced Materials, Год журнала: 2023, Номер 36(11)

Опубликована: Дек. 14, 2023

Human-machine interaction (HMI) technology shows an important application prospect in rehabilitation medicine, but it is greatly limited by the unsatisfactory recognition accuracy and wearing comfort. Here, this work develops a fully flexible, conformable, functionalized multimodal HMI interface consisting of hydrogel-based sensors self-designed flexible printed circuit board. Thanks to component regulation structural design hydrogel, both electromyogram (EMG) forcemyography (FMG) signals can be collected accurately stably, so that they are later decoded with assistance artificial intelligence (AI). Compared traditional multichannel EMG signals, human-machine method based on combination FMG significantly improves efficiency increasing information entropy signals. The decoding from only two channels for different gestures reaches 91.28%. resulting AI-powered active system control pneumatic robotic glove assist stroke patients completing movements according recognized human motion intention. Moreover, further generalized applied other remote sensing platforms, such as manipulators, intelligent cars, drones, paving way future robot systems.

Язык: Английский

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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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Myasthenia gravis—Pathophysiology, diagnosis, and treatment

Handbook of clinical neurology, Год журнала: 2024, Номер unknown, С. 283 - 305

Опубликована: Янв. 1, 2024

Язык: Английский

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The Lived Experience of Myasthenia Gravis: A Patient-Led Analysis

Neurology and Therapy, Год журнала: 2021, Номер 10(2), С. 1103 - 1125

Опубликована: Окт. 23, 2021

A greater understanding of the reality living with myasthenia gravis (MG) may improve management and outcomes for patients. However, there is little published data on patient perspective how MG impacts life. Our objective was to reveal lived experience from perspective. This analysis led by an international Patient Council comprising nine individuals who serve as local/national advocates in seven countries (Europe United States). Insights into were consolidated three sources (a qualitative research study 54 people or their carers countries; a previous meeting [September 2019]; literature review). prioritised Council, discussed during virtual workshop (August 2020) articulated series statements organised domains. Overarching themes that describe identified authors. From 114 insights supporting quotes, defined 44 summary Five overarching MG. These include fluctuating unpredictable symptoms; constant state adaptation, continual assessment trade-offs all aspects life; treatment inertia, often resulting under-treatment; sense disconnect healthcare professionals; feelings anxiety, frustration, guilt, anger, loneliness depression. patient-driven enriches our

Язык: Английский

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The humanistic burden of myasthenia gravis: A systematic literature review

Journal of the Neurological Sciences, Год журнала: 2022, Номер 437, С. 120268 - 120268

Опубликована: Апрель 21, 2022

While the clinical manifestations of myasthenia gravis (MG) are well understood, its humanistic impact is not. The objective this systematic literature review (SLR) was to provide a comprehensive understanding burden MG with regards psychological symptoms and health-related quality life (HRQoL) according patients caregivers.A search conducted on December 27, 2019, in MEDLINE Embase identify English-language studies that were published from January 1, 2009-December 2019 presented relevant information among adults their caregivers. Title/abstract full-text screening performed by two investigators, any discrepancies resolved third investigator.Sixty-seven publications included SLR. Compared general population, experienced worse HRQoL. Studies reporting MG, including depression, anxiety, fatigue, sleep, heterogeneous terms scales instruments used assess patients, as patient populations themselves. However, those more severe hospitalization days had depression fatigue sleep improved disease remission and/or improvement. Scores for females compared males where evaluated, HRQoL scores generally following treatment.While demonstrates associated get better improvement remission, additional options efficacious therapy adequately address disease-related also improve may contribute beneficial outcomes greater number MG.

Язык: Английский

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The effectiveness and side effects of pyridostigmine in the treatment of myasthenia gravis: a cross-sectional study

Neuromuscular Disorders, Год журнала: 2022, Номер 32(10), С. 790 - 799

Опубликована: Сен. 7, 2022

Pyridostigmine is the most commonly used drug in symptomatic treatment of myasthenia gravis (MG); however, research into its effectiveness and side effects scarce. The aim this study was to assess effectiveness, prevalence net benefit pyridostigmine. All MG patients participating Dutch-Belgian patient registry were included. A dynamic online questionnaire developed Out 642 invited patients, 410 (64%) fully completed questionnaire; 61% reported that they currently pyridostigmine, 36% had discontinued pyridostigmine 2% never have Patients a median 60, IQR 28-78 65, 45-84. Of all using 91% (vs. 55% control group). Most frequently flatulence, urinary urgency, muscle cramps, blurred vision hyperhidrosis. In group who reason for discontinuation 26%. Diarrhea, abdominal cramps twitching cited reasons discontinue These results can be guide shared decision making prior starting MG.

Язык: Английский

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Myasthenia gravis, respiratory function, and respiratory tract disease

Journal of Neurology, Год журнала: 2023, Номер 270(7), С. 3329 - 3340

Опубликована: Апрель 26, 2023

Abstract Myasthenia gravis (MG) is characterized by muscle weakness caused autoantibodies that bind to the postsynaptic membrane at neuromuscular junction and impair acetylcholine receptor function. Weakness of respiratory muscles represents most severe MG manifestation, 10–15% all patients experience an crisis with need mechanical ventilatory support least once in their life. active immunosuppressive drug treatment long term, they regular specialist follow-up. Comorbidities affecting function attention optimal treatment. Respiratory tract infections can lead exacerbations precipitate crisis. Intravenous immunoglobulin plasma exchange are core treatments for exacerbations. High-dose corticosteroids, complement inhibitors, FcRn blockers represent fast-acting effective patients. Neonatal myasthenia a transient condition newborn mother’s antibodies. In rare cases, baby required.

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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