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Magnesium sulfate and calcium channel blocking drugs as antidotes for acute organophosphorus insecticide poisoning - a systematic review and meta-analysis.

INTRODUCTION: Treatment of acute organophosphorus or carbamate insecticide self-poisoning is often ineffective, with tens of thousands of de … orus poisoned patients. A small neurophysiological study of magnesium sulfate reported reversion of neuromuscular junction effects of organophosphorus insecticide exposure. Comparative clinical studies: Only four of eight studies were randomized controlled trials; all studies were of magnesium sulfate, of small to modest size, and at substantial risk of bias. They inclu …

Abstracts of the 19th International Congress on Neuromuscular Diseases 7th – 11th July 2026

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… Abstract Topic Groups (Submission Categories) Topic Group 2 - Diseases of Neuromuscular Junction: Clinical Features, Pathophysiology, Therapy: Myasthenia Gravis OS04.05 Efficacy of Complement vs. … junction. …

Regenerative Peripheral Nerve Interface (RPNI) Surgery for Mitigation of Neuroma and Postamputation Pain.

BACKGROUND: A neuroma occurs when a regenerating transected peripheral nerve has no distal target to reinnervate. Symptomatic neuromas are a … ly of denervated muscle targets for regenerating nerve axons and facilitates the reestablishment of neuromuscular junctions without sacrificing denervation of any residual muscles. EXPECTED OUTCOMES: Articles describing RPNI surgery for postamputation pain have shown favorable outcomes, with significant reduction in neuroma pain and phantom pain scores at approximately …

Genetic and Mechanistic Insights Inform Amyotrophic Lateral Sclerosis Treatment and Symptomatic Management: Current and Emerging Therapeutics and Clinical Trial Design Considerations.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting both upper and lower motor neurons. ALS is classically characterized by painless progressive weakness, causing impaired function of limbs, speech, swallowing, and respiratory function. The disease is fatal within 2-4 years, often the result of respiratory failure. The pathologic hallmark for a majority of ALS cases is aberrant cytoplasmic accumulations of the nuclear protein TAR-DNA binding protein (TDP-43). A total of 10-15% of ALS can be attributed to a single gene mutation, known as genetic or "familia

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… Junction and Axonal Transport The neuromuscular junction (NMJ) is the crucial connection between the motor neuron axon terminal and the muscle. … Table 8 Investigational therapeutics in ALS phase 1, 2, and 3 clinical trials targeting the neuromuscular junction and axonal transport Abbreviations: ASO, antisense oligonucleotide; ER, endoplasmic reticulum; est, estimated; MuSK, muscle-specific kinase; NMJ, neuromuscular junction; TDP-43, transactive …

Autoimmune Neuromuscular Disorders at a Molecular Crossroad: Linking Pathogenesis to Targeted Immunotherapy.

Myasthenia gravis, chronic inflammatory demyelinating polyneuropathy, and idiopathic inflammatory myopathies are among the most widely recognized autoimmune neuromuscular disorders. Although they differ in clinical presentation, shared immunopathogenic mechanisms place them at a molecular crossroads. Evidence of overlapping pathways has led to the development of targeted strategies including complement inhibition, FcRn antagonism, B-cell depletion, and the CAR-T cell approach. In this review, we analyze current knowledge regarding pathogenic mechanisms and their link to immunotherapy, extensiv

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… Tacrolimus, a calcineurin inhibitor, serves as a steroid-sparing agent and may further benefit patients by stabilizing acetylcholine receptor clustering at the neuromuscular junction [ 70 , 71 ]. … This identifies a complement-injured niche at the neuromuscular junction and highlights the mechanism of C5 inhibition [ 107 ]. …

Эффективность неконцентрированных действий Координирующие Шоковые Волны в сочетании с ботулинумтоксином на спасти у пациентов с умом.

Спастичность — это обычное осложнение у пациентов с неврологическими расстройствами, возрастающей инвалидностью и ухудшающей качество жизни. Было продемонстрировано, что комбинированная обработка с сфокусированными ударными волнами (FESWT) и ботулиновым токсином (BONT-A) повышает эффективность и продолжительность воздействия BONT-A на спасти у пациентов с травмами мозга. Мы изучили эффективность сочетания неконцентрированных ударных волн (uESWT) и BONT-A по спастисти. Это перспективное исследование с систематической случайностью включает в себя 24 :: пациенты с травмой мозга, небольшой размер

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… Thus, the postsynaptic effect on the neuromuscular junction, reducing acetylcholine receptors, would be added to the already known presynaptic effect of BoNT-A. … These results would support a summation of the effects of the toxin at the presynaptic level and of the focal and unfocused shock wave at the postsynaptic level in the neuromuscular junction. …

Comprehensive analysis of acute flaccid paralysis with and without myelitis in Taiwanese children.

BACKGROUND: Acute flaccid paralysis (AFP) is a clinical syndrome marked by the sudden onset of muscle weakness or paralysis, requiring immediate medical intervention due to its potential for significant morbidity and mortality. Despite extensive studies on AFP, comparative analyses between cases with myelitis (M-AFP) and non-myelitis (NM-AFP) remain scarce. This study seeks to address this gap by analyzing demographic, clinical, and etiological distinctions between these groups. METHODS: A retrospective study was conducted on 39 pediatric AFP patients diagnosed between 2012 and 2021. Particip

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… However, AFP can also occur without myelitis, with a diverse array of underlying etiologies, including neuromuscular junction disorders, peripheral neuropathies, metabolic disturbances, and even hematologic malignancies. … For example, inflammatory myopathies may initially resemble viral myositis, while neuromuscular junction disorders like myasthenia gravis may be mistaken for behavioral fatigue [ 24 ]. …

VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations.

Mutations in VCP cause multisystem degeneration impacting the nervous system, muscle, and/or bone. Patients may present with ALS, Parkinsonism, frontotemporal dementia, myopathy, Paget's disease, or a combination of these. The disease mechanism is unknown. We developed a Drosophila model of VCP mutation-dependent degeneration. The phenotype is reminiscent of PINK1 and parkin mutants, including a pronounced mitochondrial defect. Indeed, VCP interacts genetically with the PINK1/parkin pathway in vivo. Paradoxically, VCP complements PINK1 deficiency but not parkin deficiency. The basis of this pa

Outcomes of Conservative Versus Surgical Treatment of Dropped Head Syndrome in a Single Institution: A Case Series and Review of the Literature.

Dropped head syndrome (DHS), also known as camptocephalia, is characterized by a mobile chin-on-chest deformity from hypotonia of the cervical extensor muscle or hypertonia of the anterior neck muscles. There remains a paucity of quality published literature on this topic, particularly its management. The objective of this study is to identify radiographic and clinical outcomes of surgery as compared to nonsurgical treatment, to summarize the literature, and to create a decision-making paradigm for managing patients with DHS. As such, we report outcomes of our retrospective chart review series

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… EMG studies showed motor neuron disease, neuromuscular junction disorder, or generalized myopathy. He was diagnosed with neck flexor dystonia in the setting of his parkinsonism and early dementia. He decided to proceed with PSIF C2-T6. …

Immunological Targets in Generalized Myasthenia Gravis Treatment: Where Are We Going Now?

BackgroundGeneralized myasthenia gravis (gMG) is a heterogeneous autoimmune disorder marked by antibody-mediated disruption of neuromuscular transmission. Despite advancements in immunosuppressive therapies and biologics, a subset of patients remains refractory, necessitating more targeted and personalized treatment strategies.ObjectiveThis review aims to synthesize current knowledge of the immunopathological mechanisms across gMG subtypes and to explore emerging therapeutic targets tailored to these diverse disease phenotypes.MethodsA narrative review was conducted, integrating recent finding

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… Autoantibodies play a central role in the pathogenesis of MG due to their well-established direct pathogenic effects at the neuromuscular junction (NMJ) [ 15 ]. … junction. …

CAR T клеточная терапия в неврологических расстройствах, вызванных автоантибиозами: многообещающая стратегия.

Клеточная терапия химерного антигенного рецептора (CAR) Т становится перспективным подходом к неврологическим аутоиммунным расстройствам на базе клеток B, особенно тем, которые характеризуются патогенными автоантиорганизмами, направленными на ключевые нейронные структуры. Эти заболевания, включая расстройство спектрального спектра нейромиелита, миастения гравис, синдром Ламберта-Итона миастеника, болезнь, связанная с антителами MOG, энцефалит рецептора против NMDA, диацилглицирол губазы альфа антитела, ассоциируемый с энцефалитом, синдромом жесткого человека и множественным склерозом, могут бы

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… MG is caused by autoantibodies against AChR or MuSK at the neuromuscular junction, impairing synaptic transmission. LEMS is mediated by VGCC antibodies, reducing calcium influx and acetylcholine release. … MG is caused by autoantibodies against AChR or MuSK at the neuromuscular junction, impairing synaptic transmission. LEMS is mediated by VGCC antibodies, reducing calcium influx and acetylcholine release. …

Mechanisms and effects of metformin on skeletal muscle disorders.

Skeletal muscle disorders are mostly genetic and include several rare diseases. With disease progression, muscle fibrosis and adiposis occur, resulting in limited mobility. The long course of these diseases combined with limited treatment options affect patients both psychologically and economically, hence the development of novel treatments for neuromuscular diseases is crucial to obtain a better quality of life. As a widely used hypoglycemic drug in clinical practice, metformin not only has anti-inflammatory, autophagy-regulating, and mitochondrial biogenesis-regulating effects, but it has a

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… The neuromuscular junction (NMJ), which connects motor neurons to skeletal muscle fibers, plays a crucial role in controlling muscle contractions ( 1 ). Fragmentation of NMJs has been observed in DMD patients and mdx mouse models ( 105 ). … Alterations of neuromuscular junctions in Duchenne muscular dystrophy. Neurosci Lett. (2020):737. doi: 10.1016/j.neulet.2020.135304 106. Pratt SJP, Shah SB, Ward CW, Kerr JP, Stains JP, Lovering RM. Recovery of altered neuromuscular junction morphology and muscle function in mdx mice after injury. …

Biomolecular condensates as dynamic regulators of musculoskeletal homeostasis, disease, and therapeutic challenges.

Biomolecular condensates are membraneless assemblies that concentrate proteins, nucleic acids, and other biomolecules into dynamic cellular compartments. Liquid-liquid phase separation (LLPS) is one important route by which such condensates form, particularly when multivalent interactions generate liquid-like, reversible assemblies. However, not every condensate or disease-associated assembly should be explained solely by LLPS. In the musculoskeletal system, condensates have been linked to transcription, signal transduction, RNA metabolism, stress responses, tissue development, homeostasis, an

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… The neuromuscular junction provides a clear example. … Tissue access is another barrier: mineralized bone, avascular cartilage, fibrotic muscle, and the neuromuscular junction impose different diffusion, retention, and clearance constraints. …

Chimeric Antigen Receptor T-Cells in Myasthenia Gravis: Advances, Safety Challenges, and Future Directions.

This review examines the emerging application of chimeric antigen receptor (CAR) T-cell therapy in myasthenia gravis (MG), with emphasis on safety, efficacy signals, and future therapeutic potential in treatment-refractory disease. A comprehensive literature search was conducted across PubMed, medRxiv, bioRxiv, and Google Scholar for studies published between January 1, 2010 and July 1, 2025, using the terms "CAR T-cell," "chimeric antigen receptor," and "myasthenia gravis." Eligible reports included clinical trials, case reports, case series, and mechanistic studies describing CAR T-cell ther

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… Early progress was grounded in detailed pathophysiologic insights that linked specific antibody subclasses to neuromuscular‐junction injury and thereby rationalized new targets [ 1 , 4 ]. … Bril , “Autoimmune Neuromuscular Junction Disorders 3 Advances and Ongoing Research in the Treatment of Autoimmune Neuromuscular Junction Disorders,” 21 , no. 2 ( 2022 ): 189 – 202 . 10.1016/S1474-4422(21)00463-4 35065041 60 K. Hofmann , A. K. Clauder , and R. A. …

The Role of Cullin-RING Ligases in Striated Muscle Development, Function, and Disease.

The well-orchestrated turnover of proteins in cross-striated muscles is one of the fundamental processes required for muscle cell function and survival. Dysfunction of the intricate protein degradation machinery is often associated with development of cardiac and skeletal muscle myopathies. Most muscle proteins are degraded by the ubiquitin-proteasome system (UPS). The UPS involves a number of enzymes, including E3-ligases, which tightly control which protein substrates are marked for degradation by the proteasome. Recent data reveal that E3-ligases of the cullin family play more diverse and c

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… Summarized, data on the inhibition of neddylation through Mln4924 and siRNA point towards crucial roles for CRL activity during skeletal muscle formation and neuromuscular junction development. … While these results demonstrate the importance of neddylated CRL for neuronal synapses, they may also suggest potential roles for Nae1 functions in the development and function of motorneurons and the neuromuscular junction. …

Benefit and danger from immunotherapy in myasthenia gravis.

In the last years, significant advances have improved the knowledge of myasthenia gravis (MG) immunopathogenesis and have enabled to realize new molecules with a selective action targeting compounds of the immunological system. This review discusses emerging treatments for MG, including complement inhibitors, neonatal Fc receptor targeting agents, and B cell interfering drugs, focusing on benefit and danger. In the second section of the review, several related adverse events of immunotherapy, including MGonset, are debated.

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… Introduction Myasthenia gravis (MG) is an autoimmune disease caused by autoantibodies targeting neuromuscular junction (NMJ) components as the acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and lipoprotein-related peptide 4 (LRP4). … Effect of D-penicillamine on neuromuscular junction in patients with Wilson disease. Neurology. 2004;63:935–936. doi: 10.1212/01.WNL.0000137021.90567.37. 56. Krenn M, Grisold A, Wohlfarth P, Rath J, Cetin H, Koneczny I, Zimprich F. Neuromuscular disorders induced by medical treatments. …

Резюме 18th Неевромускульная научно-исследовательская конференция Соединенного Королевства 15th и 16th апрель 2025.

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… A hallmark of motor unit pathology in SMA involves the loss of motor neurons and disruption of neuromuscular junctions (NMJs). … Initial fluorescence microscopy of P16 diaphragms revealed neuromuscular junction morphology indistinguishable from age-matched WT, with robust staining for AChR. …

Химерический антигенный рецептор T-клеточная терапия в нейроиммунных болезнях: механистическое обоснование, клинический опыт и проблемы с переводом.

Нейроиммунные заболевания представляют собой разнородную группу расстройств, при которых клетки В, плазменные удары, плазменные клетки, автоантитела и Т клетки поразному влияют на повреждение тканей. Хотя биологика B-клеток улучшила контроль за заболеваниями, некоторые пациенты по-прежнему сталкиваются с реанимацией, рецидивом после лечения или кумулятивной неврологической инвалидностью. Химерическая антигенная рецепторная T-клетка (CAR-T-клетка) является средством достижения более глубокого и потенциально устойчивого истощения определенных патогенных иммунных клеток. Этот описательный обзор в

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… The relevance of this limitation is disease dependent, however, and does not apply equally to disorders centered in the peripheral nervous system or at the neuromuscular junction. … The central schematic represents CAR-T cell manufacture and infusion and does not imply direct targeting of astrocytes, oligodendrocytes, neurons, or the neuromuscular junction. …

Neurology

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… Neuromuscular Diseases Disorders of the motor unit are rare in cats and affect skeletal muscle, the neuromuscular junction, or the lower motor neuron. …

New Targeted Agents in Myasthenia Gravis and Future Therapeutic Strategies.

Myasthenia gravis (MG) is a chronic autoimmune disease for which multiple immunomodulatory therapies are available. Nevertheless, MG has a significant impact on patient quality of life. In recent years, experts' main efforts have focused on optimizing treatment strategies, since disease burden is considerably affected by their safety and tolerability profiles, especially in patients with refractory phenotypes. This article aims to offer neurologists caring for MG patients an overview of the most innovative targeted drugs specifically designed for this disease and summarizes the recent literatu

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… Pathophysiology of Myasthenia Gravis MG is caused by the failure of neuromuscular transmission resulting from the binding of autoantibodies to signaling proteins—mostly the nicotinic acetylcholine receptor (AChR)—at the neuromuscular junction (NMJ). … Complement Deposition at the Neuromuscular Junction in Seronegative Myasthenia Gravis Acta Neuropathol. 2020 139 1119 1122 10.1007/s00401-020-02147-5 32157386 PMC7244604 24. Koneczny I. Herbst R. …