Результаты поиска

Preliminary establishment of a spinal stability scoring system for multiple myeloma.

BackgroundMultiple myeloma is an incurable malignant plasma cell disorder that represents the most common primary malignant bone tumor. It commonly involves bone metastasis in multiple vertebral bodies, and the Spinal Instability Neoplastic Score scoring system may not be fully applicable to multiple myeloma (MM) patients.AimTo evaluate the spinal stability of patients with MM spinal involvement to guide their clinical treatment.MethodsBy using the Delphi method, we collected and extracted information through a series of questionnaires and improved it via feedback. We also preliminarily establ

Физическая активность, упражнения и реабилитация в Миастении Гравис: систематический обзор и сводный анализ.

Миастения гравис вызывает колебания, оживляемую слабость, создавая неопределенность в отношении физической активности и физических упражнений. В ходе этого систематического обзора были изучены обычная физическая активность и оседлое поведение, препятствия и факторы, препятствующие занятиям спортом, структурированные упражнения, мышечная подготовка дыхательных путей и физическая реабилитация в аутоиммунной миастении. С самого начала был проведен поиск в PubMed, Scopus и Web of Science Core Collection. 30 июль 2026, без даты базы данных или лингвистических фильтров; поиск в обратном направлении

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… Introduction and background Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Antibodies most often target the acetylcholine receptor and, in smaller subgroups, muscle-specific kinase or low-density lipoprotein receptor-related protein 4. …

Clinical management and therapeutic development for the rare disease rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is a rare disease that arises from skeletal muscle mainly affects children and adolescents. Patients with RMS have diverse symptoms and prognosis based on tumor sizes, tumor anatomical locations, histological subtypes of the tumors and genetic testing of paired-box-forkhead box O1 (PAX-FOXO1) fusion gene. The 4 subtypes of RMS include embryonal RMS (eRMS), alveolar RMS (aRMS), spindle cell/sclerosing RMS (scRMS) and pleomorphic RMS (pRMS). Treatment for RMS patients remains challenging due to its heterogeneous nature. Thus, a combinatory approach is likely to warrant bet

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… Neuromuscular disorders affect the peripheral nerves that control voluntary muscles or neuromuscular junction. Muscular dystrophy results in wasting and loss of muscle tissue, disability and possible deformity. …

Posttraumatic Vernet syndrome without fracture: A case report and short literature review.

RATIONALE: The aim of this case is to emphasize the need to include nerve traction in the differential diagnosis of nerve deficits associated with Vernet syndrome. This mechanism of injury has been described only once, but must not be overlooked and should be considered and included as a possible cause in diagnostic algorithms. PATIENT CONCERNS: A patient presenting with dysphagia, extreme hoarseness, and limited shoulder movement after head injury was admitted to the emergency department. DIAGNOSES: Multidisciplinary evaluation was performed, and nerve traction-induced Vernet syndrome was e

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… Neuromuscular junction disease was excluded by a negative prostigmine test. An analysis of anti-acetylcholine receptor and anti-muscle specific kinase antibodies also proved to be negative as well as a multidisciplinary specialist assessment. …

Rapid Fire

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Ячейки памяти в инфекциях и автономном иммунитете: механизмы, функции и терапевтические последствия.

Клетки памяти имеют ключевое значение для способности адаптивной иммунной системы запоминать и эффективно реагировать на ранее встречавшиеся патогены. В то время как клетки памяти обеспечивают надежную защиту от инфекций, они могут также способствовать автоматическому иммунитету в тех случаях, когда регулирование оказывается неэффективным. Здесь мы анализируем роль клеток памяти Т и В в инфекции и автоматическом иммунитете, с уделением особого внимания их дифференциации, активации, функций воздействия и основополагающих механизмов регулирования. Мы подробно остановимся на конкретных механизмах

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… In MG, the immune system produces autoantibodies that target and block the acetylcholine receptors (AChRs) on the postsynaptic membrane of neuromuscular junctions. These autoantibodies hinder the normal communication between nerve and muscle cells, leading to muscle weakness. …

Геномический диагноз детских расстройств: революция и эволюция.

Мощные, последние достижения в технологиях анализа генома оказали глубокое воздействие на практику медицинской генетики как в лаборатории, так и в клинике. Более широкое использование общегеномных тестов, таких, как анализ хромосомных микроарра и последовательность экзомов, привело к переходу к "генотипному" подходу. В настоящее время имеются многочисленные методы диагностики конкретного синдрома или фенотипа, и, хотя традиционные методы остаются эффективными инструментами в некоторых ситуациях, технологии с более высокой производительностью фактически стали лабораторным инструментом диагности

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… Likewise, neuromuscular conditions are a highly heterogeneous group of disorders that include neuropathies, myopathies, motor neuron disease, and neuromuscular junction disorders. …

Association of Immune Checkpoint Inhibitors With Neurologic Adverse Events: A Systematic Review and Meta-analysis.

ImportanceNeurologic adverse events (NAEs) due to immune checkpoint inhibitors (ICIs) can be fatal but are underexplored.ObjectiveTo compare NAEs reported in randomized clinical trials (RCTs) of US Food and Drug Administration-approved ICIs with other forms of chemotherapy and placebo.Data sourcesBibliographic databases (Embase, Ovid, MEDLINE, and Scopus data) and trial registries (ClinicalTrials.gov) were searched from inception through March 1, 2020.Study selectionPhase II/III RCTs evaluating the use of ICIs were eligible for inclusion. Unpublished trials were excluded from the analysis.Data

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… NAE includes the manifestation of any neurologic toxicity secondary to ICI use in the central nervous system, including the brain, brainstem, and spinal cord, extending to the peripheral nervous system, which includes the neuromuscular junction and muscle fibers. …

Effects of Physical Cues on Stem Cell-Derived Extracellular Vesicles toward Neuropathy Applications.

The peripheral nervous system undergoes sufficient stress when affected by diabetic conditions, chemotherapeutic drugs, and personal injury. Consequently, peripheral neuropathy arises as the most common complication, leading to debilitating symptoms that significantly alter the quality and way of life. The resulting chronic pain requires a treatment approach that does not simply mask the accompanying symptoms but provides the necessary external environment and neurotrophic factors that will effectively facilitate nerve regeneration. Under normal conditions, the peripheral nervous system self-r

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… Once a nerve experiences interrupted stimulation to the neuromuscular junction, the release of neurotransmitters for muscular contraction is halted [ 145 ]. Therefore, voluntary and involuntary muscular contractions are impaired, increasing neuropathic symptoms [ 146 ]. …

A review of neurotoxicities associated with immunotherapy and a framework for evaluation.

Immuno-oncology agents, including immune checkpoint inhibitors (ICIs) and chimeric antigen receptor T (CAR-T) cell therapies, are increasing in use for a growing list of oncologic indications. While harnessing the immune system against cancer cells has a potent anti-tumor effect, it can also cause widespread autoimmune toxicities that limit therapeutic potential. Neurologic toxicities have unique presentations and can progress rapidly, necessitating prompt recognition. In this article, we review the spectrum of central and peripheral neurologic immune-related adverse events (irAEs) associated

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… Neuromuscular junction disorders Myasthenia gravis (MG) can occur after ICI treatment, either due to preexisting disease exacerbation or de novo disease. …

Paraneoplastic Neurological Syndromes: Advances and Future Perspectives in Immunopathogenesis and Management.

Paraneoplastic neurological syndromes (PNSs) are immune-mediated disorders caused by an antitumor response that cross-reacts with the nervous system, leading to severe and often irreversible neurological disability. Once considered exceedingly rare, PNSs are now increasingly recognized owing to the identification of novel neural autoantibodies, wider use of commercial testing, and the emergence of immune checkpoint inhibitor (ICI)-related neurotoxicity that phenotypically overlaps with classic PNS. In this narrative review, we performed a structured search of PubMed/MEDLINE, Scopus, Web of Sci

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… Clinical Characteristics The clinical spectrum of PNS is vast, potentially affecting any level of the neuraxis from the cerebral cortex to the neuromuscular junction. … Finally, Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic disorder of the neuromuscular junction causing proximal weakness that improves with exercise. Approximately 60% of cases are paraneoplastic, almost exclusively associated with SCLC [ 20 , 48 ]. 5.2. …

Гипоксия в множестве склерозов.

Низкая доступность кислорода (гипоксии) является заметной, но плохо понимаемой особенностью множественного склероза (МС). Неизвестно, вызывает ли гипоксия патологию и симптомы MS или она является следствием других патологических явлений, таких, как воспаление и сосудистая дисфункция. Здесь мы резюмируем имеющуюся литературу о взаимосвязи между гипоксией и как патологическими, так и симптомными характеристиками MS. Острая экологическая гипоксия (т.е. высота) может вызвать или облегчить события, связанные с MS, возможно, путем усиления гипоксии тканей в центральной нервной системе. Соответственн

Вирусы и "Мозг-отношение к беде".

Неврологические расстройства, некоторые из которых связаны с вирусными инфекциями, растут из-за старения и увеличения численности населения. Несмотря на крепкую защиту центральной нервной системы, некоторые вирусы эволюционировали, чтобы пробить их, что часто приводит к ужасным последствиям. В этом обзоре мы рассказываем о различных способах проникновения различных вирусов в ЦНС и о последствиях таких вторжений. Последствия могут проявляться в виде острых заболеваний, таких, как энцефалит, менингит, или приводить к долгосрочным последствиям, таким, как нервно - мышечная дисфункция, как это име

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… After inoculation by a bite or scratch from an infected animal, the virus enters a motor neuron of the PNS via receptor-mediated endocytosis at the neuromuscular junction [ 38 , 39 ]. RABV retrogradely traverses the neuron by hijacking the microtubule transport machinery [ 40 , 41 ]. …

The role of heat shock protein 90 in idiopathic pulmonary fibrosis: state of the art.

Heat shock protein 90 (HSP 90) and its isoforms are a group of homodimeric proteins that regulate several cellular processes, such as the elimination of misfolded proteins, cell development and post-translational modifications of kinase proteins and receptors. Due to its involvement in extracellular matrix (ECM) remodelling, myofibroblast differentiation and apoptosis, HSP 90 has been investigated as a key player in the pathogenesis of lung fibrosis. Idiopathic pulmonary fibrosis (IPF) is the most common and deadly interstitial lung disease, due to the progressive distortion of lung parenchyma

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… HSP 90β regulates embryonic development, the innate immune response, muscle cell differentiation and regeneration, hepatocyte formation, neuromuscular junction maintenance, and the control of glucose and cholesterol metabolisms [ 34 ]. … junction maintenance Control of glucose and cholesterol metabolisms Cell differentiation Cellular interaction Activity of actin and tubulin in cytoskeleton | M aiti et al . [ 34 ] B asset et al . [ 35 ] Extracellular HSP 90 | Ubiquitous | Tumourigenesis Tumour cell migration and invasion Tissue repair …

Intensive Care Unit-Acquired Weakness: Not just Another Muscle Atrophying Condition.

Intensive care unit-acquired weakness (ICUAW) occurs in critically ill patients stemming from the critical illness itself, and results in sustained disability long after the ICU stay. Weakness can be attributed to muscle wasting, impaired contractility, neuropathy, and major pathways associated with muscle protein degradation such as the ubiquitin proteasome system and dysregulated autophagy. Furthermore, it is characterized by the preferential loss of myosin, a distinct feature of the condition. While many risk factors for ICUAW have been identified, effective interventions to offset these ch

B cell lineage reconstitution underlies CAR-T cell therapeutic efficacy in patients with refractory myasthenia gravis.

B-cell maturation antigen (BCMA), expressed in plasmablasts and plasma cells, could serve as a promising therapeutic target for autoimmune diseases. We reported here chimeric antigen receptor (CAR) T cells targeting BCMA in two patients with highly relapsed and refractory myasthenia gravis (one with AChR-IgG, and one with MuSk-IgG). Both patients exhibited favorable safety profiles and persistent clinical improvements over 18 months. Reconstitution of B-cell lineages with sustained reduced pathogenic autoantibodies might underlie the therapeutic efficacy. To identify the possible mechanisms un

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… Autoreactive B cells with autoantibody formation play a key role in the pathogenesis of MG, and the autoantibodies in the majority of MG patients are directed against the muscle acetylcholine receptor (AChR) of the neuromuscular junction, while in 6% antibodies against the muscle-specific kinase (MuSK … JJ, Palace J, Murai H, Tannemaat MR, Kaminski HJ, Bril V (2022) Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders. …

Oral Presentations

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Устные доклады

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Is dosing of therapeutic immunoglobulins optimal? A review of a three-decade long debate in europe.

The consumption of immunoglobulins (Ig) is increasing due to better recognition of antibody deficiencies, an aging population, and new indications. This review aims to examine the various dosing regimens and research developments in the established and in some of the relevant off-label indications in Europe. The background to the current regulatory settings in Europe is provided as a backdrop for the latest developments in primary and secondary immunodeficiencies and in immunomodulatory indications. In these heterogeneous areas, clinical trials encompassing different routes of administration,

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… Auto-antibodies are directed to signaling proteins at the neuromuscular junction, in particular, the nicotinic acetylcholine receptor (AChR). … At least three mechanisms have been proposed to explain how anti-AChR antibodies compromise neuromuscular transmission: (i) complement binding and activation at the neuromuscular junction; (ii) accelerated degradation of AChR molecules (antigenic modulation); and (iii) functional block of AChR-binding …

Exploring the role of Transcranial magnetic stimulation in cognitive impairment and sarcopenia: a narrative review.

Sarcopenia and cognitive impairment (CI) are major contributors to disability among the elderly, diminishing their quality of life and placing also a significant burden on healthcare systems and societies. There are currently no effective treatments for sarcopenia and CI. Interestingly, recent research has revealed a strong connection between these two conditions. Although the exact mechanisms of this relationship remain unclear, factors such as chronic inflammation, mitochondrial oxidative stress, and impaired communication through the muscle-brain axis have been put forward. Transcranial mag

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… In addition, the accumulation of RNS/ROS can alter the morphology of the neuromuscular junction, leading to muscle denervation ( Baumann et al., 2016 ). … Evidence provided by Stölting et al. (2016) indicates that magnetic stimulation can promote nerve and muscle regeneration by reducing inflammation, facilitating the aggregation of acetylcholine receptors, and inducing the maturation of neuromuscular junctions. …