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Here, we report a case of myasthenia gravis and myopathy in a patient treated with nivolumab. A 76-year-old man who had been treated with four doses of nivolumab because of non-small cell lung cancer (NSCLC) presented with proximal-dominant muscle weakness and fluctuating ptosis and diplopia. Serologic studies revealed increased levels of muscle enzymes including creatine phosphokinase (2934 U/L), and acetylcholine receptor antibody was positive (1.31 nmol/L). Following electrodiagnostic study, he was diagnosed with myasthenia gravis and active stage of myopathy. After discontinuation of nivolumab, he was treated with corticosteroids, intravenous immunoglobulin G, and pyridostigmine. The neuromuscular symptoms and serologic abnormalities of the patient markedly improved. Currently, he is taking oral steroids and pyridostigmine without further immunotherapy.
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http://dx.doi.org/10.1111/1759-7714.13177 | DOI Listing |
J Neuroimmunol
August 2025
Department of Neurology, Tianjin Medical University General Hospital, Tianjin 300052, China; Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin 300052, China. Electronic address:
We present a clinically instructive case of a 50-year-old woman with acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (MG) who subsequently developed glutamic acid decarboxylase 65 (GAD65) antibody-associated neurological disorders alongside a type B2 thymoma. This rare coexistence highlights the profound immune dysregulation induced by thymomas, wherein loss of self-tolerance emergence multiple concurrent autoimmune phenomena. The patient's favorable response to multimodal immunotherapy-including efgartigimod, high-dose corticosteroids, and rituximab-underscores the therapeutic imperative for early, targeted immunomodulation in such complex neuroimmunological syndromes.
View Article and Find Full Text PDFNeurotherapeutics
September 2025
Department of Neurology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China. Electronic address:
Early intervention in impending myasthenic crisis (IMC) is critical to avert life-threatening progression. This study compared the clinical effectiveness and safety of the novel FcRn antagonist efgartigimod versus intravenous immunoglobulin (IVIg) in IMC management. In this retrospective cohort study, we analyzed 51 acetylcholine receptor antibody-positive (AChR-Ab+) IMC patients who received either efgartigimod (n = 30) or IVIg (n = 21) from June 2023 to November 2024.
View Article and Find Full Text PDFAnn Neurol
September 2025
Department of Neurology & Rehabilitation Medicine, The George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
Objective: Approximately 80% of patients with myasthenia gravis (MG) have autoantibodies against acetylcholine receptor, and clinical characteristics may vary between early-onset (symptom onset age <50 years) and late-onset MG (EOMG and LOMG), but the pathophysiological differences between EOMG and LOMG are not well established.
Methods: We performed an exploratory in-depth proteomics analysis examining 768 inflammatory proteins utilizing the baseline serum samples from the BeatMG study (NCT02110706). We performed pathway enrichment analysis to assess the pathways involved in LOMG.
J Biosci Bioeng
September 2025
Graduate School of Interdisciplinary Science and Engineering in Health Systems, Okayama University, Okayama 700-8530, Japan.
Blood purification using immunoadsorbent columns is a therapeutic strategy for removing pathogenic autoantibodies in autoimmune diseases. Currently available columns have limitations: Trp/Phe columns offer cost-effectiveness and sterilizability, but lack antigen specificity and have limited capacity to remove diverse pathogenic autoantibodies; whereas Protein A/peptide/anti-human IgG columns target all antibodies, regardless of pathogenicity, limiting specificity, and often require sterile production due to low stability under sterilization conditions, except for peptide ligands. Full-length autoantigen-immobilized immunoadsorbent columns have great potential to specifically adsorb targeted autoantibodies, because autoantibodies recognize diverse epitopes that vary among individuals.
View Article and Find Full Text PDFCureus
August 2025
Department of Anaesthesiology and Critical Care, Faculty of Medicine, University of Peradeniya, Kandy, LKA.
Congenital myasthenic syndromes (CMS) are a group of rare inherited neuromuscular disorders caused by defects in neuromuscular transmission. Unlike autoimmune myasthenia gravis, CMS typically presents in childhood with variable severity and symptoms, and does not involve autoantibodies. This report presents the management of a primigravida diagnosed with CMS who was generally stable on pyridostigmine therapy but experienced a severe exacerbation during pregnancy.
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