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Unmasking Anti‐CASPR2 Syndrome in a Patient Treated for Myasthenia Gravis in the Era of New Treatments.

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Title: Unmasking Anti‐CASPR2 Syndrome in a Patient Treated for Myasthenia Gravis in the Era of New Treatments.
Authors: Fabry, Vincent (AUTHOR), Rual, Celso (AUTHOR), Bost, Chloé (AUTHOR), Acket, Blandine (AUTHOR), Maquet, Julien (AUTHOR), Cintas, Pascal (AUTHOR)
Source: European Journal of Neurology. Jul2025, Vol. 32 Issue 7, p1-3. 3p.
Subjects: Myasthenia gravis, Thymoma, Monoclonal antibodies, Immunomodulators, Isaacs syndrome, Immunoregulation, Autoimmune diseases
Abstract: Introduction: Myasthenia gravis (MG) is an autoimmune disorder characterised by autoantibodies against the acetylcholine receptor (AChR‐Ab). Morvan syndrome (MoS) is a rarer autoimmune disease with neuromyotonia, dysautonomia and encephalopathy, associated with antibodies targeting contactin‐associated protein‐like 2 (CASPR2) and may coexist with MG, particularly in patients with thymoma. Case Report: A 57‐year‐old man with AChR‐Ab MG was treated with pyridostigmine and prednisone for one year and then presented with a severe exacerbation. The symptoms were not controlled despite intravenous immunoglobulins and plasmapheresis. Chest CT revealed a thymoma. Zilucoplan (a C5 complement inhibitor) was started, with rapid improvement. Efgartigimod (a neonatal Fc receptor (FcRn) antagonist) was added to stabilise residual symptoms prior to thymectomy. Three weeks after the third and final efgartigimod cycle, the patient had no symptoms of MG but began to develop profuse sweating, then generalised hypertonia, fasciculations, myoclonus and dysautonomia, consistent with MoS, which were confirmed by the presence of anti‐CASPR2 antibodies. Symptoms improved markedly after resumption of efgartigimod. Conclusion: This case provides the first evidence of the efficacy of efgartigimod in the treatment of MoS and suggests that FcRn inhibition may be beneficial in IgG4‐mediated disorders beyond MG. It also highlights the importance of considering coexisting autoimmune conditions in thymoma, particularly when new symptoms occur under selective immune modulation. Finally, it emphasises the need to understand immunopathological mechanisms when choosing immunomodulatory treatment. [ABSTRACT FROM AUTHOR]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Data: Unmasking Anti‐CASPR2 Syndrome in a Patient Treated for Myasthenia Gravis in the Era of New Treatments.
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  Data: <searchLink fieldCode="AR" term="%22Fabry%2C+Vincent%22">Fabry, Vincent</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Rual%2C+Celso%22">Rual, Celso</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Bost%2C+Chloé%22">Bost, Chloé</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Acket%2C+Blandine%22">Acket, Blandine</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Maquet%2C+Julien%22">Maquet, Julien</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Cintas%2C+Pascal%22">Cintas, Pascal</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22European+Journal+of+Neurology%22">European Journal of Neurology</searchLink>. Jul2025, Vol. 32 Issue 7, p1-3. 3p.
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  Data: <searchLink fieldCode="DE" term="%22Myasthenia+gravis%22">Myasthenia gravis</searchLink><br /><searchLink fieldCode="DE" term="%22Thymoma%22">Thymoma</searchLink><br /><searchLink fieldCode="DE" term="%22Monoclonal+antibodies%22">Monoclonal antibodies</searchLink><br /><searchLink fieldCode="DE" term="%22Immunomodulators%22">Immunomodulators</searchLink><br /><searchLink fieldCode="DE" term="%22Isaacs+syndrome%22">Isaacs syndrome</searchLink><br /><searchLink fieldCode="DE" term="%22Immunoregulation%22">Immunoregulation</searchLink><br /><searchLink fieldCode="DE" term="%22Autoimmune+diseases%22">Autoimmune diseases</searchLink>
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  Data: Introduction: Myasthenia gravis (MG) is an autoimmune disorder characterised by autoantibodies against the acetylcholine receptor (AChR‐Ab). Morvan syndrome (MoS) is a rarer autoimmune disease with neuromyotonia, dysautonomia and encephalopathy, associated with antibodies targeting contactin‐associated protein‐like 2 (CASPR2) and may coexist with MG, particularly in patients with thymoma. Case Report: A 57‐year‐old man with AChR‐Ab MG was treated with pyridostigmine and prednisone for one year and then presented with a severe exacerbation. The symptoms were not controlled despite intravenous immunoglobulins and plasmapheresis. Chest CT revealed a thymoma. Zilucoplan (a C5 complement inhibitor) was started, with rapid improvement. Efgartigimod (a neonatal Fc receptor (FcRn) antagonist) was added to stabilise residual symptoms prior to thymectomy. Three weeks after the third and final efgartigimod cycle, the patient had no symptoms of MG but began to develop profuse sweating, then generalised hypertonia, fasciculations, myoclonus and dysautonomia, consistent with MoS, which were confirmed by the presence of anti‐CASPR2 antibodies. Symptoms improved markedly after resumption of efgartigimod. Conclusion: This case provides the first evidence of the efficacy of efgartigimod in the treatment of MoS and suggests that FcRn inhibition may be beneficial in IgG4‐mediated disorders beyond MG. It also highlights the importance of considering coexisting autoimmune conditions in thymoma, particularly when new symptoms occur under selective immune modulation. Finally, it emphasises the need to understand immunopathological mechanisms when choosing immunomodulatory treatment. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
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  Data: <i>Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.1111/ene.70285
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      – SubjectFull: Myasthenia gravis
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      – SubjectFull: Thymoma
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      – SubjectFull: Monoclonal antibodies
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      – SubjectFull: Autoimmune diseases
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      – TitleFull: Unmasking Anti‐CASPR2 Syndrome in a Patient Treated for Myasthenia Gravis in the Era of New Treatments.
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              Text: Jul2025
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