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Efgartigimod treatment for therapy-refractory autoimmune encephalitis with coexistent NMDAR and LGI1 antibodies: a case report and literature review.

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Title: Efgartigimod treatment for therapy-refractory autoimmune encephalitis with coexistent NMDAR and LGI1 antibodies: a case report and literature review.
Authors: Xu, Jiaming (AUTHOR), Guo, Zhenli (AUTHOR), Zhao, Jie (AUTHOR), Chen, Yun (AUTHOR), Liu, Zhijun (AUTHOR), Wu, Yan (AUTHOR)
Source: Neurological Sciences. May2025, Vol. 46 Issue 5, p1995-2005. 11p.
Subjects: Autoimmune encephalitis, HLA histocompatibility antigens, Medical sciences, Methyl aspartate receptors, Cerebrospinal fluid, FC receptors
Abstract: The Fc receptor (FcRn) inhibitors can ameliorate autoimmune conditions such as myasthenia gravis through a rapid and specific clearance of serum IgG levels, and they also have potential for future use in a wider variety of antibody-mediated autoimmune diseases. Some patients with therapy-refractory autoimmune encephalitis (AE) continue to be unresponsive to initial and secondary treatment regimens. A 32-year-old male presented with predominant psychiatric symptoms and seizures, along with imaging evidence indicating multifocal cerebral cortical involvement. Neural antibody testing revealed dual positivity for N-methyl-D-aspartate receptor (NMDAR) and leucine-rich glioma-inactivated 1 (LGI1) antibodies in both serum and cerebrospinal fluid (CSF). Human leukocyte antigen (HLA) genotyping revealed the presence of the DQB1*03:01 and DQB1*06:01 alleles in the patient. Treatment with efgartigimod, the FcRn inhibitor, led to significant clinical improvements accompanied by a significant decrease in both anti-NMDAR and anti-LGI1 antibody levels. Herein, we report a rare case of therapy-refractory anti-NMDAR AE coexisting with positive LGI1 antibodies. Efgartigimod demonstrates promising potential for treating antibody-mediated AE. Clinical trial number Not applicable. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature 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: Efgartigimod treatment for therapy-refractory autoimmune encephalitis with coexistent NMDAR and LGI1 antibodies: a case report and literature review.
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  Data: <searchLink fieldCode="AR" term="%22Xu%2C+Jiaming%22">Xu, Jiaming</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Guo%2C+Zhenli%22">Guo, Zhenli</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Zhao%2C+Jie%22">Zhao, Jie</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Chen%2C+Yun%22">Chen, Yun</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Liu%2C+Zhijun%22">Liu, Zhijun</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Wu%2C+Yan%22">Wu, Yan</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. May2025, Vol. 46 Issue 5, p1995-2005. 11p.
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  Data: <searchLink fieldCode="DE" term="%22Autoimmune+encephalitis%22">Autoimmune encephalitis</searchLink><br /><searchLink fieldCode="DE" term="%22HLA+histocompatibility+antigens%22">HLA histocompatibility antigens</searchLink><br /><searchLink fieldCode="DE" term="%22Medical+sciences%22">Medical sciences</searchLink><br /><searchLink fieldCode="DE" term="%22Methyl+aspartate+receptors%22">Methyl aspartate receptors</searchLink><br /><searchLink fieldCode="DE" term="%22Cerebrospinal+fluid%22">Cerebrospinal fluid</searchLink><br /><searchLink fieldCode="DE" term="%22FC+receptors%22">FC receptors</searchLink>
– Name: Abstract
  Label: Abstract
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  Data: The Fc receptor (FcRn) inhibitors can ameliorate autoimmune conditions such as myasthenia gravis through a rapid and specific clearance of serum IgG levels, and they also have potential for future use in a wider variety of antibody-mediated autoimmune diseases. Some patients with therapy-refractory autoimmune encephalitis (AE) continue to be unresponsive to initial and secondary treatment regimens. A 32-year-old male presented with predominant psychiatric symptoms and seizures, along with imaging evidence indicating multifocal cerebral cortical involvement. Neural antibody testing revealed dual positivity for N-methyl-D-aspartate receptor (NMDAR) and leucine-rich glioma-inactivated 1 (LGI1) antibodies in both serum and cerebrospinal fluid (CSF). Human leukocyte antigen (HLA) genotyping revealed the presence of the DQB1*03:01 and DQB1*06:01 alleles in the patient. Treatment with efgartigimod, the FcRn inhibitor, led to significant clinical improvements accompanied by a significant decrease in both anti-NMDAR and anti-LGI1 antibody levels. Herein, we report a rare case of therapy-refractory anti-NMDAR AE coexisting with positive LGI1 antibodies. Efgartigimod demonstrates promising potential for treating antibody-mediated AE. Clinical trial number Not applicable. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature 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: 10.1007/s10072-024-07843-8
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        Text: English
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        StartPage: 1995
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              M: 05
              Text: May2025
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