Immunotherapy-responsive Non-paraneoplastic Encephalitis with Antibodies against GAD, LGI1, and GABA<sub>A</sub> Receptor

  • Nakano Takahiro
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Chihara Norio
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Matoba Kento
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Tachibana Hisatsugu
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Okuda Shiho
    Department of Neurology, Hyogo Rehabilitation Center Hospital, Japan
  • Otsuka Yoshihisa
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Ueda Takehiro
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Sekiguchi Kenji
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Kowa Hisatomo
    Department of Rehabilitation Science, Kobe University Graduate School of Health Sciences, Japan
  • Leypoldt Frank
    Institute of Clinical Chemistry, University Hospital Schleswig-Holstein, Germany
  • Wandinger Klaus-Peter
    Institute of Clinical Chemistry, University Hospital Schleswig-Holstein, Germany
  • Matsumoto Riki
    Division of Neurology, Kobe University Graduate School of Medicine, Japan

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Abstract

<p>A 62-year-old man showed abnormal behavior. Brain magnetic resonance imaging revealed multifocal lesions on T2-weighted images. Initial screening revealed that he was seropositive for antibodies against glutamate decarboxylase, which usually indicates treatment resistance to autoimmune encephalitis (AE). Intensive immunosuppressive therapies, however, improved the neurological symptoms. In line with this, we also detected seropositivity for antibodies against leucine-rich glioma-inactivated 1 and gamma-aminobutyric acid A receptor (GABAAR). Brain imaging and treatment responsiveness suggested that antibodies against GABAAR were the main cause of symptoms. Furthermore, the patient showed the presence of triple anti-neural antibodies in the absence of malignancy and had a favorable clinical course. </p>

Journal

  • Internal Medicine

    Internal Medicine 61 (3), 419-423, 2022-02-01

    The Japanese Society of Internal Medicine

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