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anti-iglon5 disease: a novel topic beyond neuroimmunology

Neural Regeneration Research 2023年第5期18卷 1017-1022页

作者： Yi-ZongHeng Zhang You Ni Yi-Ning Gao Ding-Ding Shen Lu He Dou Yin Huan-Yu Meng Qin-Ming Zhou Ji Hu Sheng Chen Department of Neurology&Institute of Neurology Ruijin HospitalAffiliated with Shanghai Jiao Tong University School of MedicineShanghaiChina Co-Innovation Center of Neuroregeneration Nantong UniversityNantongJiangsu ProvinceChina School of Life Science and Technology ShanghaiTech UniversityShanghaiChina Shanghai Key Laboratory of Psychotic Disorders Shanghai Mental Health CenterShanghaiChina

anti-iglon5 disease is a recently defined autoimmune disorder of the nervous system associated with autoantibodies against iglon5. Given its broad clinical spectrum and extremely complex pathogenesis, as well as difficulties in its early diagnosis and treatment, anti-iglon5 disease has become the subject of considerable research attention in the field of neuroimmunology. anti-iglon5 disease has characteristics of both autoimmunity and neurodegeneration due to the unique activity of the antiiglon5 antibody. Neuropathologic examination revealed the presence of a tauopathy preferentially affecting the hypothalamus and brainstem tegmentum, potentially broadening our understanding of tauopathies. In contrast to that seen with other autoimmune encephalitis-related antibodies, basic studies have demonstrated that iglon5 antibody-induced neuronal damage and degeneration are irreversible, indicative of a potential link between autoimmunity and neurodegeneration in antiiglon5 disease. Herein, we comprehensively review and discuss basic and clinical studies relating to anti-iglon5 disease to better understand this complicated disorder.

关键词： anti-iglon5 disease autoimmune encephalitis human leukocyte antigen IgG4-related diseases iglon5 antibody iglons immunotherapy inflammation neurodegeneration neuroimmunology tauopathy

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