Neural Regeneration Research ›› 2023, Vol. 18 ›› Issue (5): 1017-1022.doi: 10.4103/1673-5374.355742

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Anti-IgLON5 disease: a novel topic beyond neuroimmunology

Yi-ZongHeng Zhang1, #, You Ni1, #, Yi-Ning Gao1, #, Ding-Ding Shen1, 2, Lu He1, Dou Yin1, Huan-Yu Meng1, Qin-Ming Zhou1, *, #br# Ji Hu2, 3, 4, *, Sheng Chen1, 2, *#br#   

  1. 1Department of Neurology & Institute of Neurology, Ruijin Hospital, Affiliated with Shanghai Jiao Tong University School of Medicine, Shanghai, China;  2Co-Innovation Center of Neuroregeneration, Nantong University, Nantong, Jiangsu Province, China;  3School of Life Science and Technology, ShanghaiTech University, Shanghai, China;  4Shanghai Key Laboratory of Psychotic Disorders, Shanghai Mental Health Center, Shanghai, China
  • Online:2023-05-15 Published:2022-11-01
  • Contact: Sheng Chen, MD, PhD, mztcs@163.com; Ji Hu, PhD, huji@shanghaitech.edu.cn; Qin-Ming Zhou, MD, PhD, zqmm2005@163.com.
  • Supported by:
    This work was supported by Shanghai Shuguang Plan Project, No. 18SG15, Shanghai Outstanding Young Scholars Project, Shanghai Talent Development Project, No. 2019044, Clinical Research Plan of SHDC, No. SHDC 2020CR2027B (all to SC).

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Abstract: 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 anti-IgLON5 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 anti-IgLON5 disease. Herein, we comprehensively review and discuss basic and clinical studies relating to anti-IgLON5 disease to better understand this complicated disorder.

Key words: anti-IgLON5 disease, autoimmune encephalitis, human leukocyte antigen, IgG4-related diseases, IgLON5 antibody, IgLONs, immunotherapy, inflammation, neurodegeneration, neuroimmunology, tauopathy