摘要Anti-IgLON5 disease is a recently defined autoimmune disorder of the nervous system associated with autoantibodies against lgLON5.Given its broad clinical spectrum and extremely complex pathogenesis,as well as difficulties in its early diagnosis and treatment,anti-lgLON5 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-lgLON5 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 lgLON5 antibody-induced neuronal damage and degeneration are irreversible,indicative of a potential link between autoimmunity and neurodegeneration in anti-lgLON5 disease.Herein,we comprehensively review and discuss basic and clinical studies relating to anti-lgLON5 disease to better understand this complicated disorder.