从抗IgLON5病看神经免疫与神经变性间的联系

Anti-IgLON5 disease:relationship between neuroimmunology and neurodegeneration

抗IgLON5病(anti-IgLON5 disease)是一种罕见的自身免疫性脑炎,以血清和(或)脑脊液中存在抗IgLON5抗体为主要特征,于2014年被首次报道。与传统的自身免疫性脑炎不同,抗IgLON5病兼具神经免疫和神经变性2种疾病特征,部分患者的尸检结果提示其下丘脑和脑干被盖等区域存在磷酸化tau蛋白沉积。抗IgLON5病的病理发现揭示了神经免疫与神经变性之间的潜在关联,前者可能为后者的启动因素,即免疫炎症诱发磷酸化tau蛋白的沉积,神经免疫可能参与并促进了神经变性的发生、发展。

Anti-IgLON5 disease is a rare autoimmune encephalitis(AE)with anti-IgLON5 antibodies in serum and/or CSF,which was first reported in 2014.Different from the traditional AE,anti-IgLON5 disease presents dual characteristics of neuroimmunity and neurodegeneration.Autopsy of some patients showed phosphorylated tau deposition,mainly in hypothalamus and brainstem tegmentum.The discovery of anti-IgLON5 disease reveals a potential crosslink between neuroimmunology and neurodegeneration,and may become a breakthrough in studying the interplay between the two mechanisms,which means that the immune inflammation induces the deposition of phosphorylated tau protein.Neuroimmunity may participate in and promote the occurrence and development of neurodegeneration.Neuroimmunity may be the trigger of neurodegeneration.