抗神经元抗体相关自身免疫性脑炎免疫治疗策略OA

Autoimmune encephalitis(AE)refers to a group of CNS inflammatory diseases mediated by abnormal immune mechanisms.Most patients present with prodromal infectious symptoms such as fever and headache before onset,and core clinical manifestations include neuropsychiatric abnormalities,cognitive impairment,epileptic seizures,sleep disorders,and autonomic dysfunction.Its pathogenesis is associated with tumors,infections,genetics,and the use of immune checkpoint inhibitors,and its pathological changes are dominated by inflammatory damage to brain parenchyma.The detection of AE antibodies in serum and CSF serves as an important diagnostic criterion.Broadly,AE encompasses anti-neuronal cell antigen AE and anti-glial cell antigen AE.The former is classified into anti-neuronal intracellular antigen AE and anti-neuronal surface antigen AE based on target antigen localization.Anti-neuronal intracellular antigen antibodies mediate cellular immunity via T cells,leading to irreversible neuronal damage.This subtype is often complicated by tumors,with poor response to immunotherapy and unfavorable prognosis.In contrast,anti-neuronal surface antigen antibodies directly bind to target antigens,causing reversible neuronal dysfunction through humoral immunity and showing a good response to immunotherapy.Treatment of AE includes immunotherapy and anti-epileptic therapy,among which immunotherapy plays a pivotal role.Due to differences in pathological mechanisms and clinical phenotypes among different antibody subtypes,precise immunotherapy is required for AE patients:anti-neuronal surface antigen AE prioritizes therapeutic regimens targeting humoral immunity,while anti-neuronal intracellular antigen AE necessitates broad-spectrum and potent immunosuppressive regimens.This review focuses on anti-neuronal cell antigen AE,analyzing differences in pathological mechanisms mediated by various antibodies,and elaborating on the selection and efficacy of stepwise,individualized immunotherapy.Anti-glial cell antigen AE and antibody-negative AE are not included in the discussion.