Mattar Adonis, Honnorat Jérôme, Joubert Bastien
Centre français de référence des syndromes neurologiques paranéoplasiques et des encéphalites auto-immunes, Hospices civils de Lyon ; Hôpital neurologique de Bron ; Hôpital neurologique de Lyon, France.
Hôpital neurologique de Bron ; Hôpital neurologique de Lyon, France Équipe Synaptopathies et auto-anticorps (SynatAc), institut NeuroMyoGène, Inserm U1217/CNRS UMR 5310, université Claude-Bernard, Lyon, France.
Rev Prat. 2022 Jan;72(1):85-90.
Autoimmune encephalitis: an update. Autoimmune encephalitis (AE) are rare autoimmune disorders of the central nervous system associated with anti-neuron antibodies. Patients classically present with anterograde amnesia, temporal lobe seizures, and/or behavioral changes, along with a variety of possible other symptoms, depending on the autoantibody. AE with antibodies targeting intracellular proteins are usually paraneoplastic and carry a poor prognosis. AE with antibodies against neuron cell-surface proteins associate with cancer less frequently and usually have better outcomes. Diagnosis relies on the detection of associated anti-neuron antibodies, while management focuses on the treatment of underlying neoplasia along with immune-suppressive therapies.
最新进展。自身免疫性脑炎（AE）是与抗神经元抗体相关的罕见中枢神经系统自身免疫性疾病。典型情况下，患者会出现顺行性遗忘、颞叶癫痫和/或行为改变，以及根据自身抗体不同而出现的各种其他可能症状。针对细胞内蛋白的抗体所致的AE通常为副肿瘤性，预后较差。针对神经元细胞表面蛋白的抗体所致的AE与癌症的关联较少，通常预后较好。诊断依赖于相关抗神经元抗体的检测，而治疗则侧重于潜在肿瘤的治疗以及免疫抑制疗法。
