Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Semin Neurol. 2011 Apr;31(2):144-57. doi: 10.1055/s-0031-1277985. Epub 2011 May 17.
Autoimmune encephalopathy represents a complex category of disease with diverse immunologic associations, clinical manifestations, and therapeutic outcomes. Three main subgroups include autoimmune encephalopathies without cancer but with neural nonspecific serologic evidence of autoimmunity (encompassing "Hashimoto's encephalopathy") that is the main focus of this review, paraneoplastic encephalopathies, and central nervous system (CNS) vasculitis (primary or secondary). Diagnosis of autoimmune encephalopathy can be suspected based on the clinical course, serologic evidence of autoimmunity, severe but nonspecific slowing on electroencephalography, and evidence of intrathecal inflammation in the cerebrospinal fluid. Rarely, there will be evidence of meningeal enhancement or increased fluid attenuated inversion-recovery signal in symptomatic regions on magnetic resonance imaging, but diagnosis may require brain biopsy demonstration of perivascular lymphocytic infiltrates. Nonspecific autoimmune encephalopathies are generally much more therapeutically responsive than paraneoplastic and vasculitic encephalopathies, so that high-dose corticosteroids may produce dramatic improvement within as little as a few days, although exceptional patients can require months of therapy. Paraneoplastic syndromes typically require tumor removal and often prove fatal. CNS vasculitides may respond to steroid therapy, but often require a second agent such as cyclophosphamide.
自身免疫性脑炎是一类具有多种免疫关联、临床表现和治疗效果的复杂疾病。主要分为三大类：无癌症但存在神经非特异性自身免疫血清学证据的自身免疫性脑炎（包括“桥本脑病”），是本次综述的重点；副肿瘤性脑病；以及中枢神经系统（CNS）血管炎（原发性或继发性）。自身免疫性脑炎的诊断可基于临床病程、自身免疫血清学证据、脑电图上严重但非特异性的减慢，以及脑脊液中鞘内炎症的证据来推测。在症状区域的磁共振成像上，脑膜增强或液体衰减反转恢复信号增加很少见，但诊断可能需要脑活检显示血管周围淋巴细胞浸润。非特异性自身免疫性脑炎通常比副肿瘤性和血管炎性脑炎更具治疗反应性，因此大剂量皮质类固醇可能在短短几天内产生显著改善，尽管极个别患者可能需要数月的治疗。副肿瘤综合征通常需要肿瘤切除，往往是致命的。中枢神经系统血管炎可能对类固醇治疗有反应，但通常需要第二种药物，如环磷酰胺。
