成人重症自身免疫性脑炎的临床特点及治疗

Clinical Characteristics and Therapy Strategy of Severe Autoimmune Encephalitis in Adults

目的:探讨重症自身免疫性脑炎患者临床特点、辅助检查、免疫治疗及转归。方法:分析14例重症自身免疫性脑炎患者的临床特征、脑脊液检查、影像学表现、脑电图特征以及免疫治疗方案和转归。结果:14例患者平均发病年龄35岁,主要表现为认知功能减退(14例)、意识障碍(12例)、严重精神症状(8例)、自主神经功能障碍(6例)、癫痫持续状态(5例)、呼吸衰竭(5例),13例患者首次脑电图(EEG)异常,4例患者头颅核磁共振(MRI)检查示颞叶、顶叶或海马异常信号;全部患者均接受免疫治疗,12例患者症状改善,2例死亡。结论:成人重症自身免疫性脑炎表现主要以认知功能减退、意识障碍为主,大多首次可检出EEG异常;大部分患者免疫治疗效果良好。

Objective： To observe the clinical manifestations,auxiliary examinations,immunotherapy and prognosis of patients with severe autoimmune encephalitis. Methods： A total of 14 hospitalized cases with severe autoimmune encephalitis were retrospectively analyzed. Clinical manifestations,cerebrospinal fluid examinations,imaging,electroencephalogram,immunotherapy and prognosis were recorded. Results： Fourteen patients were 35 years old in average. The mainly clinical characteristics were cognitive decrease（ in 14 cases）,disturbance of consciousness（ in 12 cases）,severe mental disorder（ in 8 cases）,autonomic nerve dysfunction（ in 6 cases）,status epilepticus（ in 5 cases） and respiratory failure（ in 5 cases）. The first EEG was abnormal in 13 patients. The brain MRI indicated abnormal signals in temporal lobe,parietal lobe or hippocampus in 4 patients. All patients were treated with immune therapy. 12 patients well responded to immune therapy,and 2 patients died. Conclusions： Severe autoimmune encephalitis in adults may mainly present cognitive decrease,disturbance of consciousness,the first EEG of most patients was abnoormal. Most patients with severe autoimmune encephalitis treated with active immune therapy can get good prognosis.