阴性精神症状自身免疫性脑炎患者的临床分析

Clinical features of autoimmune encephalitis patients with negative psychiatric symptoms

摘要:

目的 研究阴性精神症状自身免疫性脑炎(AE)患者的临床特征，提高对疾病的认识，以优化其治疗。方法 收集2018年1月至2021年12月武汉大学人民医院神经内科收治的40例确诊为AE的患者，其中4例无精神行为异常。精神行为异常表现为胡言乱语、幻觉、容易激惹、攻击行为和性格改变等。将有精神行为异常的36例患者分为阴性精神症状组（N组，9例）和非阴性精神症状组（P组，27例）。比较2组患者的临床表现、实验室检查、影像学特征、治疗及预后等指标。结果 40例患者中抗NMDAR脑炎最多见，为32例(80%)；抗LGI1脑炎4例(10%)；抗GABABR脑炎2例(5%)；抗CASPR2脑炎1例(2.5%)；抗NMDAR脑炎合并抗MOG脑炎抗体阳性1例(2.5%)。40例AE患者的常见临床表现有精神行为异常36例(90%)、癫痫发作20例(50%)、运动功能障碍4例(10%)、认知障碍7例(17.5%)、言语障碍6例(15%)、意识障碍8例(20%)等。40例AE患者均接受了一线免疫治疗，单用激素冲击治疗23例(57.5%)，激素冲击联合静脉注射免疫球蛋白(IVIg)治疗15例(37.5%)，激素+IVIg+血浆置换联合治疗2例(5%)。N组较少出现前驱头痛(P<0.05)。N组与P组在白细胞及中性粒细胞计数方面差异存在统计学意义(P<0.05)。N组9例患者预后均为良好；P组22例患者(81.5%)预后良好，5例预后不佳。结论 自身免疫性脑炎可表现出复杂多样的精神症状，应在诊断的早期进行阴性精神症状筛查，并尽早制定个体化治疗方案，以期改善预后。 [国际神经病学神经外科学杂志, 2022, 49(6): 1-6]

Abstract:

Objective To investigate the clinical features of autoimmune encephalitis （AE） patients with negative psychiatric symptoms， to improve the awareness of this disease， and to optimize its treatment.Methods A total of 40 patients who were admitted to Department of Neurology， Renmin Hospital of Wuhan University， from January 2018 to December 2021 and were diagnosed with AE were enrolled， among whom 4 patients had no mental and behavioral disorders. The manifestations of mental and behavioral disorders included babbling， hallucination， irritability， aggressive behavior， and personality change. The 36 patients with mental and behavioral disorders were further divided into negative psychiatric symptom group （N group with 9 patients） and non-negative psychiatric symptom group （P group with 27 patients）. The two groups were compared in terms of the indicators such as clinical manifestation， laboratory examination， radiological features， treatment， and prognosis.Results Among the 40 patients， there were 32（80%） patients with anti-NMDAR encephalitis， 4（10%） with anti-LGI1 encephalitis， 2（5%） with anti-GABABR encephalitis， 1（2.5%） with anti-CASPR2 encephalitis， and 1（2.5%） with anti-NMDAR encephalitis and positive anti-MOG antibody. As for the 40 patients with AE， common clinical manifestations included mental and behavioral disorders in 36 patients （90%）， seizure in 20 patients （50%）， dyskinesia in 4 patients （10%）， cognitive impairment in 7 patients （17.5%）， speech and language impairment in 6 patients （15%）， and disturbance of consciousness in 8 patients （20%）. All 40 patients received first-line immunotherapy， among whom 23（57.5%） received hormone shock therapy alone， 15（37.5%） received hormone shock therapy combined with intravenous immunoglobulin （IVIG）， and 2（5%） received hormone shock therapy+IVIG+plasma exchange. The N group had a significantly lower proportion of patients with prodromal headache （P<0.05）. There were significant differences in white blood cell count and neutrophil count between the N group and the P group （P<0.05）. In the N group， all 9 patients had good prognosis； in the P group， 22（81.5%） had good prognosis and 5 had poor prognosis.Conclusions AE can have various complex psychiatric symptoms. Negative psychiatric symptom screening should be performed in the early stage of diagnosis and an individualized treatment regimen should be developed as early as possible to improve prognosis. ［Journal of International Neurology and Neurosurgery, 2022, 49(6): 1-6］