【摘要】 目的 抗N-甲基-D-天冬氨酸受体脑炎(NMDAR)患者疾病分期4个阶段。文中探讨NMDAR患者不同疾病阶段的临床特征及一线免疫治疗的短期预后。方法 回顾性分析2017年3月—2021年6月期间于安徽省立医院确诊的22例抗NMDAR脑炎患者临床资料，并按临床表现分为早期阶段7例和进展阶段15例。观察患者不同阶段的临床指标(脑脊液白细胞数、蛋白浓度、氯化物浓度、糖浓度),脑电图，头颅磁共振，重症监护和一线免疫治疗6个月后随访的mRS评分，并观察一线免疫治疗6个月后的临床症状改善情况。结果 (1)临床特征：所有患者均存在精神行为异常、言语障碍，其次以癫痫发作(63.6%)、运动障碍(54.5%)、意识障碍(50.0%)、记忆障碍(45.5%)和自主神经功能障碍(36.4%)较为常见。早期阶段以前驱症状(57.1%)、脑脊液白细胞数升高(85.7%)为最主要特征，早期阶段前驱症状与白细胞数升高情况明显多于进展阶段(P<0.05);而进展阶段则以mRS>2分(60.0%)、脑电图中-重度异常(40.0%)、头颅磁共振异常(33.3%)、重症监护(33.3%)为主要特征，进展阶段mRS>2分患者明显多于早期阶段(P<0.05)。其中，脑电图中-重异常和磁共振异常者均处于进展阶段。(2)短期预后：22例抗NMDAR脑炎患者一线免疫治疗6个月后随访，癫痫发作、不自主运动和意识障碍症状均消失，仅存在精神行为异常、记忆障碍各8例，言语障碍6例，除记忆障碍外，其他临床症状较急性发作期明显改善(P<0.05)。结论 抗NMDAR脑炎不同疾病阶段具有不同的临床特征，早期阶段最主要症状包括前驱症状及脑脊液白细胞数升高，进展阶段脑电图中-重度异常、头颅磁共振异常较多，短期预后差；其疾病分期有助于早期诊断；早期阶段患者及时一线免疫治疗可抑制病情进展，改善短期预后。更多还原

【Abstract】 Objective To analyze the clinical characteristics of patients with anti-N-methyl-D-aspartate receptor(NMDAR) encephalitis at different disease stages and the short-term prognosis of patients at different disease stages and with first-line immunotherapy. Methods The clinical data of 22 patients with anti-NMDAR encephalitis were collected and divided into 7 at the early stage and 15 at the progressive stage by clinical manifestations. Patients were observed in different stages of clinical outcome: Leukocyte count, protein concentration, chloride concentration and sugar concentration in cerebrospinal fluid, EEG, cranial magnetic resonance, mRS score at 6-month follow-up after intensive care and first-line immunotherapy, and for clinical improvement after 6 months of first-line immunotherapy. Results(1) Clinical features: psychobehavioral abnormalities and speech disturbances were present in all patients, followed by seizures(63.6%), motor disturbances(54.5%), impaired consciousness(50.0%), memory disturbances(45.5%) and autonomic dysfunction(36.4%) being more common. The early stage was dominated by prodromal symptoms(57.1%) andthe number of leukocytes in cerebrospinal fluid increased(85.7%). The increase of prodromal symptoms and leukocyte count in the early stage was significantly more than that in the progressive stage(P<0.05); However, the progressive stage was mainly characterized by mRS score > 2(60.0%), Moderate to severe EEG abnormalities(40.0%), cranial magnetic resonance abnormality(33.3%), and intensive care admission(33.3%). Patients with advanced Mrs greater than 2 points were significantly more than early patients(P<0.05). Of these, those with moderate to severe EEG and abnormal cranial magnetic resonance, were in a progressive stage.(2) Short term outcome: at follow-up, 6 months after first-line immunotherapy in 22 patients with anti-NMDAR encephalitis, symptoms including seizures, involuntary movements and impaired consciousness all disappeared, and only psychobehavioural abnormalities, memory disturbances were present in 8 patients each, and speech disturbances in 6 patients, and after treatment, except for memory impairment, other clinical symptoms were significantly improved compared with those in acute attack stage(P<0.05). Conclusion Anti-NMDAR encephalitis has different clinical features in different disease stages. The main symptoms in the early stage include prodromal symptoms and the number of leukocytes in cerebrospinal fluid increased. In the advanced stage, there are more moderate to severe EEG abnormalities and cranial magnetic resonance abnormalities, and the short-term prognosis is poor; Its disease stage is helpful for early diagnosis; Timely first-line immunotherapy for patients at early stages can inhibit disease progression and improve short-term prognosis.更多还原