20例抗N-甲基-D-天冬氨酸受体脑炎患者临床特点分析

目的回顾性分析总结20例抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者的临床特点,增强对抗NMDAR脑炎的认识。方法对90例临床疑似脑炎患者的血清和脑脊液进行抗NMDAR-IgG检测,分析确诊为抗NMDAR脑炎的20例患者的临床表现、实验室检查、治疗及预后。结果抗NMDAR脑炎患者男女比例为6:14,中位年龄24岁,首发症状及主要精神症状多有不同。20例抗NMDAR脑炎患者中有12例患者血清和脑脊液中抗NMDAR-IgG抗体均阳性,其他8例仅在血清或脑脊液中检测到抗NMDAR-IgG抗体。5例盆腔检查异常,其中1例病理确诊为成熟囊性畸胎瘤。6例患者脑电图异常,7例头颅MRI异常。除1例患者未接受免疫治疗死亡外,其余患者接受免疫治疗后症状均有不同程度的缓解,其中3例未伴畸胎瘤的患者用二线免疫治疗后复检血清和脑脊液中抗NMDAR-IgG抗体水平下降。结论抗NMDAR脑炎患者中年轻女性发病率较高。二线免疫治疗可能对不伴有畸胎瘤患者的疗效更好。CSF中的抗NMDAR-IgG抗体的阳性率高于血清,同时检测血清和脑脊液中抗NMDAR抗体可以提高疾病的诊断效率。     

抗NMDAR脑炎相关炎性指标的研究现状及进展

抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是一种中枢神经系统自身免疫性疾病,多急性或亚急性起病,临床表现为癫痫发作、精神行为异常、认知障碍等症状,目前临床尚缺乏与该疾病临床特征及预后相关的指标。本文对抗NMDAR脑炎相关的炎性指标的研究现状及进展进行综述,以期了解抗NMDAR脑炎的临床严重程度及预后与脑炎相关炎性指标的关系,进一步明确抗NMDAR脑炎的发病机制。     

抗NMDAR脑炎8例临床特点及文献复习

目的 总结抗NMDAR脑炎的临床特点、辅助检查、诊断、治疗及预后。方法 分析2015年1月～2016年12月在武汉大学人民医院神经内科就诊的8例抗NMDAR脑炎患者的临床特点及诊治过程,随访患者观察患者的转归预后。结果 6例患者为青年女性,2例为男性,3例女性患者合并畸胎瘤并切除,3例患者行气管切开,8例患者均有精神症状和不自主运动,8例均预后良好。结论 抗NMDAR脑炎是重症脑炎中常见的类型,需要给予足够的时间来观察患者的疗效,积极控制并发症,早期诊断和治疗可改善患者的预后。     

抗N-甲基-D-天冬氨酸受体脑炎患者临床特点分析

目的 探讨抗N-甲基-D-天冬氨酸受体(N-methyl- D-aspartate receptor,NMDAR)脑炎的临床特征与抗NMDAR抗体在诊断该病中的意义.方法 选择62例各种病因的脑炎、脑病及其他中枢神经系统疾病患者,采用转染细胞间接免疫荧光法检测其血清及脑脊液抗NMDAR抗体,同时对该病的临床表现、实验室检查、治疗及预后进行分析.结果 28％(9/32)的临床诊断脑炎病例组患者血清或脑脊液抗NMDAR抗体为阳性.脑脊液抗体的阳性率高于血清,其中5例抗体滴度较高的患者伴有血脑屏障破坏.这些患者均未发现肿瘤,临床上以发热、精神异常、癫痫、肌张力障碍与自主神经功能障碍表现突出,并有头颅MRI与脑电图异常,早期免疫治疗有效.结论 脑脊液及血清中抗NMDAR抗体检测有助于自身免疫性抗NMDAR脑炎患者的早期诊断与治疗.     

抗NMDAR及抗LGI1相关脑炎急性期癫痫发作、临床特征及短期预后的对照研究

目的回顾性对照研究抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)及抗富亮氨酸胶质瘤失活1蛋白(Leucinie-rich gliomain activated 1,LGI1)相关脑炎患者急性期癫痫发作、临床特征及短期预后,为临床早期诊断和治疗提供参考。方法连续纳入2018年1月—2020年6月于四川省人民医院神经内科就诊的抗NMDAR及抗LGI1相关脑炎患者,回顾分析患者一般信息、临床表现、急性期癫痫发作情况及发作类型,评估两种自身免疫性脑炎在急性期癫痫发作的特点与短期预后的差异。结果共纳入75例抗NMDAR相关脑炎及抗LGI1相关脑炎患者,其中男41例,女34例,平均年龄(32.8±17.9)岁,平均病程(1.8±1.1)个月,其中抗NMDAR和抗LGI1抗体阳性分别59例和16例。75例中56例(74.7%)在急性期出现了癫痫发作,56例癫痫发作患者中伴有意识障碍38例(67.8%)、自主神经功能障碍5例(8.9%)、氧合能力下降24例(42.9%)、入住神经内科重症监护病房(NICU) 20例(35.7%),与无癫痫发作组比较有统计学差异(P0.05)。抗NMDAR脑炎在急性期癫痫发作中位数年龄为23岁,抗LGI1脑炎为56.5岁(P0.05)。抗NMDAR脑炎和抗LGI1脑炎在急性期癫痫发作均以全面性发作为常见(55.9%vs.53.8%),抗NMDAR脑炎在急性期更多出现反复癫痫发作及癫痫持续状态(P0.05)。在早期合理使用抗癫痫药物(AEDs)及抗免疫等对症支持治疗后,56例患者中70%出院时癫痫得到有效控制,3个月后随访,18例(32.1%)患者停用AEDs,而30例(53.5%)患者仍继续接受AEDs治疗,其中25例患者(44.6%)癫痫无发作。结论抗NMDAR相关脑炎及抗LGI1相关脑炎急性期癫痫发作风险均较高,伴有癫痫发作患者更容易出现意识障碍、氧合能力下降、入住NICU比例更高。抗NMDAR脑炎更常见于30岁左右年轻人群,抗LGI1脑炎更易于60岁左右发病。抗NMDAR脑炎患者更容易出现脑电图异常、平均住院天数更长,在急性期更容易出现癫痫反复发作与癫痫持续状态,及时诊断及干预治疗后,大部分患者癫痫发作能得到良好控制,急性期过后约1/3患者可停用AEDs。     

抗N-甲基-D-天冬氨酸受体脑炎的抗体检测及临床意义

目的探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的抗体检测意义及临床特征。方法选取本院收治的脑炎病人35例及对照组30例,分析临床资料,采用转染细胞间接免疫荧光法检测两组患者其血清及脑脊液抗NMDAR机体结果抗NMDAR抗体检测仅1例边缘叶脑炎患者结果阳性,该患者腑脊液细胞数增多、蛋白轻度升高、脑电图见双侧慢波、其余检查无异常。精神症状及意识水平障碍明显,免疫治疗有效。结论抗NMDAR脑炎发病率低,临床表现复杂多样,怀疑该病时需行抗NMDAR抗体检测。     

抗NMDAR脑炎并发隐球菌性脑膜炎一例

抗N-甲基-D-天门冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)脑炎是由NMDAR抗体介导的自身免疫性脑炎,常以发热、头痛为前驱症状,主要表现为精神行为异常、癫痫发作、意识水平下降或昏迷。隐球菌性脑膜炎是中枢神经系统常见的真菌感染,其在免疫功能低下时易发病,常以发热、头痛、呕吐为首发症状。抗NMDAR脑炎并发隐球菌性脑膜炎目前尚未见相关报道,本文对作者医院收治的1例抗NMDAR脑炎并发隐球菌性脑膜炎病例进行报道。1病例报告患者女,60岁,因“头痛6个月,头痛加重伴有精神行为异常1个月”于2018-08-08入作者医院。     

抗N-甲基-D-天冬氨酸受体脑炎14例临床分析

目的 分析14例抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者的临床资料,总结其临床特点。方法 纳入14例确诊为抗NMDAR脑炎的患者为研究对象,收集患者的人口统计学、临床表现及实验室检查等临床资料进行分析,使用改良Rankin量表(mRS)对其神经功能进行评价。结果 本研究14例患者中男性6例,女性8例,平均年龄(9.90±7.16)岁。85.71%患者表现为精神行为异常,64.29%有癫痫发作。重症患者与普通患者脑脊液抗NMDAR抗体滴度差异无统计学意义。所有患者均接受一线免疫治疗,有4例患者接受了二线免疫治疗,治疗有效率78.57%(P<0.05),随访复发率为21.43%。其中重症患者组治疗总有效率40.00%。结论抗NMDAR脑炎常见于青少年及儿童,以精神行为异常、癫痫发作、运动功能障碍等为主要临床表现,早期进行免疫治疗有利于患者的预后。对于重症抗NMDAR脑炎患者,适时采用二线免疫治疗及多学科综合治疗可能是提高治疗有效率的途径之一。     

抗NMDAR脑炎临床分析(附11例病例报告及文献复习)

目的总结分析抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特征以提高对该病的认识和重视。方法搜集国内206例抗NMDAR脑炎患者的报道,及在吉林大学白求恩第一医院就诊的11例抗NMDAR脑炎患者的详细病例资料,回顾性总结抗NMDAR脑炎的临床特点、实验室检查、治疗及预后,并结合文献予以分析讨论。结果患者多以抽搐或精神症状起病,病程中可出现意识障碍、运动障碍、中枢性通气不足、自主神经功能障碍等临床表现,头部MRI多无明显特异性改变,有的表现为皮质异常信号,脑电图常提示异常,血和(或)脑脊液NMDAR抗体阳性,多数预后较好。结论加强对该病的认识,尽早诊断及治疗有利于改善患者的预后。     

抗NMDAR脑炎的脑脊液Aβ和tau蛋白生物标志物与疾病严重程度及预后的关系

目的 探究脑脊液Aβ和tau在抗NMDAR脑炎患者中的临床价值。方法 以2019-07—2022-06郑州大学第一附属医院神经内科收治的34例抗NMDAR脑炎患者和13例非炎症性神经疾病患者为研究对象,收集临床资料和随访资料进行回顾性研究。结果 抗NMDAR脑炎组患者较对照组出现明显的发热、意识障碍、癫痫、认知障碍、精神行为异常（P<0.05）;脑脊液Aβ1-42、Aβ1-40明显低于对照组（P<0.001）。抗NMDAR脑炎患者脑脊液Aβ1-42与MoCA评分（r=0.433,P=0.031）相关。多因素回归分析表明抗NMDAR脑炎患者脑脊液Aβ1-42对疾病严重程度（OR=0.979,95%CI:0.962～0.997,P=0.023）、3个月疾病预后情况（OR=0.992,95%CI:0.986～0.999,P=0.020）有影响。结论 抗NMDAR脑炎患者脑脊液Aβ水平明显降低,且与认知水平相关,可能是抗NMDAR脑炎患者疾病严重程度和预后情况的独立危险因素。     

Suppression of synaptic plasticity by cerebrospinal fluid from anti-NMDA receptor encephalitis patients

The functional effects of cerebrospinal fluid (CSF) from patients with anti-NMDA receptor (NMDAR) encephalitis on the NMDAR-mediated synaptic plasticity were evaluated by using mouse hippocampus slices. Anti-NMDAR antibody detection system was established by immunostaining recombinant NMDAR heteromers expressed in HEK cell culture as well as native NMDARs in cultured hippocampal neurons. Under a complete blind manner for the clinical information, CSF and sera collected from 36 pre-diagnosed patients were tested for anti-NMDAR antibodies. With this test, thirteen patients were diagnosed as anti-NMDAR encephalitis. CSF positive for anti-NMDAR antibodies suppressed induction of long-term potentiation (LTP) at Schaffer collateral-CA1 synapses in mouse hippocampal slices. LTP induction was not suppressed by CSF collected from herpes simplex virus (HSV) encephalitis or non-encephalitis control patients. Antibody absorption with NMDAR-expressing HEK cell culture reversed the suppression of LTP by anti-NMDAR encephalitis patients' CSF, confirming that anti-NMDAR antibodies suppressed LTP. The present experiments firmly support the proposal that the anti-NMDAR encephalitis autoantibody is responsible for cognitive disorders like amnesia accompanying this disease.     

Japanese encephalitis-induced anti-N-methyl-d-aspartate receptor encephalitis: A hospital-based prospective study

ObjectivesA hospital-based prospective study was performed to determine: 1) whether Japanese encephalitis (JE) normally triggers anti-N-methyl-d-aspartate receptor (NMDAR) immunoglobulin G (IgG) synthesis, especially in monophasic JE patients; and 2) the incidence of JE-induced anti-NMDAR encephalitis in pediatric patients with JE.MethodsWe detected the level of anti-NMDAR IgG in the serum and cerebral spinal fluid (CSF) of JE patients within one week of onset. If patients relapsed during the convalescence phase, we detected JE virus RNA in the CSF and anti-NMDAR IgG in both the serum and CSF. For patients who did not relapse during the convalescence phase, serum was collected and anti-NMDAR IgG was detected during the 30–60-day course of the disease.ResultsWe enrolled 65 JE patients, who were negative for anti-NMDAR IgG in the serum and CSF during the acute phase, of which 63 patients were successfully followed up. Five patients relapsed during the convalescence phase, for whom JE virus RNA in the CSF was negative and excluded latent JE reactivation. The distinctive symptoms of four younger patients were choreoathetosis, whereas the psychiatric and behavioral manifestations were the distinctive symptoms experienced by the teenager. Anti-NMDAR IgG in the CSF of three patients was positive and they were diagnosed with anti-NMDAR encephalitis. The other two patients were negative for anti-NMDAR IgG in both the serum and CSF. For the 58 patients who did not relapse during the convalescence phase, anti-NMDAR IgG was negative in the serum of all patients at 30–60 days during the course of the disease.ConclusionsJE does not typically trigger anti-NMDAR IgG synthesis. Besides anti-NMDAR IgG, other unknown autoantibodies can also cause autoimmune encephalitis in the convalescence phase of JE. The incidence of JE-induced autoimmune encephalitis in pediatric patients with JE was 7.9%, and the incidence of JE-induced anti-NMDAR encephalitis was 4.7%.     

Relapsing Anti-NMDAR Encephalitis after a gap of eight years in a girl from North-East India

It has been just 7 years since the discovery of anti-NMDAR encephalitis as distinct immune-mediated encephalitis and we have such cases being reported from our country. Herein, we describe a case of a 13-year-old girl who had relapsing encephalitis consisting of multiple types of difficult-to-control seizures, abnormal behavior, language disintegration, memory loss and abnormal movements eight years after the first clinical attack. In 2005, when she was 5 yearsold, anti-NMDAR encephalitis was not yet discovered and she was provisionally diagnosed as a case of viral encephalitis. During her second attack in 2013, antibodies against NMDAR were demonstrated by immunofluoresence in serum (1:10). This is the first report from our country of a case of relapsing anti-NMDAR encephalitis of such a long duration, successfully treated by immunotherapy.     

Idiopathic autonomic neuropathy: comparison of cases seropositive and seronegative for ganglionic acetylcholine receptor antibody

BACKGROUND: The clinical characteristics of autoimmune autonomic neuropathy are only partially defined. More than 50% of patients with high levels of ganglionic acetylcholine receptor (AChR) autoantibodies have a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal tract symptoms, and neurogenic bladder. OBJECTIVE: To compare patients with idiopathic autonomic neuropathy who were seropositive (n = 19) and seronegative (n = 87) for ganglionic AChR antibodies. DESIGN: Retrospective review of autonomic programmatic database. SETTING: Autonomic Disorders Program Project at Mayo Clinic College of Medicine, Rochester, Minn. PATIENTS: We evaluated a cohort of 87 patients with idiopathic autonomic neuropathy who had undergone full autonomic testing and neurological evaluation and who had a complete panel of paraneoplastic and ganglionic AChR antibodies. We compared patients seropositive (n = 19) and seronegative (n = 87) for ganglionic AChR antibodies. RESULTS: The seropositive group had a significant overrepresentation of abnormal pupillary responses (12/18 [67%] vs 12/87 [14%]; P<.001), sicca complex (9/15 [60%] vs 11/47 [23%]; P =.01), and lower gastrointestinal tract dysautonomia (16/19 [84%] vs 48/85 [56%]; P =.02). A subacute mode of onset was more common in the seropositive group (12/19 [63%] vs 23/84 [27%]; P =.004). Results of quantitative autonomic function tests differed significantly in the 2 groups only in the cardiovagal domain. Because subacute onset was overrepresented in the seropositive group, we analyzed the data separately, controlling for temporal profile (ie, the relationship between antibody status and symptoms while controlling for rate of onset). The relationships between antibody status and clinical profile (eg, presence of sicca complex, pupillary abnormalities, and lower gastrointestinal tract symptoms) generally remained significant regardless of onset rate, indicating that the associations are not due to temporal profile. CONCLUSIONS: These observations support the concept that ganglionic AChR antibodies are diagnostically and pathophysiologically important. Patients with orthostatic hypotension and prominent cholinergic dysautonomia are most likely to be seropositive for ganglionic AChR antibody.     

Altered perception might be a symptom of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis

ABSTRACT

Most patients with N-methyl-D-aspartate receptor (NMDAR) encephalitis initially present with psychiatric symptoms. Although a delayed diagnosis may lead to a poor outcome, psychiatric symptoms that could differentiate anti-NMDAR encephalitis from other psychoses have not been fully investigated. We evaluated two patients with anti-NMDAR encephalitis who were observed by psychiatrists from onset throughout the course of disease. Both patients exhibited disorientation, memory deficits, perceptual disturbances, hallucinations, and mood liability. Among those, altered perceptions were most prominent - in particular, altered time perceptions without disorganization syndrome. The information obtained for these patients may help clinicians differentiate anti-NMDAR encephalitis from other psychoses, e.g., schizophrenia.     

The high frequency and clinical feature of seronegative myasthenia gravis in Southern China

Anti-acetylcholine receptor antibodies (anti-AChR-Ab) are responsible for the failure of neuromuscular junction in myasthenia gravis (MG). Some anti-AChR-Ab-seronegative MG patients have anti-muscle-specific tyrosine kinase antibodies (anti-MuSk-Ab). Here, the anti-AChR-Ab was tested in 250 MG outpatients from Southern China. While anti-MuSk-Ab was tested in 66 patients who had no anti-AChR-Ab in blood serum, but none of them was positive. The antibodies were measured by a radioimmunoprecipitation assay. The frequency of anti-AChR-Ab was 51.2 %. The percentage of anti-AChR-Ab in ocular type was lower than generalized type (44.9 vs. 66.2 %, P = 0.002). Seronegative MG was characterized by a lower percentage of thymoma than seropositive patients (P = 0.013). It seemed to be less severe in seronegative MG than seropositive MG in these 250 patients. In ocular type, seronegative MG mainly manifesting blepharoptosis but seldom diplopia or eyeball fixation related to ocular movement disability (P = 0.016). While in generalized type, seronegative MG was characterized by a lower percentage of bulbar muscle involvements than seropositive patients (P = 0.005). Logistic regression analysis revealed that bulbar weakness was affected by the existence of anti-AChR antibodies (OR = 3.524, P = 0.015). Besides, seronegative MG tended to be characterized by a lower percentage of neck extensor involvement, but this did not reach significance. The percentage of anti-AChR antibodies was much lower than other countries. Seronegative MG has characteristic clinical features that are different from features of the remaining seropositive MG. This emphasises the predictive value of anti-AChR antibodies analysis in MG patients.     

Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong

Aim

The study aims to analyze the incidence, clinical features, investigation findings and treatment outcomes of anti-N-methyl-d-aspartate receptor encephalitis in children from Hong Kong.

Absent anti-N-methyl-D-aspartate receptor NR1a antibodies in herpes simplex virus encephalitis and varicella zoster virus infections

Purpose: A 2012 report and subsequent case series described anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in patients during the acute phase and relapse of herpes simplex virus 1 (HSV1) encephalitis (HSV1E). However, the prevalence of this phenomenon is unknown and systematic studies on other viral infections of the nervous system are missing. Materials and methods: We retrospectively analyzed serial cerebrospinal fluid (CSF) and serum samples of consecutive patients treated for neurological HSV1, HSV2 and varicella zoster virus (VZV) infections in our tertiary care university hospital between 2003 and 2013 for the presence of antibodies directed against the NR1a subunit of the NMDAR using indirect immunofluorescence. Results: In total, 88 patients with the following infections were identified through an electronic database search: HSV1 (24 with encephalitis), HSV2 (6 with meningitis, 3 with encephalitis and 1 with myelitis), or VZV (3 with meningitis, 33 with encephalitis, 17 with radiculitis and 1 with myelitis). Two patients with HSV1E and HSV2E, respectively, experienced a clinical relapse. Clinical follow-up was for up to 85 months, and repetitive serum and CSF analyses for up to 43 months. However, at no time did any of the 88 patients exhibit anti-NMDAR NR1a antibodies. Conclusions: In this study, we did not detect anti-NMDAR NR1a antibodies in serial CSF and serum samples of HSV1E patients or patients with other viral infections (HSV2 and VZV). However, the presence of antibodies directed against other epitopes of the NMDAR and other neuronal cell surface antigens cannot be excluded, necessitating further studies.     

乌司他丁对大鼠脑缺血再灌注后海马NMDAR1表达的影响

目的探索乌司他丁对脑缺血再灌注大鼠海马N-甲基-D-天门冬氨酸受体(NMDAR)表达的影响。方法将72只雄性SD大鼠随机分为假手术组(S组)、模型组(I/R组)和乌司他丁干预组(U组)。采用线栓法阻断大脑右侧中动脉建立脑缺血损伤模型。U组于再灌注即刻腹腔注射乌司他丁20000 U/kg,再灌注3 h后进行神经功能评分(NDS),采用TUNEL法检测脑组织凋亡细胞数,RT-PCR检测NMDAR1 mRNA的表达水平,采用免疫组化染色计算平均光密度值(MOD)观察NMDAR1的表达。结果与S组(0)比较,I/R组(2.58±0.35)和U组(1.34±0.41)的NDS升高(P<0.05);与S组(0)比较,I/R组(52.10±3.25)和U组(26.40±2.70)的凋亡细胞数增加(P<0.05);与S组(0.8902±0.0136)比较,I/R组(1.0398±0.0211)和U组(0.9073±0.0142)NMDAR1 mRNA的表达上调(P<0.05);与S组(0.0066±0.0007)比较,I/R组(0.0594±0.0111)和U组(0.0068±0.0004)NMDAR1表达的MOD值升高(P<0.05)。与I/R组(2.58±0.35)比较,U组(1.34±0.41)的NDS降低(P<0.05);与I/R组(52.10±3.25)比较,U组(26.40±2.70)凋亡细胞数减少(P<0.05);与I/R组(1.0398±0.0211)比较,U组(0.9073±0.0142)NMDAR1 mRNA的表达下降(P<0.05);与I/R组(0.0594±0.0111)比较,U组(0.0068±0.0004)NMDAR1表达的MOD值降低(P<0.05)。结论乌司他丁减轻脑缺血再灌注损伤的机制可能与抑制NMDAR1表达有关。     

Clinical Characteristics and Prognosis of Severe Anti-<Emphasis Type="Italic">N</Emphasis>-methyl-<Emphasis Type="SmallCaps">d</Emphasis>-aspartate Receptor Encephalitis Patients

Background and purpose

Data concerning the characteristics and duration of the critical manifestations, treatment response, and long-term outcomes of severe anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis patients compared to those of non-severe patients are limited. This observational study was performed to explore the clinical characteristics and long-term outcomes of severe anti-NMDAR encephalitis patients.

Methods

According to their characteristics on admission to the neurology intensive care unit, patients with anti-NMDAR encephalitis were divided into a severe group and a non-severe group. The demographics, clinical manifestations, main accessory examinations, immunotherapy, and outcomes of patients were recorded. Statistical analyses were employed to examine the differences in each observed indicator between the severe and non-severe groups.

Results

This study enrolled 111 patients with anti-NMDAR encephalitis, including 59 males and 52 females with a mean age of 27.7?±?13.7 years; 39 (35.1%) patients were in the severe group, and 72 (64.9%) patients were in the non-severe group. Compared to the non-severe group, the severe group exhibited a higher proportion of epilepsy, involuntary movement, disturbance of consciousness, autonomic dysfunction, and central hypoventilation. The cerebrospinal fluid (CSF) of all patients was positive for the NMDAR antibody, but only 57 patients (51.4%) tested positive for the NMDAR antibody in the blood. The proportion of patients with a strong positive NMDAR antibody titer in the severe group (48.7%) was higher than that in the non-severe group (29.2%). The proportion of patients receiving intravenous gamma immunoglobulin in the severe group was higher than that in the non-severe group (P?=?0.003), and only patients in the severe group received plasma exchange, intravenous rituximab, and cyclophosphamide treatment. No significant difference was observed in the prognosis between the severe group and the non-severe group after 6 months and during long-term follow-up.

Conclusion

Most severe anti-NMDAR encephalitis patients will eventually achieve good long-term prognoses after receiving early, positive and unremitting combined immunotherapy and life support.