抗体阳性自身免疫性脑炎的临床特点及预后

目的 分析抗体阳性自身免疫性脑炎的临床特点,探讨影响其短期预后的因素。方法 搜集2018-01—2022-09于郑大五附院神经内科确诊的抗体阳性自身免疫性脑炎患者的临床资料,对其临床特征进行回顾性分析,总结各例AE的临床特点、影像学特征及实验室检验特点。同时,根据m RS评分将患者分为预后良好组（0～2分）及预后不佳组（>2分）,分析2组患者性别、年龄、腰穿压力、脑脊液白细胞计数及脑脊液白蛋白定量等因素对患者短期预后的影响。结果 抗体阳性自身免疫性脑炎占脑炎比例的23.1%(22/95),发病年龄13～86岁,平均52.5岁;抗NMDAR脑炎（8/22,36.3%）、抗LGI1抗体相关脑炎（6/22,27.2%）、抗GABABR抗体相关脑炎（3/22,13.6%）相对常见,临床表现特点包括认知功能下降、精神行为异常、癫痫、言语障碍、运动障碍及自主神经功能障碍等;3例抗LGI1相关抗体脑炎患者头颅磁共振出现海马异常信号,2例抗NMDAR脑炎患者头颅磁共振分别出现双侧小脑及双侧丘脑异常信号;性别、年龄、腰穿脑脊液压力、脑脊液白细胞计数及脑脊液白蛋白定量对患者短期预后的无显著影响。结论...     

重叠抗体自身免疫性脑炎的研究进展

近年来对于重叠抗体自身免疫性脑炎的报道越来越多,其重叠抗体形式多种多样.常见重叠形式为自身免疫性脑炎叠加神经胶质抗体、自身免疫性脑炎与其他全身性自身免疫性疾病并存、自身免疫性脑炎相关重叠抗体,但各类型的临床意义尚未明确.文中收集国内外对重叠抗体自身免疫性脑炎的研究现状,对该类脑炎可能的发病机制、重叠类型、临床表现特点及...     

抗谷氨酸脱羧酶抗体阳性自身免疫性脑炎三例临床特征分析

目的探讨抗谷氨酸脱羧酶(glutamic acid decarboxylase,GAD)抗体阳性自身免疫性脑炎的临床症状及相关辅助检查特点,为临床正确诊疗抗GAD相关脑炎提供依据。方法对作者医院自2016年1月至2018年8月期间明确诊断的3例抗GAD抗体阳性自身免疫性脑炎患者进行回顾性研究,分析其一般人口学、临床症状学、脑脊液细胞学、影像学、脑电图、治疗及预后等特征。结果3例患者均为女性,发病年龄分别为44岁、26岁、27岁,其中2例患者以难治性癫痫状态起病,1例以头痛起病,病程中均出现一定程度意识障碍及显著认知功能下降,其中1例患者病情恢复期出现类似吉兰-巴雷综合征样周围神经损害;头颅MRI均表现为双侧海马、颞叶等边缘叶系统核异常信号,且进行性加重。3例均检测到GAD65抗体,其中1例发病初期即检测到该抗体,2例患者发病初期神经元抗体检测为阴性,分别于发病43 d、79 d脑脊液检测到;3例患者发病初期脑电图表现中-重度广泛性、多灶样异常,中-高波幅3~5 Hz慢波阵发,杂有稍多量尖棘波发放。1例患者发病初期脑脊液白细胞计数增高(淋巴细胞97%),10 d后复查恢复正常,余2例患者脑脊液常规生化免疫及细胞学未见明显异常。三例患者经甲泼尼龙及大剂量免疫球蛋白治疗后认知功能较症状高峰期基本恢复,未再有癫痫发作。结论此3例抗GAD抗体阳性自身免疫性脑炎患者均为女性,中青年发病,初期表现难治性癫痫持续状态或者耐药性癫痫,恢复期出现认知功能减退,病程中均无显著精神症状;影像学以双侧额颞叶、海马受累较为明显;病程中脑脊液抗GAD 65抗体均阳性,且脑脊液中该抗体发病初期可为阴性。     

自身免疫性脑炎临床特点及治疗分析

目的 总结自身免疫性脑炎的临床特点及治疗转归。方法 收集2017-10—2022-03在聊城市人民医院住院治疗的18例自身免疫性脑炎患者的临床资料,回顾性分析其临床表现、实验室检查、影像学检查、脑电图和治疗转归。结果 18例患者中男12例,女6例,平均发病年龄41.8岁（15～68岁）。临床表现：16例患者有癫痫发作史,13例患者认知功能下降,15例患者精神行为异常。脑脊液检查：3例患者脑脊液压力超过300 mmH2O,8例患者白细胞计数升高,均低于100×106个/L,以淋巴细胞和单核细胞为主,部分可见激活单核细胞和转化淋巴细胞,8例脑脊液蛋白轻度升高,脑脊液相关抗体检测均为阳性。影像学检查：8例抗NMDAR脑炎患者发现异常,1例抗GABABR抗体相关脑炎及2例抗LGI1抗体相关脑炎患者可见颞叶及海马异常信号。脑电图检查：10例患者存在异常,重度异常者2例,中度异常者2例。治疗转归：所有患者均予以糖皮质激素联合免疫球蛋白的一线治疗,12例患者出院时改良Rankin评分0～2分。结论 自身免疫性脑炎特征性临床表现较少,仍以癫痫发作、认知障碍、精神异常等表现为主,对于临床上有此表现的患者...     

抗体阳性自身免疫性脑炎的临床特征与预后的研究进展

<正>自身免疫性脑炎(autoimmune encephalitis,AE)是由一种中枢神经系统(central nervous system,CNS)抗体相关免疫反应引起的脑炎综合征。它是一种罕见的、严重的、但能治疗的自身免疫性疾病,其主要依赖于神经元抗体检测的特异性和敏感性进行确定诊断。根据抗体的分布情况,AE可分为神经元表面抗原阳性(如NMDAR、GABABR、AMPAR、LGI1等)的AE抗体和胞内或核抗原阳性(如Hu、Ma2、GAD等)的AE抗体。     

15例边缘性脑炎临床分析

目的观察边缘性脑炎的临床特点,探讨不同类型边缘性脑炎的临床表现及诊断,避免诊断治疗延误。方法对15例诊断为边缘性脑炎患者的临床症状,体征、血及脑脊液的免疫学检查及脑电图、核磁共振结果等方面进行比较分析。结果15例患者都表现出主要累及海马及中央颞叶的临床症状,其中6例患者出现边缘系统外症状;10例患者出现癫痫发作;15例患者均表现出CSF的异常;6例存在双侧边缘系统受累的影像学表现,其中3例还存在边缘系统外神经系统病灶;7例患者发现肿瘤,其中5例为肺癌,2例为畸胎瘤;3例Hu抗体阳性,1例CV2／CRMP5抗体阳性,7例患者NMDA抗体阳性。结论LE的临床症状或MRI、EEG、CSF等表现复杂多样,对初期症状的辨别对于LE的诊断有很重要的帮助作用,同时能够一定程度上提示LE的类型,对于及时制定合理的治疗方案提供依据。     

蛋白A免疫吸附治疗自身免疫脑炎疗效的回顾性分析

目的 探索蛋白A免疫吸附治疗对自身免疫性脑炎(A E)的疗效及安全性.方法 回顾性收集作者医院收治的24例对一线治疗反应不佳的AE住院患者,其中抗N-甲基-D-天冬氨酸受体(NMDAR)抗体阳性20例,抗γ-氨基丁酸B型受体(GABAB R)阳性2例,抗富含亮氨酸胶质瘤失活蛋白1(LGI1)阳性1例,抗IgLON5阳性...     

8例自身免疫性脑炎的病例分析并文献复习

目的探讨自身免疫性脑炎的临床表现、磁共振(MRI)图像、脑脊液特点及治疗转归。方法回顾性分析我院8例(自2012年1月-2016年1月)自身免疫性脑炎患者的临床资料。结果 8例患者平均发病年龄为44岁,其中有4例抗NMDA受体脑炎,3例LGI1蛋白抗体阳性边缘系统脑炎,1例抗GABABR脑炎,所有患者(100%)均有认知功能损害和精神症状;5例(62%)出现癫痫发作;3例(37%)出现不自主运动和自主神经功能障碍。4例(50%)患者脑电图存在异常;4例(50%)患者有磁共振检查异常信号;1例发现肺癌。结论以认知功能损害、精神行为异常、癫痫发作为主要表现的患者,要警惕自身免疫性脑炎的可能,以免误漏诊。     

抗Ma2抗体相关脑炎临床和影像学分析

目的探讨抗Ma2抗体相关脑炎的临床表现、实验室检查和神经影像学特点,以期为临床诊治该病提供参考。方法回顾性收集作者医院2015年1月至2017年12月诊断的3例抗Ma2抗体相关脑炎患者的临床资料。结果3例抗Ma2抗体相关脑炎临床表现包括记忆下降、精神行为异常、癫痫发作等,1例患者存在睡眠障碍。3例患者血清抗Ma2抗体均呈阳性,脑脊液中该抗体阳性1例。3例患者均有头颅MRI多发异常信号,主要位于边缘系统和脑干上部。1例患者发现肺癌。结论抗Ma2抗体与边缘性脑炎或脑干脑炎关系密切。对于抗Ma2抗体相关脑炎患者,需要重点排查潜在恶性肿瘤。     

论自身免疫性脑炎责任抗体的致病性

自身免疫性脑炎是一种中枢神经系统自身免疫性疾病,大量新抗体的发现扩增其临床疾病谱,但也为临床表型的精准识别带来一定困难.责任抗体系指同一例患者病程中与一个或多个临床表型有对应因果关系的致病性抗体,这一概念的提出,在自身抗体与临床表型之间建立联系,体现出现代精准医学的理念.明确责任抗体的致病性是理解抗体-临床表型因果关系...     

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.

Clinical manifestations,treatment outcomes,and prognostic factors of pediatric anti-NMDAR encephalitis in tertiary care hospitals: A multicenter retrospective/prospective cohort study

ObjectiveAnti-NMDAR encephalitis is an acute autoimmune neurological disorder that is increasingly recognized in pediatric populations. Several studies of the disorder have been conducted worldwide but there are few publications in Thailand. Here, we describe the clinical manifestations, treatment outcomes, and prognostic factors in children with anti-NMDAR encephalitis.MethodsBetween January 2007 and September 2017, we conducted a retrospective/prospective cohort study of children diagnosed with anti-NMDAR encephalitis from three tertiary care hospitals in Thailand: King Chulalongkorn Memorial Hospital, Chonburi Hospital, and Prapokklao Hospital. We assessed the Modified Rankin Score (mRS) score for each participant to measure severity of disease and treatment outcome at baseline, 12, and 24 months.ResultsWe recruited 14 participants (1–13 years with median age 8.4 years). Participants were followed up for a median of 20.5 months. Clinical manifestations included behavioral dysfunction (100%), movement disorder (93%), speech disorder (79%), sleep disorder (79%), and seizures (79%). All patients received first-line immunotherapy (corticosteroids: 100%, intravenous immunoglobulin: 79%, plasma exchange: 21%). Second-line immunotherapy (cyclophosphamide) was administered to 57% of patients. During the first 12 months, 8 patients (62%) achieved a good outcome (mRS ≤ 2). At 24 months, 9 patients (81%) had achieved a good outcome. Altered consciousness and central hypoventilation were predictors of poor outcome. (p < 0.05).ConclusionsWe observed similar clinical manifestation of anti-NMDAR encephalitis in Thai children to those reported in other countries. Furthermore, the percentage of patients with good outcomes in our study was comparable with previous studies. Further studies are required to investigate other populations in other regions of Thailand.     

Acute encephalitis with intrathecal synthesis of measles antibodies

Summary In two cases of acute encephalitis, there was an intrathecal antibody synthesis against measles virus, as determined with an enzyme-linked immunosorbent assay. The clinical findings were not consistent with a usual measles infection, subacute sclerosing panencephalitis or acute multiple sclerosis.     

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.     

伴有精神症状的自身免疫性脑炎的临床特点分析

目的 分析不同抗体类型自身免疫性脑炎患者精神症状的特点.方法 回顾性研究了自身免疫性脑炎患者的临床特点.对自免脑患者的精神症状进行分析.对象:2019年9月到2020年12月就诊于中国医科大学附属第一医院神经内科确诊为自身免疫性脑炎的患者.结果 抗体阳性为20例,临床表现中最常见的依次是抽搐发作、精神症状、意识障碍、记...     

Validity and prognostic utility of clinical assessment scale for autoimmune encephalitis (CASE) score in children with autoimmune encephalitis

IntroductionThe clinical assessment scale for autoimmune encephalitis (CASE) is a recently developed and validated scale to rate the severity of autoimmune encephalitis (AE) in adults. But it is yet to be validated in pediatric AE cases.MethodsIn a prospective observational study, we determined the validity and prognostic utility of CASE in the pediatric population with a diagnosis of probable or definite AE. We also determined clinical, neuroimaging, or laboratory-based prognostic factors for favorable clinical outcomes at 3 months after presentation. We used weighted kappa statistics and the intra-class correlation coefficient of individual item scores and total scores for determining inter-observer and intra-observer reliability respectively.ResultsWe enrolled a total of 54 patients (28 girls, probable [45%] or definite [55%] AE). Functional status score (FSS), CASE score, and other scores showed significant improvement at the time of discharge and 3-months, as compared to baseline (p < 0.0001). The intra-observer and interobserver reliability of the total scores was excellent (k = 0.94 and 0.95 respectively). CASE was also found to have good internal consistency (Cronbach-α = 0.83). The corrected item-total correlations of all items were >0.40. The correlation between the total CASE score and FSS score at admission, at discharge, and at 3 months was strong (r = 0.90, 0.92, and 0.94, p < 0.001). In multivariate analysis, only seropositivity or definite AE and CASE score at baseline was found to be significant predictive factors for functional status at 3 months (p = 0.03, 0.01).ConclusionCASE score can be used for monitoring the severity of pediatric AE patients. It also has prognostic usefulness for predicting functional independence on follow-up.     

Characterisation of a syndrome of autoimmune adult onset focal epilepsy and encephalitis

We report a series of patients with a clinical syndrome characterised by the explosive onset in adulthood of recurrent focal seizures of frontotemporal onset and features suggestive of autoimmune encephalitis. We propose that this presentation of “autoimmune adult onset focal epilepsy and encephalitis” is a recognisable clinical syndrome, and provide evidence it may be associated with heterogeneous immunological targets. Between 2008 and 2011 we encountered six patients with new-onset epilepsy in whom we suspected an autoimmune aetiology. We first characterised the clinical, electroencephalographic, cerebrospinal fluid (CSF), imaging, and pathological findings of this syndrome. We subsequently tested them for antibodies against both intracellular and neuronal cell surface antigens. All patients presented with recurrent seizures with focal frontotemporal onset, refractory to multiple anticonvulsants. Four had focal T2-weighted hyperintensities on MRI. CSF mononuclear cells were variably elevated with positive oligoclonal bands in four. Brain biopsy in one patient demonstrated perivascular lymphocytic infiltration. Two were treated with immunosuppression and went on to achieve complete seizure control and return to baseline cognition. Three of four patients who received only pulsed steroids or no treatment had ongoing frequent seizures, with two dying of sudden unexpected death in epilepsy. Subsequently, three had antibodies identified against neuronal cell surface antigens including N-methyl-d-aspartate receptor and leucine-rich glioma inactivated 1. We suggest that patients with such a presentation should be carefully evaluated for a suspected autoimmune aetiology targeting cell surface antigens and have a therapeutic trial of immunosuppression as this may improve their long-term outcome.