1例成人抗N-甲基-D-天冬氨酸受体抗体合并抗髓鞘少突胶质细胞糖蛋白抗体双阳性脑炎病例报告

1例44岁男性因发作性意识不清伴左上肢麻木、抽搐入院。脑电图提示左侧各导棘波频发,影像学检查提示右侧顶颞叶局部脑回肿胀、强化,脑脊液、血清样本提示抗N-甲基-D-天冬氨酸受体(NMDAR)抗体和抗髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性,诊断为抗NMDAR抗体合并抗MOG抗体双阳性脑炎。予以免疫球蛋白、糖皮质激素联合奥卡西平治疗,随访5个月未见明显症状发作。临床报道该类双抗体阳性脑炎患者多为儿童,成年较罕见,及时诊断治疗患者可以取得良好的临床预后。     

抗N- 甲基-D- 天冬氨酸受体脑炎的筛查及临床分析

目的 分析不明原因脑炎患者的脑脊液及血清学相关参数,探讨抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)脑炎在不明原因脑炎中的患病比例和临床特点。方法 收集2017年1月～2018年12月就诊入院的128例不明原因脑炎的病例资料,对患者脑脊液标本进行抗NMDAR抗体、常规及生化检测,对血清标本进行抗NMDAR抗体和肿瘤标志物检测,对筛选结果及确诊为抗NMDAR脑炎患者的临床资料进行分析。结果 128例不明原因脑炎患者,脑脊液抗NMDAR抗体阳性22例(17.2%),脑脊液常规及生化分析阳性42例(32.8%),血清抗体阳性20例(15.6%),肿瘤标志物检测阳性7例(5.5%)。确诊抗NMDAR脑炎患者22例,其中血清抗NMDAR抗体检测阳性率90.9%,脑脊液抗体阳性率100%,脑脊液常规及生化分析阳性率77.3%,肿瘤标志物检测阳性率为9.1%。确诊患者临床表现多为精神行为异常和癫痫样发作; 脑电图异常多为双额、颞、中央导联为慢波。颅脑磁共振成像(MRI)多为额颞叶T2加权像及液体衰减反转恢复序列异常信号,部分显示双侧或单侧颞角扩大。结论 脑脊液抗NMDAR抗体检查是确诊抗NMDAR脑炎的金标准,不明病因脑炎患者结合临床症状进行相关检测,对抗NMDAR脑炎的早期确诊有重要意义。     

儿童自身免疫性脑炎的早期脑电图特点

目的 探讨儿童自身免疫性脑炎早期脑电图特点,为免疫性脑炎的早期诊治提供客观依据.方法 回顾性分析32例自身免疫性脑炎患儿[8例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎,24例为抗体阴性自身免疫性脑炎]的视频脑电图及动态脑电图资料,分析脑电图背景、临床发作期放电部位、发作间期癫痫样放电及亚临床癫痫发作情况.结果 ...     

成人MOG抗体病和抗NMDAR脑炎叠加综合征1例并文献复习

目的:探讨成人髓鞘少突胶质细胞糖蛋白(MOG)抗体病和抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎叠加综合征的临床表现、影像学特点及愈后。方法:报导1例成人MOG抗体病和抗NMDAR脑炎叠加综合征病例,并以“MOG、NMDAR”为关键词在CNKI、Sinomed数据库及PubMed(建库至2021年4月)检索中英文文献,结合既往文献报道病例总结分析。结果:共收集到15例成人病例,男性9例,平均始发年龄34.7岁,13例呈复发缓解病程,平均复发间隔时间14.4月;首发症状以头痛、抽搐、发热多见。15例患者共有39次发作事件,急性发作临床症状以头痛、视力下降、精神行为异常最常见。根据抗NMDAR脑炎及MOG抗体病的诊断标准,39次发作临床分型为:MOG抗体病1例次,抗NMDAR脑炎4例次,未定型10例次,重叠型24例次。共收集到急性发作期头颅MRI影像33次,异常32例次。皮质受累32例次、脑干12例次、放射冠8例次、视神经7例次。所有患者对一线免疫治疗均敏感,5例患者在激素减量过程中出现复发,8例患者加用二线免疫抑制剂。15例患者中有2例中断治疗,1例遗留双眼视物模糊及被害妄想,1例遗留右眼视力下降,其余13例中,改良Rankin量表(MRS)评分0~1分11例,2分2例。结论:成人MOG抗体病和抗NMDAR脑炎叠加综合征并不少,多见于男性,大多呈复发缓解病程,发作期症状以头痛、视力下降、精神行为异常多见,头颅MRI阳性率高,主要累及皮质、脑干、放射冠、视神经,所有患者对一线免疫治疗敏感但大多需要2种以上的免疫治疗且易反复,可能需要更强或更长时间的免疫治疗。建议抗NMDA受体脑炎或MOG抗体病患者同时进行自身免疫性脑炎相关抗体及MOG抗体的检测,以制定最佳的治疗方案,减少复发。     

抗N-甲基-D-天冬氨酸受体脑炎八例分析

目的：探讨抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎患者的临床表现、辅助检查特点、治疗和预后。 方法：回顾性分析我院8例确诊为抗NMDAR脑炎患者的临床资料。结果：5例患者出现前驱症状;所有患者 临床症状均出现快速进展的精神行为异常、认知障碍,4例为首发症状,此外表现有言语障碍、癫痫发作、运 动障碍、意识水平下降及自主神经功能障碍等;8例患者脑脊液抗NMDAR抗体阳性,3例头MRI检查显示异 常病灶,位于大脑皮质、丘脑、海马、脑干等部位。6例患者脑电图异常,为弥漫性慢波或局灶性痫样放电。 所有患者均接受一线免疫治疗,延误诊治的1例患者对治疗反应差并出现复发。结论：抗NMDAR脑炎临床 表现复杂多样,但具有其特点,对于出现不明原因的精神行为异常或认知障碍的青年患者,及时行抗NMDAR抗体筛查十分必要,早期治疗预后良好。     

抗NMDAR脑炎的临床特点分析

目的 探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点及治疗预后.方法 收集2018年12月至2020年6月于北京朝阳中西医结合急诊抢救中心神经内二科诊治的12例抗NMDAR脑炎患者的临床资料,回顾性分析其一般资料、脑脊液检测、抗NMDAR抗体检测、影像学表现、脑电图表现及治疗预后.结果 12例患者中男性3...     

抗NMDAR脑炎二例临床误诊分析

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,减少误诊误治。方法对我院近期收治的抗NMDAR脑炎2例的临床资料进行回顾性分析并复习相关文献。结果本组2例因精神行为异常、意识障碍、癫痫发作就诊,例1曾按病毒性脑炎予抗病毒治疗;例2曾按分离性精神障碍予抗精神药物治疗。入我科后经综合分析,考虑自身免疫相关脑炎可能,行腰椎穿刺脑脊液及血液检查示NMDAR抗体均强阳性,确诊为抗NMDAR脑炎。予糖皮质激素、免疫球蛋白等治疗,例1合并畸胎瘤行手术切除后予利妥昔单抗、环磷酰胺及抗癫痫等对症治疗,后2例症状均明显改善出院。随访3个月~1年,例1畸胎瘤复发;例2病情出现反复,再次入院予对症治疗后好转出院。结论抗NMDAR脑炎临床表现无特异性,易误诊。及时行相关抗体检测、伴有肿瘤者及时切除肿瘤并早期予免疫抑制剂是避免误诊误治的关键。     

自身免疫性脑炎临床特征初探

目的:探讨自身免疫性脑炎(AE)的临床特征、治疗及预后。方法:对7例AE患者的临床表现、实验室检查、治疗及预后等临床资料进行回顾性分析。结果:7例AE患者中,4例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎,其中2例伴畸胎瘤;2例为抗富含亮氨酸胶质瘤失活蛋白1(LGI1)脑炎;1例为抗γ-氨基丁酸B型受体(GABABR)脑炎。以精神行为异常(5/7)、癫痫发作(4/7)、意识清晰度下降(4/7)、面-臂肌张力障碍发作(FBDs)(1/7)、近事记忆力下降(2/7)为主要临床表现。2例颅脑MRI检查异常。2例脑脊液(CSF)抗NMDAR抗体阳性,2例CSF抗LGI1抗体阳性,1例CSF抗GABABR抗体阳性。2例血清抗NMDAR抗体阳性,1例血清抗GABABR抗体阳性。7例经治疗后4例基本恢复,2例遗留癫痫发作(1例伴精神行为异常),复发1例。结论:以急性发作的精神行为异常、癫痫发作及FBDs为主要临床表现的患者要警惕AE可能。AE临床治疗以激素、免疫球蛋白及手术(伴有畸胎瘤患者)治疗为主,早期诊疗多数预后良好。     

不伴肿瘤的抗NMDA受体脑炎临床特点

目的:通过回顾性分析8例不伴肿瘤的抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的临床特点,提高临床对不伴肿瘤的抗NMDA受体脑炎的认识。方法:回顾性分析我院2015-01-2016-06收治的8例不伴肿瘤的抗NMDA受体脑炎的一般资料、主要表现、头部MRI、脑电图、血清及脑脊液抗NMDA抗体等资料。结果:本组8例患者平均年龄38.4岁,主要表现为精神行为异常8例(100%)、癫痫发作7例(87.5%)、植物神经功能紊乱7例(87.5%)等,脑脊液抗NMDA抗体均阳性,MRI异常2例(25.0%),脑电图出现极度δ刷状波2例(25.0%),免疫抑制治疗有效7例(87.5%),完全恢复2例(25.0%),好转5例(62.5%),死亡1例(12.5%)。结论:本组不伴发肿瘤的抗NMDA受体脑炎与既往报道的伴发肿瘤的抗NMDA受体脑炎临床特点相似,亦主要表现为精神行为异常、癫痫发作、植物神经功能紊乱等,脑电图可出现特征性极度δ刷状波,及时免疫治疗预后较好。     

抗N-甲基-D-天冬氨酸受体脑炎研究进展

抗N-甲基-D-天冬氨酸受体脑炎(抗NMDA受体脑炎)是一种自身免疫介导性脑炎,病程呈多阶段进展,临床表现为急性或亚急性起病,以近记忆缺损、自主神经功能障碍、精神行为异常及癫痫发作为特点。抗NMDA受体脑炎病因不明确,大多有不典型的临床症状,漏诊误诊率高,诊断上需要检测血清和脑脊液抗NMDAR抗体,治疗上以尽早免疫治疗、合并肿瘤手术切除、支持治疗,ICU监护等,预后大多数良好,疾病可能复发,需要长期随访。     

以癫痫发作为首发症状的自身免疫性脑炎8例临床分析

目的:分析以癫痫发作为首发症状的自身免疫性脑炎的临床资料。方法:回顾性分析以癫痫发作为首发症状的自身免疫性脑炎8例患者的临床表现、实验室检测结果、影像学检查、脑电图特点、治疗结果及随访记录,并结合文献讨论。结果:8例患者中,抗NMDAR脑炎5例,抗GABAR脑炎2例,抗LGI1脑炎1例;男6例,女2例;发病年龄18～66岁,平均50岁;均以癫痫发作为首发症状,均有反应迟钝、近记忆力下降的症状,其中出现精神行为异常3例,言语障碍1例,顽固性低钠血症1例;均有≥1种的癫痫发作形式,脑电图异常6例;头颅MRI检查异常4例。结论:对于以癫痫发作为首发症状的自身免疫性脑炎患者,早期免疫治疗及抗癫痫治疗,对于控制症状、改善预后具有重要意义。     

Electroconvulsive therapy and/or plasmapheresis in autoimmune encephalitis?

Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes - anti-N-Methyl-D-Aspartate receptor (NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment (and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment. We discuss several previously published case reports and one new case. These reports examined the effects of electroconvulsive therapy (ECT) on patients with autoimmune encephalitis, particularly those in whom psychiatric symptoms are especially debilitating and refractory to standard treatment. We also discuss factors predicting good outcome and possible mechanisms by which ECT may be effective. Numerous cases, such as those presented by Wingfield, Tsutsui, Florance, Sansing, Braakman and Matsumoto, demonstrate effective use of ECT in anti-NMDAR encephalitis patients with severe psychiatric symptoms such as catatonia, psychosis, narcolepsy and stupor who had failed to respond to standard treatments alone. We also present a new case of a 71-year-old female who presented to a psychiatric unit initially with depression, which escalated to catatonia, delusions, nihilism and auditory hallucinations. After anti-NMDAR antibodies were isolated, she was treated by the neurology team with plasmapheresis and steroids, with a partial response. She received multiple sessions of ECT and her psychiatric symptoms completely resolved and she returned to her premorbid state. For this reason, we suggest that ECT should be considered, particularly in those patients who are non-responders to standard therapies.     

Adjunct therapeutic plasma exchange for anti-N-methyl-D-aspartate receptor antibody encephalitis: a case report and review of literature

Encephalitis associated with autoantibodies directed against the N-methyl-D-aspartate receptor (NMDAR) is usually a paraneoplastic syndrome that presents in young females with ovarian teratomas. We report a case of a previously healthy 14-year-old girl with sudden-onset paranoia, hallucinations, hyperactivity, increased speech, decreased sleep, seizures, and violent behavior deteriorating to catatonia. Her cerebrospinal fluid tested positive for anti-NMDAR antibodies. She was treated with five sessions of therapeutic plasma exchange (TPE) after having failed therapy with antibiotics, intravenous steroids, intravenous immunoglobulin (IVIG), one dose of rituximab, and seven sessions of electroconvulsive therapy (ECT). The American Society for Apheresis assigns a Category III (Grade 2C) recommendation for TPE in paraneoplastic neurologic syndromes; however, apheresis specifically for anti-NMDAR encephalitis has not been well studied. Literature review revealed two case reports describing outstanding improvement in patients with anti-NMDAR encephalitis following TPE. We report no improvement in our patient's symptoms after plasma exchange and discuss possible reasons for why it failed along with review of the literature.     

Four-year-old anti-N-methyl-D-aspartate receptor encephalitis patient with ovarian teratoma: A case report

BACKGROUNDA population-based comparative study in United States shows that the prevalence and incidence of autoimmune encephalitis are comparable to those of infectious encephalitis and its detection is increasing over time. Some patients are complicated with ovarian teratoma. The younger the patient is, the less likely a tumor will be present.CASE SUMMARYThis case report describes the successful treatment of anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis by early laparoscopic ovarian cystectomy and immunotherapy in a 4-year-old female child. And to the best of our knowledge, this detailed case report describes the youngest patient to date with anti-NMDAR encephalitis who underwent laparoscopic ovarian cystectomy.CONCLUSIONAlthough the younger the patient is, the less likely a tumor will be detected, we still emphasize that all patients with suspected or confirmed anti-NMDAR encephalitis should be screened for ovarian tumors if possible. Prompt initiation of immunotherapy and tumor removal are crucial for good outcomes.     

儿童自身免疫性脑炎MRI特点

目的 观察儿童自身免疫性脑炎（AE）的头部MRI表现。方法 回顾性分析16例临床确诊AE患儿的头部MRI表现，并进行MRI分型：1型，颅内无病变；2型，边缘叶型；3型，边缘叶外型；4型，混合型；2~4型均为异常表现。结果 16例AE中，头部MRI诊断为1型8例，3、4型各4例，未见2型病例。8例3、4型AE中，5例主要累及额叶、颞叶、岛叶、顶叶及基底核、海马等，表现为点状或斑片状T2WI及T2液体衰减反转恢复（FLAIR）高信号、弥散加权成像（DWI）等信号；2例主要累及颞叶、额叶、顶叶、岛叶及海马，表现为点状或斑片状T2WI及T2 FLAIR高信号、DWI高信号，其中1例大脑皮层增厚；1例小脑萎缩。结论 50% AE患儿存在脑部异常，主要累及是颞叶、额叶、岛叶及海马，MRI主要表现为T2WI、T2 FLAIR高信号。     

二代测序诊断EB病毒性脑炎继发自身免疫性脑炎1例报道

病毒性脑炎与自身免疫性脑炎在临床中常难以鉴别,临床表现较为相似,但治疗方案不同,因此早期识别尤为重要。本文报道1例影像学提示边缘叶脑炎,经二代测序技术确诊为EB病毒感染的病毒性脑炎患者,治疗后6个月继发癫痫,自身免疫性抗体抗谷氨酸受体(NMDA型)抗体阳性,诊断为成人病毒性脑炎后自身免疫性脑炎(PVEAE)。结合相关文献,本文描述了二代测序技术早期诊断病毒性脑炎及自身免疫性脑炎的临床应用,讨论病毒性脑炎继发自身免疫性脑炎的临床特征,旨在更好地实现二者的早期诊断与鉴别,改良患者诊疗方案,为患者带来更好的预后结局。