发病酷似单纯疱疹病毒脑炎的抗N-甲基-D-天冬氨酸受体脑炎

目的 提高对发病酷似单纯疱疹病毒脑炎(Herpes simplex encephalitis,HSE)的抗N-甲基-D-天冬氨酸(N-methyl-D-aspartate,NMDA)受体脑炎的认识。方法 报道2例发病酷似单纯疱疹病毒脑炎的抗NMDA受体脑炎,并复习相关文献。结果 2例患者均急性起病,表现为发热、抽搐、认知功能减退、精神行为异常等。脑MRI均未见异常。病例1、病例2脑脊液白细胞最高分别为20×10⁶/L、21×10⁶/L。脑脊液蛋白、葡萄糖以及氯化物水平均正常。2例患者早期均被诊断为HSE,并接受阿昔洛韦抗病毒治疗,但病情仍进展。抗NMDA受体抗体检测显示例1脑脊液及血液均阳性,例2脑脊液阳性,从而明确了抗NMDA 受体脑炎的诊断。结论 对脑MRI无异常,脑脊液白细胞无明显升高的边缘系统脑炎,应想到抗NMDA 受体脑炎可能。     

易误诊为病毒性脑炎的成人抗N-甲基-D-天门冬氨酸受体脑炎(附16例临床分析)

目的成人抗N-甲基-D-天门冬氨酸(NMDA)受体脑炎易误诊为病毒性脑炎,本文总结确诊的16例成人抗NMDA受体脑炎临床特征及治疗与预后,以提高对该病认识。方法对自2014年12月至2016年6月在湘雅医院神经内科诊治的16例抗NMDA受体脑炎患者的临床表现、实验室检查、影像学资料、治疗经过及预后进行回顾性分析。结果 16例患者表现为显著的神志障碍、精神行为异常、癫痫发作,部分患者伴随自主神经功能异常。头部MRI示正常或多发病灶。其中11例患者脑脊液和血清NMDA受体抗体均阳性,另外5例患者仅脑脊液抗体阳性。经免疫治疗,10例患者预后良好,6例患者预后不良。结论抗NMDA受体脑炎早期易被误诊为病毒性脑炎、癫痫、原发精神疾病,可疑患者应尽早行抗NMDA受体抗体检查,及早治疗能够改善患者预后。     

抗 N －甲基－D －天门冬氨酸受体脑炎57例临床分析

抗N－甲基－D－天门冬氨酸（NMDA）受体脑炎是一组免疫介导的神经精神综合征,属于自身免疫性脑炎。2007年Dalmau和Bataller［1］首先报道了表现为神经精神症状的12例抗NMDA受体脑炎患者,均行血清或脑脊液检测,发现了抗NMDA受体抗体,依据临床上表现出的精神症状、癫痫发作、记忆障碍、意识水平下降以及低通气状态等一系列特征,称为抗NMDA受体脑炎。并明确了这类脑炎的异常靶点在NMDA受体的NRl亚基上。随着对疾病的认识和检查手段的不断丰富,近两年来,诊断为抗NMDA受体脑炎患者的数量也大量增多,本研究汇总了2013年1月～2015年11月经抗NMDA受体抗体筛查,脑脊液NMDA受体抗体阳性,伴或不伴血清NMDA受体抗体阳性,同时排除其他诊断如病毒性脑炎、脑肿瘤、代谢性疾病、药物中毒等,确诊为抗NMDA受体脑炎的非儿童患者57例。现对其临床特点进行分析,以提高对抗NMDA受体脑炎的认识。     

抗N-甲基-D-天冬氨酸受体脑炎一例

目的 探讨抗N-甲基-M-天冬氨酸(NMDA)受体脑炎的临床特点、诊断及治疗.方法报道1例伴卵巢畸胎瘤的抗NMDA受体脑炎患者的临床表现及诊治过程,并总结其临床特征.结果患者为青年女性,表现为精神行为异常及抽搐,意识障碍及通气不足.早期发现畸胎瘤,脑脊液中抗NMDA受体抗体阳性.行畸胎瘤手术切除,联合静脉用免疫球蛋白和激素冲击治疗,患者临床痊愈.结论 抗NMDA受体脑炎是一种自身免疫性的、能用血清学方法诊断的疾病.早期明确诊断和给予及时治疗,预后良好.     

儿童复发性抗N-甲基-D-天门冬氨酸受体脑炎的研究进展

抗N-甲基-D-天门冬氨酸(N-methyl-D-aspar-tate,NMDA)受体脑炎是一种由抗NMDA 受体抗体介导的自身免疫性脑炎.2007 年 Dalmau 等[1]发现本病的致病性抗体和诊断标志物,即抗 NM-DA受体抗体,明确了抗N MDA 受体脑炎的概念.目前,抗N MDA受体脑炎是最常见的自身免疫性脑...     

抗N-甲基-D-天冬氨酸受体脑炎

抗N-甲基-D-天冬氨酸(NMDA)受体脑炎是近几年发现的边缘叶脑炎,为抗NMDA受体抗体介导的一种自身免疫性疾病,常合并肿瘤表现为副肿瘤综合征。该病患者可出现边缘叶损害症状,颅脑MRI可见边缘叶异常信号,脑脊液及血清能检测到NMDA受体抗体。该疾病较其它副肿瘤综合征发病率高、易误诊且病情重,但及时治疗预后良好。本文就抗NMDA受体脑炎发病机制、临床特点、辅助检查、诊断治疗等研究现状进行综述,进而提高对该疾病的认识,指导临床治疗。     

以单侧肢体无力起病的不典型抗NMDA受体脑炎1例报告

<正>抗NMDA受体脑炎(Anti-NMDA receptor encephalitis)是与抗N-甲基-D-天冬氨酸受体(NMDA受体)抗体相关的自身免疫性脑炎~([1]),于2007年被Dalmau等首次报道~([2]),随着对该病认识的深入,临床发现率逐渐增多,但对于具有不典型临床表现的病例仍易漏诊误诊,现报道1例以单侧肢体无力起病,早期抗体检测阴性的不典型抗NMDA受体脑炎患者,旨在加强临床对该类患者的早期认识,以免延误病情,影响患者预后。1病例资料     

自身免疫性脑炎临床鉴别诊断及治疗研究

目的分析自身免疫性脑炎的鉴别诊断及治疗措施。方法选取2015-01—2016-02我院治疗的病毒性脑炎患者为病毒性脑炎组,自身免疫性脑炎患者为自身免疫性脑炎组,每组50例,进行相关鉴别诊断;同时将自身免疫性脑炎患者按随机数字表法分为观察组(25例)与对照组(25例),观察组给予免疫球蛋白、激素治疗,对照组仅给予激素治疗。观察并比较自身免疫性脑炎的鉴别诊断及效果。结果自身免疫性脑炎组脑脊液蛋白质含量正常率(74.00%)、亚急性发作率(52.00%)、脑脊液与血清中抗NMDA抗体(54.00%)均高于病毒性脑炎组,影像学累及颞叶率(12.00%)低于病毒性脑炎组;观察组总有效率(84.00%)优于对照组(56.00%),差异具有统计学意义(P0.05)。结论自身免疫性脑炎大多亚急性发作,其脑脊液蛋白质含量基本正常,自身免疫性脑炎患者在脑脊液与血清中均存在不同程度的自身免疫性抗体,同时免疫球蛋白联合激素有更好的治疗效果。     

抗N-甲基-D-天冬氨酸受体脑炎1例报道

<正>抗N-甲基-D-天冬氨酸受体脑炎(anti-N-methyl-D-aspartate receptor encephalitis,抗NMDA受体脑炎)是一种与NMDA受体有关的新型自身免疫性边缘叶脑炎,对治疗反应良好。该病多以精神症状首发,血与脑脊液可检测出抗NMDA受体抗体,临床上易与单纯疱疹病毒性脑炎、桥本氏脑病、急性脱髓鞘等疾病混淆。现将本科收治的1例抗NMDA受体脑炎病例资料报道如下。     

抗N-甲基-D-天冬氨酸受体脑炎29例误诊分析

目的提高对抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的认识,减少临床误诊。方法对2013年11月~2016年10月成都上锦南府医院-华西上锦分院神经内科收治的29例确诊的抗NMDA受体脑炎病例进行回顾性分析,总结误诊的原因。结果 29例抗NMDA受体脑炎初期均出现误诊,平均误诊时间17 d,住院时间37 d。进行抗NMDA受体抗体检测确诊抗NMDA受体脑炎后,予以丙种球蛋白和/或糖皮质激素治疗,多数患者预后较好。少数发病初期即出现昏迷、难治性癫痫持续状态的重症患者预后较差。结论抗NMDA受体脑炎临床误诊率极高,早期行CSF和/或血液抗NMDA受体抗体检测对于该病的确诊及指导治疗具有重要的临床价值。     

Two cases of anti-NMDA receptor encephalitis

Anti-NMDA receptor encephalitis, reported by Dalmau et al., is a paraneoplastic encephalitis frequently associated with ovarian teratoma. After the manifestation of schizophrenia-like psychotic symptoms in the initial stage, serious neurological symptoms such as convulsions and central hypoventilation develop. We report two cases of 17-year-old girls with anti-NMDA receptor encephalitis who exhibited different clinical courses. Case 1 showed a typical course of anti-NMDA receptor encephalitis associated with sustained consciousness disturbance requiring long-term artificial respiration. Case 2 underwent surgery for an ovarian teratoma in the early stages of the disorder, did not show convulsions or central hypoventilation, and recovered without any sequelae. Early resection of the ovarian teratoma and the immune suppression therapy may have contributed to the rapid recovery and favorable outcome in case 2. Psychiatrists are the first to see a majority of patients with anti-NMDA receptor encephalitis because of psychiatric symptoms and behavioral changes observed in the initial stage. For successful treatment, psychiatrists need to cooperate with neurologists and gynecologists early in the course of this disorder. Psychiatrists' knowledge of the symptoms and clinical course of this form of encephalitis is essential for early detection and adequate treatment, which may be life-saving and contribute to good functional outcomes.     

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。     

自身免疫性脑炎临床表现与MRI特征分析

目的对自身免疫性脑炎的临床症状与MRI特征进行分析,提高临床医师对自身免疫性脑炎的临床特征及MRI表现的认识。方法纳入自身免疫性脑炎33例,收集一般临床资料,采用MRI观察颅内病变情况,分析其主要症状及影像学特征。结果本组自身免疫性脑炎的主要症状为精神行为异常27例(81.8%),癫痫发作26例(78.8%),18例(54.5%),发热11例(33.3%),中枢性低通气9例(27.3%)等,其中不自主运动和发热发生率LE组较NMDA组低。MRI阳性者10例,脑实质受累8例,脑膜受累3例,脑实质依次累及枕叶、双侧海马、额叶、顶叶、颞叶及丘脑、小脑等部位。结论精神行为异常及癫痫发作为自身免疫性脑炎最常见的两个症状,MRI可发现边缘系统异常信号,其中FLAIR序列最为敏感。     

视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎1例

目的 探讨视神经脊髓炎合并抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的临床特征。方法 报道本院收治的1例视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎的临床特点及诊治经过,并结合文献分析该病的特征。结果 患者为21岁女性,表现为腹泻后急性起病的下肢麻木无力、昏睡、视物模糊; MRI示双侧基底节、海马、桥臂、齿状回、延髓背侧多发异常信号,同时伴有颈髓多发条片状长T₂异常信号。脑脊液NMDA受体抗体阳性,血清和脑脊液水通道蛋白4(AQP4)抗体阳性,合并心动过缓并发症,筛查未发现肿瘤; 经激素冲击、丙种球蛋白及免疫抑制剂治疗后症状逐渐好转至痊愈。结论 视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎可以在感染后起病,症状和影像学主要表现为两种疾病的叠加,可出现心动过缓并发症,早期积极行免疫治疗效果较好。     

Neuropsychological characterization of three adolescent females with anti-NMDA receptor encephalitis in the acute,post-acute,and chronic phases: an inter-institutional case series

Objective: Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an acute, immune-mediated paraneoplastic syndrome that often presents with psychobehavioral changes, abnormal movements, autonomic instability, seizures, and cognitive dysfunction. While the disease continues to be more readily identified and appropriately treated, the course of cognitive deficits from the acute to post-acute to chronic phase has not been well described, particularly in the pediatric population. This case series describes the neuropsychological functioning of three adolescent females with anti-NMDA receptor encephalitis from its early presentation to long-term follow-up.Method: All three cases are adolescent females with antibody-confirmed anti-NMDA receptor encephalitis. A review of the literature is provided summarizing the disorder and its known cognitive sequelae, pathophysiology, treatment, and prognostic factors, as well as each patient’s relevant history, symptom presentation, and disease course. Neuropsychological functioning of each patient was evaluated from her initial inpatient hospitalization to long-term follow-up (3.5–12 months after acute evaluation).Results: All three patients demonstrated clear improvement in cognitive functioning during the course of their recovery, though selected deficits in executive functioning, fine motor dexterity, language, and memory were observed at long-term follow-up in some of our patients.Conclusions: Findings are consistent with studies in adults that found cognitive deficits following anti-NMDA receptor encephalitis. Though gradual recovery was noted over time, all three patients reported no clinically significant difficulties during their final evaluation, despite showing mild impairment in some areas, emphasizing the importance of ongoing neuropsychological follow-up.     

Cytokine/chemokine elevation during the transition phase from HSV encephalitis to autoimmune anti-NMDA receptor encephalitis

A 3-year-old girl suffered from anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis after resolution of herpes simplex virus encephalitis (HSE). Methylprednisolone pulse and immunoglobulin therapies showed little effect, but the patient completely recovered after six courses of monthly cyclophosphamide pulse therapy and successive maintenance on mycophenolate mofetil for one year. Anti-NMDA receptor antibody in the cerebrospinal fluid (CSF) was minimally detected during the prodromal febrile period and then was seen to be markedly elevated at the onset of second encephalopathy phase. CSF interleukin (IL)-6, and 10, tumor necrosis factor-α, interferon gamma, C-X-C motif ligands (CXCL)10 and 13, chemokine ligand 2, and migration inhibitory factor showed a second peak during the prodromal period and were reduced at the onset of anti-NMDA receptor encephalitis. These suggest the presence of cytokine/chemokine phase between the initial HSE and the secondary autoimmune encephalitis phases. Treatment strategy during the early stage of this entity should be further explored.     

Anti-N-methyl-D-aspartate receptor encephalitis associated with hepatic neuroendocrine carcinoma: A case report

Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis can present with and without tumor. Tumor associations are less common in older patients. We report a 65-year-old gentleman who presented with one week history of cough, chills, rigor and altered behavior, followed by florid visual and auditory hallucinations. Mini mental status examination score was 16/30. Both cerebrospinal fluid and plasma anti-NMDA receptor antibodies were detected. A course of intravenous methylprednisolone was given with partial symptom improvement. A hepatic neuroendocrine carcinoma was detected and confirmed on biopsy. Unfortunately, he developed several medical complications: non-ST elevation myocardial infarction, infected foot gangrene and peripheral vascular disease, which made him unsuitable for both surgery and chemotherapy. He passed away 6 months later due to the progression of the malignancy. This case illustrated that NMDAR encephalitis may be associated with an uncommon hepatic neuroendocrine carcinoma in an older person, which is responsive to early treatment.