抗γ-氨基丁酸B型受体脑炎老年患者临床特点分析

目的回顾分析抗γ-氨基丁酸B型受体(GABABR)脑炎老年患者的病例资料,提高对该病的认识。方法回顾2014年12月至2015年12月在该院就诊的7例60岁以上抗GABABR脑炎患者的病例资料,总结临床特点、影像、电生理、实验室检查、治疗及预后。结果 7例抗GABABR脑炎患者多以抽搐起病,病程中可出现精神行为异常、记忆力下降等临床表现。6例脑脊液检查异常,白细胞或蛋白轻度升高。6例患者血清和脑脊液GABABR抗体均为阳性,1例患者仅血清GABABR抗体阳性。头部MRI多表现为海马、颞叶、丘脑异常信号,脑电图常提示部分性发作。1例患者合并肺癌。免疫治疗后多数患者预后好。结论当临床怀疑边缘叶脑炎时,需完善血和脑脊液相关免疫抗体检测、脑脊液常规检查、头部MRI、脑电图以明确诊断。以加强对抗GABABR脑炎的关注,尽早诊断及治疗有利于改善患者的预后。     

老年自身免疫性脑炎临床特点分析

目的比较老年患者与青年患者自身免疫性脑炎的临床症状,总结老年患者的特点,提高对老年患者自身免疫性脑炎的识别。方法回顾性分析2013年10月~2015年10月在解放军总医院神经内科诊断为自身免疫性脑炎的患者20例,老年组5例,其中男性4例,女性1例,平均年龄(65.0±4.8)岁;青年组15例,其中男性12例,女性3例,平均年龄(34.3±14.5)岁。对2组患者的临床表现、血清免疫抗体、脑脊液、头颅磁共振成像及脑电图等方面进行比较分析,总结老年性自身免疫性脑炎的临床特点。结果老年组患者多以癫痫症状为主要表现,老年组患者同时存在癫痫、精神行为异常和认知功能障碍3大临床症状患者比例高于青年组(80.0%vs 60.0%,P=0.417),不自主运动症状患者比例低于青年组(20.0%vs 33.3%,P=0.573),但均无统计学差异。老年组脑脊液白细胞数明显低于青年组[(5.20±3.11)×106/Lvs(26.20±24.47)×106/L,P=0.000],2组间脑脊液蛋白水平、复发率无统计学差异(P0.05)。结论老年自身免疫性脑炎患者多以癫痫为首发症状,脑脊液常规多无异常表现,自身免疫相关抗体的检测有助于诊断,确诊后应尽早治疗。     

γ-氨基丁酸受体基因多态性与特发性癫痫的相关性研究

目的探讨GABA受体基因GABBR1多态性在特发性癫痫发病（IGE）中的作用。方法采用PCR、琼脂糖凝胶电泳技术分析46例IGE患者（研究组）rs3025627、rs3025628和rs29220基因型频率变化,并与50例健康查体者（对照组）比较。结果观察组与对照组rs3025627TT基因型频率分别为0．362、0．245,P〈0．01,rs3025627AT基因型频率分别为0．354、0．447,P〈0．05。两组rs3025628和rs29220基因型频率无统计学差异（P〉0．05）。结论rs3025627基因型频率改变可能是导致GABBR1基因外显子7在IGE病中起作用的原因。     

γ-氨基丁酸能信号系统与胰岛素抵抗

γ-氨基丁酸(GABA)是一种重要的抑制性神经递质,研究发现,除了神经系统外,GABA还广泛分布于脂肪、肝脏、肌肉等对胰岛素敏感的组织。GABA可通过对糖脂代谢、炎性反应、免疫应答、肠道菌群等途径的调控,增强外周组织胰岛素信号转导,从而改善胰岛素抵抗,为2型糖尿病的防治提供了新的方向。     

老年人抗N-甲基-D-天冬氨酸受体脑炎五例

<正>抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是与自身抗体介导的突触功能障碍有关的自身免疫性脑炎。该病是备受关注的边缘性脑炎的一种,多见于育龄期女性和儿童,老年、男性则相对少见~([1])。本研究回顾性分析5例老年抗NMDAR脑炎患者的临床资料。1临床资料收集2015年1月～2018年9月在赣南医学院第一附属医院确诊的抗NMDAR脑炎患者5例,男性4例,女性1例,年龄60～70岁,平均年龄63.4岁。所有患者诊断和治疗均按2017年中国自身免疫性脑炎诊治专家共识及2016年自身免疫性脑炎诊治指南[2-3]。病例1男性,70岁;病例2男     

抗NMDA受体脑炎二例

<正>1病例介绍病例1,男性,13岁,学生;因"精神行为异常、抽搐5 d"于2014-07-06入院。患者自7月1日中午出现精神行为异常、懒言、不愿交流、反应迟钝,伴言语不清、右上肢抽搐,抽搐持续数分钟后自行缓解。当地医院头颅CT检查未见异常。7月6日下午又出现右下肢不自主抖动,伴口角不自主抽动。无双眼上翻、口吐白沫,无意识丧失、大小便失禁等。1     

老年人心绞痛型癫痫的临床特点

老年人心绞痛型癫痫的临床特点济南市中心医院（２５００１３）于志远孙勇郁阿丽张玉珍李庆红荆瑞云老年人心绞痛型癫痫较少见，临床容易误诊。１９９２年以来，我们收治１２例患者，现将其临床特点分析报告如下。１临床资料１．１一般资料本组男５例，女７例；年龄６１～...     

感染相关的抗N-甲基-D-天冬氨酸受体脑炎的临床特点分析

目的:总结感染后出现的抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点。方法:收集5例感染后出现的抗NMDAR脑炎患者临床病历资料,并检索感染性因素相关的NMDAR脑炎的文献资料,分析此类疾病的临床特点。结果:5例(男4,女1)呼吸道感染后出现的抗NMDAR脑炎患者,平均年龄23.4岁,所有患者均未发现肿瘤,3例合并其他自身免疫性抗体。感染至发病的时间(5.2±2.8)d。主要症状包括发热、精神行为异常、癫痫发作、小便障碍,平均确诊时间达(40.6±25.4)d。血清和脑脊液的NMDAR抗体滴度均达到()。脑脊液检查以轻度细胞数升高和/或蛋白升高为主,有4例出现头颅磁共振及脑电图检查异常,2例进重症监护病房。经过免疫治疗后,有3例预后良好,2例预后一般。5例患者病程中均出现反复发作,2年内总复发次数达11次。结论:感染因素可以诱发抗NMDA抗体脑炎,其多在感染后1周内发病。主要症状仍以发热、精神行为异常为主,容易误诊为病毒性脑炎,脑脊液NMDAR抗体检测有助于明确诊断,免疫治疗后复发率高、预后欠佳。     

Cross-reactivity of a pathogenic autoantibody to a tumor antigen in GABAA receptor encephalitis

Encephalitis associated with antibodies against the neuronal gamma-aminobutyric acid A receptor (GABA_A-R) is a rare form of autoimmune encephalitis. The pathogenesis is still unknown but autoimmune mechanisms were surmised. Here we identified a strongly expanded B cell clone in the cerebrospinal fluid of a patient with GABA_A-R encephalitis. We expressed the antibody produced by it and showed by enzyme-linked immunosorbent assay (ELISA) and immunohistochemistry that it recognizes the GABA_A-R. Patch-clamp recordings revealed that it tones down inhibitory synaptic transmission and causes increased excitability of hippocampal CA1 pyramidal neurons. Thus, the antibody likely contributed to clinical disease symptoms. Hybridization to a protein array revealed the cross-reactive protein LIM-domain-only protein 5 (LMO5), which is related to cell-cycle regulation and tumor growth. We confirmed LMO5 recognition by immunoprecipitation and ELISA and showed that cerebrospinal fluid samples from two other patients with GABA_A-R encephalitis also recognized LMO5. This suggests that cross-reactivity between GABA_A-R and LMO5 is frequent in GABA_A-R encephalitis and supports the hypothesis of a paraneoplastic etiology.

A shared feature of autoimmune encephalitis syndromes are autoantibodies recognizing either intracellular antigens or extracellular epitopes of cell-surface antigens (1–5). Intracellular antigens are primarily released by cellular immune responses and later bound by autoantibodies, whereas cell surface antigens are assumed to be targets of direct humoral immune responses. For both processes, paraneoplastic mechanisms are surmised, and tumors are indeed often detected in conjunction with both types of autoimmunity albeit at different frequencies. However, in some patients a tumor is identified neither at the onset nor during the course of the neurological disease. Here, the immune reaction may have already erased the malignancy or have kept it below the diagnostic detection limit (6, 7).Encephalitis associated with autoantibodies against the gamma-aminobutyric acid A receptor (GABA_A-R) has recently been described (8–11). Anti–GABA_A-R antibodies cause GABA_A-R cross-linking and internalization of the antibody–receptor complex with a selective reduction of postsynaptic GABA_A-R clusters at inhibitory GABAergic synapses (9, 12). This is supposed to cause hyperexcitability and dysfunction of neuronal networks, and thus clinical disease symptoms (1). The immunopathogenesis is heterogeneous and may include viral as well as tumoral triggers of GABA_A-R encephalitis: Viral infections of the central nervous system (CNS) may represent triggers of the disease in some patients (13, 14), whereas peripheral tumors are clinically detectable in about one-third of all patients at the time of GABA_A-R encephalitis diagnosis (14). The malignancies comprise thymoma, Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, multiple myeloma, small cell lung cancer, and rectal cancer (8–10, 13, 15, 16).Here we describe cloning and functional analysis of a pathogenic antibody from the cerebrospinal fluid (CSF) B cells of a patient with “idiopathic” GABA_A-R encephalitis, who has been described earlier and was termed “index patient 2” (IP2) (9). In the CSF and the hippocampus of this patient we have recently detected a strongly expanded CD8+ T cell clone (17). We expressed the dominant antibody from the CSF B cells of patient IP2 (termed Ab-IP2) recombinantly and showed that it binds recombinant α1-subunits of the GABA_A-R, recognizes hippocampal neuropil structures, and dampens phasic GABAergic inhibitory synaptic activity and increases excitability in hippocampal CA1 pyramidal neurons. Hybridization to a protein array and confirmation by enzyme-linked immunosorbent assay (ELISA) and immunoprecipitation revealed that Ab-IP2 also recognized LIM-domain-only protein 5 (LMO5, synonym: “Cysteine and glycine-rich protein 2,” CSRP2). We detected cross-reactivity between GABA_A-R and LMO5 also in CSF samples from two other patients with GABA_A-R encephalitis. Strikingly, all LMO proteins are related to tumor growth and spread (18), and this has also been demonstrated specifically for LMO5 (19–24). This may hint toward a paraneoplastic pathogenesis (induced by detectable or occult tumors) and linked T and B cell responses as a major principle in the immunopathogenesis of GABA_A-R encephalitis.     

老年性癫癎的临床特征和脑电图分析

目的分析和总结老年性癫癎的临床特征、脑电图特点及临床意义。方法对92例60岁后发病的老年性癫癎患者的临床、脑电图及神经影像学资料等进行分析。结果 92例患者中,复杂部分性发作是最常见的发作类型。82例为症状性癫癎,10例为隐源性癫癎。脑血管病、脑肿瘤、代谢中毒性疾病、颅内感染、脑外伤等是常见病因。脑电图异常以局灶性慢波最常见。56例随访患者中,47例发作控制良好。结论老年人群是癫癎的高发人群,正确认识临床特征和脑电图特点是诊断治疗的关键。     

重庆市303例儿童流行性乙型脑炎的临床特征

目的分析重庆地区流行性乙型脑炎的临床特征。方法运用回顾性调查研究的方法,收集2007-2010年重庆医科大学附属儿童医院住院的流行性乙型脑炎患儿资料,分析其临床特征。结果 303例患儿,男女比例2.03∶1,1～4岁115例,4～7岁103例,7～10岁43例,发病季节7-9月分别为77、207和19例。未接种疫苗与接种疫苗比为8.7∶1,发病地区分布35个区县。普通型180例,占59.4%﹔重型100例,占33.0%﹔极重型23例,占7.6%.临床表现发热303例（100%）、意识障碍252例（83.2%）、抽搐214例（70.6%）、呕吐137例（45.2%）及头痛112例（37.0%）。血清与脑脊液乙脑抗体lgM阳性率分别为44.61%和79.21%,异常脑电图、头颅CT及头颅MRI分别占98.1%、53.0%和68.3%。47.2%病例在住院过程中出现并发症,出院时好转98.3%,5例死亡。结论重庆市流行性乙型脑炎常见于7月、8月,均为农村地区且大多数无疫苗接种史,以普通型为主。临床表现主要有发热、抽搐及意识障碍,多伴有脑电图、头颅CT及头颅MRI异常,脑脊液乙脑抗体lgM阳性率高于血清,大多数病例预后良好。     

三种核酸扩增方法检测临床乙脑标本的比较

目的探索三种核酸扩增方法应用于临床乙脑标本检测的敏感性,建立能够适合于临床标本的检测方法,同时比较病毒核酸检测与血清学检测的相关性。方法分别采用普通逆转录PCR、逆转录套式PCR和SyBr Green I实时荧光PCR方法对48份疑似乙脑病例的血清或脑脊液标本进行检测,并比较了三种方法的检出限。结果血清学检测表明,48份标本中有18份标本IgM抗体阳性;3种病毒核酸检测,普通逆转录PCR和SyBr Green I实时荧光PCR方法未能检出阳性标本,而逆转录套式PCR检出15份阳性标本。结论三种核酸检测方法中,逆转录套式PCR最为敏感;单独依赖血清学检测会产生相当一部分假阴性,证明了临床实验室加强乙脑病毒核酸检测对临床诊断具有重要意义。     

GABAA receptor encephalitis associated with human parvovirus B19 virus infection: Case report

Rationale:Human parvovirus B19 (B19) infection can produce a spectrum of clinical syndromes, including neurological manifestations, most notably encephalitis. Although symptoms suggestive of autoimmune disease in patients with B19 infection have been previously described, a clear association of autoimmune encephalitis with B19 infection has yet to be established.Patient concerns:We describe the case of a 6-year-old boy who was hospitalized due to status epilepticus, which evolved to super-refractory status epilepticus that was only mildly responsive to anticonvulsant drugs.Diagnosis:A cerebrospinal fluid study identified slight pleocytosis and B19 positivity. A subsequent autoimmunity cerebrospinal fluid study revealed the presence of anti-γ-aminobutyric acid type A (GABA_A) receptor antibodies.Interventions:After pulse therapy with methylprednisolone and continuous therapy with prednisolone with cyclosporine, the patient experiencing seizure persistence with disordered motor function manifestations and only minor improvement in consciousness, and so, plasmapheresis was performed. With continued immunosuppressive treatments with cyclosporine and prednisolone, the patient''s clinical picture showed progressive improvement, with good control of seizures. Although the patient tolerated withdrawal of the anticonvulsant drugs well, he developed seizures when corticosteroid therapy withdrawal was attempted, so was started on azathioprine.Outcomes:After immunosuppressive therapy, the patient evolved with complete remission of symptoms, normal neurological examination and age-appropriate neuropsychomotor development.Lessons:The present case characteristics, together with previous findings, support the hypothesis that autoimmunity may be triggered by extensive antigen release due to degeneration of infected neurons. This case highlights the importance of early clinical suspicion and treatment.     

老年人癫痫发作的临床特征分析

目的探讨老年人癫痫发作的临床表现、病因及其诊断与鉴别诊断。方法回顾性分析139例患者的临床资料,其中癫痫发作125例,非癫痫发作误诊为癫痫发作14例。结果主要病因为脑血管病(46.4%),其次为病因不详(29.6%)。125例癫痫发作患者中,部分性发作86例(68.8%),全面性发作39例(31.2%);53例行常规脑电图检查,12例(22.6%)出现非特异性异常,1例(1.9%)出现癫痫样电发放;43例行长程视频脑电图检查,38例(88.4%)出现癫痫样电发放。14例非癫痫发作均误诊为癫痫发作。结论脑血管病是老年患者癫痫发作最常见的病因,部分性发作为老年患者癫痫发作最常见的发作形式。Todd麻痹和非抽搐性癫痫持续状态为癫痫发作的特殊形式,极易误诊。代谢障碍性疾病、晕厥、短暂性全面性遗忘、短暂性脑缺血发作所致的非癫痫发作与癫痫发作的鉴别诊断较为困难。     

高龄缺血性脑白质损害患者的临床分析

目的探讨高龄缺血性脑白质损害患者的危险因素和临床特点。方法将248例>75岁的缺血性脑白质损害患者分为3组:轻度脑白质损害组(轻度损害组,88例)、中度脑白质损害组(中度损害组,104例)和重度脑白质损害组(重度损害组,56例)。采用logistic回归分析其危险因素,并对临床特征进行分析比较。结果轻度、中度和重度脑白质损害患者所占比例分别为35.5%、41.9%和22.6%;年龄和血压与脑白质损害程度相关;非特异性神经系统症状(34.7%)、运动功能减退(24.6%)和认知功能减退(21.4%)为主要就诊原因,差异显著(P<0.05)。结论年龄和高血压是脑白质损害的危险因素;运动和认知功能障碍是脑白质损害的重要临床特征。     

云南省流行性乙型脑炎研究进展

云南省是我国流行性乙型脑炎高发区,且近年来有回升趋势。本文综述了云南省该病的发病与分布、媒介蚊种及中间宿主等研究情况,探讨了防治对策,并提出了建议。     

老年脑小血管病患者双重任务行走步态特征

目的分析老年脑小血管病(CSVD)患者双重任务行走(DTW)步态特征。方法选择解放军总医院第七医学中心住院的老年脑白质损害患者46例为CSVD组和正常老年人22例为对照组,用单独行走和DTW测试,计算跨步时间变异系数(CV)、步态不对称性(GA)及时相协调指数(PCI)。结果与单独行走比较,对照组DTW跨步长变短、步速降低,CSVD组DTW跨步时间延长,跨步长变短,步速降低,步频减慢,CV、GA、PCI明显增加(P<0.01)。单独行走时,CSVD组跨步长和步速明显低于对照组,GA和PCI明显高于对照组[(1.11±0.13)m vs(1.19±0.14)m,(1.01±0.16)m/s vs(1.10±0.15)m/s,2.54(1.58,3.55)vs 2.18(0.00,2.53),(4.44±1.40)vs(2.70±0.74),P<0.05,P<0.01]。DTW时,CSVD组跨步长、步速和步频明显低于对照组,跨步时间、CV、GA、PCI明显高于对照组(P<0.05,P<0.01)。DTW时,CSVD组每步时相φ值的离散程度较大。结论老年CSVD患者在DTW时更易出现步态障碍,步态的稳定性、对称性及协调性降低,DTW更能反映其日常生活活动能力。