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。     

感染性疾病诱发的自身免疫性脑炎四例临床特点分析

<正>自身免疫性脑炎(autoimmune encephalitis, AE)是指一类由抗体触发免疫机制介导的脑炎，此类抗体的靶抗原包括神经细胞表面抗原和神经细胞内抗原。抗神经细胞表面抗原的抗体主要结合位点位于神经细胞表面蛋白、离子通道或受体，如抗N-甲基-D-天冬氨酸受体(NMDAR)、抗富含亮氨酸胶质瘤失活蛋白1(LGI1)、抗γ-氨基丁酸B型受体(GABABR)与α氨基-3-羟基-5-甲基-4-异噁唑丙酸受体(AMPAR)等。     

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...     

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。     

抗神经元细胞表面抗原抗体相关自身免疫性脑炎7例临床分析

目的总结抗神经元表面抗原抗体(NSAbs)相关自身免疫性脑炎(AE)患者的临床特点。方法回顾性分析首都医科大学附属北京地坛医院2012-05—2017-04诊治的7例抗NSAbs相关AE患者的临床特征及实验室检查结果特点,并进行相关文献复习。结果 7例患者中男5例,女2例,年龄15~77岁。3例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎;2例抗γ氨基丁酸B型受体(GABABR)脑炎,其中1例合并抗Hu抗体阳性;2例抗富亮氨酸胶质瘤失活1蛋白(LGI1)脑炎。4例患者以癫痫为突出或惟一表现,1例患者仅表现为头痛、发热,2例患者临床表现符合经典边缘性脑炎。1例合并肺癌(抗GABABR脑炎合并抗Hu抗体阳性患者)。6例脑电图检查可见癫痫波发放,1例仅表现为头痛、发热的抗NMDAR脑炎患者脑电图未见异常。3例头MRI检查可见异常信号。7例患者经免疫治疗均好转,例1有复发。结论抗NSAbs相关AE可以癫痫为突出或惟一的临床表现,或以头痛、发热为惟一的临床症状。特异性抗NSAbs检测阳性可协助明确诊断。     

抗GABA_B R脑炎5例病例报告及文献复习

目的探讨5例抗GABABR脑炎的临床特点及诊治。方法对患者的临床表现、头部影像学、脑电图特点、脑脊液检查等进行回顾性分析及相关文献回顾。结果 5例抗GABABR脑炎患者亚急性起病、病前无感染史,均以抽搐起病,有2例伴有精神症状。1例患者头部MRI右侧海马异常高信号。脑电提示部分性发作。腰椎穿刺:CSF 2例压力升高,蛋白轻度增高或正常,白细胞(20~116×106/L,单核为主),糖正常。CSF+血抗GABABR抗体阳性。肺部CT或PET-CT扫描提示肺部占位。经糖皮质激素或丙种球蛋白治疗好转。结论 (1)头部MRI、脑电图、CSF+血抗GABABR抗体检测利于抗GABABR脑炎早期诊断;(2)抗GABABR脑炎患者应积极查找可能肿瘤,尤其肺部占位,PET-CT检查对于肺CT检查阴性者有益。     

抗N-甲基-D-天冬氨酸受体抗体脑炎复发的临床特征

目的总结复发性抗N-甲基-D-天冬氨酸受体(NMDAR)抗体脑炎的临床特点、诊断及治疗。方法回顾性分析2013年5月至2017年2月郑州大学人民医院诊治的57例抗NMDAR抗体脑炎中10例复发者的临床症状、实验室检查、影像学特点和免疫治疗。结果 10例(19%)复发中男5例,女5例,平均年龄27.5岁,10例共复发14次,其中2例复发2~3次,平均复发间隔时间7.1个月(1~19月)。复发时最常见症状为癫痫(8/14)和精神行为异常(8/14),其中1例次复发仅表现为不典型症状,如眩晕、行走不稳和复视。复发时13例次行抗NMDAR抗体检测,脑脊液抗体阳性率100%(13/13),血清抗体阳性率69%(9/13)。13例次复发再次给予免疫治疗仍有效,1例次拒绝治疗,出院后平均随访18.9月,3例完全恢复正常,4例遗留轻微后遗症,3例遗留较明显后遗症,影响日常生活。结论抗NMDAR脑炎复发时可表现为典型或不典型症状,脑脊液抗NMDAR抗体检测有助诊断,积极免疫治疗仍有效。     

抗N-甲基-D-天冬氨酸受体脑炎20例临床分析

目的分析抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者临床表现、头颅MRI、脑电图(EEG)、脑脊液(CSF)特点。方法收集20例抗NMDAR脑炎患者的临床、MRI、EEG和CSF资料。结果 20例抗NMDAR脑炎患者男性13例,女性7例。年龄3~61岁,平均年龄25±19岁。入院时头颅MRI异常率为60%(12/20),其中4例累及边缘系统,5例累及额顶颞枕叶、放射冠及半卵圆中心等结构。例4入院时头颅MRI检查未见异常,第24天复查见双顶枕叶、右侧胼胝体及扣带回区异常信号。16例患者行EEG检查,14例(88%)异常,异常的EEG主要为非特异性慢波,其中1例(例4)患者住院期间复查EEG出现大量δ波。90%(18/20)患者CSF结果异常;细胞学检查以淋巴细胞反应为主,例9有激活性单核细胞增多。11例(55%)患者血清和CSF NMDAR抗体阳性,7例(35%)患者仅CSF NMDAR抗体阳性,2例(例11、例14)患者仅血清NMDAR抗体阳性。结论本组以男性青少年多见。其主要临床表现为精神异常、癫痫、记忆力下降、行为异常、不自主运动等,严重者可出现中枢性低通气。头颅MRI病变不仅局限于边缘系统。重症患者EEG可出现δ波。早期诊断、治疗预后较好,部分患者可复发。     

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。     

Denervation study of synapses of organ of corti of old world monkeys

Fine structural characteristics of synapses in the spiral organ of Corti were examined, with reference to differences between inner and outer haircell systems, and to location of neurons of origin of efferent axons. Surgical interruption of crossed olivocochlear bundle, of vestibular nerve, of facial nerve, and excision of superior cervical ganglia were used to determine the pathways of efferent axons. Interruption of the vestibular nerve near the brainstem results in degeneration of all efferent terminals on outer hair cells. Mid-line lesions at, and caudal to, the facial colliculus result in degeneration of about half of these efferent terminals. Efferent synaptic bulbs to the inner hair-cell system are small, of the order of one micron, and form type 2 junctions with afferent dendrites. They tend to have more large dense-core vesicles (about 80 nm) than the large efferent terminals of the outer hair-cell system, and appear to be the terminals of axons in the habenula perforata, which exhibit varicosities laden with large dense core vesicles. The varicosities are unaffected by excision of the superior cervical ganglia. So far as our material can reveal, it appears that the varicosities in the habenula perforata do not survive vestibular root interruption, nor do the efferent processes in the internal spiral bundle or at the base of inner hair cells. Most interestingly, the afferent processes of the inner hair-cell system, as identified for example by their relation to pre-synaptic bodies in the inner hair cells, are subject to a trans-synaptic reaction after severance of the vestibular root. They undergo a dramatic cytological transformation, characterized by increase of volume, engorgement with microtubules, microfilaments, microvesicles of various sizes, and clusters of lysosomes. Thus, both the efferent and afferent terminals of the inner hair-cell system show marked cytological differences from the corresponding terminals of the outer hair cell system.     

Summary of legislation of 1935 of interest to the department of Mental Hygiene

Psychiatric Quarterly -     

Summary of legislation of 1941 of interest to the Department of Mental Hygiene

Psychiatric Quarterly -     

Asymmetry of on- and off-pathways of blue-sensitive cones of the retina of macaques

Macaque retinal ganglion cells whose receptive-field center recieves input from blue-sensitive cones show an overt asymmetry of the frequency of ON-center and OFF-center varieties, an asymmetry not present in ganglion cells whose center receives input from the other two cone types. A similar asymmetry of ON/OFF responses is found in the local electrotetinogram (d-wave) mediated by signals from blue-sensitive cones. ‘Blue-ON-center’ ganglion cells have larger receptive-field centers and shorter conduction latencies than other opponent-color varieties, suggesting an appreciable degree of receptor convergence and presumably large cell bodies. Intracellular stainings of these neurons with Procion Yellow show that they correspond to diffuse stratified (Parasol) ganglion cells whose flat-topped dendritic arborization stratifies in the sclerad half of the inner plexiform layer. In view of the known characteristics of macaque bipolar cells and of the ON/OFF asymmetry, it is proposed that these ganglion cells are postsynaptic to cone-specific flat bipolars possibly mediating sign-inverting synaptic contacts. The results also indicate a reversal, for the blue-cone pathway, of the ON/OFF lamination of the inner plexiform layer that has recently been described in other species.     

Mechanism of action of tubocurarine on nicotinic cholinoreceptors of neurons of the sympathetic ganglia of rats

Tubocurarine (Tc) effect on membrane currents elicited by acetylcholine (ACh) was studied in isolated superior cervical ganglion neurons of rat using patch-clamp method in the whole-cell recording mode. The "use-dependent" block of ACh current by Tc was revealed in the experiments with ACh applications, indicating that Tc blocked the channels opened by ACh. Mean lifetime of Tc-open channel complex, tau, was found to be 9.8 +/- 0.5 s (n = 7) at -50 mV and 20-24 degrees C. tau exponentially increased with membrane hyperpolarization (e-fold change in tau corresponded to the membrane potential shift by 61 mV). Inhibition of the ACh-induced current by Tc (3-30 microM/1) was completely abolished by membrane depolarization to the level of 80-100 mV. Inhibition of ACh-induced current was augmented at increased ACh doses. It is concluded that the open channel block produced by Tc is likely to be the only mechanism for Tc action on nicotinic acetylcholine receptors in superior cervical ganglion neurons of rat.