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从病例看“自身免疫（相关）性癫痫” 在临床诊治中的挑战

通过回顾性分析3例代表性临床病例的诊断、治疗及后期随访资料，以揭示目前有关“自身免疫（相关）性癫痫”在诊断和治疗方面存在的挑战和问题，并通过文献复习来探讨合理的应对策略。3例患者中，2例因反复癫痫发作就诊，在临床未明显提示免疫病因的情况下，多次送检相关抗体或启动免疫治疗。另1例患者临床除了癫痫发作，还有其他脑病表现，结合病史和影像所见高度提示免疫病因，最终经抗体检测阳性结果证实。3例患者的诊治经过提示，目前对“自身免疫（相关）性癫痫”诊断和治疗存在一定程度“过度化”情况。“自身免疫”和“癫痫”的关系较为复杂。自身免疫性脑炎中的癫痫发作和慢性自身免疫（相关）性癫痫之间的界限仍不清晰，缺乏可用于实际操作的标准（如生物标记物）是造成后者混乱临床诊疗现状的重要原因。现阶段，理清诸如“急性症状性发作”和“癫痫”等基本概念，仔细全面评估患者，尽早识别出自身免疫性脑炎中已经确定的、具有一定表型特征的综合征，以及使用可指导临床送检抗体或启动免疫治疗的相关评测量表，可帮助临床医生更加合理、有效地诊疗。

桥本脑病临床分析一例

Value of Superparamagnetic Iron Oxide in the Experimental Autoimmnune Encephalomyelitis Model

【摘要】 目的 在实验性自身免疫性脑脊髓炎(experimental autoimmune encephalomyelitis,EAE)模型中,比较常规T2加权成像(T2weighted imaging,T2WI)、钆二乙三胺五醋酸(gadoliniumdiethylenetriamine pentaacetic acid,GdDTPA)和超顺磁性氧化铁(superparamagnetic iron oxide,SPIO)增强图像之间的差异,探讨巨噬细胞在多发性硬化(multiple sclerosis,MS)炎性活动病灶中的细胞学标志。方法 在EAE模型临床症状的亚临床期、初发期、高峰期,13只复发缓解(relapsingremitting,RR)EAE大鼠模型组和13只正常对照组大鼠在注入对比剂之前均行常规T2WI扫描,接着分别在其尾静脉注入GdDTPA后5 min行T1加权成像(T1weighted imaging,T1WI),再注入SPIO,24 h后行T2WI扫描。扫描完毕后立即处死大鼠取脑,行脑组织切片的ED1免疫组织化学染色和Prussian blue染色。结果 EAE模型组大鼠在第11天出现临床症状(初发期),第14天达到高峰期;MRI检查:SPIO增强图像对EAE病灶的显示较常规T2WI和GdDTPA增强图像好。病理学检查:ED1染色,在SPIO显示为低信号的区域内出现了炎症细胞(以巨噬细胞为主)浸润;Prussian blue染色示病灶内巨噬细胞胞质内出现了蓝染颗粒,沉积部位与T2WI上低信号区对应。对照组大鼠均无异常。结论 SPIO较GdDTPA更好地显示EAE模型中炎性活动性病灶内血管周围以巨噬细胞为主的浸润。

自身免疫性肝炎/原发性胆汁性肝硬化重叠综合征误诊为药物性肝炎一例

【摘要】　目的　提高对自身免疫性肝病的认识。　方法　报道一例自身免疫性肝炎/原发性胆汁性肝硬化重叠综合征误诊为药物性肝炎，并综述相关文献总结其临床表现、诊断、治疗及预后。　结果　自身免疫性肝病是一组以肝脏病理损害和肝功能异常为主要表现的自身免疫性疾病，可分为自身免疫性肝炎（autoinmmune hepatitis，AIH）和（primary biliary cirrhosis，PBC）及原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）。由于无特异性的临床表现，容易漏诊或误诊为药物性肝炎，诊断主要依靠生化及免疫学检查。AIH/PBC重叠综合征的治疗目前无成熟经验，主要药物包括熊去氧胆酸（UDCA）、糖皮质激素和免疫抑制剂等。　结论　对于不明原因的肝损害患者应做免疫学筛查。

Association between Interleukin-23 Receptor Gene Polymorphisms and Dilated Cardiomyopathy

目的:本文通过研究白介素23受体(IL-23R)基因多态性与扩张型心肌病(DCM)的相关性,探讨DCM患者的免疫遗传学发病机制. 方法:采用PCR-RFLP方法测定DCM患者和正常对照者IL-23R基因rs7517847位点的单核苷酸多态性. 用卡方检验比较病例组与对照组之间基因型频率和等位基因频率的统计学差异。结果:IL-23R基因rs7517847位点单核苷酸多态基因型和等位基因频率在DCM组与正常对照组之间无差异。结论:本研究未发现IL-23R基因rs7517847位点多态性与DCM相关。

癫痫的免疫性病因研究进展

国际抗癫痫联盟（ILAE）于 2017 年提出了癫痫的六大病因，其中免疫性病因是目前研究的热点。系统性自身免疫性疾病、神经系统自身免疫性疾病、自身免疫性脑炎（Autoimmune encephalitis，AE）与癫痫的关系均十分密切。其中，AE 与癫痫的关系尤为复杂。从癫痫的概念及AE的特点出发，ILAE 提出了“继发于自身免疫性脑炎的急性症状性发作”及“自身免疫相关癫痫”这两个概念，可以更好地从发病机制及临床特点来认识 AE 与癫痫的关系，为后续的研究理清了思路。

Research progress on immune-related vestibular dysfunction

Vestibular dysfunction is a clinical syndrome characterized by symptoms such as dizziness or vertigo, abnormal control of eye movements, balance disorders, and autonomic nervous system symptoms. Immune-related vestibular dysfunction is caused by vestibular abnormalities triggered by autoimmune reactions or dysfunctions in the immune system. Some autoimmune diseases can present with vestibular dysfunction as the initial symptom or a typical manifestation. There is still a lack of understanding regarding the immune pathogenic factors of vestibular abnormalities both domestically and internationally. This paper provides a detailed summary of the types, onset, pathological mechanisms, symptoms, signs, and auxiliary examinations of immune-related vestibular dysfunction, aiming to offer new insights for the identification of such diseases.

脑电图检查对自身免疫性脑炎的诊断进展

对近年自身免疫性脑炎病例研究中脑电图（EEG）的特点进行综述。EEG 对于自身免疫性脑炎的诊断有着高于其他检查的敏感性，常见的异常表现为弥漫性或局灶慢波改变，且近年发现极度 δ 刷为抗 N-甲基-D-天门冬氨酸受体脑炎相对特异性 EEG 改变，有助于对自身免疫性脑炎的诊断。但其他自身免疫性脑炎的 EEG 总结资料相对较少，应进一步进行总结研究。

Clinical characteristics of autoimmune encephalitis in common antibody types and epileptic seizures

Patients with autoimmune encephalitis are mainly characterized by behavioral, mental and motor abnormalities, neurological dysfunction, memory deficits and seizures. Different antibody types of autoimmune encephalitis its pathogenesis, clinical characteristics are different, in recent years found immune related epilepsy is closely related to autoimmune encephalitis, based on autoimmune encephalitis type is more, we choose more common autoimmune encephalitis, expounds its characteristics, to help clinical diagnosis.

The beneficial effect of Bacteroides Fragilis (BF839) as a supplementary treatment in drug-resistant epilepsy: a pilot study

ObjectiveThe purpose of this study was to find a new method for the treatment of drug-resistant epilepsy, and to study the efficacy and safety of Bacteroidesfragilis (BF839) in the adjunctive treatment of refractory epilepsy, as well as the improvement of comorbidity.MethodsA prospective, single-arm, open pilot clinical study was designed for the additive treatment of drug-resistant epilepsy using BacteroidesFragilis 839 (BF839). 47 patients with refractory epilepsy, who were admitted to the epilepsy outpatient clinic of the Second Affiliated Hospital of Guangzhou Medical University from April 2019 to October 2019, were enrolled and treated with BF839 adjunct treatment. The primary efficacy endpoint was median percent reduction from baseline in monthly (28-day) seizure frequency for the 16-week treatment period. Other efficacy analysis included response rate(proportion of patients with ≥ 50% seizure reduction) in the 16 weeks period, the proportion of patients seizure free and the retention rate after12 months intervention, and the observance of the side effects and comorbidities.ResultsThe median reduction percent of all seizure types was −53.5% (P=0.002). The response rate was 61.1% (22/36). 8.5% (4/47) patients seizure free at 12 months. The retention rate at 12 months was 57.4% (27/47). The side effects were diarrhea 4.3% (2/47) and constipation 4.3% (2/47). 48.9% (23/47) of the patients reported improvement in comorbidities, with cognitive improvement of 21.2% (10/47).ConclusionBF839 can be used as an effective additive therapy to treat drug-resistant epilepsy. It is safe and beneficial to the improvement of comorbidities. This is the first time in the world that a single intestinal strain has been reported to be effective in treating drug-resistant epilepsy. This research has important implications.