anti-NMDAR autoimmune encephalitis

  • 文章类型: Journal Article
    这项研究的目的是阐明脑脊液(CSF)抗体滴度(AT)和性别对诊断为抗N-甲基-d-天冬氨酸受体自身免疫性脑炎(NMDARAE)的患者的急性脑血流量(CBF)的贡献。
    从2016年12月至2023年1月招募了45例诊断为NMDARAE的患者。使用动脉自旋标记分析了疾病早期NMDARAE患者的急性CBF。根据CSFAT和性别对各组进行比较。还在组间比较了感兴趣区域中CBF的连通性。
    与健康受试者相比,具有不同CSFAT的患者表现出CBF异常的不同脑区(p=0.001,簇水平FWE校正)。CSF中的高抗体滴度(HAT)比CSF中的低抗体滴度(LAT)的女性患者的CBF改变更多的大脑区域(p=0.001,簇水平FWE校正)。女性脑脊液HAT患者左扣带后回CBF降低,左前叶,左边的calcarine,左侧中扣带回高于CSF中具有相同AT的男性患者(p=0.001,簇水平FWE校正)。所有NMDARAE患者均显示左壳核(Putamen_L)和左杏仁核(Amygdala_L)CBF升高,右前叶(Precuneus_R)CBF降低,这表明这些是NMDARAE的诊断CBF标志物。
    CSFAT和性别导致诊断为NMDARAE的患者的CBF异常。改变的CBF可能作为NMDARAE的诊断标志物。
    The objective of this study was to elucidate the contribution of cerebrospinal fluid (CSF) antibody titers (AT) and sex to acute cerebral blood flow (CBF) in patients diagnosed with anti-N-methyl-d-aspartate receptor autoimmune encephalitis (NMDAR AE).
    Forty-five patients diagnosed with NMDAR AE were recruited from December 2016 to January 2023. The acute CBF in patients with NMDAR AE at the early stage of the disease was analyzed using arterial spin labeling. The groups were compared based on CSF AT and sex. The connectivity of the CBF in the region of interest was also compared between groups.
    The patients with different CSF AT exhibited varied brain regions with CBF abnormalities compared to the healthy subjects (p = 0.001, cluster-level FWE corrected). High antibody titers (HAT) in CSF contributed to more brain regions with CBF alterations in female patients than in female patients with low antibody titers (LAT) in CSF (p = 0.001, cluster-level FWE corrected). Female patients with HAT in CSF displayed more decreased CBF in the left post cingulum gyrus, left precuneus, left calcarine, and left middle cingulum gyrus than the male patients with the same AT in CSF (p = 0.001, cluster-level FWE corrected). All patients with NMDAR AE showed increased CBF in the left putamen (Putamen_L) and left amygdala (Amygdala_L) and decreased CBF in the right precuneus (Precuneus_R), which suggests that these are diagnostic CBF markers for NMDAR AE.
    CSF AT and sex contributed to CBF abnormalities in the patients diagnosed with NMDAR AE. Altered CBF might potentially serve as the diagnostic marker for NMDAR AE.
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  • 文章类型: Review
    原理:抗N-甲基-d-天冬氨酸受体(NMDAR)脑炎是一种自身免疫性突触性脑炎,通常由神经元表面抗体介导。临床上,它表现为各种各样的神经和精神症状,主要影响患有卵巢畸胎瘤的年轻女性,这在孕妇中很少见。患者的担忧:我们报告了一例35岁的多胎妊娠患者在妊娠38周时因癫痫发作而被送往急诊室,精神症状,如神志不清的言语和神秘的视觉和听觉幻觉,Bradylalia,逆行性健忘症.诊断:结合腰椎穿刺结果,判断自身免疫性脑炎的诊断为抗NMDA抗体。脑成像,和病人的持续症状。结果:这种情况因其罕见性和症状的广泛性而值得注意。妊娠38周时,病人做了剖腹产,在6个月的随访中观察到良好的产妇恢复和良好的新生儿适应。教训:我们的目标包括提高对这种情况的认识,并强调早期诊断的重要性。这种脑炎是可以治疗的,并且可能是可逆的,强调及时识别的重要性。
    Rationale: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a form of autoimmune synaptic encephalitis, often mediated by neuronal surface antibodies. Clinically, it manifests through a diverse range of neurological and psychiatric symptoms, primarily affecting young women with ovarian teratoma, which is rare in pregnant women. Patient concerns: We report a case of a 35-year-old multiparous pregnant patient at 38 weeks of gestation presented to the emergency room with seizure, psychiatric symptoms like delirious speech with mystical visual and auditory hallucinations, bradylalia, and retrograde amnesia. Diagnosis: The diagnosis of autoimmune encephalitis with anti-NMDA antibodies was concluded by considering the lumbar puncture results, brain imaging, and the patient\'s persistent symptoms. Outcomes: This case is noteworthy for its rarity and the symptoms\' breadth. At 38 weeks of gestation, the patient underwent a cesarean section, resulting in excellent maternal recovery observed during the 6-month follow-up and good neonatal adaptation. Lessons: Our goals include raising awareness about this condition and emphasizing the significance of early diagnosis. This encephalitis is treatable and potentially reversible, underscoring the importance of prompt identification.
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  • 文章类型: Journal Article
    背景:有关儿童带有抗N-甲基-d-天冬氨酸受体(NMDAR)抗体的自身免疫性脑炎的临床特征和严重程度的信息在儿科研究领域引起了越来越多的关注。方法:在这项回顾性队列研究中,所有病例(n=67)均来自三级儿童医院,从2017年到2020年。我们将接受重症监护病房(ICU)护理的重症病例与未接受ICU护理的非重症病例进行了比较,并使用机器学习算法来预测儿童的严重程度。以及使用免疫和病毒核酸测试来确定可能的致病触发因素。结果:儿童平均年龄为8.29(标准差4.09)岁,女孩41人(61.19%)。11人(16.42%)入住ICU,神经科病房收治56人(83.58%)。10个个体参数在重症病例和非重症病例之间具有统计学意义(P<0.05)。包括头痛,异常的精神行为或认知障碍,癫痫发作,伴随肿瘤,痰/血液病原体,血球蛋白,血尿素氮,血液免疫球蛋白G,血液免疫球蛋白M,脑脊液中多核细胞的数量。随机森林回归模型对严重程度的总体预测能力达到0.806,其中脑脊液中多核细胞的数量贡献最大。潜在致病原因表现为支原体比例最高,其次是爱泼斯坦-巴尔病毒。结论:我们的发现为早期识别儿童自身免疫性脑炎提供了证据,特别是在严重的情况下。
    Background: Information on the clinical characteristics and severity of autoimmune encephalitis with antibodies against the N-methyl-d-aspartate receptor (NMDAR) in children is attracting more and more attention in the field of pediatric research. Methods: In this retrospective cohort study, all cases (n = 67) were enrolled from a tertiary children\'s hospital, from 2017 to 2020. We compared severe cases that received intensive care unit (ICU) care with nonsevere cases that did not receive ICU care and used machine learning algorithm to predict the severity of children, as well as using immunologic and viral nucleic acid tests to identify possible pathogenic triggers. Results: Mean age of children was 8.29 (standard deviation 4.09) years, and 41 (61.19%) were girls. Eleven (16.42%) were admitted to the ICU, and 56 (83.58%) were admitted to neurology ward. Ten individual parameters were statistically significant differences between severe cases and nonsevere cases (P < .05), including headache, abnormal mental behavior or cognitive impairment, seizures, concomitant tumors, sputum/blood pathogens, blood globulin, blood urea nitrogen, blood immunoglobulin G, blood immunoglobulin M, and number of polynucleated cells in cerebrospinal fluid. Random forest regression model presented that the overall prediction power of severity reached 0.806, among which the number of polynucleated cells in cerebrospinal fluid contributed the most. Potential pathogenic causes exhibited that the proportion of mycoplasma was the highest, followed by Epstein-Barr virus. Conclusion: Our findings provided evidence for early identification of autoimmune encephalitis in children, especially in severe cases.
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