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[急性脑炎:抗离子型谷氨酸受体激活的N-甲基-D-天冬氨酸受体(NMDAR):阿根廷11例儿科病例分析(贝尼托·耶林奖)]

[Acute encephalitis anti-ionotropic glutamate receptor activated N-methyl-D-aspartate (NMDAR): analysis of eleven pediatric cases in Argentina (Benito Yelín Award)].

作者信息

Pérez Elisa, Ruggieri Víctor, Monges Soledad, Loos Mariana, Caraballo Roberto, Yerga Andrés, Rugilo Carlos, Vincent Angela, Arroyo Hugo A

机构信息

Servicio de Neurología, Hospital de Pediatría Prof. Dr. J. P. Garrahan, Buenos Aires, Argentina.

出版信息

Medicina (B Aires). 2013;73 Suppl 1:1-9.

Abstract

Encephalitis are an inflammatory processes of various origin, among which include autoimmune origin. The identification of antibodies against the N-methyl-D- aspartate, allowed clinical immunological characterization of an entity susceptible to immunomodulatory therapy. Originally described in young women associated with ovarian teratoma, is now a recognized entity in children even in the absence of detectable tumors. The aim of the study was conducted through review of medical records, was to describe the clinical, developmental and findings in further studies of eleven children with confirmed diagnosis of this entity through identification of specific antibodies. All debuted with psychiatric symptoms in nine associating seizures, and two extrapyramidal movements. In the evolution of language all had commitment nine severe autonomic symptoms, one with hypoventilation and requirements of ARM. Brain MRI was abnormal in three. Eight had voltage EEG asymmetry and / or amplitude, three of them had spikes. Six had CSF pleocytosis and three of seven positive oligoclonal bands. Five IgM serology for mycoplasma were positive. CPK increase occurred in conjunction with antisychotics in five. With immunomodulatory treatment, five had complete recovery three behavioral disorders / cognitive deficits and one severe. A patient's clinical picture resolved without treatment. In any associated tumor was detected. We conclude that in front of a child with acute encephalopathy and clinical support this entity after infectious cause were ruled out, immunomodulatory therapy should be started early, avoid the use of antipsychotic drugs and search for possible hidden tumors.

摘要

脑炎是多种病因引起的炎症过程,其中包括自身免疫性病因。抗N-甲基-D-天冬氨酸抗体的鉴定,使一种适合免疫调节治疗的疾病得以进行临床免疫学特征描述。该疾病最初在与卵巢畸胎瘤相关的年轻女性中被描述,现在即使在未检测到肿瘤的儿童中也是一种公认的疾病。本研究旨在通过查阅病历,描述11例经特异性抗体鉴定确诊为此病的儿童在进一步研究中的临床、发育及相关发现。所有患儿均以精神症状起病,9例伴有癫痫发作,2例出现锥体外系运动症状。在语言发育过程中,所有患儿均出现9种严重的自主神经症状,1例伴有通气不足及需要辅助呼吸。3例脑部MRI异常。8例脑电图有电压不对称和/或波幅异常,其中3例有棘波。6例脑脊液有细胞增多,7例中有3例寡克隆带阳性。5例支原体IgM血清学阳性。5例在使用抗精神病药物时出现肌酸磷酸激酶升高。经免疫调节治疗后,5例完全康复,3例有行为障碍/认知缺陷,1例严重。1例患者未经治疗临床症状缓解。未检测到任何相关肿瘤。我们得出结论,对于一名患有急性脑病且在排除感染性病因后有临床支持此疾病的儿童,应尽早开始免疫调节治疗,避免使用抗精神病药物,并寻找可能隐藏的肿瘤。

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