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胶质纤维酸性蛋白(GFAP)抗体在病因不明的局灶性发作患儿中的应用。

Glial fibrillary acidic protein (GFAP)-antibody in children with focal seizures of undetermined cause.

机构信息

Department of Speech and Language Therapy, Faculty of Health Science, Biruni University, İstanbul, Turkey.

Tzartos NeuroDiagnostics, Athens, Greece.

出版信息

Acta Neurol Belg. 2021 Oct;121(5):1275-1280. doi: 10.1007/s13760-020-01361-y. Epub 2020 Apr 24.

Abstract

Anti-neuronal antibodies that are related with autoimmune encephalitis syndromes may also be found in children with new onset seizures or chronic epilepsy. To unravel the significance of autoimmune astrocytopathy in epilepsy, we investigated serum antibody to glial fibrillary acidic protein (GFAP), another autoantigen described in autoimmune encephalitis with seizures, in 38 children with focal seizures of undetermined cause. GFAP antibody was screened with cell based assay and indirect immunohistochemistry and was found in two boys with normal brain MRI and unrevealing medical history prior to seizures. The 2-year-old boy had chronic treatment-resistant frontal lobe epilepsy. The 2.5-year-old boy had a single episode of focal seizures and remained seizure free thereafter in a follow-up period of 4 years. Nevertheless, he showed severe cognitive and language impairment. These results suggest that autoimmune astrocytopathy may be present in some epilepsy patients. Whether this immune response is a bystander effect generated by seizure-induced astrocytosis or directly involved in epileptogenesis needs to be further studied.

摘要

与自身免疫性脑炎综合征相关的抗神经元抗体也可能存在于新发癫痫发作或慢性癫痫的儿童中。为了解癫痫中自身免疫性星形胶质细胞病的意义,我们研究了 38 例病因不明局灶性癫痫发作儿童的血清抗神经胶质纤维酸性蛋白(GFAP)抗体,GFAP 是另一种在伴癫痫发作的自身免疫性脑炎中描述的自身抗原。使用基于细胞的检测和间接免疫组织化学法筛选 GFAP 抗体,在两名脑 MRI 正常且癫痫发作前无明显病史的男孩中发现了该抗体。2 岁的男孩患有慢性耐药性额叶癫痫。2.5 岁的男孩发作了一次局灶性癫痫发作,此后在 4 年的随访期间无癫痫发作,但他表现出严重的认知和语言障碍。这些结果表明,自身免疫性星形胶质细胞病可能存在于一些癫痫患者中。这种免疫反应是由癫痫发作引起的星形胶质细胞增生引起的旁观者效应,还是直接参与癫痫发生,需要进一步研究。

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