Division of Pediatric Neurology, Department of Neurology, University of Virginia, 1221 Lee St., Charlottesville, VA, USA.
Department of Neurology, University of Virginia, Charlottesville, VA, USA.
Mult Scler Relat Disord. 2018 Nov;26:121-123. doi: 10.1016/j.msard.2018.09.015. Epub 2018 Sep 13.
Autoimmune encephalitis has been increasingly recognized within the pediatric population, and the number of implicated autoantibodies continues to grow. The identification of characteristic clinical and paraclinical features helps direct the evaluation and increases the likelihood of making a definitive diagnosis of a specific antibody-mediated encephalitis. The finding of extreme delta brush on electroencephalogram (EEG) has been suggested to serve as a clinical clue to the diagnosis of anti-NMDA-R encephalitis. Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently described antibody-mediated meningoencephalomyelitis, reported almost exclusively in adult patients. We report a case of autoimmune GFAP astrocytopathy in a pediatric patient with extreme delta brush pattern on EEG, negative anti-NMDA-R antibodies, and distinctive MRI findings. The findings reported herein should prompt clinicians to consider the diagnosis of autoimmune GFAP astrocytopathy in patients with suspected autoimmune encephalitis.
自身免疫性脑炎在儿科人群中越来越受到重视，涉及的自身抗体数量不断增加。特征性的临床和辅助检查特征有助于指导评估，并增加明确诊断特定抗体介导性脑炎的可能性。脑电图（EEG）上出现极度 delta 刷波被认为是抗 NMDA-R 脑炎诊断的临床线索。自身免疫性神经胶质纤维酸性蛋白（GFAP）星形胶质细胞病是一种最近描述的抗体介导性脑膜脑炎，几乎仅在成年患者中报道。我们报告了一例儿科患者的自身免疫性 GFAP 星形胶质细胞病，其脑电图上出现极度 delta 刷波模式、抗 NMDA-R 抗体阴性和独特的 MRI 发现。本文报道的这些发现应促使临床医生在疑似自身免疫性脑炎的患者中考虑自身免疫性 GFAP 星形胶质细胞病的诊断。
