Guo Ya, Guo Jiamin, Wang Xueyu, Ma Aihua, Gao Yuxing, Chen Jiacheng, Nie Cuili, Chen Na
Department of Pediatric, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, 250021, China.
Heliyon. 2024 Feb 22;10(5):e26827. doi: 10.1016/j.heliyon.2024.e26827. eCollection 2024 Mar 15.
Glial fibrillary acidic protein (GFAP) astrocytopathy is a novel autoimmune neurological disorder and is diagnosed by GFAP-IgG in cerebrospinal fluid (CSF) measurement.
Herein, we described a 10-year-old boy with abnormal neurological symptoms and signs. GFAP-IgG was detected in CSF using cell-based assay (CBA), and his CSF showed an increase in lymphocytes, a slight decrease in glucose and an increase in protein level in the early stage. The cranial MRI showed multiple strips of T2-FLAIR hyperintense signal changes on the surface of medulla oblongata, pons, and gyrus in bilateral cerebral hemispheres. He was treated with immunoglobulin (IVIG) and high-dose methylprednisolone pulse treatment, and his clinical presentations gradually improved.
We highlight that patients with normal inflammatory markers in peripheral blood have obvious meningitis-like symptoms, and clinicians need to consider GFAP astrocytopathy. The early diagnosis and treatment of GFAP astrocytopathy are important for improving the prognosis.
胶质纤维酸性蛋白(GFAP)星形细胞病是一种新型自身免疫性神经疾病,通过检测脑脊液(CSF)中的GFAP-IgG进行诊断。
在此,我们描述了一名10岁有异常神经症状和体征的男孩。采用基于细胞的检测方法(CBA)在脑脊液中检测到GFAP-IgG,其脑脊液在早期显示淋巴细胞增多、葡萄糖略有降低和蛋白质水平升高。头颅磁共振成像(MRI)显示双侧大脑半球延髓、脑桥表面及脑回有多发条片状T2-FLAIR高信号改变。给予其静脉注射免疫球蛋白(IVIG)和大剂量甲泼尼龙冲击治疗,其临床表现逐渐改善。
我们强调外周血炎症指标正常但有明显脑膜炎样症状的患者,临床医生需考虑GFAP星形细胞病。GFAP星形细胞病的早期诊断和治疗对改善预后很重要。