Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
BMC Ophthalmol. 2023 Apr 26;23(1):184. doi: 10.1186/s12886-023-02927-z.
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) has been reported as a spectrum of autoimmune, inflammatory central nervous system disorders. Linear perivascular radial gadolinium enhancement patterns on brain magnetic resonance imaging (MRI) are a hallmark of these disorders. GFAP-A is associated with cerebrospinal fluid (CSF) GFAP antibody (GFAP-Ab), while the association with serum GFAP-Ab is less clear. This study aimed to observe the clinical characteristic and MRI changes of GFAP-Ab-positive optic neuritis (ON).
We performed a retrospective, observational case study at the department of neurology, Beijing Tongren Hospital, from December 2020 to December 2021. The serum of 43 patients and CSF samples of 38 patients with ON were tested for GFAP-Ab by cell-based indirect immune-fluorescence test.
Four patients (9.3%) were detected GFAP-Ab positive, and in three out of the four patients, GFAP-Abs were detected only in serum. All of them demonstrated unilateral optic neuritis. Three patients (1, 2, and 4) experienced severe visual loss (best corrected visual acuity ≤ 0.1). Two patients (2 and 4) had experienced more than one episode of ON at the time of sampling. MRI showed optic nerve hyperintensity on T2 FLAIR images in all GFAP-Ab positive patients, and orbital section involvement was the most common. During follow-up (mean 4.5 ± 1 months), only Patient 1 had a recurrent ON, and no patient developed new other neurological events or systemic symptoms.
GFAP-Ab is rare in patients with ON and may manifest as isolated, relapsing ON. This supports the notion that the GFAP-A spectrum should comprise isolated ON.
自身免疫性胶质纤维酸性蛋白（GFAP）星形胶质细胞病（GFAP-A）已被报道为一系列自身免疫性、炎症性中枢神经系统疾病。脑磁共振成像（MRI）上线性血管周围放射状钆增强模式是这些疾病的标志。GFAP-A 与脑脊液（CSF）GFAP 抗体（GFAP-Ab）相关，而与血清 GFAP-Ab 的相关性尚不清楚。本研究旨在观察 GFAP-Ab 阳性视神经炎（ON）的临床特征和 MRI 变化。
我们在北京同仁医院神经内科进行了一项回顾性、观察性病例研究，时间为 2020 年 12 月至 2021 年 12 月。采用细胞间接免疫荧光法检测 43 例 ON 患者血清和 38 例 CSF 样本中的 GFAP-Ab。
4 例（9.3%）患者 GFAP-Ab 阳性，其中 3 例仅在血清中检测到 GFAP-Abs。所有患者均表现为单侧视神经炎。3 例（1、2 和 4 号患者）出现严重视力丧失（最佳矫正视力≤0.1）。2 例（2 和 4 号患者）在取样时经历了不止一次 ON 发作。所有 GFAP-Ab 阳性患者的 MRI 显示 T2 FLAIR 图像上视神经高信号，最常见的是眶段受累。在随访期间（平均 4.5±1 个月），只有 1 号患者出现复发性 ON，没有患者出现新的其他神经系统事件或全身症状。
ON 患者中 GFAP-Ab 罕见，可能表现为孤立性、复发性 ON。这支持 GFAP-A 谱应包括孤立性 ON 的观点。
