Background: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is a recently defined autoimmune inflammatory disease of the central nervous system in which GFAP IgG is present in the cerebrospinal fluid (CSF). Its primary clinical manifestation is meningoencephalitis, and it usually responds well to corticosteroids. Herein, we report a case of a patient with GFAP-A with initial symptoms of psychological and cognitive impairment, which did not respond to high-dose methylprednisolone therapy but was successfully treated with protein A immunoadsorption (PAIA) therapy. Methods: GFAP IgG was detected by indirect immunofluorescence assay. The patient’s data were analyzed retrospectively. Results: A 48-year-old man presented with anxiety, depression, cognitive decline, tremor, gait disturbance, and fecal and urine incontinence. Autoimmune GFAP-A was diagnosed based on the following: (1) T2-weighted and fluid-attenuated inversion recovery MRI findings of hypersensitive lesions in the subcortical and deep white matter of the brain, with multiple longitudinally extensive lesions in the cervical and chest regions of the spinal cord, and (2) high levels of GFAP IgG in the CSF. Clinical symptoms and abnormalities detected on neuroimaging worsened after administration of high-dose intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG) but improved significantly after PAIA therapy. Conclusion: Psychological impairment can be the first sign of autoimmune GFAP-A. PAIA might be an effective treatment for patients with GFAP-A who respond poorly to conventional IVMP and IVIG therapy.
Neuroimmunomodulation

中文翻译：

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甲泼尼龙治疗失败后，蛋白 A 免疫吸附可缓解自身免疫性胶质纤维酸性蛋白星形细胞病

背景：自身免疫性胶质纤维酸性蛋白 (GFAP) 星形细胞病 (GFAP-A) 是一种最近定义的中枢神经系统自身免疫性炎症性疾病，其中 GFAP IgG 存在于脑脊液 (CSF) 中。其主要临床表现是脑膜脑炎，通常对皮质类固醇反应良好。在此，我们报告了一例具有心理和认知障碍初始症状的 GFAP-A 患者，该患者对大剂量甲基强的松龙治疗没有反应，但通过蛋白 A 免疫吸附 (PAIA) 治疗成功治疗。方法：采用间接免疫荧光法检测GFAP IgG。对患者资料进行回顾性分析。结果：一名 48 岁男性因焦虑、抑郁、认知能力下降、震颤、步态障碍以及大便失禁就诊。自身免疫性 GFAP-A 的诊断依据如下： (1) T2 加权和液体衰减反转恢复 MRI 发现大脑皮层下和深部白质中的过敏病变，颈部和胸部区域有多个纵向广泛病变(2) CSF 中高水平的 GFAP IgG。大剂量静脉注射甲基强的松龙 (IVMP) 和静脉注射免疫球蛋白 (IVIG) 后，神经影像学检测到的临床症状和异常恶化，但在 PAIA 治疗后显着改善。结论：心理障碍可能是自身免疫性 GFAP-A 的第一个迹象。对于对常规 IVMP 和 IVIG 治疗反应不佳的 GFAP-A 患者，PAIA 可能是一种有效的治疗方法。
神经免疫调节