Abstract

Aim

Idiopathic intracranial hypertension (IIH), a disease of obscure origin, is characterized by headache and visual disturbances due to increased intracranial pressure. Recent line of evidence has suggested involvement of inflammation in IIH pathogenesis thus bringing forward anti-glial autoimmunity as a potential contributor of IIH. Glial fibrillary acidic protein (GFAP) is a major astrocytic autoantigen associated with a specific form of meningoencephalitis.

Materials and methods

In this study, we investigated the presence of GFAP-antibody in 65 sera (49 obtained during active disease and 16 during remission) and in 15 cerebrospinal fluid (CSF) samples of 58 consecutively recruited IIH patients using cell based assay and indirect immunohistochemistry.

Results

GFAP-antibody was found in active period sera of 2 IIH patients with classical symptoms and good treatment response. Two remission period sera obtained at different time points from one of these cases showed lower titers of GFAP-antibody positivity. IgG from positive samples yielded an astrocytic immunoreactivity pattern. None of the CSF samples showed GFAP-antibodies.

Conclusions

These results suggest that anti-astrocyte autoimmunity might be present in a fraction of IIH patients. Exact pathogenic significance of this association needs to be further studied.

中文翻译：

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血清胶质纤维酸性蛋白（GFAP）-抗体在特发性颅内高压中的作用

摘要

目的

特发性颅内高压 (IIH) 是一种起源不明的疾病，其特征是颅内压升高引起的头痛和视力障碍。最近的证据表明炎症参与 IIH 发病机制，从而提出抗神经胶质自身免疫作为 IIH 的潜在贡献者。胶质纤维酸性蛋白 (GFAP) 是一种主要的星形细胞自身抗原，与特定形式的脑膜脑炎相关。

材料和方法

在这项研究中，我们使用基于细胞的测定和间接免疫组织化学研究了 58 名连续招募的 IIH 患者的 65 份血清（49 份在活动性疾病期间获得，16 份在缓解期间获得）和 15 份脑脊液 (CSF) 样本中 GFAP 抗体的存在。

结果

2例IIH患者活动期血清中发现GFAP抗体，症状典型，治疗反应良好。从这些病例之一的不同时间点获得的两种缓解期血清显示出较低的 GFAP 抗体阳性滴度。来自阳性样品的 IgG 产生星形细胞免疫反应模式。没有一个 CSF 样品显示出 GFAP 抗体。

结论

这些结果表明，部分 IIH 患者可能存在抗星形胶质细胞自身免疫。这种关联的确切致病意义需要进一步研究。