Glial Fibrillary Acidic Protein Astrocytopathy Based on a Two-Center Chinese Cohort Study

IF 3.9 2区医学 Q1 CLINICAL NEUROLOGY

Annals of Clinical and Translational Neurology Pub Date : 2025-07-10 DOI:10.1002/acn3.70118

Ti Wu, Hao Zhang, Chao Gao, Qiuhua Yu, Moli Fan, Lin-Jie Zhang, Haipeng Zhang, Hengri Cong, Yuzhen Wei, Chotima Böttcher, Alexej Verkhratsky, Friedemann Paul, Fu-Dong Shi, Tian Song

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Abstract

Objective

Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a recently defined nosological form belonging to the class of autoimmune inflammatory disorders affecting the central nervous system (CNS). Here, we report the clinical and MRI characteristics, treatment, and prognosis of a GFAP-A cohort from two centers in China.

Methods

We retrospectively analyzed the data from 38 adult patients with positive GFAP antibodies and diagnosed as GFAP-A between June 2019 and September 2024. Clinical features, semiquantitative antibody test results, MRI features, treatment approaches, and prognosis were collected.

Results

Among the 38 patients, 24 were male, and the median age at disease onset was 49.5 years. The clinical phenotype included encephalomyelitis (28.9%), myelitis (23.7%), encephalitis (18.4%), meningoencephalomyelitis (18.4%), meningitis/spinal meningitis (7.9%), and peripheral neuropathy (2.6%). In enhanced MRI images, 4 (10.5%) of the patients showed enhancement of the cerebral meninges, 2 (5.3%) had enhancement of the ependyma, and 5 (13.2%) had enhancement of the spinal cord pia mater. 77.1% of the patients responded to the glucocorticoid treatment, while 65.8% had a monophasic course. Spearman correlation analysis showed that CSF-specific oligoclonal bands were significantly correlated with 1-year relapse (CI = 0.527, p = 0.003).

Interpretation

The clinical manifestations of GFAP-A are highly diverse, encompassing encephalitis, myelitis, and meningitis, including spinal meningitis. The enhancement of the spinal pia mater and ependyma on MRI was confirmed. Most patients exhibit a positive response to glucocorticoid therapy. The presence of CSF-specific oligoclonal bands could potentially serve as an indicator for predicting recurrence.

Abstract Image

查看原文本刊更多论文

基于中国双中心队列研究的胶质纤维酸性蛋白星形细胞病。

目的：胶质原纤维酸性蛋白星形细胞病（gmap - a）是一种最近被定义的疾病形式，属于影响中枢神经系统（CNS）的自身免疫性炎症疾病。在这里，我们报告了来自中国两个中心的gmap - a队列的临床和MRI特征，治疗和预后。方法：回顾性分析2019年6月至2024年9月期间38例GFAP抗体阳性并诊断为GFAP- a的成年患者的数据。收集临床特征、半定量抗体检测结果、MRI特征、治疗方法及预后。结果：38例患者中，男性24例，中位发病年龄49.5岁。临床表型包括脑脊髓炎（28.9%）、脊髓炎（23.7%）、脑炎（18.4%）、脑膜脑脊髓炎（18.4%）、脑膜炎/脊髓膜炎（7.9%）和周围神经病变（2.6%）。MRI增强影像中，4例（10.5%）患者表现为脑膜强化，2例（5.3%）患者表现为室管膜强化，5例（13.2%）患者表现为脊髓硬膜强化。77.1%的患者对糖皮质激素治疗有反应，65.8%的患者为单相病程。Spearman相关分析显示，csf特异性寡克隆带与1年复发显著相关（CI = 0.527, p = 0.003）。结论：gmap - a的临床表现是高度多样化的，包括脑炎、脊髓炎和脑膜炎，包括脊髓性脑膜炎。MRI显示脊髓硬膜和室管膜增强。大多数患者对糖皮质激素治疗表现出积极的反应。csf特异性寡克隆条带的存在可能作为预测复发的指标。

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来源期刊

Annals of Clinical and Translational Neurology Medicine-Neurology (clinical)

CiteScore

9.10

自引率

1.90%

发文量

218

审稿时长

8 weeks

期刊介绍： Annals of Clinical and Translational Neurology is a peer-reviewed journal for rapid dissemination of high-quality research related to all areas of neurology. The journal publishes original research and scholarly reviews focused on the mechanisms and treatments of diseases of the nervous system; high-impact topics in neurologic education; and other topics of interest to the clinical neuroscience community.