脑的磁共振成像（MRI）中出现的放射状增强模式已被确认为自身免疫性胶质纤维酸性蛋白星形细胞病（GFAP-A）的典型病变。然而，作者遇到了一些没有GFAP-IgG的患者，这些患者并没有显示出这种特定的成像。在本研究中，我们报道了5例GFAP-IgG阴性患者的GFAP-A特定成像模式的临床表现。数据是从2013年6月至2023年4月进行的回顾性获取的，共招募了5名有效数据的GFAP-IgG阴性患者。临床信息要么由研究人员获取，要么从转诊医生处检索，并包括前驱症状、神经表现、合并症、辅助研究结果。共有5名GFAP-IgG阴性患者表现为"脑脑膜炎/脑炎"病症和脑放射状血管周围增强。其中一名患者患有外周淋巴瘤，四名患者血清和/或脑脊液中有其他自身免疫抗体，其中一名患者具有阳性的水通道IgG。这五名患者的临床特点包括头痛、发热、癫痫和异常行为症状。患者的MRI显示自发性GFAP-A的从侧脑室到白质的放射状血管周围钆增强。在GFAP-IgG阴性患者中，具有放射状血管周围增强的GFAP-A样疾病可能存在，无论是否有肿瘤这一发现都可以为GFAP-A的鉴别诊断提供新的见解。版权所有 © 2023 Elsevier B.V. 保留所有权利。

Brain radial enhancement pattern on magnetic resonance imaging (MRI) has been identified as typical lesions in autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). However, the authors encountered several patients without GFAP-IgG showing that such specific imaging. In the present study, we reported the clinical pictures of 5 GFAP-IgG-negative patients with GFAP-A specific imaging pattern.Data was retrospectively obtained from June 2013 through April 2023, and five GFAP-IgG-negative patients with valid data were recruited. Clinical information was either obtained by the investigators or retrieved from the referring clinicians and included prodromal symptoms, neurologic manifestations, comorbidities, results of ancillary studies.Altogether five GFAP-IgG-negative patients with "meningoencephalitis/encephalitis" manifestations and brain radial perivascular enhancement were confirmed. One patient had peripheral lymphoma. Four patients had other autoimmune antibody in serum and/or cerebrospinal fluid, of which one patient had positive aquaporin IgG. Clinical features of the five patients included headache, fever, epilepsy and abnormal behavioral symptoms. MRI of patients revealed radial perivascular gadolinium enhancement extending from the lateral ventricles to the white matter suggestive of autoimmune GFAP-A.GFAP-A-like disorders with radial perivascular enhancement could be found in GFAP-IgG-negative patients with or without neoplasm, which could provide new insight into the differential diagnosis of GFAP-A.Copyright © 2023 Elsevier B.V. All rights reserved.