Gklinos Panagiotis, Athanasopoulos Fotios, Giatrakou Vagia, Arkoudis Nikolaos-Achilleas, Pournara Dorothea, Giagkou Eirini, Tountopoulou Argyro, Vassilopoulou Sofia, Mitsikostas Dimos-Dimitrios
First Neurology Department, Eginition University Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece.
Research Unit of Radiology and Medical Imaging, National and Kapodistrian University of Athens, 11528 Athens, Greece.
Antibodies (Basel). 2024 Sep 25;13(4):79. doi: 10.3390/antib13040079.
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, which was first identified in 2016, is an immune-mediated inflammatory disorder of the nervous system characterized by antibodies targeting GFAP. The exact pathogenic mechanisms, as well as the role of anti-GFAP antibodies, remain unclear; however, it seems that neuroinflammation is mediated by specific CD8+ T-cells and that neoplasms or viral infections can act as the initial trigger. Although the clinical spectrum of the disease is broad and heterogenous, GFAP astrocytopathy most commonly presents as meningoencephalitis with or without myelitis. Other symptoms include headache, visual disturbances, extrapyramidal or brainstem syndromes, and psychiatric manifestations including psychosis. The disease has a characteristically favorable response to steroid treatment while relapses occur in approximately 20-30% of the patients.
We present two cases of GFAP astrocytopathy admitted to our hospital: a 43-year-old male with persistent headache and a 59-year-old female with acute dysarthria and swallowing difficulties followed by cognitive and behavioral symptoms.
Additionally, we conduct a comprehensive review of the literature to elucidate the role of anti-GFAP antibodies in disease pathogenesis and examine imaging characteristics, clinical manifestations, and treatment options for this recently described neuroimmunological condition.
This review presents two unusual cases of GFAP-astrocytopathy and provides evidence for the pathogenesis, clinical presentation, imaging characteristics and treatment options of the disease.
自身免疫性胶质纤维酸性蛋白（GFAP）星形细胞病于2016年首次被发现，是一种免疫介导的神经系统炎性疾病，其特征是存在靶向GFAP的抗体。确切的致病机制以及抗GFAP抗体的作用仍不清楚；然而，似乎神经炎症是由特定的CD8 + T细胞介导的，并且肿瘤或病毒感染可能是最初的触发因素。尽管该疾病临床谱广泛且具有异质性，但GFAP星形细胞病最常见的表现是伴或不伴脊髓炎的脑膜脑炎。其他症状包括头痛、视觉障碍、锥体外系或脑干综合征以及包括精神病在内的精神症状。该疾病对类固醇治疗通常有良好反应，约20 - 30%的患者会复发。
我们报告了我院收治的2例GFAP星形细胞病病例：1例43岁男性，有持续性头痛；另1例59岁女性，有急性构音障碍和吞咽困难，随后出现认知和行为症状。
此外，我们对文献进行了全面综述，以阐明抗GFAP抗体在疾病发病机制中的作用，并研究这种最近描述的神经免疫性疾病的影像学特征、临床表现和治疗选择。
本综述介绍了2例不寻常的GFAP星形细胞病病例，并为该疾病的发病机制、临床表现、影像学特征和治疗选择提供了依据。
