An Qi, Liu Limei
Department of Neurology, The Second Affiliated Hospital of Dalian Medical University, Dalian, 116000, China.
BMC Neurol. 2025 Aug 25;25(1):347. doi: 10.1186/s12883-025-04363-6.
This report provides a comprehensive overview of the clinical manifestations, diagnostic evaluations, treatment, and prognosis of a 36-year-old male patient diagnosed with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in conjunction with Epstein-Barr virus (EBV) infection at our institution. Reports of GFAP-A associated with viral infections are infrequent.
The patient exhibited a range of symptoms, including fever, gait instability resembling ataxia, a sensation akin to stepping on cotton, diminished responsiveness, cognitive decline, urinary and bowel dysfunction, and persistent hiccups. Enhanced imaging of the thoracic spine revealed patchy meningeal enhancement, with central canal-like enhancement observed in coronal views. Additionally, radiating perivascular linear enhancement was noted in the ventricular white matter, cerebellum, and other regions, alongside the aforementioned central canal-like enhancement. Next-generation sequencing (NGS) of cerebrospinal fluid (CSF) confirmed the presence of human herpesvirus type 4 (EBV). Both cell-based assay (CBA) and tissue-based assay (TBA) tests validated the presence of GFAP antibodies in the CSF. Following treatment with acyclovir for antiviral therapy and high-dose corticosteroid therapy, the patient demonstrated significant clinical improvement.
It is postulated that the viral infection may have precipitated autoimmune meningoencephalitis. Providing more related cases for the diagnosis of this disease.
本报告全面概述了我院一名36岁男性患者的临床表现、诊断评估、治疗及预后情况。该患者被诊断为自身免疫性胶质纤维酸性蛋白星形细胞病（GFAP - A）并伴有爱泼斯坦 - 巴尔病毒（EBV）感染。关于GFAP - A与病毒感染相关的报道并不常见。
患者出现了一系列症状，包括发热、类似共济失调的步态不稳、踩棉花感、反应迟钝、认知能力下降、排尿和排便功能障碍以及持续性呃逆。胸椎增强成像显示脑膜斑片状强化，在冠状位可见中央管样强化。此外，在脑室白质、小脑及其他区域除上述中央管样强化外，还可见放射状血管周围线性强化。脑脊液（CSF）的下一代测序（NGS）证实了人类4型疱疹病毒（EBV）的存在。基于细胞的检测（CBA）和基于组织的检测（TBA）均验证了脑脊液中GFAP抗体的存在。经阿昔洛韦抗病毒治疗和大剂量糖皮质激素治疗后，患者临床症状有显著改善。
据推测病毒感染可能引发了自身免疫性脑膜脑炎。为该病的诊断提供了更多相关病例。
