Scholarlink

Background <p>Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel autoimmune central nervous system (CNS) disorder. Epstein–Barr virus (EBV) has been implicated in various neurological diseases, yet GFAP-A with concomitant Epstein–Barr virus positivity is poorly understood.</p> Methods <p>We retrospectively analyzed 12 patients with concomitant EBV and GFAP positivity admitted between January 2022 and September 2025, and performed a literature review. Clinical, imaging, laboratory features, treatments, and outcomes were evaluated.</p> Results <p>In our cohort, the median age at onset was 52.5&#xa0;years (58% male). The predominant phenotype was meningo-encephalomyelitis (33%), with headache (100%) and fever (92%) as common symptoms. Peripheral nervous system involvement was noted in 16%. CSF analysis showed pleocytosis (100%) and elevated protein levels. MRI revealed T2/FLAIR hyperintensities in 67%, mainly in white matter and brainstem, and spinal lesions in 73%, including longitudinally extensive transverse myelitis (27%). All received antiviral and immunotherapy, with most showing improvement after intravenous methylprednisolone (± IVIG). At a median 32-month follow-up, only one relapsed, two died, and 80% of survivors achieved good outcomes (mRS &lt; 3). Literature review (32 cases) showed similar features and immunotherapy responsiveness. Overall recurrence and mortality rates were both 7%.</p> Conclusions <p>In this small series, cases with concomitant EBV and GFAP positivity showed overlapping clinical features with classical GFAP-A and generally favorable responses to immunotherapy. Early recognition and CSF-based GFAP antibody testing may help improve patient outcomes; however, further studies are needed to confirm these findings.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Clinical characteristics of autoimmune GFAP astrocytopathy with concomitant Epstein–Barr virus positivity: a case series of 12 patients and literature review

  • 期刊论文

  • 发表日期：

    2026年3月21日

查看全文
  • Zhentang Cao,
  • Haiman Hou,
  • Hao Liu,
  • Bo Li,
  • Xudong Zhang,
  • Lu Zhao

摘要

Background

Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel autoimmune central nervous system (CNS) disorder. Epstein–Barr virus (EBV) has been implicated in various neurological diseases, yet GFAP-A with concomitant Epstein–Barr virus positivity is poorly understood.

Methods

We retrospectively analyzed 12 patients with concomitant EBV and GFAP positivity admitted between January 2022 and September 2025, and performed a literature review. Clinical, imaging, laboratory features, treatments, and outcomes were evaluated.

Results

In our cohort, the median age at onset was 52.5 years (58% male). The predominant phenotype was meningo-encephalomyelitis (33%), with headache (100%) and fever (92%) as common symptoms. Peripheral nervous system involvement was noted in 16%. CSF analysis showed pleocytosis (100%) and elevated protein levels. MRI revealed T2/FLAIR hyperintensities in 67%, mainly in white matter and brainstem, and spinal lesions in 73%, including longitudinally extensive transverse myelitis (27%). All received antiviral and immunotherapy, with most showing improvement after intravenous methylprednisolone (± IVIG). At a median 32-month follow-up, only one relapsed, two died, and 80% of survivors achieved good outcomes (mRS < 3). Literature review (32 cases) showed similar features and immunotherapy responsiveness. Overall recurrence and mortality rates were both 7%.

Conclusions

In this small series, cases with concomitant EBV and GFAP positivity showed overlapping clinical features with classical GFAP-A and generally favorable responses to immunotherapy. Early recognition and CSF-based GFAP antibody testing may help improve patient outcomes; however, further studies are needed to confirm these findings.