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Background <p>Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a rare central nervous system (CNS) autoimmune disorder with highly heterogeneous presentations. This study aims to describe the clinical and diagnostic features in a Chinese case series.</p> Methods <p>Six patients at a single tertiary center with positive GFAP antibody were included, the patients’ data were retrospectively analyzed.</p> Results <p>The cohort included 3 males and 3 females, with a median age of 56 years (range 30–75). Prominent urinary dysfunction was observed in four patients (66.7%). Brain or spinal cord MRI abnormalities were present in only 50% (3/6) of patients. All patients exhibited CSF-specific oligoclonal bands and elevated indices of intrathecal IgG synthesis. A notable finding was the delayed detection of CSF GFAP antibody in five patients (83.3%), who tested negative at disease onset but turned positive upon follow-up testing weeks to months later.</p> Conclusion <p>This case series underscores the high frequency of autonomic urinary dysfunction and the potential for delayed antibody detection in Chinese GFAP-A patients. These findings suggest that clinical suspicion should remain high even with initial negative antibody testing, warranting repeat CSF analysis in suspected cases to avoid diagnostic delay.</p>

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Prominent urinary dysfunction and delayed antibody detection in patients with glial fibrillary acidic protein astrocytopathy: a case series analysis

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月2日

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  • Zhenyu Niu,
  • Yunchuang Sun,
  • Fan Li,
  • Zhaoxia Wang,
  • Luhua Wei,
  • Ran Liu,
  • Feng Gao,
  • Haiqiang Jin,
  • Jing Guo,
  • Nan Zhang,
  • Yiming Zheng,
  • Hongjun Hao

摘要

Background

Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a rare central nervous system (CNS) autoimmune disorder with highly heterogeneous presentations. This study aims to describe the clinical and diagnostic features in a Chinese case series.

Methods

Six patients at a single tertiary center with positive GFAP antibody were included, the patients’ data were retrospectively analyzed.

Results

The cohort included 3 males and 3 females, with a median age of 56 years (range 30–75). Prominent urinary dysfunction was observed in four patients (66.7%). Brain or spinal cord MRI abnormalities were present in only 50% (3/6) of patients. All patients exhibited CSF-specific oligoclonal bands and elevated indices of intrathecal IgG synthesis. A notable finding was the delayed detection of CSF GFAP antibody in five patients (83.3%), who tested negative at disease onset but turned positive upon follow-up testing weeks to months later.

Conclusion

This case series underscores the high frequency of autonomic urinary dysfunction and the potential for delayed antibody detection in Chinese GFAP-A patients. These findings suggest that clinical suspicion should remain high even with initial negative antibody testing, warranting repeat CSF analysis in suspected cases to avoid diagnostic delay.