A Case of Autoimmune glial fibrillary acidic protein astrocytopathy mimicking neuromyelitis optica

Author:

Fang Yanan1,Liu Gang1,He Xiaomei1,Zhang Yanqi1,Wang Zhicheng1

Affiliation:

1. Chaoyang Central Hospital

Abstract

Abstract Background Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory disease of the central nervous system (CNS), which affects various regions in the CNS, presenting by variable clinical manifestations. Diagnosis is confirmed by the presence of CSF immunoglobulin-G (IgG) against GFAP. The pathogenesis of GFAP-A is poorly understood. This report has enriched the disease spectrum of GFAP-A. Case presentation We describe a 58-year-old woman Who suffered acute neurological symptoms, including right upper limb paresthesia and right limb weakness. Cranial magnetic resonance imaging showed enhancement pattern in the cerebellum, CSF was tested for GFAP IgG antibodies and serum was tested for AQP4 IgG antibodies which were positive, and GFAP-A was finally diagnosed. She responded well to high-dose glucocorticoids. Sequential treatment with oral prednisone was prescribed. The right limb numbness of the patient recovered well, the right limb weakness returned to normal. So far, after 8 months, the patient still has no recurrence. Conclusion In conclusion, the provision of an accurate early diagnosis and appropriate treatment are crucial for improving the prognosis of patients with autoimmune GFAP astrocytopathy.

Publisher

Research Square Platform LLC

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