Generalized cortical encephalitis in autoimmune GFAP astrocytopathy
Yu Guo1,2,3, Hongjun Hao1,2,3, Yunchuang Sun4,5,6
1Department of Neurology, Peking University First Hospital, Xicheng District, Beijing, China.
Summary
We describe a rare case of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) presenting as encephalitis. Prompt immunotherapy led to significant recovery, expanding the known symptoms of GFAP-A.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is an emerging neurological disorder.
- GFAP-A is characterized by inflammation targeting glial fibrillary acidic protein, primarily affecting the central nervous system.
Observation:
- A 51-year-old woman presented with subacute cognitive decline and seizures.
- Brain MRI showed diffuse cortical abnormalities, cerebral edema, and leptomeningeal enhancement.
- Anti-GFAP antibodies were detected in serum and cerebrospinal fluid.
Findings:
- The patient was diagnosed with autoimmune GFAP-A presenting with generalized cortical encephalitis.
- Immunotherapy resulted in substantial clinical and radiological improvement.
- This case highlights a rare presentation of GFAP-A.
Implications:
- This case expands the known clinical and radiological spectrum of GFAP-A.
- It suggests potential heterogeneity in the pathogenesis of GFAP-A.
- Early diagnosis and immunotherapy are crucial for favorable outcomes in GFAP-A.
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