[Analysis of the clinical characteristics of severe autoimmune glial fibrillary acidic protein astrocytopathy].

Objective: To analyze the clinical characteristics of 14 patients with severe autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). Methods: A retrospective analysis was conducted on the clinical data of 14 patients diagnosed with severe GFAP-A in Xuanwu Hospital, Capital Medical University, between July 2023 and September 2024. Results: (1) Fourteen patients were included in the study, including 11 males and 3 females, aged 15-66 years (average: 39±13 years). The time from disease onset to consciousness impairment was 4-15 days, with an average of 10±3 days. (2) The primary initial main symptoms were fever, headache, limb weakness, and abnormal mental behavior. As the condition worsened, all patients developed consciousness disorders, and 11 experienced respiratory failure requiring tracheal intubation. (3) Intracranial lesions often involved both cerebral hemispheres, the thalamus, basal ganglia, and the brainstem. Spinal cord lesions involved the cervical and thoracic regions. (4) Most patients had elevated intracranial pressure. In the cerebral spinal fluid (CSF), all patients exhibited elevated protein levels and a high white blood cell count, predominantly monocytes, along with reduced glucose levels. (5) Treatment encompassed combinations of immunotherapies, including hormones, human immunoglobulin, plasma exchange, or immunoadsorption. Conclusions: The clinical manifestations of critically ill GFAP-A patients are heterogeneous. Disease progression is rapid, and the symptoms are increasingly severe, making early diagnosis difficult. Head and spinal cord MRI, CSF analysis, and GFAP-IgG testing are essential for diagnosis. After immunotherapy, most patients have a good prognosis.