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| Item Type: | Article |
|---|---|
| Title: | Glial fibrillary acidic protein astrocytopathy based on a two-center chinese cohort study |
| Creators Name: | Wu, T., Zhang, H., Gao, C., Yu, Q., Fan, M., Zhang, L.J., Zhang, H., Cong, H., Wei, Y., Böttcher, C., Verkhratsky, A., Paul, F., Shi, F.D. and Song, T. |
| Abstract: | OBJECTIVE: Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a recently defined nosological form belonging to the class of autoimmune inflammatory disorders affecting the central nervous system (CNS). Here, we report the clinical and MRI characteristics, treatment, and prognosis of a GFAP-A cohort from two centers in China. METHODS: We retrospectively analyzed the data from 38 adult patients with positive GFAP antibodies and diagnosed as GFAP-A between June 2019 and September 2024. Clinical features, semiquantitative antibody test results, MRI features, treatment approaches, and prognosis were collected. RESULTS: Among the 38 patients, 24 were male, and the median age at disease onset was 49.5 years. The clinical phenotype included encephalomyelitis (28.9%), myelitis (23.7%), encephalitis (18.4%), meningoencephalomyelitis (18.4%), meningitis/spinal meningitis (7.9%), and peripheral neuropathy (2.6%). In enhanced MRI images, 4 (10.5%) of the patients showed enhancement of the cerebral meninges, 2 (5.3%) had enhancement of the ependyma, and 5 (13.2%) had enhancement of the spinal cord pia mater. 77.1% of the patients responded to the glucocorticoid treatment, while 65.8% had a monophasic course. Spearman correlation analysis showed that CSF-specific oligoclonal bands were significantly correlated with 1-year relapse (CI = 0.527, p = 0.003). INTERPRETATION: The clinical manifestations of GFAP-A are highly diverse, encompassing encephalitis, myelitis, and meningitis, including spinal meningitis. The enhancement of the spinal pia mater and ependyma on MRI was confirmed. Most patients exhibit a positive response to glucocorticoid therapy. The presence of CSF-specific oligoclonal bands could potentially serve as an indicator for predicting recurrence. |
| Keywords: | Astrocytopathy, Glial Fibrillary Acid Protein, Immunotherapy, Neuroimmunology, Recurrence |
| Source: | Annals of Clinical and Translational Neurology |
| ISSN: | 2328-9503 |
| Publisher: | Wiley |
| Date: | 10 July 2025 |
| Official Publication: | https://doi.org/10.1002/acn3.70118 |
| PubMed: | View item in PubMed |
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