{"title":"基于中国双中心队列研究的胶质纤维酸性蛋白星形细胞病。","authors":"Ti Wu, Hao Zhang, Chao Gao, Qiuhua Yu, Moli Fan, Lin-Jie Zhang, Haipeng Zhang, Hengri Cong, Yuzhen Wei, Chotima Böttcher, Alexej Verkhratsky, Friedemann Paul, Fu-Dong Shi, Tian Song","doi":"10.1002/acn3.70118","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Objective</h3>\n \n <p>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.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>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.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>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, <i>p</i> = 0.003).</p>\n </section>\n \n <section>\n \n <h3> Interpretation</h3>\n \n <p>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.</p>\n </section>\n </div>","PeriodicalId":126,"journal":{"name":"Annals of Clinical and Translational Neurology","volume":"12 9","pages":"1813-1822"},"PeriodicalIF":3.9000,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.70118","citationCount":"0","resultStr":"{\"title\":\"Glial Fibrillary Acidic Protein Astrocytopathy Based on a Two-Center Chinese Cohort Study\",\"authors\":\"Ti Wu, Hao Zhang, Chao Gao, Qiuhua Yu, Moli Fan, Lin-Jie Zhang, Haipeng Zhang, Hengri Cong, Yuzhen Wei, Chotima Böttcher, Alexej Verkhratsky, Friedemann Paul, Fu-Dong Shi, Tian Song\",\"doi\":\"10.1002/acn3.70118\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Objective</h3>\\n \\n <p>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.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Methods</h3>\\n \\n <p>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.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Results</h3>\\n \\n <p>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, <i>p</i> = 0.003).</p>\\n </section>\\n \\n <section>\\n \\n <h3> Interpretation</h3>\\n \\n <p>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.</p>\\n </section>\\n </div>\",\"PeriodicalId\":126,\"journal\":{\"name\":\"Annals of Clinical and Translational Neurology\",\"volume\":\"12 9\",\"pages\":\"1813-1822\"},\"PeriodicalIF\":3.9000,\"publicationDate\":\"2025-07-10\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://onlinelibrary.wiley.com/doi/epdf/10.1002/acn3.70118\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Annals of Clinical and Translational Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1002/acn3.70118\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Clinical and Translational Neurology","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1002/acn3.70118","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Glial Fibrillary Acidic Protein Astrocytopathy Based on a Two-Center Chinese Cohort Study
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.
期刊介绍:
Annals of Clinical and Translational Neurology is a peer-reviewed journal for rapid dissemination of high-quality research related to all areas of neurology. The journal publishes original research and scholarly reviews focused on the mechanisms and treatments of diseases of the nervous system; high-impact topics in neurologic education; and other topics of interest to the clinical neuroscience community.