Research progress of autoimmune glial fibrillary acidic protein astrocytopathy
Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP ⁃ A) is a novel autoimmune inflammatory disease of the central nervous system first reported in 2016. The predominant clinical syndrome of GFAP ⁃A is one or more of meningitis, encephalitis and myelitis. The initial symptoms are fever,...
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| Format: | Article |
| Language: | English |
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Tianjin Huanhu Hospital
2022-03-01
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| Series: | Chinese Journal of Contemporary Neurology and Neurosurgery |
| Subjects: | |
| Online Access: | http://www.cjcnn.org/index.php/cjcnn/article/view/2476 |
| _version_ | 1819157006661976064 |
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| author | WANG Wen⁃wen LI Mei |
| author_facet | WANG Wen⁃wen LI Mei |
| author_sort | WANG Wen⁃wen |
| collection | DOAJ |
| description | Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP ⁃ A) is a novel autoimmune inflammatory disease of the central nervous system first reported in 2016. The predominant clinical syndrome of GFAP ⁃A is one or more of meningitis, encephalitis and myelitis. The initial symptoms are fever, headache, disturbance of consciousness and positive meningeal irritation. However, the pathogenesis of GFAP ⁃ A is unknown and there are no uniform diagnostic criteria for it. At present, it is generally accepted that positive glial fibrillary acidic protein ⁃immunoglobulin G (GFAP ⁃ IgG) in serum and cerebrospinal fluid (CSF) is the diagnostic standard. The positive predictive value of GFAP ⁃ IgG in CSF is higher than that in serum. Cell ⁃ based assay (CBA) and tissue ⁃ based assay (TBA) are both recommended methods for detecting GFAP ⁃ IgG. GFAP ⁃ IgG positive in serum and/or CSF is used to distinguish it from other autoimmune diseases in central nervous system, intracranial inflammatory and neoplastic disease. Most patients respond well to steroid therapy although a small number of patients may leave sequelae or relapse. This paper makes a systematic review on the etiology, pathogenesis, clinical features, diagnosis, differential diagnosis, treatment and prognosis of this disease, hoping that this review can deepen clinicians' understanding of the disease, discover and treat the disease in time. |
| first_indexed | 2024-12-22T16:01:54Z |
| format | Article |
| id | doaj.art-bee9bce7a2d14cb2815cfcc821cb1569 |
| institution | Directory Open Access Journal |
| issn | 1672-6731 |
| language | English |
| last_indexed | 2024-12-22T16:01:54Z |
| publishDate | 2022-03-01 |
| publisher | Tianjin Huanhu Hospital |
| record_format | Article |
| series | Chinese Journal of Contemporary Neurology and Neurosurgery |
| spelling | doaj.art-bee9bce7a2d14cb2815cfcc821cb15692022-12-21T18:20:41ZengTianjin Huanhu HospitalChinese Journal of Contemporary Neurology and Neurosurgery1672-67312022-03-0122320521010.3969/j.issn.1672⁃6731.2022.03.014Research progress of autoimmune glial fibrillary acidic protein astrocytopathyWANG Wen⁃wen0 LI Mei1Department of Neurology, Children's Hospital of Chongqing Medical University; National Clinical Research Center for Child Health and Disorders; Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing 400014, ChinaDepartment of Neurology, Children's Hospital of Chongqing Medical University; National Clinical Research Center for Child Health and Disorders; Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing 400014, ChinaAutoimmune glial fibrillary acidic protein astrocytopathy (GFAP ⁃ A) is a novel autoimmune inflammatory disease of the central nervous system first reported in 2016. The predominant clinical syndrome of GFAP ⁃A is one or more of meningitis, encephalitis and myelitis. The initial symptoms are fever, headache, disturbance of consciousness and positive meningeal irritation. However, the pathogenesis of GFAP ⁃ A is unknown and there are no uniform diagnostic criteria for it. At present, it is generally accepted that positive glial fibrillary acidic protein ⁃immunoglobulin G (GFAP ⁃ IgG) in serum and cerebrospinal fluid (CSF) is the diagnostic standard. The positive predictive value of GFAP ⁃ IgG in CSF is higher than that in serum. Cell ⁃ based assay (CBA) and tissue ⁃ based assay (TBA) are both recommended methods for detecting GFAP ⁃ IgG. GFAP ⁃ IgG positive in serum and/or CSF is used to distinguish it from other autoimmune diseases in central nervous system, intracranial inflammatory and neoplastic disease. Most patients respond well to steroid therapy although a small number of patients may leave sequelae or relapse. This paper makes a systematic review on the etiology, pathogenesis, clinical features, diagnosis, differential diagnosis, treatment and prognosis of this disease, hoping that this review can deepen clinicians' understanding of the disease, discover and treat the disease in time.http://www.cjcnn.org/index.php/cjcnn/article/view/2476autoimmune diseases of the nervous systemglial fibrillary acidic proteinastrocytesreview |
| spellingShingle | WANG Wen⁃wen LI Mei Research progress of autoimmune glial fibrillary acidic protein astrocytopathy Chinese Journal of Contemporary Neurology and Neurosurgery autoimmune diseases of the nervous system glial fibrillary acidic protein astrocytes review |
| title | Research progress of autoimmune glial fibrillary acidic protein astrocytopathy |
| title_full | Research progress of autoimmune glial fibrillary acidic protein astrocytopathy |
| title_fullStr | Research progress of autoimmune glial fibrillary acidic protein astrocytopathy |
| title_full_unstemmed | Research progress of autoimmune glial fibrillary acidic protein astrocytopathy |
| title_short | Research progress of autoimmune glial fibrillary acidic protein astrocytopathy |
| title_sort | research progress of autoimmune glial fibrillary acidic protein astrocytopathy |
| topic | autoimmune diseases of the nervous system glial fibrillary acidic protein astrocytes review |
| url | http://www.cjcnn.org/index.php/cjcnn/article/view/2476 |
| work_keys_str_mv | AT wangwenwen researchprogressofautoimmuneglialfibrillaryacidicproteinastrocytopathy AT limei researchprogressofautoimmuneglialfibrillaryacidicproteinastrocytopathy |