Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis
ObjectiveAutoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel steroid sensitive autoimmune disease, without a diagnostic consensus. The purpose of this study was to improve early GFAP-A diagnosis by increasing awareness of key clinical characteristics and imaging manifestati...
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Frontiers Media S.A.
2024-12-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2024.1466847/full |
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| author | Xiaomeng Li Jiacun Li Han Xu Xiaohui Liu Meilin Li Jingzhen He Jianjun Xiu |
| author_facet | Xiaomeng Li Jiacun Li Han Xu Xiaohui Liu Meilin Li Jingzhen He Jianjun Xiu |
| author_sort | Xiaomeng Li |
| collection | DOAJ |
| description | ObjectiveAutoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel steroid sensitive autoimmune disease, without a diagnostic consensus. The purpose of this study was to improve early GFAP-A diagnosis by increasing awareness of key clinical characteristics and imaging manifestations.MethodsMedical records of 13 patients with anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) were reviewed for cross-sectional and longitudinal analysis of clinical and magnetic resonance imaging (MRI) findings.ResultsThe predominant GFAP-A clinical manifestations are limb weakness/numbness and fever. GFAP-A has a propensity in the early stage for meningeal and leptomeningeal lesions on the brainstem surface, with a typical pattern of periventricular linear radial and leptomeningeal enhancement. The clinical manifestations and leptomeningeal enhancement were rapidly alleviated after treatment with high doses of corticosteroids or/and intravenous immunoglobulin, although, there are patients who may present with increased brain parenchymal lesions. On 3T MRI, the spinal cord demonstrated extensive longitudinal T2-weighted hyper-intensity, central distribution, and gray matter involvement. Optic nerve involvement in some patients was also noted with optic nerve swelling and abnormal enhancement. In addition to the classic reversible splenium of corpus callosum syndrome (type I), this study found the much rarer type II with diffusion restriction on DWI (Diffusion Weighted Imaging) in the corpus callosum. Positive anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) are important for GFAP-A diagnosis with overlapping antibodies commonly noted. This study found anti-GM3 antibodies, a rare finding also previously reported.ConclusionThis study correlates GFAP-A clinical and imaging features, noting a “delay” phenomenon between clinical manifestations, treatment response, and radiographic MRI findings. MRI T2-FLAIR brainstem hyperintensity and T2-FLAIR gadolinium enhanced images, and subtraction techniques were valuable for early lesion detection and accurate diagnosis. |
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| id | doaj-art-906505bc90c8455eb73fed53df0ec2a2 |
| institution | OA Journals |
| issn | 1664-3224 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Immunology |
| spelling | doaj-art-906505bc90c8455eb73fed53df0ec2a22025-08-20T01:59:17ZengFrontiers Media S.A.Frontiers in Immunology1664-32242024-12-011510.3389/fimmu.2024.14668471466847Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosisXiaomeng Li0Jiacun Li1Han Xu2Xiaohui Liu3Meilin Li4Jingzhen He5Jianjun Xiu6Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, ChinaDepartment of Radiology, People’s Hospital of RiZhao, RiZhao, Shandong, ChinaDepartment of Radiology, Jinan Third People’s Hospital, Jinan, Shandong, ChinaDepartment of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, ChinaDepartment of Radiology, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, Shandong, ChinaDepartment of Radiology, Qilu Hospital of Shandong University, Jinan, Shandong, ChinaDepartment of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, ChinaObjectiveAutoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel steroid sensitive autoimmune disease, without a diagnostic consensus. The purpose of this study was to improve early GFAP-A diagnosis by increasing awareness of key clinical characteristics and imaging manifestations.MethodsMedical records of 13 patients with anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) were reviewed for cross-sectional and longitudinal analysis of clinical and magnetic resonance imaging (MRI) findings.ResultsThe predominant GFAP-A clinical manifestations are limb weakness/numbness and fever. GFAP-A has a propensity in the early stage for meningeal and leptomeningeal lesions on the brainstem surface, with a typical pattern of periventricular linear radial and leptomeningeal enhancement. The clinical manifestations and leptomeningeal enhancement were rapidly alleviated after treatment with high doses of corticosteroids or/and intravenous immunoglobulin, although, there are patients who may present with increased brain parenchymal lesions. On 3T MRI, the spinal cord demonstrated extensive longitudinal T2-weighted hyper-intensity, central distribution, and gray matter involvement. Optic nerve involvement in some patients was also noted with optic nerve swelling and abnormal enhancement. In addition to the classic reversible splenium of corpus callosum syndrome (type I), this study found the much rarer type II with diffusion restriction on DWI (Diffusion Weighted Imaging) in the corpus callosum. Positive anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) are important for GFAP-A diagnosis with overlapping antibodies commonly noted. This study found anti-GM3 antibodies, a rare finding also previously reported.ConclusionThis study correlates GFAP-A clinical and imaging features, noting a “delay” phenomenon between clinical manifestations, treatment response, and radiographic MRI findings. MRI T2-FLAIR brainstem hyperintensity and T2-FLAIR gadolinium enhanced images, and subtraction techniques were valuable for early lesion detection and accurate diagnosis.https://www.frontiersin.org/articles/10.3389/fimmu.2024.1466847/fullautoimmune glial fibrillary acidic protein astrocytopathymeningeal enhancementbrain FLAIR gadolinium enhancementspinal cord longitudinal T2 hyperintensityoverlapping antibodies |
| spellingShingle | Xiaomeng Li Jiacun Li Han Xu Xiaohui Liu Meilin Li Jingzhen He Jianjun Xiu Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis Frontiers in Immunology autoimmune glial fibrillary acidic protein astrocytopathy meningeal enhancement brain FLAIR gadolinium enhancement spinal cord longitudinal T2 hyperintensity overlapping antibodies |
| title | Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis |
| title_full | Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis |
| title_fullStr | Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis |
| title_full_unstemmed | Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis |
| title_short | Review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis |
| title_sort | review of clinical and imaging findings in autoimmune glial fibrillary acidic protein astrocytopathy to aid in early diagnosis |
| topic | autoimmune glial fibrillary acidic protein astrocytopathy meningeal enhancement brain FLAIR gadolinium enhancement spinal cord longitudinal T2 hyperintensity overlapping antibodies |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2024.1466847/full |
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