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Hokkaido University Collection of Scholarly and Academic Papers >
Graduate School of Medicine / Faculty of Medicine >
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Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Area Postrema Syndrome-Like Symptoms without Medulla Oblongata Lesions
| Title: | Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Area Postrema Syndrome-Like Symptoms without Medulla Oblongata Lesions |
| Authors: | Iwami, Kosuke Browse this author | | Nomura, Taichi Browse this author | | Seo, Sho Browse this author | | Nojima, Shingo Browse this author | | Tsuzaka, Kazufumi Browse this author | | Kimura, Akio Browse this author | | Shimohata, Takayoshi Browse this author | | Yabe, Ichiro Browse this author →KAKEN DB |
| Keywords: | Glial fibrillary acidic protein | | Astrocytopathy | | Autoantibody | | Area postrema syndrome | | Hiccups |
| Issue Date: | 1-Dec-2022 |
| Publisher: | Karger |
| Journal Title: | Neuroimmunomodulation |
| Volume: | 29 |
| Issue: | 4 |
| Start Page: | 433 |
| End Page: | 438 |
| Publisher DOI: | 10.1159/000524344 |
| Abstract: | Introduction: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently described steroid-responsive meningoencephalomyelitis positive for cerebrospinal fluid (CSF) anti-GFAP antibody. Area postrema syndrome (APS) involves intractable hiccups, nausea, and vomiting, which is caused by medulla oblongata (MO) impairment. APS is a characteristic symptom of aquaporin-4 (AQP4) autoimmunity, and it helps to differentiate between AQP4 and GFAP autoimmunity. Conversely, although 6 cases of autoimmune GFAP astrocytopathy with APS and MO lesions have been reported, the association between GFAP autoimmunity and APS is unclear. We report the case of a patient with autoimmune GFAP astrocytopathy presenting with APS-like symptoms without MO lesions and discuss the mechanisms underlying the symptoms. Methods: CSF anti-GFAP antibody was detected using cell-based assays and immunohistochemical assays. Results: A 54-year-old Japanese man developed persistent hiccups, intermittent vomiting, fever, anorexia, and inattention. Brain magnetic resonance imaging (MRI) showed periventricular lesions with radial linear periventricular enhancement, suggesting autoimmune GFAP astrocytopathy. However, no obvious MO lesions were identified on thin-slice images. Spinal cord MRI revealed hazy lesions with patchy enhancement along the cervical and thoracic cord. CSF analysis demonstrated inflammation, with positive results for anti-GFAP antibodies. Anti-AQP4 antibodies in the serum and CSF were negative. Esophagogastroduodenoscopy revealed gastroparesis and gastroesophageal reflux disease, and vonoprazan, mosapride, and rikkunshito were effective only against persistent hiccups. Steroid therapy was initiated, allowing clinical and radiological improvements. Repeated MRIs demonstrated no obvious MO lesions. Conclusion: This report suggests that autoimmune GFAP astrocytopathy presents with APS-like symptoms without obvious MO lesions. The possible causes of hiccups were gastroparesis and cervical cord lesions. Gastroesophageal reflux disease was not considered a major cause of the hiccups. Intermittent vomiting appeared to be associated with gastroparesis, cervical cord lesions, and viral-like symptoms. Testing for anti-GFAP antibodies should be considered in patients with APS-like symptoms in the context of typical clinical-MRI features of autoimmune GFAP astrocytopathy. |
| Rights: | This is the peer-reviewed but unedited manuscript version of the following article: Iwami K, Nomura T, Seo S, Nojima S, Tsuzaka K, Kimura A, Shimohata T, Yabe I: Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Area Postrema Syndrome-Like Symptoms without Medulla Oblongata Lesions. Neuroimmunomodulation 2022;29:433-438. The final, published version is available at http://www.karger.com/?doi=10.1159/000524344. |
| Type: | article (author version) |
| URI: | http://hdl.handle.net/2115/90948 |
| Appears in Collections: | 医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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Submitter: 矢部 一郎
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