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      Overlapping Clinical Syndromes in Patients with Glial Fibrillary Acidic Protein IgG

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          Objective: The aim of this paper is to report 2 cases with overlapping syndromes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Methods: Antibodies were detected by indirect immunofluorescence assay. Patient data were analyzed retrospectively. Results: One patient presented with overlapping neuromyelitis optica spectrum disorder (NMOSD) and positive GFAP-IgG and aquaporin-4-IgG. His main symptoms included vision loss, hiccups, fever, headache, and ataxia. High leukocyte count and protein levels were found in cerebrospinal fluid. Brain magnetic resonance imaging (MRI) revealed abnormalities in the hippocampus, midbrain, pons, medulla, and meninges. Characteristic radial enhancing patterns were seen. The other patient was a male with relapsing polychondritis (RP) and positive GFAP-IgG. His main manifestations were meningoencephalitis and dementia. MRI showed extensive abnormalities in the white matter around the ventricles, temporal lobe, and thalamus, with enhancement. Both patients responded well to the treatment with steroids and immunosuppressants. Conclusions: Although overlapping syndromes are rare, we report positive GFAP-IgG in 2 cases with NMOSD or RP. Both patients had clinical features of GFAP astrocytopathy, but diagnosis of the condition was very challenging because of the overlapping presentation.

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          Most cited references 15

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          Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients

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            Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis.

            A novel astrocytic autoantibody has been identified as a biomarker of a relapsing autoimmune meningoencephalomyelitis that is immunotherapy responsive. Seropositivity distinguishes autoimmune glial fibrillary acidic protein (GFAP) meningoencephalomyelitis from disorders commonly considered in the differential diagnosis.
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              Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients


                Author and article information

                S. Karger AG
                July 2020
                26 February 2020
                : 27
                : 1
                : 69-74
                aDepartment of Neurology, The Affiliated Dongguan Houjie Hospital of Guangdong Medical University, Dongguan, China
                bDepartment of Radiology, The Affiliated Dongguan Houjie Hospital of Guangdong Medical University, Dongguan, China
                cKey Laboratory of Oral Medicine, Guangzhou Institute of Oral Disease, Affiliated Stomatology Hospital of Guangzhou Medical University, Guangzhou, China
                Author notes
                *Yihong Huang, Department of Neurology, The Affiliated Dongguan Houjie Hospital of, Guangdong Medical University, Dongguan 523945 (China), sums_hyh@sina.cn
                505730 Neuroimmunomodulation 2020;27:69–74
                © 2020 S. Karger AG, Basel

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