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Overlapping Clinical Syndromes in Patients with Glial Fibrillary Acidic Protein IgG.
Neuroimmunomodulation ( IF 2.4 ) Pub Date : 2020-02-26 , DOI: 10.1159/000505730
Han Lin 1 , Yihong Huang 2 , Huanzhong Zeng 3 , Mingxia Wang 1 , Shaobing Guan 1 , Guohua Chen 1 , Hongbing Guan 4
Affiliation  

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.
Neuroimmunomodulation


中文翻译:

胶质纤维酸性蛋白IgG患者的临床症状重叠。

目的:本文旨在报道2例自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病变重叠综合征。方法:采用间接免疫荧光法检测抗体。回顾性分析患者数据。结果:一名患者表现为重叠性神经脊髓炎视神经光谱障碍(NMOSD),且GFAP-IgG和aquaporin-4-IgG阳性。他的主要症状包括视力下降,打ic,发烧,头痛和共济失调。脑脊液中发现高白细胞计数和蛋白质水平。脑磁共振成像(MRI)显示海马,中脑,脑桥,延髓和脑膜异常。看到了特征性的径向增强图案。另一例是男性,患有复发性多发性软骨炎(RP)和GFAP-IgG阳性。他的主要表现为脑膜脑炎和痴呆。MRI显示脑室,颞叶和丘脑周围的白质广泛异常,并增强。两名患者对类固醇和免疫抑制剂的治疗均反应良好。结论:尽管重叠综合征很少见,但我们报告2例NMOSD或RP阳性GFAP-IgG。两名患者均具有GFAP星形细胞病变的临床特征,但由于重叠的表现,对该病的诊断非常具有挑战性。
神经免疫调节
更新日期:2020-02-26
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