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作 者:王勋[1] 张蒙蒙 王慧敏 代大伟[1] 张黎明[1] WANG Xun;ZHANG Mengmeng;WANG Huimin(The First Affiliated Hospital of Harbin Medical University,Harbin 150001,China)
机构地区:[1]哈尔滨医科大学附属第一医院,黑龙江哈尔滨163700
出 处:《中风与神经疾病杂志》2024年第11期1029-1034,共6页Journal of Apoplexy and Nervous Diseases
摘 要:目的总结脑脊液(CSF)和血清中抗胶质纤维酸性蛋白(GFAP)抗体阳性患者的临床特点。方法收集2020年12月—2023年12月于哈尔滨医科大学附属第一医院收治并确诊的16例抗GFAP抗体阳性患者的临床表现、实验室检查、影像学检查、神经电生理检查等资料并进行回顾性分析。结果16例患者中男12例,女4例,年龄22~68岁。临床表现符合脑炎、脑膜脑炎7例,脱髓鞘疾病7例,急性脊髓炎1例,周围神经受累伴呃逆1例;有糖尿病史3例,癫痫病史1例,大细胞贫血史1例。CSF压力升高6例,有核细胞数升高8例,蛋白升高11例。抗MOG抗体阳性2例,抗神经节苷脂抗体阳性1例,抗β2糖蛋白Ⅰ抗体阳性2例。结论CSF抗GFAP抗体体阳性的(脑膜)脑炎是GFAP星形细胞病的典型临床表现,部分病例可伴胼胝体受累。GFAP抗体可出现在脱髓鞘疾病中,诊断需结合临床表型。合并其他抗体时,应以核心表型为基础确认责任抗体。虽然GFAP抗体致病性尚无定论,但抗GFAP抗体阳性有助于神经免疫疾病的诊断及避免延误免疫治疗。Objective To investigate the clinical features of patients with positive anti-glial fibrillary acidic protein(GFAP)antibody in cerebrospinal fluid(CSF)and serum.Methods A retrospective analysis was performed for the data of 16 patients with positive anti-GFAP antibody who were admitted and diagnosed from December 2020 to December 2023,including clinical manifestations,laboratory examination,neuroimaging data,and neural electrophysiological examination.Results Among the 16 patients,there were 12 male patients and 4 female patients,with an age of 22‒68 years.In terms of clinical manifestations,there were 7 patients with encephalitis and meningoencephalitis,7 with demyelinating diseases,1 with acute myelitis,and 1 with peripheral nerve involvement and hiccup,and there were 3 patients with the medical history of diabetes,1 with the medical history of epilepsy,and 1 with the medical history of macrocytic anemia.Six patients had an increase in CSF,8 had an increase in the number of nucleated cells,and 11 had an increase in protein.There were 2 patients with positive anti-MOG antibody,1 patient with positive anti-ganglioside antibodies,and 2 patients with positive anti-β2 glycoprotein I antibody.Conclusion Encephalitis or meningoencephalitis with positive anti-GFAP antibody in CSF is a typical clinical manifestation of GFAP astrocytopathy,with possible involvement of the corpus callosum in some cases.Anti-GFAP antibody can be observed in demyelinating diseases,while a confirmed diagnosis can be made with reference to clinical phenotypes.In case of the presence of other antibodies,responsible antibodies should be identified based on the core phenotype.Although no consensus has been reached on the pathogenicity of anti-GFAP antibody,positive anti-GFAP antibody may help with the diagnosis of neuro-immune diseases and the adoption of timely immunotherapy.
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