自身免疫性胶质纤维酸性蛋白星形胶质细胞病8例临床特征分析

Clinical analysis of 8 cases of autoimmune glial fibrillary acidic protein astrocytopathy

目的分析自身免疫性胶质纤维酸性蛋白(GFAP)星形胶质细胞病的临床特征。方法回顾性分析2021年1月1日至2021年10月1日于宁夏医科大学总医院收治并确诊的8例自身免疫性GFAP星形胶质细胞病患者的临床症状、辅助检查结果及转归情况。结果8例患者中男性5例,女性3例,发病年龄32~66岁,急性期以脑膜、脑、脊髓及视神经受累为主。8例患者脑脊液抗GFAP抗体均阳性,合并脑脊液及血清AQP4抗体阳性3例,合并肿瘤2例。7例患者脑脊液检查结果异常,均表现为白细胞计数升高,且以淋巴细胞为主。颅脑MRI异常信号7例,其中脑室周围放射状强化3例。脊髓MRI异常信号5例,脊膜受累者1例,脊髓受累者4例;其中长节段脊髓病变3例。7例接受甲强龙冲击治疗后症状缓解,1例复发,1例预后较差。结论GFAP星形胶质细胞病可以以自主神经功能障碍及脊髓病变为主要临床表现,特征性磁共振影像学表现(脑室周围放射状线样强化)和淋巴细胞为主的脑脊液炎性反应对该病有重要提示意义,脑脊液抗GFAP抗体检测可明确诊断。

Objective To analyze the clinical characteristics of autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy.Methods The clinical symptoms,auxiliary examination results and outcomes of 8 patients with autoimmune GFAP astrocytopathy admitted and diagnosed in the General Hospital of Ningxia Medical University from January 1,2021 to October 1,2021 were retrospectively analyzed.Results Among the 8 patients,there were 5 males and 3 females,aged 32-66 years at onset;the lesions mainly involved the meninges,brain,spinal cord and optic nerve at acute stage.The GFAP antibodies were all positive in the cerebrospinal fluid(CSF)of 8 patients,3 patients had coexisting AQP4-IgG positive in CSF and serum.2 patients were combined with tumor.7 patients were with abnormal CSF,all of which showed elevated white blood cell counts,with lymphocytes being predominant.Brain MRI abnormalities were found in 7 cases,including radial paraventricular enhancement in 3 patients.Spinal MRI abnormalities were found in 5 patients,including One case of spinal meninges involvement and 4 cases of spinal cord involvement.Among them,there were 3 patients with longitudinally extensive myelitic abnormalities.Seven patients had a symptoms relieved after methylprednisolone.1 patient had a recrudescence and 1 patient had a poor prognosis.Conclusion Autonomic dysfunction and spinal cord lesions can be main clinical manifestations of autoimmune GFAP astrocytopathy,the characteristic magnetic resonance imaging(radial paraventricular enhance-ment)and CSF findings of lymphocyte-predominant plecytosis are important indicators.CSF GFAP antibody testing can confirm the diagnosis.