The Department of Neurology, Chenzhou No.1 People's Hospital, Chenzhou, Hunan, 423000, People's Republic of China.
BMC Neurol. 2022 Jul 20;22(1):271. doi: 10.1186/s12883-022-02802-2.
Area postrema syndrome (APS) as the isolated manifestation in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been rarely reported.
A 61-year-old male patient presented with intractable hiccup. He was first admitted to the department of Gastroenterology because he had no symptoms other than hiccup. Then he was diagnosed with possible digestive system disease and started on treatment. 2 weeks later, his symptom didn't improve at all. After consultation, the patient was referred to our department. Cerebrospinal fluid (CSF) analysis revealed lymphocytes pleocytosis, elevated protein level. Cell-based assays demonstrated GFAP antibodies in blood and CSF. His symptom improved with steroid pulse therapy (methylprednisolone, 1 g for 5 days), followed by a gradual tapering of oral prednisolone. Three months after the initial presentation, he showed no relapses.
We report atypical manifestation of autoimmune GFAP astrocytopathy which presented as APS, suggesting that autoimmune GFAP astrocytopathy should be added to the list of possible cause of APS.
作为自身免疫性神经胶质纤维酸性蛋白（GFAP）星形细胞瘤病的孤立表现，后区综合征（APS）很少见。
一名 61 岁男性患者因顽固性呃逆就诊。因仅有呃逆而无其他症状，他首先被收入消化内科。随后被诊断为可能的消化系统疾病，并开始治疗。2 周后，他的症状没有任何改善。经会诊，患者被转至我科。脑脊液（CSF）分析显示淋巴细胞增多，蛋白水平升高。基于细胞的检测显示血液和 CSF 中存在 GFAP 抗体。他的症状在类固醇脉冲治疗（甲泼尼龙，1g 连用 5 天）后改善，随后逐渐减少口服泼尼松龙的剂量。初次就诊 3 个月后，他没有复发。
我们报告了一种自身免疫性 GFAP 星形细胞瘤病的非典型表现，表现为 APS，提示自身免疫性 GFAP 星形细胞瘤病应被列入 APS 的可能病因列表中。
