A case of autoimmune glial fibrillary acidic protein astrocytopathy with concurrent renal cell carcinoma

Krithika Arrabothu , Srinivas Govindan , Wilson Rodriguez , Christopher Tapia , Robert L. White III
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Abstract

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel central nervous system disorder characterized by subacute neuropsychiatric symptom development and rapid remission with corticosteroid therapy. In this paper, we describe a case of GFAP encephalitis found to have renal cell carcinoma. The patient was a 71-year-old man with a history of transient ischemic attacks, severe diabetic retinopathy and monoclonal gammopathy of undetermined significance, who presented with subacute cognitive decline. Anti-GFAP antibodies were detected in the cerebrospinal fluid. Computer Tomography (CT) of chest, abdomen and pelvis identified a renal mass that was resected. Subsequent pathology confirmed clear cell renal cell carcinoma. The patient was treated with corticosteroids with cognitive recovery near to baseline. In conclusion, we encourage the early consideration of autoimmune GFAP astrocytopathy for patients with clinical manifestation of subacute neuropsychiatric symptoms after other common conditions have been ruled out, as it usually has a great response to immunosuppressive therapy. In addition, this case highlights the importance of full body scan to identify possible underlying malignancy as a possible precipitating factor.

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一例自身免疫性胶质纤维酸性蛋白星形细胞病变并发肾细胞癌的病例
自身免疫性胶质纤维酸性蛋白(GFAP)星形胶质细胞病是一种新型中枢神经系统疾病,其特点是出现亚急性神经精神症状,并在皮质类固醇治疗后迅速缓解。本文描述了一例发现患有肾细胞癌的 GFAP 脑炎患者。患者是一名71岁的男性,有短暂性脑缺血发作、严重糖尿病视网膜病变和意义不明的单克隆丙种球蛋白病病史,出现亚急性认知功能下降。脑脊液中检测到抗-GFAP抗体。胸部、腹部和盆腔的计算机断层扫描(CT)发现了肾脏肿块,并进行了切除。随后的病理证实为透明细胞肾细胞癌。患者接受了皮质类固醇治疗,认知能力恢复接近基线。总之,我们鼓励临床表现为亚急性神经精神症状的患者在排除其他常见疾病后及早考虑自身免疫性 GFAP 星形细胞病,因为它通常对免疫抑制治疗有很好的反应。此外,本病例还强调了全身扫描的重要性,以确定潜在的恶性肿瘤可能是诱发因素。
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