非典型的GFAP Astrocytopathy

客观的描述的anti-GFAP astrocytopathy与非典型特征。

背景胶质原纤维酸性蛋白(GFAP) astrocytopathy steroid-responsive自身免疫性脑膜脑炎,通常表现为前病毒性疾病其次是脑病和视神经乳头炎对视力没有显著影响。我们描述一个典型的GFAP astrocytopathy呈现与深刻的视力丧失和颅内高血压。

设计/方法病例报告

结果31你男性有高血压病史的流感样症状的一周。随后,他经验丰富的视物模糊,提出去医院。他被发现高血压急性肾损伤。他是治疗高血压的紧迫性和排放。然而,病人的视力继续恶化,他威胁视觉幻觉。他重新去医院。眼科检查发现两国在丧失视力,视网膜出血和严重视神经乳头水肿。他的实验室检查是著名的LP发现淋巴细胞白血球增多,蛋白质和开启压力升高54。他开始对乙酰唑胺和转移到三级医疗中心。在那里,他发达与精神病性脑病。 An extensive infectious/ autoimmune/malignancy workup was completed. This included three repeat LPs showing persistent intracranial hypertension, lymphocytic pleocytosis, high protein and oligoclonal bands. He underwent imaging with CT chest/abdomen/pelvis, MRV and MRI head/orbits, which showed no evidence of malignancy, patent vasculature and pachymeningeal enhancement, respectively. His work-up was notable for positive IgG GFAP in CSF leading to diagnosis of GFAP astrocytopathy. He was treated with pulse dose IV steroids followed by slow steroid taper and concurrent PLEX therapy. Due to severity of case, he was later started on cyclophosphamide. For papilledema, he was continued on acetazolamide and underwent bilateral optic nerve sheath fenestration. Overall, his encephalopathy resolved but vision remained poor.

结论本例中描述了一个独特的表现GFAP astrocytopathy包括快速的视力丧失,持久颅内高血压和缺乏特色的磁共振成像结果。