Abstract:
A 59-year-old man had developed visual abnormality, nausea, headache, and weight loss since three months before. The ophthalmologist found severe optic disc edema in both eyes, and referred him to our hospital. The patient had mild cerebellar ataxia. Increased cerebrospinal fluid pressure, increased protein and cell counts, positive oligoclonal band, and contrast-enhanced head MRI showed multiple linear perivascular radial gadolinium enhancement around bilateral lateral ventricles. His subjective and objective findings significantly improved with steroid treatment. The cerebrospinal fluid was found to be positive for glial fibrillary acidic protein (GFAP) antibodies, and a diagnosis of GFAP astrocytopathy was obtained. When optic edema or radial contrast effects was observed on contrast-enhanced MRI, GFAP astrocytopathy should be considerd. Prompt immunotherapy is required to circumvent the development of permanent visual impairment.
摘要:
一名59岁的男子出现了视觉异常,恶心,头痛,从三个月前开始减肥。眼科医生发现双眼都有严重的视盘水肿,把他转到我们医院.患者有轻度小脑共济失调。脑脊液压力升高,增加蛋白质和细胞计数,正寡克隆带,和对比增强的头部MRI显示双侧侧脑室周围的多个线性血管周围放射状钆增强。通过类固醇治疗,他的主观和客观发现显着改善。发现脑脊液对胶质纤维酸性蛋白(GFAP)抗体呈阳性,并诊断为GFAP星形细胞病。当在对比增强MRI上观察到视神经水肿或径向对比效应时,应考虑GFAP星形细胞病。需要及时的免疫疗法来避免永久性视力障碍的发展。