Papeix C, Laloum L, Richet A, Ayache D, Moulignier A, Héran F, Bakouche P, Gout O
Service de Neurologie, Fondation Ophtalmologique Adolphe de Rothschild, Paris.
Rev Neurol (Paris). 2000 Sep;156(8-9):783-5.
We report a case of Susac's syndrome characterized by subacute encephalopathy, bilateral hearing loss and multiple bilateral branch retinal artery occlusions in a forty-year-old-white-woman. Brain Magnetic Resonance Imaging showed on T2-weighted images multiple, punctate areas of increased signal intensity in periventricular white matter, gray matter and brainstem most of them being enhanced by gadolinium. Cerebrospinal fluid was acellular but with an increased protein level (1.66 g/l). Treatment with cyclophosphamid and intravenous immmunoglobulin resulted in dramatic improvement of the clinical status over the following months and CSF normalization.
我们报告了一例Susac综合征病例，该病例发生在一名40岁的白人女性身上，其特征为亚急性脑病、双侧听力丧失以及多发性双侧视网膜分支动脉阻塞。脑磁共振成像在T2加权图像上显示，脑室周围白质、灰质和脑干中有多个点状信号强度增加区域，其中大多数区域经钆增强。脑脊液无细胞，但蛋白水平升高（1.66 g/l）。在接下来的几个月里，使用环磷酰胺和静脉注射免疫球蛋白进行治疗，使临床症状显著改善，脑脊液恢复正常。
