Yu Gang, Peng Guo-guang, Wang Li, Dong Wei-wei, Deng Qiu-ming
Department of Neurology, the First Affiliated Hospital, Chongqing University of Medical Sciences, Chongqing 400016, China.
Zhonghua Nei Ke Za Zhi. 2003 Dec;42(12):843-6.
To investigate the clinic characters and diagnosis of Susac syndrome.
Two cases were systematically studied by the authors.
All the two cases were young women. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid-to-low frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2-weighted brain magnetic resonance imaging.
This rare syndrome often can be identified at an early stage with a careful history and physical examination. MRI, SPECT, retinal fluorescein angiography and audiometry will contribute to diagnosis.
探讨Susac综合征的临床特征及诊断方法。
作者对2例患者进行了系统研究。
2例均为年轻女性。临床表现包括急性和亚急性多灶性及弥漫性脑病症状、听力丧失和视力丧失。视网膜动脉闭塞而无葡萄膜炎或角膜结膜炎、中低频单侧或双侧感音神经性听力丧失以及T2加权脑磁共振成像上白质和灰质中多个信号增强的小病灶有助于诊断。
这种罕见综合征通过仔细的病史询问和体格检查通常可在早期得以识别。磁共振成像、单光子发射计算机断层扫描、视网膜荧光血管造影和听力测定有助于诊断。