Hallervorden-Spatz综合征的临床研究(附5例病例报告)  

Clinical research on Hallervorden-Spatz syndrome

作　　者：许二赫[1] 林一聪[1] 王宪玲[1] 黄小钦[1] 贾建平[1] 

机构地区：[1]首都医科大学宣武医院神经内科,北京100053

出　　处：《中风与神经疾病杂志》2012年第11期991-994,共4页Journal of Apoplexy and Nervous Diseases

摘　　要：目的分析Hallervorden-Spatz综合征的临床,辅助检查及影像学特点。方法回顾性分析了5例Hallervorden-Spatz综合征患者的临床表现和辅助检查结果及影像学特点。5例患者均进行了MRI检查,其中第5例患者还进行了外周血涂片和脂蛋白电泳的检查。结果 5例患者均表现出椎体外系症状和典型的"虎眼征"。其中有4例患者起病年龄早,均在20岁以前,另一例患者30岁起病。结论影像学特点("虎眼征")和临床症状是临床实践中明确诊断HSD的重要证据,可以结合症状适当做外周血涂片以及脂蛋白电泳便于分型或与其他疾病相鉴别。Objective To analysis clinical characteristic,laboratory results and imaging of Hallervorden-Spatz syndrome（HSS）.Methods The clinical characteristics,laboratory results and imaging of 5 cases of HSS were analyzed.All of them undertook MRI exams and one of them took blood smear and lipoprotein electrophoresis tests.Results All patients had extrapyramid manifestations and typical ＂the eye of the tiger＂ appearance.Four of them were not more than twenty years at the onset and the others was thirty.Conclusion The imaging characteristic（the eye of the tiger）and clinical manifestations are important for definite diagnosis.We can differentiate HSS from others disease and classify it based on blood smear and lipoprotein electrophoresis.

关 键 词：HALLERVORDEN-SPATZ综合征 磁共振成像 虎眼征 

分 类 号：R749.13[医药卫生—神经病学与精神病学]