Kufs病的MRI和CT诊断  

MRI and CT manifestations of Kufs′disease

作　　者：谭利华[1] 李德泰[1] 彭隆祥[2] 卢伟[3] 刘辉[1] 周顺科[1] 徐锡萍[2] 吴晓英[2] 张淑安[2] 

机构地区：[1]中南大学湘雅二医院放射科,长沙410011 [2]中南大学湘雅医学院电镜室 [3]中南大学湘雅二医院神经内科,长沙410011

出　　处：《中华放射学杂志》2002年第6期520-522,共3页Chinese Journal of Radiology

摘　　要：目的　提高对Kufs病MRI及CT表现的认识。方法　报告 4例经病理证实的Kufs病的MRI和CT所见 ,并作文献复习。结果　双侧大脑半球及小脑皮层轻度萎缩 1例 ,大脑半球轻度萎缩1例 ,MRI显示桥脑、双侧尾状核、丘脑及屏状核有特征性的局灶长T1、长T2 信号 ,无强化 1例 ,MRI示双侧丘脑局灶异常信号灶 1例。结论　Kufs病MRI和CT主要表现为不同程度脑萎缩 。Objective To enhance the understanding of MRI and CT findings in Kufs′s disease. Methods The MRI and CT features in 4 cases of Kufs′ disease confirmed by pathology were reported, and literature was reviewed. Results Various degrees of cerebral and cerebellar atrophy were seen in 1 patient, and only the cerebral atrophy was found in another case with MRI and CT. The lesions in the pons, both caudate nuclei, thalamus, and claustra were hypointense to gray matter on T 1WI and hyperintense on T 2WI, and the lesions showed no enhancement after Gd DTPA injection in 1 case.The local abnormal signal lesions were seen on both thalamus on MRI in another case. Conclusion Cerebral and cerebellar atrophy can be found on MRI and CT in most Kufs′ diseases and characteristic signs can be seen on MRI in some patients′ brain.

关 键 词：神经元蜡样质脂褐素增多症 磁共振成像 X线计算机体层摄影术 Kufs病 CT表现 MRI表现 

分 类 号：R445.2[医药卫生—影像医学与核医学]