Joubert综合征的MRI及DTI表现(附2例报告并文献复习)  

MRI and DTI Findings of Joubert Syndrome(A Report of 2 Cases and Review of the Literature)

作　　者：李滨[1] 岳云龙[1] 金延方[1] 刘洪亮[1] 梁竸[1] 

机构地区：[1]首都医科大学附属北京世纪坛医院核磁共振室,100038

出　　处：《临床放射学杂志》2012年第3期446-448,共3页Journal of Clinical Radiology

摘　　要：目的探讨Joubert综合征的MRI及扩散张量成像(DTI)表现,提高对该病的认识。资料与方法回顾性分析2例Joubert综合征患儿的临床资料、MRI及DTI表现。结果 Joubert综合征的MRI表现包括小脑"中线裂"、中脑"臼齿征"和第四脑室扩大。DTI显示小脑上脚纤维束孤立单侧走行,在中脑内缺乏左右走向水平交叉。结论 Joubert综合征的常规MRI征象具有特征性,DTI可以直接观察纤维束走行异常,提供解剖学证据。Objective To discuss the MRI and DTI findings of Joubert Syndrome（JS）.Materials and Methods Clinic,MRI and DTI data of two cases with Joubert Syndrome were analyzed retrospectively.Results In two cases with JS,typical MR imaging features of ＂midline cleft＂ between cerebellar hemispheres,midbrain ＂molar tooth sign＂and enlargement of fourth ventricle were depicted.DTI demonstrated that the fibers of superior cerebellar peduncules did not decussate in the mesencephaon.Conclusion Joubert Syndrome has some MRI characteristics.DTI can directly observe abnormality of fiber tracts.which is helpful to provide the evidence of anatomy.

关 键 词：脑疾病 JOUBERT综合征 磁共振成像 扩散张量成像 

分 类 号：R445.2[医药卫生—影像医学与核医学]