Joubert综合征的CT和MRI表现及诊断价值

CT and MRI features of Joubert syndrome and their diagnostic values

目的 :探讨Joubert综合征临床表现及CT、MRI表现,提高对Joubert综合征的认识。方法 :对4例Joubert综合征患儿临床资料及MRI、CT表现进行回顾性分析,并复习相关文献。其中3例行MRI检查,1例行螺旋CT扫描。结果:小脑半球间"中线裂",蝙蝠翼状、三角形第四脑室以及中脑臼齿征为Joubert综合征的典型MRI及CT表现。结论:Joubert综合征MRI及CT表现具有一定的特征性,对临床诊断Joubert综合征具有重要价值。

Objective To explore the clinical, CT and MRI features of Joubert syndrome to improve the knowledge on the disease. Methods Four cases with Joubert syndrome had their clinical data and CT and MRI manifestations retrospectively analyzed, of which three ones underwent MR imaging and one case went through spiral CT scanning. Literature survey was carried out. Results It＇s found that Joubert syndrome had CT and MRI features as midline cleft between bilateral cerebellar hemispheres, bat wing and triangular-shaped fourth ventricular as well as molar tooth sign of the midbrain.Conclusion Joubert syndrome shows specificity in its MRI and CT features which are of great significance for its diagnosis.