Wu Chen-Hao, Chai Jyh-Wen, Lee Chen-Hui, Chen Wen-Hsien, Lee Tan, Chen Clayton Chi-Cheng
Department of Radiology, Taichung Veterans General Hospital, Taiwan, ROC.
J Chin Med Assoc. 2006 Jul;69(7):338-42. doi: 10.1016/S1726-4901(09)70270-9.
Lhermitte-Duclos disease (LDD) is a rare benign lesion of uncertain pathogenesis characterized by distortion of the normal cerebellar laminar cytoarchitecture. We report a 22-year-old man admitted for injury sustained in a traffic accident with the incidental finding of a cerebellar mass. Magnetic resonance imaging (MRI) revealed a mass lesion within the right cerebellar hemisphere. The final diagnosis of LDD was made by obtaining a surgical specimen and identifying the characteristic appearance of the lesion by MRI study. The images showed the typical striated pattern of hyperintensity on T2-weighted images and corresponding hypointensity on T1-weighted images, as well as the typical absence of enhancement following gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA) administration. In addition, no disturbance of water diffusion on diffusion-weighted MRI, and associations of decreases in the N-acetylaspartate/creatine (NAA/Cr) and NAA/Choline (Cho) ratios with near normal values of Cho/Cr, as well as an obvious lactate peak gave supplemental information for diagnosis.
Lhermitte-Duclos病(LDD)是一种发病机制不明的罕见良性病变,其特征是正常小脑层状细胞结构扭曲。我们报告一名22岁男性因交通事故受伤入院,偶然发现小脑肿块。磁共振成像(MRI)显示右侧小脑半球有一个肿块病变。通过获取手术标本并通过MRI研究确定病变的特征性表现,最终诊断为LDD。图像显示在T2加权图像上有典型的高信号条纹状图案,在T1加权图像上相应的低信号,以及在给予钆喷酸葡胺(Gd-DTPA)后典型的无强化。此外,扩散加权MRI上无水分子扩散受限,N-乙酰天门冬氨酸/肌酸(NAA/Cr)和NAA/胆碱(Cho)比值降低且Cho/Cr值接近正常,以及明显的乳酸峰为诊断提供了补充信息。