中文摘要：

目的分析MRI和^1H-MRS对线粒体脑肌病的诊断价值。方法经病理活检确诊的6例成人（男3例,女3例）线粒体脑肌病患者采用1.5T磁共振仪扫描,其中3例行^1H-MRS检查,并分析MRI和^1H-MRS的表现。结果6例线粒体脑肌病患者MRI显示病变累及多个脑叶,多位于皮层及皮层下白质,并可同时累及大脑深部灰质和大脑皮层。脑梗死样病灶呈斑片状或楔形,非对称性。T1WI为低信号或等低信号,T2WI为高信号。急性期病变区域脑组织轻度肿胀,慢性期全脑萎缩,并可见皮质层状坏死。4例行MR增强扫描未见强化。3例^1H-MRS检查均显示病灶内Lac峰明显升高和NAA峰不同程度的降低。结论线粒体脑肌病MRI表现具有一定的特征,^1HMRS可提供更多脑组织代谢信息,对本病诊断具有重要的价值。

英文摘要：

Objective To analyse the diagnostic value of MRI and ^1H-MRS in mitochondrial encephalomyopathy （ME）. Methods MRI was performed in six adults （3 men and 3 women） with pathologically proved ME using 1.5T magnetor and three of them were examined by ^1H-MRS. MRI and ^1H-MRS features of the entity were retrospectively analysed. Results Lesions of ME involved multiple lobes of brain on MRI in all cases. In our study both cortex and subcortical white matter were involved. Furthermore, involvement of both the deeper gray matter and the cerebral cortex were displayed on MR simultaneously. The shape of the infarct-like lesion appeared as patch or wedge-form and dissymmetry. The lesions in all cases were hypointense or iso- to hypointense on T1WI and hyperintense on T2WI. The brain tissue around the lesions swelled lightly in acute stage and atrophy in chronic phase. No enhancement was seen in four patients after contrast injection.^1H-MRS showed prominent increase of lactate peak and decrease of N-acetylaspartic acid in the lesions.Conclusion ^1H-MRS is a useful investigating tool for ME. Besides the MRI features of ME,^1H-MRS can provide additional informations about the cerebral metabolism.