线粒体脑肌病的影像诊断价值

The imaging diagnostic value of mitochondrial encephalomyopathy

目的 探讨多种影像技术对线粒体脑肌病的诊断价值。方法 回顾性分析 11例线粒体脑肌病的临床资料和影像学表现。本组病例 9例行MR扫描 ,其中 2例并行MR血管成像检查和数字减影血管造影 ,3例行CT平扫检查 ,1例行正电子发射计算机体层成像。结果 CT和MR显示9例脑内病变为多发性或游走性脑梗死样病灶 ,主要累及颞枕叶后部 ,病变与血管分布区无关 ,7例发生于颞顶枕叶 ,位于双侧丘脑 1例 ,脑干、小脑、侧脑室旁 1例。另外小脑萎缩 1例 ,胸髓空洞 1例。急性期PET显示病灶中葡萄糖代谢明显升高 ,缓解期转为低代谢。结论 结合多种影像学方法检查线粒体脑肌病 ,可提高诊断准确性。

Objective To evaluate the role of several imaging technology in the diagnosis of patients with mitochondrial encephalomyopathy.Methods Clinical and image findings of 11 cases of mitochondrial encephalomyopathy were retrospectively analyzed.9 patients were studied with MR,2 patients with MRA and DSA,3 patients with CT plain scan,and 1 patient with positron emission tomography (PET).Results CT and MR imaging showed multiple or migrating infarct-like lesions in 9 cases,mainly involving the posterior temporal and occipital regions which crossed vascular boundaries.Lesions were distributed in the temporo- occipital and parietal lobes in 7 cases,bilateral thalamus in 1 case,the brainstem,cerebellum,and the deep cerebral white matter in 1 case.Other lesions demonstrated as cerebellum atrophy in 1 case,syringomyelia in thoracic spinal cord in 1 case.PET showed the metabolic rate of glucose was elevated early in the acute phase and reduced in the recovery phase.Conclusion The examination combined with several imaging methods will help to improve the imaging diagnosis.