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目的探讨MELAS综合征的MRI表现特点。方法对13例MELAS综合征患者的临床与影像学资料进行回顾性分析。结果MRI表现为长T1长T2信号,多位于颞、顶、枕叶皮层,病变形态、大小、范围易变,呈游走性,多灶性,不按血管支配区分布。MRA没有大血管闭塞。常见基底节钙化,伴脑室扩大和脑萎缩。DWI病变多表现为ADC值升高。病灶MRS检查可见Lac峰。结论MELAS综合征的影像学表现具有一定特点,根据临床及影像学特点,结合肌肉活检可对该病做出正确诊断。
Objective To investigate the MRI features of MELAS syndrome. Methods The clinical and imaging data of 13 patients with MELAS syndrome were analyzed retrospectively. Results MRI showed long T1 and long T2 signals, mostly located in the temporal, apical and occipital cortices. The morphology, size and range of the lesions were variable. They were migratory and multifocal, and were not distributed according to the area of vascular domination. MRA no major vascular occlusion. Common basal ganglia calcification, with ventricular enlargement and brain atrophy. DWI lesions showed increased ADC values. Lesions MRS Lac peak can be seen. Conclusion The imaging findings of MELAS syndrome have certain characteristics. Based on the clinical and imaging features, combined with muscle biopsy can make a correct diagnosis of the disease.