目的探讨线粒体脑病的误诊原因。方法收集经MRI检查,实验室检查及临床病理证实的线粒体脑病7例,对其脑部MRI表现进行回顾性分析。结果 7例线粒体脑病4例被误诊,MRI显示病灶直观,有一定特征性。结论 MRI有助于线粒体脑病的诊断,可疑病例应询问病史,结合生化检查及受累组织活检,可尽早诊断。 Objective To investigate the causes of misdiagnosis of mitochondrial encephalopathy. Methods Seven cases of mitochondrial encephalopathy confirmed by MRI examination, laboratory examination and clinical pathology were collected. The MRI findings of the brain were retrospectively analyzed. Results 7 cases of mitochondrial encephalopathy were misdiagnosed in 4 cases, MRI showed that the lesions were intuitive and had certain characteristics. Conclusion MRI contributes to the diagnosis of mitochondrial encephalopathy. Suspicious cases should be asked about the medical history, combined with biochemical examination and biopsy of affected tissues, can be diagnosed as soon as possible.