线粒体脑肌病的MRI诊断价值

目的 探讨线粒体脑肌病的脑部MRI表现及其诊断价值。资料与方法 对13例线粒体脑肌病患者的临床、实验室及MRI表现进行回顾性分析。结果 13例头部MRI检查均显示异常，对病变的检出率为100％，主要MRI表现为3类：（1）大脑半球多发单侧或双侧病变（7例），4例呈对称性，病变呈片状等长T1、长T2信号，以一侧或两侧颞顶枕时皮层和皮层下白质最常受累。（2）大脑半球皮层和深部灰质核团同时出现片状等长T1、长T2信号2例。上述两类脑内病变有5例合并脑萎缩。（3）脑实质信号正常，但有脑萎缩4例，其中桥脑、延髓及小脑萎缩3例，大脑半球、脑干、小脑萎缩1例。结论 MRI对线粒体脑肌病的脑内病变显示敏感且准确，对其早期诊断、指导治疗、判断疗效和提示预后具有重要价值。

MR Diagnosis of Mitochondrial Encephalomyopathy

Objective To evaluate the MRI findings and diagnostic value of MRI in mitochondrial encephalomyopathy.Materials and Methods Clinical findings, laboratory examination and MRI manifestations of 13 cases with mitochondrial encephalomyopathy were retrospectively analyzed.Results There were abnormal findings on intracranial MRI, the detection rate was 100%, MRI findings were characterized by followings: type 1: lesions of unilateral or bilateral cerebral hemisphere in 7 cases, including symmetrical lesions in 4 cases, the cerebral foci showed long T_1 and T_2 signal and occipital lobe cortex and subcortical white matter were mostly involved (7/13); type 2: abnormal long T_1 and T_2 signal in cerebral cortex and deep gray matter were detected in 2 cases (2/13); type 3: cerebral matter signal was normal, there were 4 cases with cerebral atrophy (4/13), 3 cases were suffered from pontine, medullary and cerebellar atrophy and 1 case with cerebral hemisphere, brain stem and cerebellar atrophy. 5 cases were characterized by diffuse lesions complicated by cerebral atrophy.Conclusion MRI is sensitive to the intracranial lesions in patients with mitochondrial encephalomyopathy, which is of great value in early diagnosis, instructing treatment, judging therapeutic effect and prognosis.