中枢神经系统血管炎与线粒体脑肌病的诊断与鉴别诊断

目的: 探讨中枢神经系统血管炎和线粒体脑肌病（MELAS型）临床与影像学鉴别。方法: 分别对中枢神经系统血管炎病7例和线粒体脑肌病（MELAS型）5例进行临床、实验室、影像学和组织学分析。结果: 两组疾病影像学检查极其相似，临床表现中枢神经系统血管炎体现的是血管和炎症反应，头痛、肢体无力以及变态反应改变更为突出；线粒体脑肌病体现的是能量代谢障碍，脑的灰质损害更为突出，癫痫发作，血清乳酸升高。结论: 最终诊断依赖于实验室和组织学检查。

Differential diagnosis of central nervous system vasculitis and mitochondrial encephalomyopathy

Objective:To compare the clinical and imaging characteristics between central nervous system vasculitis(CNSV)and mitochondrial encephalomyopathy (ME),so as to analyze the differential diagnosis of the two disorders.Methods: Clinical data on seven patients with CNSV and five with ME were retrospectively analyzed.The clinical manifestations,laboratory parameters,imaging features and histological characteristics were compared to screen for the evidence of their differential diagnosis.Results: The MRI results of both CNSV patients and ME patients (MELAS type) showed a multi-lesion pattern.The symptoms of CNSV patients included headache,limbs weakness,and erythrocyte sedimentation rate (ESR) increase.The symptoms of MELAS patients included epilepsy and increased serum lactic acid.The electroencephalographic manifestations of both diseases were abnormal:CNSV patients mainly had diffused lesions accompanied with limited alterations; ME patients had evidence of epileptic discharge,which was consistent with the clinical symptoms.Conclusion: Clinical manifestations of CNSV and ME patients are more valuable than imaging findings in the diagnosis of the two diseases.CNSV is characterized by vascular disorders and inflammatory reactions; ME is characterized by abnormal energy metabolism and severe damage of gray matter.The final diagnosis should depend on laboratory and histological examinations.