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目的:比较中枢神经系统血管炎(central nervous systemvasculitis,CNSV)与线粒体脑肌病(MELAS型)临床及影像学特点,分析二者的鉴别诊断特点。方法:回顾性分析我科收治的7例CNSV患者和5例线粒体脑肌病(MELAS型)患者的临床资料,比较二者临床表现、实验室检查指标、影像学表现及组织学分析的特点,筛选二者的鉴别诊断要点。结果:CNSV患者和线粒体脑肌病(MELAS型)患者头颅MRI检查均为多发病灶。CNSV临床表现以头痛、肢体无力及变态反应改变为主;线粒体脑肌病(MELAS型)以癫发作、血清乳酸升高为主要特点。两组疾病的脑电图均异常:CNSV患者以弥漫损害为主,伴局限性改变;线粒体脑肌病患者可捕捉到性放电证据,与临床表现基本一致。结论:CNSV和线粒体脑肌病的临床鉴诊较影像学更有意义,CNSV表现为血管病损害和炎症反应,线粒体脑肌病表现为能量代谢障碍,脑灰质损害更为突出,最终诊断依赖于实验室和组织学检查。
Objective: To compare the clinical features and imaging features of central nervous system vasculitis (CNSV) and mitochondrial encephalomyopathy (MELAS), and to analyze their differential diagnostic features. Methods: The clinical data of 7 patients with CNSV and 5 patients with mitochondrial encephalomyopathy (MELAS) who were treated in our department were analyzed retrospectively. The clinical manifestations, laboratory tests, imaging findings and histological features of the two patients were compared. Screening the two differential diagnosis points. Results: The cranial MRI in patients with CNSV and in patients with mitochondrial encephalomyopathy (MELAS) were all multifocal. CNSV clinical manifestations to headache, limb weakness and allergy changes; mitochondrial encephalomyopathy (MELAS type) to epileptic seizures, elevated serum lactate as the main features. The EEGs were abnormal in both groups of diseases: mainly diffuse lesions in CNSV patients with limited changes; mitochondrial encephalomyopathy patients could capture the evidence of pseudo-discharge, which is consistent with the clinical manifestations. Conclusion: The clinical diagnosis of CNSV and mitochondrial encephalomyopathy is more meaningful than imaging. The CNSV manifests as vascular injury and inflammatory reaction. Mitochondrial encephalomyopathy manifests as energy metabolism disorders, and the damage of cerebral gray matter is more prominent. The final diagnosis depends on Laboratory and histological examination.