摘要
目的线粒体脑肌病伴高乳酸血症及卒中样发作(MELAS)是最常见的线粒体脑肌病类型之一,具有明显的临床和遗传异质性。MELAS最显著的核心症状是卒中样发作,容易与缺血性脑卒中混肴。方法通过分析10例曾误诊为MELAS的青年脑梗死患者的临床表现和辅助检查,并与MELAS患者相对比。同时结合文献复习,分析卒中发作与卒中样发作的相同与不同之处。结果所有10例患者均出现一次以上卒中发作,以偏侧肢体无力最常见,其次为偏侧肢体麻木、偏盲、失语等。其中8例患者存在一种以上血管病相关的危险因素。头部MRI发现皮质和皮质下白质病变,均累及多个脑叶,数月后复查可见软化灶形成。所有患者均同时存在基底节和/或深部白质病变。MRA均发现动脉闭塞或狭窄。结论本组患者卒中发作以偏瘫最常见。影像学上以皮质受累为主,同时常出现深部白质和/或基底节病变,后期可形成软化灶。
Objective Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes（MELAS） is one of the most common mitochondrial encephalomyopathy with clinical and hereditary heterogeneity.Stroke-like episodes are the most distinctive core features of MELAS,which are easily confused with ischemic stroke.Methods Ten patients of ischemic stroke in young adults mimicking MELAS were analyzed in terms of clinical,laboratory and radiological data to detect the similarity and difference of stroke and stroke-like episodes.Meanwhile,corresponding literatures were reviewed.Results Ten patients all had episodes of stroke at least once,with hemiparesis being the most common presentation followed by hemianesthesia,hemianopsia and aphasia.More than one kind of vascular risk factors were detected in 8 patients.Both cortex and subcortical white matter lesions coexisted in all patients involving multiple lobes,which evolved into cerebral malacia several months later.Simultaneously,basal ganglia and/or deep white matter were involved.Magnetic resonance angiography disclosed stenosis or occlusion of intracranial arteries.Conclusion Hemiparesis was the most common clinical type of our patients.The radiological findings showed multiple lesions located mainly at cerebral cortex,also at deep white matter and basal ganglia,which could turn into malacia later.
出处
《中风与神经疾病杂志》
北大核心
2017年第10期910-913,共4页
Journal of Apoplexy and Nervous Diseases
关键词
线粒体脑肌病伴乳酸血症及卒中样发作
缺血性脑卒中
青年卒中
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes
Ischemic stroke
Stroke in young adults
