CAJ | 학술논문

目的探讨线粒体脑肌病伴乳酸血症和脑卒中发作(MELAS综合征)的临床、病理和动态影像学表现特点. 方法回顾性分析经肌肉活检病理证实的10例MELAS综合征患者的临床、病理及动态影像学资料. 结果 MELAS综合征患者的主要临床表现为头痛、癫痫、恶心呕吐、眼球震颤、视力障碍.CT显示病变较差,MR能清晰显示病变,病变多位于颞叶、顶叶、枕叶皮层及皮层下,呈脑回状,具有多灶性、非对称性、游走性、不按血管分布特点,MRA多无明显血管狭窄,病变区呈现高灌注和血管源性水肿,MRS可见乳酸(Lac)峰.肌肉病理活检光镜下显示破碎红纤维、强阳性血管,电镜下见线粒体数量增多、大小和形态异常. 结论 MELAS综合征的临床和影像学表现具有一定特点,结合肌肉病理活检可对该病进行早期诊断和鉴别诊断.
목적 탐토선립체뇌기병반유산혈증화뇌졸중발작(MELAS종합정)적림상、병리화동태영상학표현특점. 방법 회고성분석경기육활검병리증실적10례MELAS종합정환자적림상、병리급동태영상학자료. 결과 MELAS종합정환자적주요림상표현위두통、전간、악심구토、안구진전、시력장애.CT현시병변교차,MR능청석현시병변,병변다위우섭협、정협、침협피층급피층하,정뇌회상,구유다조성、비대칭성、유주성、불안혈관분포특점,MRA다무명현혈관협착,병변구정현고관주화혈관원성수종,MRS가견유산(Lac)봉.기육병리활검광경하현시파쇄홍섬유、강양성혈관,전경하견선립체수량증다、대소화형태이상. 결론 MELAS종합정적림상화영상학표현구유일정특점,결합기육병리활검가대해병진행조기진단화감별진단.
Objective To investigate the clinical, pathological and dynamic imaging characteristics of patients with mitochondrial myopathy,encephalopathy,lactic acidosis and stroke-like episodes (MELAS). Methods A retrospective analysis was performed on the clinical,pathological and dynamic imaging data of 10 patients with MELAS confirmed by muscle biopsy. Results The clinical manifestations included headache,seizures,nausea,vomiting,nystagrnus and visual disturbances.CT showed less lesions,and MRI could clearly show multiple lesions which mainly located in the temporal,parietal,occipital cortex and sub-cortex,having multifocal,asymmetric,migratory characterstics and not following the distribution of blood vessels.MRA showed no significant stenosis,and the lesion showed hyperperfusion and vasogenic edema,and Lae peak was visible.Muscle biopsy showed ragged red fiber optical microscope (RRF) and strongly SDH-reactive vessel (SSV),and electron microscope showed increased mitochondria number, and abnormal size and shape. Conclusion MELAS has certain clinical and imaging characteristics; by combining the muscle biopsy,we can diagnose the disease early and make differential diagnosis.