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线粒体脑肌病是一组由于线粒体功能障碍导致的一种多系统疾病。线粒体肌病的临床表现多样、复杂且发病率低，但因多伴精神症状而被精神科接诊。本文中我们分析1例以精神行为异常为主诉的线粒体脑肌病伴高乳酸血症和卒中样发作综合征的临床资料、诊疗过程，结合文献复习该病的临床特点、诊查手段，以提高临床医师对其认识，避免误诊误治的发生。“,”Mitochondrial encephalomyopathy (ME) is a group of multisystem disorders caused by mitochondrial dysfunction. The clinical manifestations of ME are diverse, complex and the prevalence of the disease is low. However, ME patients generally first visit the mental health department for their complicated psychiatric symptoms. This paper introduced the clinical information, diagnosis and treatment of a case who suffered from mitochondrial encephalomyopathy presenting with lactic acidosis and stroke-like episode （MELAS） with psychiatric complications, and further reviewed the literatures on the clinical features and the diagnostic methods of the disease to raise the clinicians′ awareness and to avoid any misdiagnosis and mistreatment.