患者女,57岁,因“精神异常伴左侧肢体无力12天”入院,住院期间行头颅MR平扫及MRA检查(图1A~D),生化检查显示血糖及乳酸增高。给予静滴葡萄糖加ATP和辅酶A注射液治疗后,病情好转。患者既往有神经性耳聋十多年,糖尿病史3年,家族母亲及兄妹5人,其中患者大姐死于脑部疾病(具体不详),家族中3人仅表现为神经性耳 Female patient, 57 years old, due to “mental abnormalities with left limb weakness 12 days” hospital admission, MR skull MR scan and MRA examination (Figure 1A ~ D), biochemical tests showed elevated blood glucose and lactate. Give intravenous glucose plus ATP and coenzyme A injection treatment, the condition improved. In the past, he had deafness for more than ten years, had a history of diabetes for three years, and five mothers and siblings. Among them, the eldest sister died of brain disease (unknown in detail), and only three of the family members showed neurogenic ears