目的 探讨伴预激综合征的线粒体脑肌病伴高乳酸血症和卒中样发作(mitochondrial encephalopathy,lactic acido-sis,and stroke-like episodes,MELAS)综合征的临床及肌肉病理特征.方法 回顾性分析5例伴有预激综合征的MELAS综合征患者的临床特征、心电图、颅脑MRI及肌肉病理等.结果伴有预激综合征的MELAS综合征患者中女性2例,男性3例;发病年龄6～20岁,平均(12.4±4.6)岁;其中癫痫是最常见的首发症状(80％);颅脑MRI均显示异常,以枕颞顶叶受累为主;肌肉病理示5例患者均可见强反应性血管现象(strong reactive vessel,SSV),3例可见破碎红纤维;基因检测示5例均存在A3243G点突变.结论伴预激综合征的MELAS综合征患者临床以癫痫为首发症状最多见,颅脑枕、颞叶均有损害,肌肉病理均存在SSV,基因突变均是A3243G点突变.

神经心理学是研究人类心理活动神经机制、高级认知功能的一门学科,是通过研究脑损害患者高级认知功能,加深对脑的各种疾病症状谱认识,通过神经心理学检测为认知功能康复寻找突破口.更为重要的是,脑结构和功能的问题是当今科学时代的研究热点,神经心理学研究对脑科学发展具有重要的意义.中医院校毕业的学生主要从事临床中医医疗工作,服务对象是病人,而病人的神经心理变化,直接关系到就诊的最终预后,故学习神经心理学对于医学生有着重要意义.神经心理学涉及神经病学、精神病学、心理学等多学科,具有更新较快、实践性强等特点,如何让医学生在轻松易懂的过程中学好神经心理学,是每一位教学工作者一直努力追求的.论文从中医院校的神经心理学教学中目前存在问题、对策措施及教学效果进行思考和建议.

Objective To report a case of peripheral neuropathy secondary to copper deficiency (CD) by long‐term decoppering chelation in Wilson′s disease (WD) to enhance understanding of the disease, and to pay more attention to individualized treatment of WD. Methods A case of WD diagnosed 12 years ago confirmed by gene detection and since then treated with anti‐copper agent was diagnosed as CD based peripheral neuropathy and significant neutropenia and followed up for six months, and the clinical manifestations, laboratory examination, electrophysiology, imaging features were summarized. The related literatures were reviewed. Results A total of 16 cases of WD complicated with CD were reviewed and analyzed, including seven males and nine females aged 13-56 years. All of them were treated with zinc for 1-38 years, and nine cases with peripheral neuropathy. Hematological indicators can be significantly improved and neurological symptoms can be partially alleviated after stopping copper removal treatment. Conclusions Peripheral neuropathy in a WD with treatment‐related CD may occur in blind treatment, irregular treatment monitoring and without individualized treatment adjustment. It is necessary to monitor blood routine, copper and zinc metabolism regularly and advocate individualized treatment of WD.

磷脂酶 A2 第 6 型基因(phospholipase A2 type 6, PLA2G6 )突变引起的疾病具有高度遗传与临床异质性,根据其临床表现归类为肌张力障碍—帕金森综合征(dysto-nia-parkinsonism,DP)、常染色体隐性遗传早发性帕金森综合征和早发性帕金森病[1].DP 患者多于青年期起病,临床主要表现为肌张力障碍,首发症状可为精神障碍、认知功能减退等,早期易误诊为精神分裂症,用抗精神病药后肌张力障碍可能被诱发或者加重,治疗主要是改善肌张力和控制精神症状,总体预后一般[2].现报告我院收治的1例以精神症状起病的帕金森综合征患者.

Objective To investigate the requirement and satisfaction of medical students on the experimental teaching of medical statistics. Methods 325 students were selected in a medical university of Anhui Province.Through a semester of learning in the course of medical statistical experiment,the requirement and satisfaction of medical students on the experimental teaching of medical statistics were investigated.Results 68.3% of the students required the teachers with master degree.80.0% of the students required associate professors or professors for teaching.75.4% of students thought that teachers aged 30 to 50 years old was appropriate.86.5% of the students hoped that experimental program should use the combination method of multimedia and traditional teaching.86.8% of students want to carry out experimental course by interactive discussion.96.9% of the students thought that there should be more using of case-report teaching.70.7% of students thought that three classes can increase the efficiency of learning.72.4% of the students thought that 30 to 40 minutes explaining the experimental course was accepted.89.8% of students called for more advanced statistical techniques and knowledge.72.9% of students want to increase the software content in the experimental teaching.96.9% of students understood the importance of the experimental teaching.94.4% of students hoped to get the guide of teacher in the experimental teaching.56.3% of students were satisfied with the teachers.Conclusions The quality of the experimental teaching of medical statistics should be improved.It is still need to improve the ability of teachers,the design of curriculum, and the method of teaching.