帕金森病患者不同脑区质子磁共振波谱研究

目的:探讨~1H-MRS对早期PD的诊断价值。方法:采用~1H-MRS技术检测早期PD、晚期PD、帕金森综合征、偏侧ET以及年龄匹配的正常对照组基底节区和皮质运动区感兴趣区的NAA/Cr和Cho/Cr比值。结果:早期或晚期PD、偏侧ET及帕金森综合征患者受累较重基底节区或运动皮质区NAA/Cr及Cho/Cr比值与受累较轻侧相比无显著性差异;上述各组患者受累较重基底节区或运动皮质区NAA/Cr及Cho/Cr比值与正常对照组相应感兴趣区NAA/Cr及Cho/Cr比值相比也均无显著性差异。结论:~1H-MRS无助于早期PD的诊断。     

β淀粉样蛋白_（1-42）促进转染人α-突触核蛋白A53T突变基因的PC12细胞内α-突触核蛋白的表达

目的：探讨β淀粉样蛋白1-42（Aβ1-42）促进转染人α-突触核蛋白（α-Syn）A53T突变基因的PC12细胞（A53T细胞）内α-Syn的表达及维生素C对A53T细胞保护作用的机制。方法：对高表达α-Syn的A53T细胞分别采用PBS、Aβ1-42和Aβ1-42＋维生素C干预。用MTT法检测细胞活性和Western blot检测不同干预后A53T细胞α-Syn表达量。结果：①随着Aβ1-42浓度增加,细胞活性呈浓度依赖性降低;维生素C可减轻细胞损伤。②Western blot检测结果提示维生素C可减轻α-Syn的谱带密度;Aβ1-42干预后A53T细胞内α-Syn表达量相对值为（0.76±0.13）,维生素C干预后α-Syn表达量相对值为（0.23±0.08）,两者差异有统计学意义（P〈0.01）。结论：Aβ1-42可导致高表达α-Syn的A53T细胞损伤,并促进α-Syn的表达;维生素C可部分拮抗该效应,对神经细胞具有一定的保护作用。     

MRI环形增强的颅内占位病例讨论

病例资料患者住院号:699×××,女性,42岁,汉族,福建省人。因"头痛1个月,反复肢体抽搐25 d"于2011年4月11日入住复旦大学附属华山医院(我院)神经科。现病史1个月前(2011-03-10)患者因过度疲劳后出现持续性头痛,为弥漫性胀痛,伴恶心、呕吐1次(非喷射性,为胃内容物)。疼痛可忍受,无畏光、畏声,无视物变形,无发热。2011-03-15患者头痛加重,伴畏寒、     

中国汉族人群CACNA1A基因CAG重复数目分布特点及其在脊髓小脑性共济失调6型基因诊断中的应用

目的 探讨我国汉族人群CACNA1A基因CAG重复数目分布特点及其在脊髓小脑性共济失调6型(spinocerebellar ataxias type 6,SCA6)基因诊断中的应用.方法 应用"两步PCR法"、变性聚丙烯酰胺凝胶电泳(DPAGE)和测序等方法对300名健康对照及109例无血缘关系的SCA患者进行CACNA1A基因CAG三核苷酸重复数目分析.结果 300名健康对照的CAG重复次数范围为3～18次,以13次最常见.在109例SCA患者中,发现1例SCA6患者,其CAG异常重复次数为24次,该患者的母亲和哥哥亦为SCA6患者,临床上均表现为缓慢进展的小脑性共济失调、构音障碍、眼震、轻度的振动及本体觉减退,遗传早现现象较明显.结论 SCA6病例在我国较少见,进行CACNA1A基因突变分析有助于临床诊断."两步PCR法"可提高CACNA1A基因突变分析的效率.     

突触前多巴胺能PET显像结果判读要素

突触前多巴胺能PET显像是帕金森综合征诊断的有用手段。该文在2020年《多巴胺转运蛋白PET脑显像技术操作和临床应用专家共识》的基础上, 主要对突触前多巴胺能PET显像的结果判读要素予以总结和推荐。     

多巴胺能激动剂的透皮给药法

左旋多巴是目前治疗帕金森病（PD）最有效的药物，但长期使用会引起药物的疗效下降并出现相应的并发症。随着持续性多巴胺（DA）能刺激可以避免不随意运动等并发症的发生这一观念的出现，人们开始寻找能够给予DA受体持续性、生理性刺激作用的给药方法。透皮制剂不仅能够持续性给药，更是一种非创伤性的相对简单的给药方法。过去一些透皮给药治疗PD的试验并不成功，如吡贝地尔和那高利特等，但对于罗替戈汀进行的随机双盲对照试验则取得了阳性结果。DA能激动剂的透皮给药法完成了抗PD药物的第3阶段的发展，并且在不久的将来会被逐渐完善。     

胰岛素对百草枯诱导的PC12细胞的保护作用

Objective To observe dopamine (DA) D2R protein expression in the cultured PC 12 cells and the effect of insulin on the survival rate,morphology,and DA of paraquat (PQ)-induced PC12 cells. Methods Immunoprecipitate Western blotting method was performed to observe DA D2R protein expression in PC 12 cells,MTT assay was used to analyze the changes in viability and morphology of PC 12 ceils which were exposed to different concentrations of PQ and insulin. Results (1) DA D2R protein was expressed in PC 12 ceils. (2) Normal PCI2 cells bodies showed fusiform shape and the synapses were in-tegrity. The cells which were exposed to the 600 μmol/L PQ became ball-like, vacuolar degeneration oc-urred,and the synapse became shorter or disappeared. But the morphology of PC12 cells had a little difference between the normal PC12 and the insulin groups,except that the cells were fusiform shape or a-nomalism but not round shape,and the synapses grew. (3) With the increase of the concentration of PQ, the viability of the cells was decreased. Insulin increased the viability of the ceils which were exposed to 600 μmol/L PQ. Insulin elevated DA concentration both in the normal PC12 cells and those exposed to 600 μmol/L PQ,but there was no significant difference ( P > 0.05 ). Conclusion Insulin could protect the PC12 dopaminergic neurons from injury induced by PQ.     

原发性帕金森病的诊断标准（2005年）

考虑本病时必须同时具备3个条件:1.中年、老年人中逐渐出现进行性加重的活动和动作缓慢,持久活动后动作更慢、幅度更小。2.颈和(或)肢体肌张力增高。3.4～6Hz的静止性震颤或姿势不稳。确诊本病时必须在上述条件中再附加至少3个或3个以上的下列条件:1.偏侧肢体起病。2.一侧肢体受累后,较长时间才扩散到另一侧肢体,病情呈现明显不对称性。3.良好的左旋多巴试验反应(评分记分法判断,可好转70%以上)。4.左旋多巴制剂的良好疗效可持续5年以上。5.病程中体征呈现十分缓慢地进行性加重,但病程至少9年以上。6.PET、SPECT检查显示黑质纹状体区多…     

胰岛素对百草枯诱导的PC12细胞的保护作用

Objective To observe dopamine (DA) D2R protein expression in the cultured PC 12 cells and the effect of insulin on the survival rate,morphology,and DA of paraquat (PQ)-induced PC12 cells. Methods Immunoprecipitate Western blotting method was performed to observe DA D2R protein expression in PC 12 cells,MTT assay was used to analyze the changes in viability and morphology of PC 12 ceils which were exposed to different concentrations of PQ and insulin. Results (1) DA D2R protein was expressed in PC 12 ceils. (2) Normal PCI2 cells bodies showed fusiform shape and the synapses were in-tegrity. The cells which were exposed to the 600 μmol/L PQ became ball-like, vacuolar degeneration oc-urred,and the synapse became shorter or disappeared. But the morphology of PC12 cells had a little difference between the normal PC12 and the insulin groups,except that the cells were fusiform shape or a-nomalism but not round shape,and the synapses grew. (3) With the increase of the concentration of PQ, the viability of the cells was decreased. Insulin increased the viability of the ceils which were exposed to 600 μmol/L PQ. Insulin elevated DA concentration both in the normal PC12 cells and those exposed to 600 μmol/L PQ,but there was no significant difference ( P > 0.05 ). Conclusion Insulin could protect the PC12 dopaminergic neurons from injury induced by PQ.     

帕金森病的影像学研究进展

帕金森病的病理和生化研究肯定了黑质纹状体区的多巴胺能系的变性是确定本病的重要依据。在帕金森病患者中临床上通过下述许多方法取得这种依据：PET和SPECT基底节核素显像[多巴胺代谢、多巴胺转运体(DAT)、多巴胺受体等]、局部脑血流(rCBF)、葡萄糖代谢、磁共振成像(MRI)、甚至超声测量黑质一纹状体体积、磁共