进行性核上性眼肌麻痹七例临床及影像学特点分析

目的 总结临床诊断为进行性核上性眼肌麻痹(PSP)患者的临床及影像学特点.方法 回顾性分析7例PSP患者的临床及影像学资料.结果 7例患者中男5例,女2例,发病年龄(62.4±5.6)岁,病程(3.7±1.2)年.其中死亡3例,失访1例.7例患者均起病隐匿,首发为运动障碍/帕金森综合征表现,逐渐加重,中晚期出现垂直性核上性眼肌麻痹、跌倒、假性球麻痹和认知障碍/痴呆.早期头颅MRI缺乏特异性;晚期2例中脑被盖部萎缩,MRI矢状位上可见中脑被盖部呈"蜂鸟征";1例中脑轴面上可见"牵牛花征"典型表现.神经保护剂及对症治疗对PSP无明显改善.结论 PSP的早期临床表现应引起重视,影像学特异性改变有助于诊断.     

痴呆行为和精神症状的诊断与治疗现状

老龄化社会的到来使痴呆成为神经精神科及老年科最为常见的问题之一[1].尽管早在1906年,Alzheimer医师即已指出,精神症状是痴呆的常见伴随症状,但长期以来尚缺乏统一的描述和定义.     

肌萎缩侧索硬化症治疗的研究进展

肌萎缩侧索硬化症是以大脑和脊髓运动神经元退行性改变为特征的进行性麻痹性疾病。目前,尚无有效治疗方法可以使肌萎缩侧索硬化症患者有实质性获益。对肌萎缩侧索硬化症患者干预的主要观点为尽早进行症状干预,包括营养支持、防止误吸、呼吸支持。随着遗传及药物的技术新进展,一些创新的治疗方法已进入临床前和临床研究。文中就肌萎缩侧索硬化症的药物治疗、非药物症状干预、新型治疗方法予以介绍。     

儿童视神经脊髓炎研究现状和进展

视神经脊髓炎(neuromyelitis optica,NMO)是一种中枢神经 系统原发炎性脱髓鞘疾病,主要特征为单发或反复发作的视神 经炎(optic neuritis,ON)和急性脊髓炎[1] .该病常难以与多发性 硬化(multiple sclerosis,MS)相鉴别,但疾病进展更快,对针对MS 的免疫调节治疗无反应.2004 年,一种NMO 特异性自身抗体即 水通道蛋白4(AQP4)抗体被发现,可以区分NMO 和其他脱髓鞘 疾病[2] ,并推动了NMO 诊断标准的更新[3] .NMO 与MS 病理机 制不同,越来越受到关注,而儿童期发病患者的发病率和临床特 征最近才有相关报道[4-6] ,其临床表现、神经影像、诊断及预后更 复杂.本文将对儿童NMO 研究现状和进展做一综述.     

高分辨率磁共振评估椎-基底动脉粥样硬化斑块对后循环缺血进展的影响

目的探讨3.0 T高分辨率磁共振(HR-MRI)评估椎-基底动脉粥样硬化斑块在后循环短暂性脑缺血发作(TIA)病情进展中的价值。方法前瞻性连续纳入2013年7月至2014年7月常熟市第一人民医院神经科门诊及住院的后循环TIA患者30例,均经头部MR血管成像证实存在椎-基底动脉粥样硬化斑块。使用3.0 T HR-MRI对椎-基底动脉斑块进行检查,评估斑块性质及稳定性,根据斑块稳定性结果分为稳定组(16例)与不稳定组(14例)。随访24个月,记录并比较两组患者首次就诊至发生后循环梗死的时间及比例。结果不稳定组发生后循环梗死时间明显短于稳定组,差异有统计学意义[11.5(7.0,20.0)个月比24.0(22.5,24.0)个月,Z=-2.755,P0.05]。稳定组和不稳定组发生后循环梗死的比例分别为5/16、8/14,组间差异无统计学意义(P0.05)。结论应用HR-MRI评估椎-基底动脉粥样硬化斑块结果对后循环缺血疾病的进展有一定参考价值。     

磁共振帕金森综合征指数在进行性核上性麻痹预后中的价值

目的研究磁共振帕金森综合征指数(MRPI)对进行性核上性麻痹(PSP)预后的提示价值。方法收集临床诊断为拟诊PSP的患者17例,行MRI检查,并计算MRPI;采用PSP评定量表(PSP-RS)评分,并与MRPI值行相关分析;对患者进行电话随访,分析MRPI值与生存时间的关系。结果 17例拟诊PSP患者PSP-RS评分为(42.2±9.4),MRPI为(17.3±4.9);MRPI与PSP-RS评分呈正相关(r=0.921,P=0.004);以MRPI 15.29为界值,MRPI>15.29患者生存时间(12.6±3.6)个月,MRPI≤15.29患者生存时间(25.0±7.3)个月(P<0.05)。结论 MRPI可以提示PSP患者的预后及生存时间。     

高清磁共振评估椎基底动脉粥样硬化斑块在后循环脑梗死预后中的应用

目的应用3.0T高分辨磁共振(HRMRI)评估椎基底动脉粥样硬化斑块,探讨其在后循环脑梗死预后中的价值。方法连续入组经磁共振血管成像(MRA)检查考虑存在椎基底动脉粥样硬化斑块狭窄的后循环脑梗死患者45例,稳定型斑块25例为稳定组,不稳定型斑块20例为不稳定组。使用3.0T HRMRI对椎基底动脉斑块进行检查,评估斑块稳定性,对治疗效果进行评估,分析斑块评估结果与预后的关系。结果稳定组基线量表得分(9.64±5.19)分,不稳定组为(9.35±5.02)分,差异无统计学意义(t=0.189,P=0.851);治疗后稳定组评分为(7.2±5.2)分,不稳定组为(10.95±6.81)分,差异有统计学意义(t=2.096,P=0.042);稳定组治疗前后差异有统计学意义(t=2.909,P=0.008),不稳定组治疗前后差异无统计学意义(t=1.367,P=0.188);稳定组治疗前后差值为(2.44±4.19),不稳定组为(-1.6±5.24),差异有统计学意义(t=2.876,P=0.006)。结论 HRMRI评估椎基底动脉粥样硬化斑块结果可提示后循环脑梗死患者预后。     

磁共振帕金森指数对进行性核上性麻痹的诊治提示

目的 研究磁共振帕金森指数(magnetic resonance Parkinsonism index,MRS)对进行性核上性麻痹(progressive supranuclear plasy,PSP)诊断及预后的提示价值.方法 搜集临床PSP 8例,额颞叶痴呆(frontotemporal dementia,FTD)5例,帕金森病(Parkinson's disease,PD)10例,多系统萎缩(multiple system atrophy,MSA)6例,行MR检查并计算MRPI值;采用PSP评定量表(progressive supranuclear plasy rating scale,PSPRS)评分,并与MRPI值行相关分析;对患者进行电话随访,分析MRPI值与生存时间关系.结果 PSP组MRPI值显著高于其余各组,PSP组数值与各组进行t检验(PD组P=0.0024;FTD组P=0.0032;MSA组P=0.0028);PSP组MRPI值与PSPRS得分呈正相关(r=0.7946,经t检验P=0.017);以MRPI值18.175分为界值将PSP组分为两个亚组,亚组间平均生存时间无统计学差异(Log-Rank Test检验,P=0.308).结论 MRPI可以提高PSP的诊断准确率.     

动态血压变异性与主观认知下降进展的相关性CSCD

Objective To analyze the correlation between ambulatory blood pressure variability and the progression of subjective cognitive decline (SCD). Methods In this prospective observational study, the overall sampling method was used to continuously select 100 patients with SCD in the Department of Neurology, Changshu First People′s Hospital and Changshu Xinzhuang People′s Hospital from January 1 2016 to June 30 2017. The baseline demographic characteristics of the patients were collected. The Chinese version of SCD‑Q9 questionnaire was used to self‑evaluate SCD, and the Montreal Cognitive Assessment Scale (MoCA) was used to evaluate objective cognitive impairment. All patients received 24 h ambulatory blood pressure monitoring, and 24 h systolic coefficient of variation (SCV) and diastolic coefficient of variation (DCV) were calculated. The follow‑up period was 4 years after the first visit, and the MoCA scale was evaluated once a year. Finally, 83 patients completed the follow‑up and were included in this study. According to the MoCA score at the end of follow‑up (<26 or ≥26), the patients were divided into progression group (39 cases) and non‑progression group (44 cases). The difference of MoCA score between baseline and last follow‑up was calculated in the progression group. The difference in demographic characteristics between the two groups was compared with χ2 test. The difference of 24 h SCV and 24 h DCV between the two groups were compared by rank sum test. The correlation between 24 h SCV and MoCA score difference or SCD‑Q9 score in the progression group were tested by multiple linear regression analysis. Results The 4‑year progression rate of SCD patients was 46.99% (39/ 83). There was no significant differences in baseline age, gender, education level, medical history, smoking history, SCD‑Q9 score and MoCA score between the progressive group and the non‑progressive group (all P>0.05). The 24 h SCV in the progressive group was significantly higher than that in the non‑progressive group [13.4% (9.9%, 15.6%) vs 10.9% (9.7%, 12.7%), U=594.50, P= 0.016]. There was no significant difference in 24 h DCV between the two groups (P>0.05). In progressive group, the 24 h SCV was negatively correlated with MoCA score difference (r=-0.368, P= 0.021). Conclusion There is a correlation between ambulatory blood pressure variability and SCD progression, high 24 h SCV may be one of the factors of SCD progression and has certain predictive value. © 2023 Chinese Journal of Health Management. All rights reserved.     

多发性硬化诊断标准现状与进展

2010年爱尔兰都柏林国际多发性硬化(MS)诊断会议推出最新MS诊断标准,主要修订为:规定在2～4个MS相关特征性部位(靠近皮质部位、脑室周围、幕下和脊髓)中每个至少存在1个T2相病灶可以提示病变存在空间多发性,与首次发病期MRI比较出现新的T2相病灶可以提示病变存在时间多发性;进一步探讨脑脊液检查的诊断价值,并将新研究结果整合进去;进一步简化原发性进行性MS(PPMS)的诊断流程;对该标准在不同人群中的应用进行详细说明。