目的比较几种磁共振线性测量指标在鉴别进行性核上性麻痹(progressive supranuclear palsy,PSP)与帕金森病(Parkinson disease,PD)上的应用价值。方法纳入临床可能的和很可能的PSP患者28例、PD患者30例和健康对照30名,测量计算中脑与桥...目的比较几种磁共振线性测量指标在鉴别进行性核上性麻痹(progressive supranuclear palsy,PSP)与帕金森病(Parkinson disease,PD)上的应用价值。方法纳入临床可能的和很可能的PSP患者28例、PD患者30例和健康对照30名,测量计算中脑与桥脑直径比(midbrain diameter/pons diameter,Md/Pd)、第三脑室宽度与额角最大间距比(width of third ventricle/distance of frontal horns,V3rd/FH)、中脑被盖部直径(midbrain tegmentum diameter,MTEG)以及大脑脚角度(angle of cerebral peduncle,ACP),并对它们作为PSP诊断指标的敏感性和特异性进行比较。结果 PSP组患者Md/Pd和MTEG小于PD组和健康对照组,V3rd/FH和ACP大于PD组和健康对照组,且差异有统计学意义。PD组与健康对照组的各项指标差异无统计学意义。通过曲线下面积(area under curve,AUC)的计算,Md/Pd诊断PSP的价值最高,其次是MTEG,其中Md/Pd在约登指数最高点诊断PSP的敏感性和特异性分别达到100%和98.2%。结论 Md/Pd、V3rd/FH、MTEG和ACP几种线性测量指标均对PSP与PD的鉴别有诊断价值,Md/Pd敏感性和特异性最高,最适于临床推广应用,MTEG测量也简单直观,敏感性和特异性也比较高,V3rd/FH和ACP对鉴别诊断也有一定的提示作用。展开更多
The clinical diagnosis of progressive supranuclear palsy(PSP) relies on the id entification of characteristic signsand symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, pr...The clinical diagnosis of progressive supranuclear palsy(PSP) relies on the id entification of characteristic signsand symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagn ose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and t o compare the biochemical and genetic characteristics of these groups. In 103 co nsecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson’s syndrome (RS) a nd PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54%of all cases, an d were characterized by the early onset of postural instability and falls, supra nuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32 %) were characterized by asymmetric onset, tremor, a moderate initial therapeut ic response to levodopa and were frequently confused with Parkinson’s disease ( PSP-P). Fourteen cases (14%)-could not be separated according to these criter ia. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 year s, P < 0.001)-and age at death earlier (72.1 versus 75.5 years, P=0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basa l pons also differed significantly. In RS, the mean four-repeat: three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P< 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical su bgroups was not significant. There were no differences in apolipoprotein E genot ypes. The classic clinical description of PSP, which includes supranuclear gaze palsy, early falls and dementia, does not adequately describe one-third of case s in this series of pathologically confirmed cases. We propose that PSP-P repre sents a second discrete clinical phenotype that needs to be clinically distingui shed from classical PSP (RS). The different tau isoformdeposition in the basal p ons suggests that this may ultimately prove to be a discrete nosological entity.展开更多
文摘目的比较几种磁共振线性测量指标在鉴别进行性核上性麻痹(progressive supranuclear palsy,PSP)与帕金森病(Parkinson disease,PD)上的应用价值。方法纳入临床可能的和很可能的PSP患者28例、PD患者30例和健康对照30名,测量计算中脑与桥脑直径比(midbrain diameter/pons diameter,Md/Pd)、第三脑室宽度与额角最大间距比(width of third ventricle/distance of frontal horns,V3rd/FH)、中脑被盖部直径(midbrain tegmentum diameter,MTEG)以及大脑脚角度(angle of cerebral peduncle,ACP),并对它们作为PSP诊断指标的敏感性和特异性进行比较。结果 PSP组患者Md/Pd和MTEG小于PD组和健康对照组,V3rd/FH和ACP大于PD组和健康对照组,且差异有统计学意义。PD组与健康对照组的各项指标差异无统计学意义。通过曲线下面积(area under curve,AUC)的计算,Md/Pd诊断PSP的价值最高,其次是MTEG,其中Md/Pd在约登指数最高点诊断PSP的敏感性和特异性分别达到100%和98.2%。结论 Md/Pd、V3rd/FH、MTEG和ACP几种线性测量指标均对PSP与PD的鉴别有诊断价值,Md/Pd敏感性和特异性最高,最适于临床推广应用,MTEG测量也简单直观,敏感性和特异性也比较高,V3rd/FH和ACP对鉴别诊断也有一定的提示作用。
文摘The clinical diagnosis of progressive supranuclear palsy(PSP) relies on the id entification of characteristic signsand symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagn ose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and t o compare the biochemical and genetic characteristics of these groups. In 103 co nsecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson’s syndrome (RS) a nd PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54%of all cases, an d were characterized by the early onset of postural instability and falls, supra nuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32 %) were characterized by asymmetric onset, tremor, a moderate initial therapeut ic response to levodopa and were frequently confused with Parkinson’s disease ( PSP-P). Fourteen cases (14%)-could not be separated according to these criter ia. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 year s, P < 0.001)-and age at death earlier (72.1 versus 75.5 years, P=0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basa l pons also differed significantly. In RS, the mean four-repeat: three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P< 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical su bgroups was not significant. There were no differences in apolipoprotein E genot ypes. The classic clinical description of PSP, which includes supranuclear gaze palsy, early falls and dementia, does not adequately describe one-third of case s in this series of pathologically confirmed cases. We propose that PSP-P repre sents a second discrete clinical phenotype that needs to be clinically distingui shed from classical PSP (RS). The different tau isoformdeposition in the basal p ons suggests that this may ultimately prove to be a discrete nosological entity.
