Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant
Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant
复制标题
DOI：
10.1111/nan.12037
复制
发表时间：
2014-02-01
影响因子：
5
通讯作者：
Revesz, T.
中科院分区：
文献类型：
--
作者：
Ling, H.;de Silva, R.;Revesz, T.
自1963年首次描述进行性核上性麻痹（PSP）的经典表现以来，现在被称为Richardson综合征（PSP-RS），几种不同的临床综合征与PSP-tau病理学相关。与其他神经退行性疾病一样，磷酸化tau病理的严重程度和分布与PSP变体的临床异质性密切相关。据报道，PSP与皮质基底综合征表现（PSP-CBS）有更多的tau负载在中额和前顶叶皮质比PSP-RS。然而，这是不确定的，如果差异存在于其他脑区的tau病理分布或如果整体tau负荷增加的大脑中的PSP-CBS.MethodsWe试图比较的临床和病理特征的PSP-CBS和PSP-RS，包括定量评估tau负荷在15皮质，基底神经节和小脑regions.ResultsIn除了相似的发病年龄和疾病持续时间，我们证明了在PSP-CBS和PSP-RS之间tau病理学的总体严重性是相同的。我们发现tau负荷向远离基底神经节的皮质区域转移;支持PSP-CBS是皮质PSP变体的观点。与PSP-RS相比，PSP-CBS在黑质背外侧和腹外侧亚区的神经元丢失较轻，皮质脊髓束的小胶质细胞反应较重;但是，在此情况下，结论更好地了解不同PSP变异体中影响选择性病理脆弱性的因素将为进一步了解神经退行性变过程提供依据潜在的tau蛋白病
AimsSince the first description of the classical presentation of progressive supranuclear palsy (PSP) in 1963, now known as Richardson's syndrome (PSP-RS), several distinct clinical syndromes have been associated with PSP-tau pathology. Like other neurodegenerative disorders, the severity and distribution of phosphorylated tau pathology are closely associated with the clinical heterogeneity of PSP variants. PSP with corticobasal syndrome presentation (PSP-CBS) was reported to have more tau load in the mid-frontal and inferior-parietal cortices than in PSP-RS. However, it is uncertain if differences exist in the distribution of tau pathology in other brain regions or if the overall tau load is increased in the brains of PSP-CBS.MethodsWe sought to compare the clinical and pathological features of PSP-CBS and PSP-RS including quantitative assessment of tau load in 15 cortical, basal ganglia and cerebellar regions.ResultsIn addition to the similar age of onset and disease duration, we demonstrated that the overall severity of tau pathology was the same between PSP-CBS and PSP-RS. We identified that there was a shift of tau burden towards the cortical regions away from the basal ganglia; supporting the notion that PSP-CBS is a cortical' PSP variant. PSP-CBS also had less severe neuronal loss in the dorsolateral and ventrolateral subregions of the substantia nigra and more severe microglial response in the corticospinal tract than in PSP-RS; however, neuronal loss in subthalamic nucleus was equally severe in both groups.ConclusionsA better understanding of the factors that influence the selective pathological vulnerability in different PSP variants will provide further insights into the neurodegenerative process underlying tauopathies.