Neuroscience Research Australia and the University of New South Wales, Sydney, New South Wales, Australia.
Mov Disord. 2011 Feb 1;26(2):256-63. doi: 10.1002/mds.23295. Epub 2010 Dec 13.
To determine whether brain atrophy differs between the two subtypes of progressive supranuclear palsy (PSP), Richardson's syndrome (PSP-RS), and PSP parkinsonism (PSP-P), and whether such atrophy directly relates to clinical deficits and the severity of tau deposition. We compared 24 pathologically confirmed PSP cases (17 PSP-RS and 7 PSP-P) with 22 controls from a Sydney brain donor program. Volume loss was analyzed in 29 anatomically discrete brain regions using a validated point-counting technique, and tau-immunoreactive neurons, astrocytes and oligodendrocytes/threads semiquantified. Correlations between the two pathological measures and the presence or absence of cardinal PSP symptoms were investigated. Cortical atrophy was more severe in PSP-RS than PSP-P and affected more frontal lobe regions (frontal pole, inferior frontal gyrus). The supramarginal gyrus was atrophic in both subtypes. Additionally, atrophy of the internal globus pallidus, amygdala, and thalamus was more severe in PSP-RS. As expected, more severe frontal lobe tau pathology differentiated PSP-RS from PSP-P. No correlations were found between the degree of atrophy and severity of tau pathology in any region assessed, or between the severity of atrophy or tau pathology and the presence or absence of cardinal PSP symptoms. Our study shows that thalamocortical atrophy is a defining feature of PSP-RS, but this atrophy does not correlate with the presence of any specific cardinal clinical feature. Interestingly, there is a disassociation between tau pathology and atrophy in the brain regions affected in PSP-RS that requires further investigation.
为了确定两种进行性核上性麻痹(PSP)亚型(Richardson 综合征 [PSP-RS] 和 PSP 帕金森病 [PSP-P])之间是否存在脑萎缩,以及这种萎缩是否与临床缺陷和 tau 沉积的严重程度直接相关。我们比较了 24 例经病理证实的 PSP 病例(17 例 PSP-RS 和 7 例 PSP-P)和来自悉尼脑捐赠计划的 22 例对照。使用经过验证的点数计数技术分析了 29 个解剖上离散的脑区的体积损失,并对半定量分析了 tau 免疫反应性神经元、星形胶质细胞和少突胶质细胞/线程。研究了这两种病理测量方法与 PSP 主要症状的存在与否之间的相关性。PSP-RS 的皮质萎缩比 PSP-P 更严重,影响更多的额叶区域(额极、下额回)。两个亚型的缘上回都有萎缩。此外,PSP-RS 的内苍白球、杏仁核和丘脑的萎缩更为严重。正如预期的那样,更严重的额叶 tau 病理学将 PSP-RS 与 PSP-P 区分开来。在评估的任何区域中,都没有发现萎缩程度与 tau 病理学严重程度之间的相关性,也没有发现萎缩或 tau 病理学严重程度与 PSP 主要症状的存在与否之间的相关性。我们的研究表明,丘脑皮质萎缩是 PSP-RS 的一个特征,但这种萎缩与任何特定的主要临床特征的存在无关。有趣的是,在 PSP-RS 受累的脑区中,tau 病理学与萎缩之间存在分离,这需要进一步研究。
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