Quantification of cerebellar hemispheric purkinje cell linear density: 32 ET cases versus 16 controls.

Quantification of cerebellar hemispheric purkinje cell linear density: 32 ET cases versus 16 controls.
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DOI:
10.1002/mds.25629
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发表时间:
2013-11
期刊:
影响因子:
8.6
通讯作者:
Vonsattel, Jean-Paul G.
Vonsattel, Jean-Paul G.
中科院分区:
医学1区
文献类型:
--
作者:
Louis, Elan D.;Babij, Rachel;Lee, Michelle;Cortes, Etty;Vonsattel, Jean-Paul G.

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虽然原发性震颤(ET)是最常见的神经系统疾病之一,但其确切的发病机制尚不清楚。浦肯野细胞丢失在一些研究中被观察到,也是人们感兴趣和争论的焦点。将这些数据表示为浦肯野细胞/层长度允许人们根据小脑叶固有的弯曲性质进行调整。利用Essential Tremor中央脑库,我们量化了病例和对照组中浦肯野细胞的线密度。用兔抗Calbindin D28k多克隆抗体对10 0μm矢状面旁小脑半球自由漂浮切片,随机选取10个视野/脑进行蒲肯野细胞/mm−1蒲肯野细胞层的定量。32例ET患者浦肯野细胞线密度低于16例对照组(1.14±0.32vs.1.35±0.31/mm−1,p=0.03)。浦肯野细胞线密度与鱼雷计数呈负相关(r=−0.38,p=0.028)。目前的ET病例样本显示,与对照组相比,浦肯野细胞数量减少。在浦肯野细胞缺失最多的个体中发现了更大的浦肯野细胞轴突重构(鱼雷)。浦肯野细胞丢失在本病发病机制中的作用值得进一步研究。
Although essential tremor (ET) is among the most prevalent neurological diseases, its precise pathogenesis is not understood. Purkinje cell loss has been observed in some studies and is the focus of interest and debate. Expressing these data as Purkinje cells/layer length allows one to adjust for the inherent curved nature of the cerebellar folia. Capitalizing on the Essential Tremor Centralized Brain Repository, we quantified Purkinje cell linear density in cases vs. controls. Free-floating, 100 μm, parasagittal cerebellar hemispheric sections were subjected to rabbit polyclonal anti-Calbindin D28k antibody, and 10 random fields/brain were selected for quantification of Purkinje cells/mm−1 Purkinje cell layer. Purkinje cell linear density was lower in 32 ET cases than 16 controls (1.14 ± 0.32 vs. 1.35 ± 0.31 per mm−1, p = 0.03). Purkinje cell linear density was inversely associated with torpedo count (r = −0.38, p = 0.028). The current sample of ET cases demonstrates a reduction in Purkinje cell number relative to that of controls. Greater Purkinje cell axonal remodeling (torpedoes) was found in individuals who had the most Purkinje cell drop out. The role of Purkinje cell loss in the pathogenesis of this disorder merits additional study.
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发表时间: 2003-04-01
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