GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, NY, USA; Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York, USA; Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York, USA; Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, New York, USA.
Mov Disord. 2013 Nov;28(13):1854-9. doi: 10.1002/mds.25629. Epub 2013 Aug 7.
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 versus 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 in16 controls (1.14 ± 0.32 vs. 1.35 ± 0.31/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.
虽然特发性震颤（ET）是最常见的神经疾病之一，但它的确切发病机制尚不清楚。一些研究观察到浦肯野细胞丢失，这是关注和争议的焦点。将这些数据表示为浦肯野细胞/层长度，可以调整小脑叶片固有的弯曲性质。利用特发性震颤集中脑库，我们对病例与对照组的浦肯野细胞线性密度进行了量化。对 100μm 游离的矢状旁小脑半球切片进行兔多克隆抗钙结合蛋白 D28k 抗体处理，并选择 10 个随机视野/大脑进行浦肯野细胞/mm(-1) 浦肯野细胞层的定量。32 例 ET 病例的浦肯野细胞线性密度低于 16 例对照组（1.14±0.32 比 1.35±0.31/mm(-1)，P=0.03）。浦肯野细胞线性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。在浦肯野细胞脱落最多的个体中，发现了更多的浦肯野细胞轴突重塑（鱼雷）。浦肯野细胞丢失在该疾病发病机制中的作用值得进一步研究。
