GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
Eur J Neurol. 2012 Apr;19(4):625-30. doi: 10.1111/j.1468-1331.2011.03598.x. Epub 2011 Dec 5.
Essential Tremor (ET) is among the most prevalent neurologic disorders. Growing clinical and neuro-imaging evidence implicates cerebellar dysfunction in the pathogenesis of ET and emerging postmortem studies have identified structural changes in the cerebellum, particularly in Purkinje cells. In this study we systematically quantified focal Purkinje cell dendritic swellings (DS) in 20 ET vs. 19 control brains.
In each brain, a standard parasagittal neocerebellar tissue block was harvested. DS were quantified in one 7-μm thick section stained with Luxol Fast Blue/Hematoxylin and Eosin (LH&E) and one section stained with Bielschowsky method.
The number of DS were higher in cases than controls by LH&E (1.50 ± 1.79 vs. 0.05 ± 0.23, P = 0.002) and Bielschowsky methods (2.70 ± 3.10 vs. 0.37 ± 0.50, P = 0.002). The number of DS was correlated with the number of torpedoes and marginally inversely correlated with the number of Purkinje cells.
The current study documents and quantifies an additional structural abnormality in the ET cerebellum, adding to the growing list of such changes in this disease. The mechanisms that underlie this and other structural changes observed in ET are currently unknown, and they deserve additional exploration.
特发性震颤(ET)是最常见的神经疾病之一。越来越多的临床和神经影像学证据表明小脑功能障碍与 ET 的发病机制有关,新兴的尸检研究已经确定了小脑的结构变化,特别是浦肯野细胞。在这项研究中,我们系统地量化了 20 例 ET 与 19 例对照大脑中的局灶性浦肯野细胞树突肿胀(DS)。
在每个大脑中,收获一个标准的矢状旁新小脑组织块。用 Luxol Fast Blue/Hematoxylin and Eosin(LH&E)和 Bielschowsky 染色法对 1 个 7μm 厚的切片进行 DS 量化。
LH&E(1.50 ± 1.79 比 0.05 ± 0.23,P = 0.002)和 Bielschowsky 方法(2.70 ± 3.10 比 0.37 ± 0.50,P = 0.002)下,病例中的 DS 数量高于对照组。DS 的数量与 torpedoes 的数量呈正相关,与浦肯野细胞的数量呈负相关。
本研究记录并量化了 ET 小脑的另一种结构异常,这增加了该疾病中此类变化的清单。目前尚不清楚导致这种和 ET 中观察到的其他结构变化的机制,它们值得进一步探索。