Essential tremor (ET) is the most common tremor disorder globally and is characterized by kinetic tremor of the upper limbs, although other clinical features can also occur. Postmortem studies are a particularly important avenue for advancing our understanding of the pathogenesis of ET; however, until recently, the number of such studies has been limited. Several recent postmortem studies have made important contributions to our understanding of the pathological changes that take place in ET. These studies identified abnormalities in the cerebellum, which primarily affected Purkinje cells (PCs), basket cells and climbing fibres, in individuals with ET. We suggest that some of these pathological changes (for example, focal PC axonal swellings, swellings in and regression of the PC dendritic arbor and PC death) are likely to be primary and degenerative. By contrast, other changes, such as an increase in PC recurrent axonal collateral formation and hypertrophy of GABAergic basket cell axonal processes, could be compensatory responses to restore cerebellar GABAergic tone and cerebellar cortical inhibitory efficacy. Such compensatory responses are likely to be insufficient, enabling the disease to progress. Here, we review the results of recent postmortem studies of ET and attempt to place these findings into an anatomical-physiological disease model.

中文翻译：

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原发性震颤病理学：神经变性和神经元连接的重组。

特发性震颤 (ET) 是全球最常见的震颤疾病，其特征是上肢运动性震颤，但也可能出现其他临床特征。尸检研究是促进我们了解 ET 发病机制的一个特别重要的途径。然而，直到最近，此类研究的数量仍然有限。最近的几项尸检研究对我们理解 ET 中发生的病理变化做出了重要贡献。这些研究确定了小脑的异常，主要影响 ET 个体的浦肯野细胞 (PC)、篮细胞和攀爬纤维。我们建议这些病理变化中的一些（例如，局灶性 PC 轴突肿胀，PC 树突状乔木的肿胀和消退和 PC 死亡）可能是原发性和退行性的。相比之下，其他变化，例如 PC 复发性轴突侧枝形成的增加和 GABA 能篮细胞轴突突起的肥大，可能是恢复小脑 GABA 能张力和小脑皮质抑制功效的补偿反应。这种代偿性反应可能是不够的，从而使疾病得以进展。在这里，我们回顾了最近对 ET 的尸检研究结果，并试图将这些发现放入解剖生理疾病模型中。可能是恢复小脑 GABA 能张力和小脑皮质抑制功效的代偿反应。这种代偿性反应可能是不够的，从而使疾病得以进展。在这里，我们回顾了最近对 ET 的尸检研究结果，并试图将这些发现放入解剖生理疾病模型中。可能是恢复小脑 GABA 能张力和小脑皮质抑制功效的代偿反应。这种代偿性反应可能是不够的，从而使疾病得以进展。在这里，我们回顾了最近对 ET 的尸检研究结果，并试图将这些发现放入解剖生理疾病模型中。