Movement Disorders Program Saskatchewan, Neurology, Department of Medicine, University of Saskatchewan/Saskatoon Health Region, Saskatoon, Saskatchewan, Canada.
Parkinsonism Relat Disord. 2012 Jun;18(5):626-8. doi: 10.1016/j.parkreldis.2012.01.013. Epub 2012 Feb 3.
The pathophysiology of essential tremor (ET) is unknown but recent studies report that the majority of ET cases has cerebellar Purkinje cell (PC) degeneration and its sequelae.
To perform PC counts in ET, and normal and Parkinson's disease (PD) controls to determine the relationship of PC loss to ET.
All ET cases and PD controls were followed at our clinic. Normal controls had no history of neurological disease and had normal standard neuropathological studies. The PC counts were done by a neuropathologist who was blinded to the clinical diagnosis. Three different methods were used for counting PC; section through any part of the PC, through any part of the PC nucleus, and through any part of PC nucleolus. The counts were done in five non-contiguous microscopic fields.
59 brains were studied. These included 12 ET, 41 PD controls, and six normal controls. The mean age at death was 82.7 in ET, 79.1 in PD, and 75.7 years in the normal controls. The mean duration of symptoms was 34 years in ET and 15.7 years in the PD cases. The mean PC counts through any part of the neuron were 64.8 in ET, 56.2 in PD, and 58.0 in normal controls. Differences were not significant.
Cerebellar PC loss does not distinguish ET from controls. It is concluded that PC loss is neither a pathological basis for, nor the distinctive feature of ET.
对特发性震颤(ET)患者、正常对照和帕金森病(PD)对照者进行浦肯野细胞(PC)计数,以确定 PC 丢失与 ET 的关系。
所有 ET 病例和 PD 对照者均在我科随访。正常对照者无神经病史,且标准神经病理学检查正常。由一位对临床诊断不知情的神经病理学家进行 PC 计数。采用三种不同的方法对 PC 进行计数:PC 的任意部分、PC 核的任意部分和 PC 核仁的任意部分。在五个非连续的显微镜视野中进行计数。
共研究了 59 例脑。包括 12 例 ET、41 例 PD 对照和 6 例正常对照。ET 的平均死亡年龄为 82.7 岁,PD 为 79.1 岁,正常对照为 75.7 岁。ET 的平均症状持续时间为 34 年,PD 病例为 15.7 年。PC 通过神经元的任何部分计数,ET 为 64.8,PD 为 56.2,正常对照为 58.0。差异无统计学意义。
小脑 PC 丢失不能将 ET 与对照组区分开来。因此,PC 丢失既不是 ET 的病理基础,也不是其特征性表现。