期刊
BRAIN
卷 130, 期 -, 页码 3297-3307出版社
OXFORD UNIV PRESS
DOI: 10.1093/brain/awm266
关键词
essential tremor; cerebellum; Purkinje cell; Lewy body; neurodegeneration; locus ceruleus
资金
- NIA NIH HHS [P01 AG07232, P50 AG08702] Funding Source: Medline
- NINDS NIH HHS [R01 NS42859, R24 NS056062] Funding Source: Medline
Despite its being one of the most commonly observed neurological disorders, neuropathological studies of essential tremor ( ET) are rare. There have been surprisingly few autopsy studies and even fewer case-control comparisons. The primary objective was to describe and quantify the pathological changes in 33 ET and 21 control brains. A secondary objective was to correlate clinical and pathological features. We examined autopsy tissue from the Essential Tremor Centralized Brain Repository. Eight ( 24.2%) of the 33 ET brains had Lewy bodies in the brainstem, mainly in the locus ceruleus. However, the majority of ET brains ( 25/33, 75.8%) had no Lewy bodies, but had pathological changes in the cerebellum. The mean number of Purkinje cells per 1003 field was reduced in ET cases without Lewy bodies ( 6.6 +/- 2.4 versus 9.6 +/- 3.4, P < 0.01), and there were similar to 7 more Purkinje cell torpedoes per section ( 12.6 +/- 7.9 versus 1.7 +/- 1.4, P < 0.001) compared to controls. ET cases without Lewy bodies also had degeneration of the dentate nucleus ( two cases). Other findings in ET cases were Purkinje cell heterotopias and dendrite swellings. Lewy body ET cases were older than ET cases without Lewy bodies. Several trends were observed in ET cases without Lewy bodies, including a younger age of onset of tremor and higher proportions with gait difficulty and family history of ET. The pathological changes of ET seem to be heterogeneous and degenerative. The majority have cerebellar changes without Lewy bodies; a smaller proportion has brainstem Lewy bodies. The clinical differences between cases with versus without Lewy bodies require additional study.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据