Neuropathological changes in essential tremor: 33 cases compared with 21 controls
Neuropathological changes in essential tremor: 33 cases compared with 21 controls
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DOI：
10.1093/brain/awm266
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发表时间：
2007-12-01
期刊：
影响因子：
14.5
通讯作者：
Hernandez, Nora
中科院分区：
文献类型：
--
作者：
Louis, Elan D.;Faust, Phyllis L.;Hernandez, Nora
尽管原发性震颤 (ET) 是最常见的神经系统疾病之一，但其神经病理学研究却很少。令人惊讶的是，尸检研究很少，病例对照比较也更少。主要目标是描述和量化 33 个 ET 和 21 个对照大脑的病理变化。次要目标是将临床和病理特征关联起来。我们检查了特发性震颤集中脑存储库的尸检组织。 33 个 ET 大脑中的 8 个（24.2%）在脑干中有路易体，主要在蓝斑。然而，大多数ET大脑（25/33，75.8%）没有路易体，但小脑有病理改变。在没有路易体的 ET 病例中，每 1003 个视野的浦肯野细胞平均数量减少（6.6 +/- 2.4 与 9.6 +/- 3.4，P < 0.01），并且与对照组相比，每个切片多了 7 个浦肯野细胞鱼雷（12.6 +/- 7.9 与 1.7 +/- 1.4，P < 0.001）。无路易体的 ET 病例也有齿状核变性（两例）。 ET 病例中的其他发现包括浦肯野细胞异位和树突肿胀。路易体 ET 病例比无路易体的 ET 病例年龄更大。在无路易体的 ET 病例中观察到了一些趋势，包括震颤发病年龄更小、步态困难和 ET 家族史的比例更高。 ET的病理变化似乎是异质性和退行性的。大多数人有小脑改变，但没有路易体；较小部分具有脑干路易体。有路易体病例与无路易体病例之间的临床差异需要进一步研究。
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.