Essential Tremor versus "ET-plus": A Detailed Postmortem Study of Cerebellar Pathology.
Essential Tremor versus "ET-plus": A Detailed Postmortem Study of Cerebellar Pathology.
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基本震颤与“ ET-Plus”：小脑病理的详细死后研究。
DOI：
10.1007/s12311-021-01263-6
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发表时间：
2021-12
期刊：
影响因子：
--
通讯作者：
Louis ED
中科院分区：
文献类型：
--
作者：
Gionco JT;Hartstone WG;Martuscello RT;Kuo SH;Faust PL;Louis ED
特发性震颤（ET）是最普遍的运动障碍之一，并且根据某些说法，是小脑变性的最常见形式。在过去的15年里，我们仔细记录了大量的死后小脑内的变化，这些小脑的变化显着不同ET和控制。最近的震颤共识分类提出，除了动作性震颤外，具有其他神经学体征的ET患者（例如，帕金森综合征、共济失调、认知改变、肌张力障碍）应作为“ET+”分开。这一诊断概念引起了相当大的争议，其有效性尚未确定。事实上，“ET+”并没有基于遗传学、病理学或预后的差异而与ET区分开。在这里，我们确定是否ET的情况下不同的“ET+”的情况下，在死后的大脑中的潜在病理变化。我们研究了死后的大脑从50例ET（24 ET和26 ET+），使用一组14个定量指标的小脑病理组织学和免疫组化方法确定。这些指标反映了浦肯野细胞（PC）体（PC计数，空篮，异位），PC树突（saponins），PC轴突（鱼雷和相关的轴突变化），篮细胞轴突肥大和攀爬纤维PC树突突触变化的变化。ET和ET+在14个小脑病理指标中的13个方面相似（p > 0.05）。只有一个指标，增厚的PC轴突轮廓的线密度，在这些组之间存在差异（ET = 0.529 ± 0.397，ET += 0.777 ± 0.477，p = 0.013），尽管在校正多重比较后，没有差异。如果ET+确实是一个不同的实体，那么其潜在的病理基础应该与ET不同。本研究表明ET与ET+患者的小脑皮质无病理学差异。这些数据不支持ET和ET+代表不同临床病理实体的概念。
Essential tremor (ET) is among the most prevalent movement disorders, and by some accounts, the most common form of cerebellar degeneration. Over the past 15 years, we have carefully documented a large number of postmortem changes within the cerebellum; these cerebellar changes differ significantly between ET and controls. A recent Consensus Classification of tremor proposed that ET patients with other neurological signs aside from action tremor (e.g., parkinsonism, ataxia, cognitive changes, dystonia) should be segregated off as “ET-plus”. This diagnostic concept has raised considerable controversy and its validity is not yet established. Indeed, “ET-plus” has not been distinguished from ET based on differences in genetics, pathology or prognosis. Here we determine whether ET cases differ from “ET-plus” cases in underlying pathological changes in the postmortem brain. We examined postmortem brains from 50 ET cases (24 ET and 26 ET-plus), using a set of 14 quantitative metrics of cerebellar pathology determined by histologic and immunohistochemical methods. These metrics reflect changes across the Purkinje cell (PC) body (PC counts, empty baskets, heterotopias), PC dendrites (swellings), PC axon (torpedoes and associated axonal changes), basket cell axonal hypertrophy and climbing fiber-PC dendrite synaptic changes. ET and ET-plus were similar with respect to 13 of 14 cerebellar pathologic metrics (p > 0.05). Only one metric, the linear density of thickened PC axon profiles, differed between these groups (ET = 0.529 ± 0.397, ET-plus = 0.777 ± 0.477, p = 0.013), although after correcting for multiple comparisons, there were no differences. If ET-plus were indeed a different entity, then the underlying pathological basis should be distinct from that of ET. This study demonstrated there were no pathological differences in cerebellar cortex between ET versus ET-plus cases. These data do not support the notion that ET and ET-plus represent distinct clinical-pathological entities.
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影响因子：
14.5
作者：
Louis, Elan D.;Lee, Michelle;Faust, Phyllis L.
通讯作者：
Faust, Phyllis L.
影响因子：
14.5
作者：
Babij, Rachel;Lee, Michelle;Louis, Elan D.
通讯作者：
Louis, Elan D.
影响因子：
3.5
作者：
Louis, Elan D.;Kuo, Sheng-Han;Faust, Phyllis L.
通讯作者：
Faust, Phyllis L.
DOI：
10.1007/s12311-016-0826-5
发表时间：
2017-04
期刊：
Cerebellum (London, England)
影响因子：
--
作者：
Kuo SH;Wang J;Tate WJ;Pan MK;Kelly GC;Gutierrez J;Cortes EP;Vonsattel JG;Louis ED;Faust PL
通讯作者：
Faust PL
DOI：
10.1111/j.1532-5415.1968.tb02103.x
发表时间：
1968-01-01
影响因子：
6.3
作者：
LINN, BS;LINN, MW;GUREL, L
通讯作者：
GUREL, L