Cerebellar Pathology in Early Onset and Late Onset Essential Tremor.
Cerebellar Pathology in Early Onset and Late Onset Essential Tremor.
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DOI：
10.1007/s12311-016-0826-5
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发表时间：
2017-04
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影响因子：
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通讯作者：
Faust PL
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作者：
Kuo SH;Wang J;Tate WJ;Pan MK;Kelly GC;Gutierrez J;Cortes EP;Vonsattel JG;Louis ED;Faust PL
早发性特发性震颤（ET）病例和晚发性特发性震颤（ET）病例在几个方面有所不同。它们在小脑病理变化方面是否不同仍有待确定。我们对 30 例震颤发作年龄 <50 岁的 ET 病例、30 例震颤发作年龄≥50 岁的 ET 病例和 30 例对照（总计 n = 90）的广泛死后特征（浦肯野细胞 (PC) 计数、PC 轴突鱼雷和相关轴突变化、异位 PC 和毛篮评级）进行了量化。我们还使用了两个替代的发病年龄切点（<40 岁与≥40 岁，以及<60 岁与≥60 岁）来定义早发 ET 与晚发 ET。我们发现震颤发作 <50 年和震颤发作≥50 年的 ET 病例具有相似的 PC 计数（8.78 ± 1.70 与 8.86 ± 1.24，p = 0.839）、PC 轴突鱼雷计数（17.87 ± 18.27 [中位数=13.00] vs. 12.90 ± 10.60 [中位数] =9.0]，p = 0.486）和相关的轴突病理学 （所有 p 值 >0.05）、异位 PC 计数（9.90 ± 11.55 [中位数 =6.00] 与 5.40 ± 5.10 [中位数 =3.50]，p = 0.092）和毛篮评级（1.95 ± 0.62 [中位数 =2.00] 与 2.05 ± 0.92 [中位数=2.00]，p = 0.314）。当使用 40 岁或 60 岁作为发病年龄切点时，结果相似。早发和晚发 ET 病例具有相似的小脑尸检特征。这些数据并不支持这些与发病年龄相关的 ET 形式代表不同的临床病理实体的观点。
Early onset and late onset essential tremor (ET) cases differ in several respects. Whether they differ with respect to cerebellar pathologic changes remains to be determined. We quantified a broad range of postmortem features (Purkinje cell (PC) counts, PC axonal torpedoes and associated axonal changes, heterotopic PCs, and hairy basket ratings) in 30 ET cases with age of tremor onset <50 years, 30 ET cases with age of tremor onset ≥50 years, and 30 controls (total n = 90). We also used two alternative age of onset cut-points (<40 vs. ≥40 years, and <60 vs. ≥60 years) to define early onset vs. late onset ET. We found that ET cases with tremor onset <50 years and tremor onset ≥50 years had similar PC counts (8.78 ± 1.70 vs. 8.86 ± 1.24, p = 0.839), PC axonal torpedo counts (17.87 ± 18.27 [median =13.00] vs. 12.90 ± 10.60 [median =9.0], p = 0.486) and associated axonal pathology (all p values >0.05), heterotopic PC counts (9.90 ± 11.55 [median =6.00] vs. 5.40 ± 5.10 [median =3.50], p = 0.092), and hairy basket ratings (1.95 ± 0.62 [median =2.00] vs. 2.05 ± 0.92 [median =2.00], p = 0.314). When using the age of onset cut-points of 40 or 60 years, results were similar. Early onset and late onset ET cases share similar cerebellar postmortem features. These data do not support the notion that these age-of-onset related forms of ET represent distinct clinical-pathological entities.