特发性震颤患者小脑浦肯野细胞数量增加。
Increased number of heterotopic Purkinje cells in essential tremor.
机构信息
Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, USA.
出版信息
J Neurol Neurosurg Psychiatry. 2011 Sep;82(9):1038-40. doi: 10.1136/jnnp.2010.213330. Epub 2010 Aug 27.
OBJECTIVE
Recent postmortem studies reveal degenerative changes, including Purkinje cell (PC) loss, in most brains from individuals with essential tremor (ET). Heterotopic PCs (HPCs) (ie, PC bodies displaced into the molecular layer) may be found in neurodegenerative diseases with PC loss. HPCs have been observed in ET but no quantitative case control analysis has been performed.
METHODS
HPCs were counted in 35 ET brains and 32 control brains (including 21 non-diseased controls and 11 diseased controls with progressive supranuclear palsy (PSP)) using a standard 20 × 25 mm cerebellar cortical section stained with a modified Bielscholwsky method.
RESULTS
The median number of HPCs per section was three times higher in 35 ET cases (median 3, mean ± SD 3.8 ± 3.6, range 0-14) versus 32 controls (median 1, mean ± SD 1.6 ± 1.7, range 0-5) (p = 0.007). The number of HPCs was similarly low in the 21 non-diseased controls and 12 PSP brains (median 1 in each group) (p = 0.04 and p = 0.01 compared with ET). In ET, the number of HPCs was inversely related to the number of PCs (Spearman's rho -0.36, p = 0.038) (ie, cases with more HPCs had fewer PCs).
CONCLUSION
PC heterotopia, which occurs in cerebellar degenerative disorders, is also a feature of ET. These findings further contribute to our understanding of the postmortem changes in this common neurological disease.
目的
最近的尸检研究显示，在大多数特发性震颤（ET）患者的大脑中存在退行性变化，包括浦肯野细胞（PC）丢失。在伴有 PC 丢失的神经退行性疾病中，可能会发现异位浦肯野细胞（HPCs）（即 PC 体位移到分子层）。已经在 ET 中观察到 HPCs，但尚未进行定量病例对照分析。
方法
使用改良 Bielscholwsky 染色的 20×25mm 小脑皮质切片，对 35 例 ET 脑和 32 例对照脑（包括 21 例非疾病对照和 11 例进展性核上性麻痹（PSP）疾病对照）进行 HPC 计数。
结果
与 32 例对照（中位数 1，均值±标准差 1.6±1.7，范围 0-5）相比，35 例 ET 病例中每节 HPC 的中位数高出三倍（中位数 3，均值±标准差 3.8±3.6，范围 0-14）（p=0.007）。在 21 例非疾病对照和 12 例 PSP 脑（每组中位数为 1）中，HPC 数量也较低（与 ET 相比，p=0.04 和 p=0.01）。在 ET 中，HPC 数量与 PC 数量呈负相关（Spearman's rho -0.36，p=0.038）（即，HPC 数量较多的病例 PC 数量较少）。
结论
在小脑退行性疾病中发生的 PC 异位，也是 ET 的一个特征。这些发现进一步有助于我们了解这种常见神经疾病的尸检变化。
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