Cerebellar dentate nucleus in Alzheimer's disease with myoclonus
Cerebellar dentate nucleus in Alzheimer's disease with myoclonus
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DOI：
10.1159/000017106
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发表时间：
1999-03-01
影响因子：
2.4
通讯作者：
Lantos, PL
中科院分区：
文献类型：
--
作者：
Fukutani, Y;Cairns, NJ;Lantos, PL
虽然肌阵挛通常发生在阿尔茨海默病（AD）的晚期，但这种症状的病理基础尚不清楚。为了阐明阿尔茨海默病中肌阵挛的神经病理底物，我们定量评估了8例患有肌阵挛的阿尔茨海默病患者、10例无肌阵挛的阿尔茨海默病患者和9例对照者小脑齿状核吻侧和尾侧神经元的密度和体积，并区分了大小神经元。肌阵挛性阿尔茨海默病组鼻侧大神经元数量密度和总数（吻侧和尾侧）显著大于非肌阵挛性阿尔茨海默病组。两组间小神经元密度差异无统计学意义，有肌阵挛性AD小神经元与大神经元总数之比（S/L比）明显低于无肌阵挛性AD，肌阵挛性AD吻部大神经元平均体积明显大于非肌阵挛性AD。相反，肌阵挛性AD的吻侧小神经元体积明显低于非肌阵挛性AD。本研究首次发现AD伴肌阵挛患者大神经元平均体积增大，小神经元平均体积减小，齿状核S/L比发生变化。S/L比失衡以及齿状核内这些神经元的形态学改变可能是AD肌缩的病理底物之一。
Although myoclonus commonly occurs in a later stage of Alzheimer's disease (AD), the pathological basis of this symptom is still unclear. In order to elucidate the neuropathological substrate of myoclonus in AD, we quantitatively assessed neuronal density and volume, with a discrimination between small and large neurons, at the rostral and caudal parts in the cerebellar dentate nucleus of 8 AD patients with myoclonus, 10 AD patients without myoclonus and 9 controls, using stereological probes. The neuronal numerical density of the large neurons at the rostral part and of total counts (rostral and caudal parts) in the myoclonic AD group were significantly greater than in the nonmyoclonic AD group. There were no significant differences in the density of small neurons between the two AD groups, The ratio of small neurons to large neurons (S/L ratio) of total counts was significantly lower in AD with myoclonus than in AD without myoclonus, The mean neuronal volume of the large neurons at the rostral part was significantly greater in myoclonic AD than in nonmyoclonic AD. Conversely, the volume of the small neurons at the rostral part was significantly lower in myoclonic AD than in nonmyoclonic AD. This study, for the first time, shows an increase in mean volume of large neurons and a decrease in mean volume of small neurons as well as a change in the S/L ratio in the dentate nucleus in AD with myoclonus. An im balance in the S/L ratio as well as morphological changes of these neurons in the dentate nucleus may contribute to the pathological substrate of myoclonus in AD.