The role of the cerebellum in the pathogenesis of cortical myoclonus
The role of the cerebellum in the pathogenesis of cortical myoclonus
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DOI：
10.1002/mds.25867
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发表时间：
2014-04-01
影响因子：
8.6
通讯作者：
Bhatia, Kailash P.
中科院分区：
文献类型：
--
作者：
Ganos, Christos;Kassavetis, Panagiotis;Bhatia, Kailash P.
小脑参与皮质性肌阵挛综合征的发病机制一直是一个假设，因为皮质性肌阵挛患者的神经病理学变化最常见于小脑，而不是在怀疑的罪魁祸首，初级躯体感觉皮质。已经提出了一个模型，增加皮质兴奋性，由于小脑抑制控制通过小脑丘脑皮层连接的损失，但证据仍然模棱两可。在这里，我们探讨这一假设，通过检查综合征，目前与皮质肌阵挛和共济失调。我们首先描述共同的临床特征和潜在的神经病理学。我们批判性地看待小脑生理学的信息，关于运动皮质输出和比较结果之间的假设和报告的神经生理学变化的条件下，皮质肌阵挛和共济失调。我们综合知识，专注于这些条件下的神经化学变化。最后，我们提出，抑制性神经传递的改变和小脑病理的存在相结合的皮质肌阵挛的发病机制中的重要因素。(c)2014运动障碍协会
The putative involvement of the cerebellum in the pathogenesis of cortical myoclonic syndromes has been long hypothesized, as neuropathological changes in patients with cortical myoclonus have most commonly been found in the cerebellum rather than in the suspected culprit, the primary somatosensory cortex. A model of increased cortical excitability due to loss of cerebellar inhibitory control via cerebello-thalamo-cortical connections has been proposed, but evidence remains equivocal. Here, we explore this hypothesis by examining syndromes that present with cortical myoclonus and ataxia. We first describe common clinical characteristics and underlying neuropathology. We critically view information on cerebellar physiology with regard to motorcortical output and compare findings between hypothesized and reported neurophysiological changes in conditions with cortical myoclonus and ataxia. We synthesize knowledge and focus on neurochemical changes in these conditions. Finally, we propose that the combination of alterations in inhibitory neurotransmission and the presence of cerebellar pathology are important elements in the pathogenesis of cortical myoclonus. (c) 2014 Movement Disorder Society