Dystonia as a network disorder: what is the role of the cerebellum?

Dystonia as a network disorder: what is the role of the cerebellum?
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肌张力障碍是一种网络障碍:小脑的作用是什么?

DOI:
10.1016/j.neuroscience.2013.11.062
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发表时间:
2014-02-28
期刊:
影响因子:
3.3
通讯作者:
Jinnah, H. A.
Jinnah, H. A.
中科院分区:
医学3区
文献类型:
--
作者:
Prudente, C. N.;Hess, E. J.;Jinnah, H. A.

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肌张力障碍是一组由持续或间歇性肌肉收缩引起的疾病,导致不自主的姿势或重复运动。有许多不同的临床表现和原因。虽然他们传统上被归因于基底神经节功能障碍,最近的证据表明,功能障碍可能源于其他地区,特别是小脑。最近的证据导致了一种新的观点,即肌张力障碍是一种涉及多个大脑区域的网络障碍。肌张力障碍发病机制的新网络模型提出了许多问题,特别是关于小脑的作用。例如,如果肌张力障碍可能是由小脑功能障碍引起的,那么为什么肌张力障碍中没有小脑症状?为什么局灶性小脑病变或退行性小脑疾病更常与共济失调而不是肌张力障碍有关?为什么肌张力障碍更常见于基底节病变而不是小脑病变?从动物身上得到的答案能外推到人类身上吗?有证据表明小脑没有参与吗?最后,这种新的发病机制模型对神经科学家和临床医生有什么实用价值?本文探讨了这些问题的潜在答案。
The dystonias are a group of disorders defined by sustained or intermittent muscle contractions that result in involuntary posturing or repetitive movements. There are many different clinical manifestations and causes. Although they traditionally have been ascribed to dysfunction of the basal ganglia, recent evidence has suggested dysfunction may originate from other regions, particularly the cerebellum. This recent evidence has led to an emerging view that dystonia is a network disorder that involves multiple brain regions. The new network model for the pathogenesis of dystonia has raised many questions, particularly regarding the role of the cerebellum. For example, if dystonia may arise from cerebellar dysfunction, then why are there no cerebellar signs in dystonia? Why are focal cerebellar lesions or degenerative cerebellar disorders more commonly associated with ataxia rather than dystonia? Why is dystonia more commonly associated with basal ganglia lesions rather than cerebellar lesions? Can answers obtained from animals be extrapolated to humans? Is there any evidence that the cerebellum is not involved? Finally, what is the practical value of this new model of pathogenesis for the neuroscientist and clinician? This article explores potential answers to these questions.
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