小脑对肌张力障碍的贡献:揭示浦肯野细胞和小脑核的作用。

Dystonia Pub Date : 2025-01-01 Epub Date: 2025-02-16 DOI:10.3389/dyst.2025.14006
Nichelle N Jackson, Jacob A Stagray, Heather D Snell
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引用次数: 0

摘要

肌张力障碍是一组神经退行性疾病,导致与运动相关的生理改变。基底神经节和小脑都是参与运动学习、感觉知觉整合和奖励的大脑区域,它们与肌张力障碍的病理有关,但细胞和亚细胞机制仍然不同,对于某些形式的肌张力障碍,尚不清楚。本综述的目的是总结最近小脑参与不同亚型肌张力障碍的证据,重点是浦肯野细胞(PC)和小脑核(CN)功能障碍,发现病理上的共性,为肌张力障碍患者治疗的未来发展奠定基础。在这里,我们将简要讨论基底节区和小脑之间的物理和功能连接,以及这些连接如何导致张力障碍症状。我们继续使用人类和动物模型数据来讨论小脑细胞类型对特定肌张力障碍和运动障碍的贡献,其中肌张力障碍是次要症状。最终,我们认为PC和CN的不规则可能是肌张力障碍的一个位点,通过钙动力学受损。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cerebellar contributions to dystonia: unraveling the role of Purkinje cells and cerebellar nuclei.

Dystonias are a group of neurodegenerative disorders that result in altered physiology associated with motor movements. Both the basal ganglia and the cerebellum, brain regions involved in motor learning, sensory perception integration, and reward, have been implicated in the pathology of dystonia, but the cellular and subcellular mechanisms remain diverse and for some forms of dystonia, elusive. The goal of the current review is to summarize recent evidence of cerebellar involvement in different subtypes of dystonia with a focus on Purkinje cell (PC) and cerebellar nuclei (CN) dysfunction, to find commonalities in the pathology that could lay the groundwork for the future development of therapeutics for patients with dystonia. Here we will briefly discuss the physical and functional connections between the basal ganglia and the cerebellum and how these connections could contribute to dystonic symptoms. We proceed to use human and animal model data to discuss the contributions of cerebellar cell types to specific dystonias and movement disorders where dystonia is a secondary symptom. Ultimately, we suggest PC and CN irregularity could be a locus for dystonia through impaired calcium dynamics.

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