Neurology Unit, Department of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, Italy.
Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), Essen University Hospital, University of Duisburg-Essen, 45147 Essen, Germany.
Cells. 2023 Apr 20;12(8):1193. doi: 10.3390/cells12081193.
The degenerative ataxias comprise a heterogeneous group of inherited and acquired disorders that are characterized by a progressive cerebellar syndrome, frequently in combination with one or more extracerebellar signs. Specific disease-modifying interventions are currently not available for many of these rare conditions, which underscores the necessity of finding effective symptomatic therapies. During the past five to ten years, an increasing number of randomized controlled trials have been conducted examining the potential of different non-invasive brain stimulation techniques to induce symptomatic improvement. In addition, a few smaller studies have explored deep brain stimulation (DBS) of the dentate nucleus as an invasive means to directly modulate cerebellar output, thereby aiming to alleviate ataxia severity. In this paper, we comprehensively review the clinical and neurophysiological effects of transcranial direct current stimulation (tDCS), repetitive transcranial magnetic stimulation (rTMS), and dentate nucleus DBS in patients with hereditary ataxias, as well as the presumed underlying mechanisms at the cellular and network level and perspectives for future research.
退行性共济失调包括一组异质性的遗传性和获得性疾病，其特征是进行性小脑综合征，常伴有一个或多个小脑外征象。目前，许多这些罕见疾病都没有特定的疾病修正干预措施，这凸显了寻找有效对症治疗的必要性。在过去五到十年中，已经进行了越来越多的随机对照试验，以研究不同的非侵入性脑刺激技术诱导症状改善的潜力。此外，一些较小的研究也探讨了深部脑刺激（DBS）齿状核作为一种侵入性手段来直接调节小脑输出，从而旨在减轻共济失调的严重程度。在本文中，我们全面回顾了经颅直流电刺激（tDCS）、重复经颅磁刺激（rTMS）和齿状核 DBS 在遗传性共济失调患者中的临床和神经生理学效应，以及在细胞和网络水平上的假定潜在机制，以及未来研究的展望。
