Phang Michael Weng Lok, Lew Sze Yuen, Chung Ivy, Lim William Kiong-Seng, Lim Lee Wei, Wong Kah Hui
Department of Anatomy, Faculty of Medicine, University of Malaya, Kuala Lumpur, 50603, Malaysia.
Department of Pharmacology, Faculty of Medicine, University of Malaya, Kuala Lumpur, 50603, Malaysia.
Chin Med. 2021 Jan 28;16(1):15. doi: 10.1186/s13020-020-00414-x.
Hereditary ataxia (HA) represents a group of genetically heterogeneous neurodegenerative diseases caused by dysfunction of the cerebellum or disruption of the connection between the cerebellum and other areas of the central nervous system. Phenotypic manifestation of HA includes unsteadiness of stance and gait, dysarthria, nystagmus, dysmetria and complaints of clumsiness. There are no specific treatments for HA. Management strategies provide supportive treatment to reduce symptoms.
This systematic review aimed to identify, evaluate and summarise the published literature on the therapeutic roles of natural remedies in the treatment of HA to provide evidence for clinical practice.
A systematic literature search was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Web of Science, PubMed and Science Direct Scopus were thoroughly searched for relevant published articles from June 2007 to July 2020.
Ten pre-clinical and two clinical studies were eligible for inclusion in this systematic review. We identified the therapeutic roles of medicinal plants Brassica napus, Gardenia jasminoides, Gastrodia elata, Ginkgo biloba, Glycyrrhiza inflata, Paeonia lactiflora, Pueraria lobata and Rehmannia glutinosa; herbal formulations Shaoyao Gancao Tang and Zhengan Xifeng Tang; and medicinal mushroom Hericium erinaceus in the treatment of HA. In this review, we evaluated the mode of actions contributing to their therapeutic effects, including activation of the ubiquitin-proteasome system, activation of antioxidant pathways, maintenance of intracellular calcium homeostasis and regulation of chaperones. We also briefly highlighted the integral cellular signalling pathways responsible for orchestrating the mode of actions.
We reviewed the therapeutic roles of natural remedies in improving or halting the progression of HA, which warrant further study for applications into clinical practice.
遗传性共济失调（HA）是一组由小脑功能障碍或小脑与中枢神经系统其他区域之间的连接中断引起的基因异质性神经退行性疾病。HA的表型表现包括姿势和步态不稳、构音障碍、眼球震颤、辨距不良以及笨拙感。目前尚无针对HA的特效治疗方法。管理策略主要是提供支持性治疗以减轻症状。
本系统评价旨在识别、评估和总结已发表的关于天然药物治疗HA的文献，为临床实践提供证据。
采用系统评价和Meta分析的首选报告项目（PRISMA）进行系统文献检索。对Web of Science、PubMed和ScienceDirect Scopus进行全面检索，以获取2007年6月至2020年7月期间的相关已发表文章。
十项临床前研究和两项临床研究符合纳入本系统评价的标准。我们确定了药用植物甘蓝型油菜、栀子、天麻、银杏、胀果甘草、白芍、葛根和熟地黄；中药配方芍药甘草汤和镇肝熄风汤；以及药用蘑菇猴头菇在治疗HA中的作用。在本评价中，我们评估了有助于其治疗效果的作用方式，包括泛素-蛋白酶体系统的激活、抗氧化途径的激活、细胞内钙稳态的维持以及伴侣蛋白的调节。我们还简要强调了负责协调作用方式的整体细胞信号通路。
我们综述了天然药物在改善或阻止HA进展方面的治疗作用，这些作用值得进一步研究以应用于临床实践。
