Przewodowska Dominika, Marzec Weronika, Madetko Natalia
Students' Scientific Association of the Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
Front Mol Neurosci. 2021 Aug 26;14:720220. doi: 10.3389/fnmol.2021.720220. eCollection 2021.
Atypical parkinsonian syndromes are rare, fatal neurodegenerative diseases associated with abnormal protein accumulation in the brain. Examples of these syndromes include progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration. A common clinical feature in parkinsonism is a limited improvement with levodopa. So far, there are no disease-modifying treatments to address these conditions, and therapy is only limited to the alleviation of symptoms. Diagnosis is devastating for patients, as prognosis is extremely poor, and the disease tends to progress rapidly. Currently, potential causes and neuropathological mechanisms involved in these diseases are being widely investigated. The goal of this review is to summarize recent advances and gather emerging disease-modifying therapies that could slow the progression of atypical parkinsonian syndromes. PubMed and Google Scholar databases were searched regarding novel perspectives for atypical parkinsonism treatment. The following medical subject headings were used: "atypical parkinsonian syndromes-therapy," "treatment of atypical parkinsonian syndromes," "atypical parkinsonian syndromes-clinical trial," "therapy of tauopathy," "alpha-synucleinopathy treatment," "PSP therapy/treatment," "CBD therapy/treatment," "MSA therapy/treatment," and "atypical parkinsonian syndromes-disease modifying." All search results were manually reviewed prior to inclusion in this review. Neuroinflammation, mitochondrial dysfunction, microglia activation, proteasomal impairment, and oxidative stress play a role in the neurodegenerative process. Ongoing studies and clinical trials target these components in order to suppress toxic protein accumulation. Various approaches such as stem cell therapy, anti-aggregation/anti-phosphorylation agent administration, or usage of active and passive immunization appear to have promising results. Presently, disease-modifying strategies for atypical parkinsonian syndromes are being actively explored, with encouraging preliminary results. This leads to an assumption that developing accurate, safe, and progression-halting treatment is not far off. Nevertheless, the further investigation remains necessary.
非典型帕金森综合征是罕见的致命性神经退行性疾病，与大脑中异常蛋白质积聚有关。这些综合征的例子包括进行性核上性麻痹、多系统萎缩和皮质基底节变性。帕金森病的一个常见临床特征是左旋多巴治疗效果有限。到目前为止，尚无改善病情的治疗方法来应对这些病症，治疗仅局限于缓解症状。诊断对患者来说是灾难性的，因为预后极差，且疾病往往进展迅速。目前，这些疾病的潜在病因和神经病理机制正在广泛研究中。本综述的目的是总结近期进展，并收集可能减缓非典型帕金森综合征进展的新兴改善病情疗法。通过检索PubMed和谷歌学术数据库以获取非典型帕金森病治疗的新观点。使用了以下医学主题词：“非典型帕金森综合征-治疗”、“非典型帕金森综合征的治疗”、“非典型帕金森综合征-临床试验”、“tau蛋白病治疗”、“α-突触核蛋白病治疗”、“进行性核上性麻痹治疗”、“皮质基底节变性治疗”、“多系统萎缩治疗”以及“非典型帕金森综合征-病情改善”。所有检索结果在纳入本综述之前均经过人工审核。神经炎症、线粒体功能障碍、小胶质细胞激活、蛋白酶体损伤和氧化应激在神经退行性过程中起作用。正在进行的研究和临床试验针对这些成分以抑制毒性蛋白积聚。各种方法，如干细胞治疗、给予抗聚集/抗磷酸化药物或使用主动和被动免疫，似乎都有前景良好的结果。目前，正在积极探索非典型帕金森综合征的改善病情策略，初步结果令人鼓舞。这使得人们认为开发准确、安全且能阻止疾病进展的治疗方法为期不远。然而，仍有必要进行进一步研究。
