前驱期多系统萎缩的研究进展

Research progress on prodromal multiple system atrophy

多系统萎缩(MSA)是一种进展迅速的神经系统退行性疾病,和帕金森病(PD)、路易体痴呆(DLB)同属于α-突触核蛋白病。由于同类疾病早期临床症状的重叠以及目前诊断标准对疾病早期缺乏敏感性,开展疾病早期修饰治疗药物的临床试验困难重重,疾病修饰治疗药物的研发以及诊断工具的开发和验证进展缓慢。因此,国际运动障碍协会(MDS)开发了新的MSA诊断标准,首次提出可能的前驱期MSA的定义和诊断标准。本综述介绍了可能的前驱期MSA诊断标准以及与其他前驱期α-突触核蛋白病鉴别诊断,以提高疾病在早期阶段诊断的准确性,有利于疾病早期研究的开展。

Multiple system atrophy(MSA)is a rapidly progressive neurodegenerative disease and,along with Parkinson's disease(PD)and dementia with Lewy bodies(DLB),belongs to the group ofα-synucleinopathies.Due to an overlap of clinical symptoms of similar diseases in the early stages and a lack of sensitivity of the current diagnostic criteria to detect the disease in the early stages,difficulties abound in conducting clinical trials on disease-modifying therapeutic agents for its early treatment,and progress in the development of disease-modifying therapeutic agents as well as the development and validation of diagnostic tools has been slow.Consequently,the International Movement Disorder Society(MDS)has introduced new diagnostic criteria for MSA,which,for the first time,propose a category known as possible prodromal MSA and its diagnostic criteria.This review described the diagnostic criteria for possible prodromal MSA and its differential diagnosis from other prodromalα-synucleinopathies,in order to improve the accuracy of diagnosis in the early stages and to promote research on the early stages of the disease.