Bhidayasiri Roongroj, Ling Helen
Division of Neurology, Chulalongkorn Comprehensive Movement Disorders Center, Chulalongkorn Univerisity Hospital, Bangkok, Thailand.
Neurologist. 2008 Jul;14(4):224-37. doi: 10.1097/NRL.0b013e318167b93f.
It has been almost 4 decades since the descriptions of the 3 parts of multiple system atrophy (MSA) have taken place, characterized clinically by dysautonomia, parkinsonism, and cerebellar dysfunction. The discovery of a distinctive pathologic maker has finally provided the conceptual synthesis of these 3 entities into the universal designation of MSA as a distinct disease process with a complex combination of clinical presentations. Although advances have been made in terms of awareness and knowledge concerning the clinical features and pathophysiology of MSA, it remains challenging for neurologists who treat these patients to differentiate MSA from its mimics as well as providing them with effective treatment.
The aim of this review is to provide an overview of the advances in the knowledge of the disease, to highlight typical features useful for the recognition of its entity, and to enlist different treatment options.
Despite the fact that there is still no treatment modality that can alter the disease progression, a number of useful symptomatic treatment measures are available and should be offered to patients to ameliorate the nonmotor features of MSA and even the motor features that may at least transiently respond to treatment.
自对多系统萎缩(MSA)的三个部分进行描述以来,已经过去了近40年,其临床特征为自主神经功能障碍、帕金森综合征和小脑功能障碍。一种独特病理标志物的发现最终将这三个实体概念性地整合为MSA这一通用名称,它是一种具有复杂临床表现组合的独特疾病过程。尽管在对MSA的临床特征和病理生理学的认识和了解方面取得了进展,但对于治疗这些患者的神经科医生来说,将MSA与其模仿疾病区分开来并为他们提供有效治疗仍然具有挑战性。
本综述的目的是概述该疾病知识的进展,突出有助于识别其实体的典型特征,并列出不同的治疗选择。
尽管目前仍没有能够改变疾病进展的治疗方式,但有一些有用的对症治疗措施可供采用,应提供给患者以改善MSA的非运动特征,甚至改善那些可能至少对治疗有短暂反应的运动特征。