Clinical aspects of multiple system atrophy

Abstract

I review the disease concept and recent developments in understanding multiple system atrophy (MSA). Recent studies have revealed that clinical diagnosis of MSA is not necessarily correct because of several different and varied clinical phenotypes as well as the presence of MSA mimics or look–alike syndromes. I discuss the limitations of revised consensus diagnostic criteria (Gilman et al : 2008), several atypical syndromes of MSA including mono–system atrophy, non–motor MSA, frontotemporal lobar degeneration (FTLD)–synuclein, and young–onset MSA, as well as clinical and imaging mimics. Because therapeutic interventions that target α–synuclein have started, further refinement of the diagnostic criteria is needed via biomarker researches with prospective study designs.