What's New in Multiple System Atrophy

Multiple system atrophy (MSA) is a rare, fatal, rapidly progressing neurodegenerative disorder of uncertain etiology that is clinically characterized by a variable combination of parkinsonism, cerebellar impairment, autonomic dysfunction and pyramidal tract signs. The mean age of disease onset is 56±9 years with poor prognosis and a mean survival of 9.5 years. The prevalence is 1.9 to 4.9 cases/100,000, increasing to 7.8/100,000 after age 50 and the incidence is 3 cases/100,000 [1]. Depending on the predominant initial motor presentation, MSA is classified into a parkinsonism variant (MSA-P) associated with striatonigral degeneration (SND) and a cerebellar variant (MSA-C) defined by olivopontocerebellar atrophy (OPCA) [2]. In the Western hemisphere, MSA-P involves 70% of the patients, while in Asian populations MSA-C predominates in two-thirds of patients [1].