Multiple system atrophy is also known as or subsumes Shy-Drager syndrome. -ed.
Multiple system atrophy is a sporadic degenerative disorder with a highly variable anatomic distribution and clinical picture. It typically features some combination of motor parkinsonism, ataxia, and dysautonomia, along with many other, less constant, signs. Average age at onset is 53 years, and average survival is only 8 years. Although there is no specific treatment, clinicians can palliate many aspects of the syndrome, particularly the dysautonomia and the sleep disturbances. The defining pathologic feature is aggregates of alpha-synuclein in oligodendroglia. In this clinical article, Dr. Robert Fekete of Baylor College of Medicine describes the essential features of multiple system atrophy, including recent advances in distinguishing it from the other parkinsonian disorders and in managing its many disabling clinical features.