Multiple system atrophy

Publisher Summary This chapter discusses the epidemiology, clinical features, diagnosis, and management of the multiple system atrophy (MSA). MSA is a progressive sporadic degenerative disease of the central and autonomic nervous systems of an unknown cause. Clinically, it manifests as a motor disorder, incorporating parkinsonism, cerebellar, or pyramidal features, together with urogenital or cardiovascular autonomic involvement. The population prevalence of MSA has been estimated at 4/100,000, although with wide confidence intervals. The commonest cause for misdiagnoses of MSA is Parkinson's disease (PD). Cases of progressive supranuclear palsy (PSP), corticobasal degeneration, and cerebrovascular disease, particularly when it coexists with PD, can sometimes be difficult to differentiate. It is important to make a definitive diagnosis. Unfortunately, especially early in the disease course, this may not yet be possible and the diagnosis may need to be reviewed at intervals. For many patients, paramedical intervention—physiotherapy, occupational therapy, speech therapy, social work, and an expert wheelchair clinic assessment—may give most benefits.