Measuring quality of life in multiple system atrophy

Abstract

© Cambridge University Press 2011. Introduction Multiple system atrophy (MSA) is a rare, progressive, neurodegenerative disease and a form of atypical parkinsonism. It has a prevalence of about 2 to 5 per 100,000 and has a considerable impact on patient outcomes, including reduced life expectancy, increased disability, and lower health-related quality of life (HRQoL). Average age of onset is about 54 years, and mean survival from the onset of symptoms is around 5 to 9 years. MSA often presents with features similar to those of Parkinson's disease (PD) in its early stages and consequently may be difficult to diagnose correctly. As a result, many patients may not receive appropriate treatment for their condition. However, despite the intial similarities, MSA has a wider range of symptoms and a differing and more aggressive disease progression and is poorly responsive to treatments commonly associated with PD, such as levopoda. Some evidence suggests that rates of depression are high in patients diagnosed with MSA. Currently, no treatment consistently benefits patients, resulting in increasing dependency on the patient's family and other caregivers, which in turn can have consequences for their HRQoL. MSA has two different subtypes. MSA-P is so called because of the predominance of parkinsonism symptoms, whereas MSA-C is identified by a predominance of cerebellar dysfunction. At present, few direct studies have evaluated HRQoL for patients with a diagnosis of MSA. Although Parkinson's disease has been the focus for a number of studies in the 1990s/2000s, very little research has been undertaken to study other forms of parkinsonism such as MSA. This is unfortunate because, although superficial similarities between the conditions have been noted, the more aggressive nature of MSA requires its own body of evidence to better study patients' outcomes, HRQoL, and health needs.