Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by cardiovascular and/or urinary autonomic failure and parkinsonism and/or cerebellar impairment movement disorders [1], [2]. MSA is sub-classified according to the predominant clinical phenotype. MSA with predominantly parkinsonian features is designated as MSA-P and MSA with predominant cerebellar ataxia features is designated as MSA-C [3]. The onset of MSA is aged > 30 years with a mean age of 55–60 years and the disease affects both sexes equally. MSA has a poor prognosis and its rapid progression results in severe disability with a median survival of 6–9 years. Treatment is available for some symptoms, in particular autonomic dysfunction, yet disease modification remains an urgent unmet need.

The prevalence of MSA has been examined in multiple studies conducted mostly across Europe and in few pan-European countries. Data on the prevalence of MSA are of particular interest due to the insights such studies can provide on risk factors for MSA as well as on the burden of the disease in the population. Such epidemiological data can be used to inform public health planning, especially since the longevity of the population increases and the prevalence of MSA increases with age. To date, the published literature has not included a review of prevalence studies of MSA in European and pan-European countries, especially as related to methodological variation. This review provides and overview on the prevalence of MSA in European and pan-European populations with a focus on the methodology used in the relevant studies.