Objective: This study sought to systematically review the methods and results of previous incidence and prevalence studies of MSA.

Background: High-quality incidence and prevalence studies are important for health-care planning and epidemiological research. To our knowledge, there are no existing systematic reviews summarising the current evidence on both the incidence and prevalence of multiple system atrophy (MSA).

Method: Two authors independently identified original articles that described the incidence or prevalence of MSA using several comprehensive and overlapping search strategies, assessed study quality, and extracted relevant data. Descriptive and pooled analyses were performed as appropriate.

Results: Fifteen incidence studies (including three age-restricted studies) and twenty prevalence (including nine age-restricted studies) studies were identified.  Studies were conducted in Europe (n=22), Asia (n=6), the United States of America (n=3), Africa (n=2), South America (n=1) and Russia (n=1).  Less than half (45.7%) of studies used the Gilman consensus criteria (1998 or 2008 iteration according to study year) for diagnosis and case definition. Ten incidence and six prevalence studies used at least two methods of case ascertainment. The percentage of patients examined to verify diagnoses ranged from 0 to 100%. Crude incidence rates ranged from 0.1 to 2.1 per 100,000 person-years and crude prevalence rates ranged from 1.0 to 16.6 cases per 100,000 in studies unrestricted by age.  Few studies reported age- or sex- stratified rates. Based on crude rates, there is little evidence to suggest clear sex differences in the incidence or prevalence of MSA but some evidence to suggest that both the incidence and prevalence of MSA increases with advancing age. Due to differences in study methodology, only three incidence studies were sufficiently similar to merit comparison, giving a pooled incidence rate of 0.9 per 100,000 person-years (95% CI 0.5, 1.3) while two similar prevalence studies gave a pooled prevalence rate of 3.6 per 100,000 (95% CI 2.2, 5.0).

Conclusion: There are few incidence and prevalence studies in MSA. Existing studies are methodologically and statistically heterogeneous, which restricts pooled analyses. Further high-quality studies are required using standardised methodology.

References: Gilman S, Low P, Quinn N, et al; American Autonomic Society and American Academy of Neurology. Consensus statement on the diagnosis of multiple system atrophy. Clin Auton Res. 1998;8(6):359-362.

Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008;71:670–6.

« Back to 2024 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/systematic-review-of-incidence-and-prevalence-studies-of-multiple-system-atrophy/