The prevalence of primary dystonia: a systematic review and meta-analysis

Abstract

Dystonia is a hyperkinetic movement disorder characterized by sustained muscle contractions that produce repetitive movements and abnormal postures. Specific information on the prevalence of dystonia has been difficult to establish because the existing epidemiological studies of the condition have adopted different methodologies for case ascertainment, resulting in widely differing reported prevalence. Medline and Embase databases were searched using terms specific to dystonia for studies of incidence, prevalence, and epidemiology. All population-based studies reporting an incidence and/or prevalence of primary dystonia were included. Sixteen original studies were included in our systematic review. Fifteen studies reported the prevalence of dystonia, including 12 service-based and three population-based studies. We performed a meta-analysis on the results of the service-based studies, and were able to combine data on the prevalence of several dystonia subtypes. From these studies, we calculated an overall prevalence of primary dystonia of 16.43 per 100,000 (95% confidence interval [CI]: 12.09-22.32). The prevalence of dystonia reported in the three population-based studies appears higher than that reported in the service-based studies. Only 1 of the 16 studies reported an incidence of cervical dystonia. This corresponded to a corrected incidence estimate of 1.07 per 100,000 person-years (95% CI: 0.86-1.32). Despite numerous studies on the epidemiology of dystonia, attempting to determine an accurate prevalence of the condition for health services planning remains a significant challenge. Given the methodological limitations of the existing studies, our own prevalence estimate of primary dystonia likely underestimates the true prevalence of the condition.