The Prevalence of Idiopathic or Inherited Isolated Dystonia: A Systematic Review and Meta-Analysis

Abstract

Background: A systematic review of epidemiological studies of primary dystonia from 1985 and 2010 found an overall prevalence of 16.43 per 100,000 (95% CI = 12.09–22.32). Methods: We performed a systematic review of studies from 2010 and 2022 to determine if there are important differences in epidemiology between these time periods. Results: Nineteen studies were included. Incidence of cervical dystonia, blepharospasm, and oromandibular dystonia were each reported in one study; one study reported incidence for all adult onset idiopathic focal dystonias combined. Using data from 11 studies, we performed random effects meta-analyses of the prevalence of cervical dystonia (9.95 per 100,000; 95% CI = 3.51–28.17), blepharospasm (2.82 per 100,000; 95% CI = 1.12–7.12), laryngeal dystonia (0.40 per 100,000; 95% CI = 0.09–1.83), upper limb dystonia (1.27 per 100,000; 95% CI = 0.36–4.52), oromandibular dystonia (0.57 per 100,000; 95% CI = 0.15–2.15), and idiopathic or inherited isolated dystonia all subtypes combined (30.85 per 100,000; 95% CI = 5.06–187.74). All studies reported more cases of dystonia in females. There was no significant difference in prevalence by subgroup analysis based on time of study publication (1985–2010 vs. 2010–2022). Subgroup analysis of differences in prevalence by dystonia subtype by continent using all studies published (1985–2022) revealed significant regional differences in the prevalence of cervical and laryngeal dystonia. Conclusion: The incidence and prevalence of idiopathic or inherited isolated dystonia in the last decade was not significantly different from earlier reports. Population-based studies across multiple geographic areas are needed to obtain a clearer understanding of the epidemiology of this condition.