The epidemiology of dystonia: the Hannover epidemiology study

• Published in: Journal of neurology 2022-12, Vol.269 (12), p.6483-6493
• Main Authors: Dressler, Dirk, Altenmüller, Eckart, Giess, Ralf, Krauss, Joachim K., Adib Saberi, Fereshte
• Format: Article
• Language: English
• Subjects: Arm; Complex regional pain syndrome; Dystonia; Epidemiology; Medicine; Medicine & Public Health; Neurology; Neuroradiology; Neurosciences; Original Communication
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-022-11310-9

The prevalence of dystonia has been studied since the 1980s. Due to different methodologies and due to varying degrees of awareness, resulting figures have been extremely different. We wanted to determine the prevalence of dystonia according to its current definition, using quality-approved registries and based on its relevance for patients, their therapy and the health care system. We applied a service-based chart review design with the City of Hannover as reference area and a population of 525,731. Barrier-free comprehensive dystonia treatment in few highly specialised centres for the last 30 years should have generated maximal dystonia awareness, a minimum of unreported cases and a high degree of data homogeneity. Prevalence [ n /1mio] and relative frequency is 601.1 (100%) for all forms of dystonia, 251.1 (42%) for cervical dystonia, 87.5 (15%) for blepharospasm, 55.2 (9%) for writer’s cramp, 38.0 (6%) for tardive dystonia, 32.3 (5%) for musician’s dystonia, 28.5 (5%) for psychogenic dystonia, 26.6 (4%) for generalised dystonia, 24.7 (4%) for spasmodic dysphonia, 20.9 (3%) for segmental dystonia, 15.2 (3%) for arm dystonia and 13.3 (2%) for oromandibular dystonia. Leg dystonia, hemidystonia and complex regional pain syndrome-associated dystonia are very rare. Compared to previous meta-analytical data, primary or isolated dystonia is 3.3 times more frequent in our study. When all forms of dystonia including psychogenic, generalised, tardive and other symptomatic dystonias are considered, our dystonia prevalence is 3.7 times higher than believed before. The real prevalence is likely to be even higher. Having based our study on treatment necessity, our data will allow better allocation of resources for comprehensive dystonia treatment.