Epidemiology of progressive cerebellar ataxia in South Yorkshire, UK

Background

Cerebellar ataxias are a heterogeneous group of rare neurological disorders characterised by progressive gait instability, limb incoordination, speech and oculomotor impairment. UK prevalence estimates remain rather limited, with the most widely cited population-based study performed in South East Wales over two decades ago. The objective is to estimate the current prevalence of progressive cerebellar ataxia within South Yorkshire using a comprehensive NHS (National Health Service) tertiary ataxia service database and to compare findings with previously published UK data.

Methods

A retrospective epidemiological analysis was undertaken using the Sheffield Ataxia Centre NHS clinical database. All alive patients aged over 18 years living in South Yorkshire with confirmed progressive cerebellar ataxia under active or historical follow-up were included, excluding static and structural ataxias where appropriate. Population sizes within South Yorkshire were derived from the 2024 Office for National Statistics (ONS) mid-year population estimates for everyone aged over 18 years and over 65 years.

Results

A total of 1204 individuals with progressive cerebellar ataxia were identified as resident in South Yorkshire, corresponding to a frequency prevalence of approximately 106 per 100 000 population (1:942). Prevalence varied by local authority, ranging from 1:647 in Sheffield to 1:1677 in Doncaster.

Conclusions

The prevalence of progressive cerebellar ataxia in South Yorkshire is substantially higher than previously reported UK figures, likely reflecting improvements in diagnostic capability, service centralisation and recognition of immune-mediated and new novel genetic ataxias. These figures have important implications for service planning and resource allocation within the NHS.