OBJECTIVES

Prevalence and incidence of Huntington’s disease (HD) has been previously characterised in the UK between 1990 and 2010. This study revisits this question to inform appropriate provision of health and social care for individuals with HD. We estimated the prevalence and incidence of HD in the UK between 2000 and 2018 and explored demographic features including age, sex and regional differences.

METHODS

Prevalent cases were defined as adult individuals (≥18 years old) with ≥1 diagnosis of HD or Huntington's chorea recorded before or during the study period (2000–2018) in UK primary care data. Incident cases had their first recorded diagnosis during the study period.

RESULTS

Overall, 881 diagnosed HD cases were identified from a total adult population of 8,367,580, yielding an overall prevalence of 10.5 (95% confidence interval [CI] 9.9–11.3) cases per 100,000 persons, which was similar between sexes. Median age of diagnosis was 52 years (interquartile range =22), and prevalence was highest in those aged 45–54 years (18 per 100,000 cases; 95% CI 15.3–20.03). Prevalence increased between 2000 and 2018 from 4.3 (95% CI 3.7–5.1) to 13 (95% CI 11.4–14.7) per 100,000 persons. Regional prevalence showed heterogeneity: highest in Scotland (15; 95% CI 13.3–17.9) and lowest in London (4; 95% CI 3.0–5.5) per 100,000 persons. Of the 881 cases, 586 patients were defined as incident within the study period. Overall incidence of HD was 0.85 per 100,000 person years (95% CI 0.8–0.9), which remained relatively stable over time. Incidence rates were similar for both sexes and were higher in older individuals, peaking in those aged >75 years.

CONCLUSIONS

In adult individuals with HD, demographic-specific prevalence and annual trends were consistent with earlier published research (1990–2010). Incidence remained stable over time and was higher in older individuals.