Wilson's Disease in Finland: A Nationwide Population-Based Study.

BACKGROUND

Data on the epidemiology and prognosis of Wilson's disease are scarce, and no clinical data are available from Finland.

METHODS

All persons diagnosed and treated for Wilson's disease in Finnish hospitals in 1998 to 2017 were identified. Data were collected from national registries and patient charts.

RESULTS

The point prevalence was 0.45/100,000 (95% confidence interval, 0.29-0.67) on December 31, 2017, but no more than 0.35/100,000 (95% confidence interval, 0.21-0.55) among native Finns. Annual incidence was 0.016/100,000 (95% confidence interval, 0.0093-0.026). Median age at diagnosis was 15.8 years (interquartile range, 8.3-32.2; range, 3.8-48.1 years). Upon presentation, liver damage was observed in 58%, neurological signs and symptoms (most often tremor and dysarthria) in 40%, and 32% of patients were asymptomatic. Patients had poorer long-term survival (hazard ratio, 2.92 for death; P = 0.005) compared with matched controls.

CONCLUSIONS

Wilson's disease is very rare in Finland. Patients have an increased risk of death indicating an unmet treatment need. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.