OBJECTIVES: Huntington’s disease (HD) is a rare, progressive, genetic neurodegenerative disorder, with a worldwide prevalence of 5.7 per 100,000 and 7-12 per 100,000 in Europe. The aim of the current systematic review was to assess the economic burden of HD in Europe.

METHODS: Medline^®, Embase^®, NHSEED, and EconLit databases were searched to identify English-language literature from database inception to May 2019 that assessed cost burden and resource utilization associated with HD

RESULTS: The review included 13 studies with majority of the studies assessing direct and indirect costs associated with HD (cost: 10, resource use: 5, HSUVs: 4). Medical treatment was found to be the major driver of cost across studies. Indirect costs varied across countries with the highest levels of resource use in France and the lowest in Poland & Italy, but caregiver cost and patient unemployment were higher in Poland & Italy as compared to other countries. Four studies reported substantial caregiver time in Spain, Poland, Italy, and France. In the UK, the mean annual cost per person was nearly five times higher in juvenile HD as compared to adult HD (£112,581 vs. £21,605) where 24% and 65% of the total cost contributed to the caregiver burden respectively. In Spain and Poland, mean monthly visits to a physician ranged from 0.13 to 2.23. Women generally had lower HSUVs than men, overall ranging from -0.11 to 0.79. In the UK, mean annual cost of £2250/person for stage I rose to £89,760 for stage IV, equating to £195 million per year in 2013.

CONCLUSIONS: The overall economic burden of HD is substantial and increases with disease severity. Medical treatment and informal care were the largest drivers of costs highlighting that caregivers needed to be considered while planning services for people with HD.