Background Huntington’s disease (HD) is a genetic neurodegenerative disease that leads to premature death. However little evidence is available regarding late-stage HD including causes and places of death.
Aims This retrospective study aims to ascertain HD causes and places of death in a European Huntington’s disease cohort – the REGISTRY.
Methods REGISTRY, the European HD Network’s prospective observational study, was used for data collection from 2001 to 2013. Our study population consisted of manifest HD patients having attended more than one study visit.
For deceased subjects data was extracted from the death report form and incorporated information such as cause and place of death. Statistical analysis included descriptive statistics and survival analysis through Kaplan Meier estimates of median survival time using SPSS and R software.
Results A total of 5164 participants were analysed (50.9% women and 98.1% Caucasians). At the end of the study period there were 533 deaths (10.3% of cases). For deceased subjects, the median age of diagnosis was 49 years (9–85) and the median age of death was 58 years (16–93).
The most frequent cause of death was pneumonia (25.6%) followed by: other infections (9.1%), suicide (8.6%), cancer (4.4%), stroke (3.4%), and trauma (1.2%). The cause of death was undetermined in 47.7% of deaths. The most frequent place of death was the hospital (29.8%) followed by: home (23.9%), nursing houses (19.8%), and hospice care facilities (3.3%). The place of death was undetermined in 23.1% of deaths.
The median survival time as time from diagnosis to death was 24 years (95% CI: 20.8–27.2); and as time from symptom onset to death was 35 years (95% CI: 29.2–40.8).
Conclusion In the REGISTRY cohort HD patients succumb to the same conditions shown in former HD and other neurodegenerative diseases studies. Still, our results show HD has its own natural history, being the age of death and the survival after the diagnosis unique among neurodegeneration.
- Huntington’s disease
- causes of death