Life Expectancy in Huntington's Disease
Adults with Huntington's disease typically survive 15-20 years after symptom onset, with death occurring at a mean age of approximately 58 years. 1, 2, 3
Survival Data from Clinical Studies
The most robust survival data comes from the European Huntington's Disease Network (REGISTRY) prospective cohort study, which analyzed 5,164 genetically confirmed HD patients and found:
- Median survival of 24 years from diagnosis 2
- Median survival of 35 years from symptom onset 2
- Mean age at death of 58 years (with mean age at diagnosis of 49 years) 2
These findings align with established clinical descriptions indicating a 15-20 year disease course from symptom onset to death. 1, 3
Geographic and Population Variations
Important caveat: A Korean study of 47 genetically confirmed HD patients found a median survival of only 14.5 years from onset (95% CI: 12.3-16.6), suggesting survival may be shorter in Asian populations or at least in the lower range of expected survival. 4 This represents the only Asian survival data available and warrants consideration when counseling patients of East Asian descent.
Age-Related Factors
- Typical adult-onset HD (age 30-50 years at onset) follows the 15-20 year survival pattern 5, 1
- Juvenile HD (onset before age 20, occurring in ~5% of cases) is a rapidly progressive variant with shorter survival, typically associated with CAG repeats exceeding 55 6, 5
Common Causes of Death
The European REGISTRY study identified the most frequent causes of death as:
These causes mirror other neurodegenerative diseases, though HD patients have a longer median time from onset to death compared to Parkinson's disease and Alzheimer's disease. 2
Clinical Implications for Prognostication
No specific factors reliably predict individual survival time. The Korean study found that gender, age at onset, CAG repeat size (both normal and mutant alleles), and presence of non-motor symptoms at onset did not significantly affect survival in Cox regression analysis. 4
The disease produces a devastating triad of progressive motor dysfunction, cognitive decline, and psychiatric disturbances over this 15-20 year course, with no currently available disease-modifying treatments. 7, 8, 6 All available therapies remain purely symptomatic. 7, 8