What is the life expectancy of Huntington's disease?

From the Guidelines

Huntington's disease has a life expectancy of 15 to 20 years after onset.

Life Expectancy Details

• The average age of onset is between 35 to 45 years [ 1 ].
• The disease is characterized by progressive behavioral symptoms, choreoathetosis and/or rigidity, and cognitive dysfunction [ 1 ].
• The genetic basis of the disease is an abnormally increased number of CAG repeats in the huntingtin gene [ 1 ].
• Key points to note are:
  • The incidence of HD is approximately 10/100,000 [ 1 ].
  • The prevalence of HD exceeds 15 per 100,000 in some populations, mostly of western European origin [ 1 ].
  • Juvenile-onset HD occurs in approximately 5% of affected patients and is a rapidly progressive variant [ 1 ].
• It's worth noting that while there is some variation in the age of onset and life expectancy, 15 to 20 years after onset is the generally accepted range [ 1 ].

From the Research

Life Expectancy of Huntington's Disease

• The life expectancy of Huntington's disease patients is approximately 20 years after diagnosis 2, 3.
• Death usually occurs due to complications such as pneumonia, followed by suicide 2.
• The disease progression leads to complete dependency in daily life, resulting in patients requiring full-time care 2.
• The duration of the disease from onset to death is typically around 15-20 years 4.

Factors Affecting Life Expectancy

• The length of the CAG repeat expansion is inversely correlated with the age of onset, with longer expansions leading to earlier onset 2, 4.
• Juvenile Huntington's disease, characterized by onset before the age of 20, often has a more rapid progression and shorter life expectancy 2.
• Symptom management and palliative care can improve the quality of life, but do not significantly alter the life expectancy 5.