Huntington’s Disease

The condition

  • Huntington’s Disease (HD) is a progressive, hereditary neurological disorder which used to be known as Huntington’s Chorea.
  • Symptoms are wide ranging and usually begin in middle adulthood, but can occur at any age.
  • HD is caused by a faulty gene on chromosome 4, which leads to damage of the nerve cells in specific areas of the brain.
  • Each person whose parent has HD is born with a 50:50 change of inheriting the faulty gene. Anyone who inherits the faulty gene will, at some stage, develop the disease.
  • The course of the disease is unpredictable and involves a triad of movement, thinking and emotional symptoms, which vary even within the same family.
  • Early symptoms might include involuntary movements, clumsiness, lack of concentration, short term memory lapses, depression, mood changes, irritability and aggressive outbursts.
  • Symptoms gradually deteriorate over approximately 10 – 20 years. In the later stages there tends to be a more global dementia, a continuation of psychological symptoms, an inability to communicate verbally, impaired swallowing and complete physical dependence.
  • There is no known cure for HD, but symptoms such as depression, mood-swings and involuntary movements can be reduced by prescribed drugs.

Incidence and prevalence

  • One in 10,000 of the UK population are symptomatic at any one time.
  • Three times this number are pre symptomatic.
  • Many more than this are living with being at risk, but have not been tested.

The impact on health and social care services

In addition to the ‘core team’, people living with HD may require support from:

  • Genetic services
  • Mental health services
  • Domiciliary and palliative care services
  • Respite services
  • Specialist equipment (eg specialised beds, chairs and wheelchairs, communication aids).