Huntington's disease

Huntington's disease is a condition which damages some of the nerve cells in the brain. The damage slowly gets worsened over time and can affect cognition (thinking, judgment, perception, awareness) and behavior. This disease is also known as Huntington’s chorea.

Risk factors

It is inherited through a faulty gene from the parents. However, such inherited cases account for only about 3% of the total cases. This is because parents die before even having babies.

Both men and women are equally at the chance of inheriting the defective genes from the parents.


The doctor initially checks for the symptoms, performs a physical examination, and takes a family history. The nervous system is also examined to check for the signs such as dementia, abnormal reflexes, hesitant speech, abnormal movements, etc.

Other tests which are performed to confirm that the person has Huntington's disease are

  • Psychological testing
  • CT or MRI scan of the head
  • PET scan of the brain

Genetic testing is also performed to check if the person carries the related genes for Huntington's disease.


After the onset of Huntington's disease, the following complications can arise after a few months to years:

Depression: The patient with Huntington's disease develops suicidal tendencies due to depression over a long period. This usually develops in the middle stage of the disease.
Complete dependence: The patient with Huntington's disease cannot carry out daily activities by oneself. In the later stages of the disease, the person gets completely confined to the bed and cannot speak but can only understand language.
Death: Nearly after 10-30 years of onset of the disease, Huntington's disease can result in death. The most common causes of death in these patients are pneumonia and other infections, falls and injuries, complications associated with swallowing difficulty.


Huntington's disease is caused due to a defect in the chromosome 4 which can be inherited. As a result of this defective gene, a part of the DNA repeatedly occurs than the normal. Larger the repeats of the DNA more are the chances of a person developing the condition at an early stage when inherited.

Huntington's disease can occur in two forms:

  1. Adult-onset: This form of Huntington's disease develops in mid- 30s or 40s.
  2. Early-onset: This form of Huntington's disease affects less number of persons and occurs in childhood or teenage.

If any one of the parents has a form of Huntington's disease, then the chances of the child developing the condition are around 50%.


Huntington's disease cannot be cured with any treatment nor can the condition be prevented from worsening. The main goal of the treatment of Huntington’s disease is to relieve the symptoms and allow the person to carry out the activities normally.

For symptomatic relief, the following medicines may be prescribed:

  • Dopamine blockers may be prescribed to reduce the abnormal movements and the behavior
  • Amantadine or tetrabenazine can help to control the extra movements

It is common for the patients with Huntington's disease to have suicidal tendencies. Therefore, the caregivers should keep an eye on the symptoms and notify the doctor immediately. With the disease progression, the person may need continuous care, even for 24 hours in a day.

Certain support groups are also available for Huntington patients.

The first features which occur in the person with Huntington's disease are the behavioral changes which include:

  • Mood swings
  • Hallucinations
  • Psychosis
  • Paranoia
  • Restlessness and fidgety movements
  • Irritability
  • Altered behavior

Following the behavioral changes, abnormal and unusual movements occur, which include:

  • Unsteady gait
  • Slow and uncontrolled movements
  • Turning head to shift eye position
  • Jerky and sudden movements of the face, arms, legs and other parts of the body
  • Facial movements such as grimaces

Dementia which slowly worsens:

  • Loss of memory
  • Speech changes
  • Loss of judgment
  • Confusion or disorientation
  • Personality changes

The other symptoms that are associated with the disease are anxiety, stress, tension, difficulty swallowing, and speech impairment.

In children, the symptoms include rigidity, tremors, and slow movements.

Huntington's disease becomes very difficult to manage as it progresses. This is because of the increased dependence of the patient on the caregivers. The following tips can be beneficial to cope up with the condition:

Diet and nutrition: As the patients with Huntington's disease usually have difficulty in maintaining a healthy weight, adequate nutrition is of high importance. At least three meals or more per day is helpful. Also, the patient should be given the foods which are easy to swallow due to their difficulty with chewing and swallowing.

Cognitive and psychiatric disorders: By following certain strategies, the families and the caregivers can avoid stress in the patients and manage the cognitive and behavioral challenges. These strategies include:
  • Using calendars and notes for maintaining regular schedules
  • Breaking down the larger tasks into smaller and manageable tasks
  • Prioritizing work and organizing the activities
  • Creating a calm and simple environment
  • Giving opportunities for social interaction with the people