Genetic Disorders

Everything you need to know about Huntington’s chorea

There are certain health ailments that tend to worsen with time as there is no known cure for the same. Huntington’s disease is a progressive condition that brings about the degeneration of the nerve cells in the brain and thereby affects the individual’s functional abilities and gives rise to the movement, cognitive, and psychiatric disorders. Huntington’s disease usually manifests itself in the 30s and the 40s. But, it is also known to affect children in their 20s, and this condition is termed as juvenile Huntington’s disease. As this condition begins at an early age, the symptoms tend to differ and the progression of the disease is faster than the regular Huntington’s disease.

Huntington’s disease is an inherited condition and is caused by an inherited defect in the single gene. In fact, it is an autosomal dominant disorder, which implies that a person needs only one copy of the defective gene to develop the disorder. A person inherits two copies of every gene, so a parent with a defective gene can pass on either the defective gene or the healthy one. Which is why the children in the family have about 50 percent chances of inheriting the gene that causes this condition.

Huntington’s disease is characterized by several movement, cognitive, and psychiatric disorders, and one of the prominent symptoms of this condition is chorea. Chorea is a movement disorder that is characterized by involuntary and unpredictable body movements. The severity of chorea can range from minor fidgeting movements to severe uncontrolled movements of the arms and the legs. Moreover, chorea is known to affect the individual’s speech, posture, ability to swallow food, and their gait.

Recognizing that the individual with Huntington’s disease has developed chorea is essential as it will help the doctor in determining the course of the treatment. The symptoms of Huntington’s chorea are as follows:

  • The most prominent symptoms of Huntington’s chorea is the “milkmaid’s grip”. The milkmaid’s grip occurs when the person doesn’t have strong hand muscles and will squeeze and release their hands as if milking.
  • Another prominent symptom of Huntington’s chorea is the involuntary sticking out of the tongue.
  • Moreover, the chorea movements can be fast or slow. For instance, the person may appear to be writhing in pain and have no bodily control.

Chorea is one of the first symptoms to manifest itself in people with adult-onset Huntington’s disease. After some time, the chorea symptoms can worsen, and it can affect the legs and the arms. As the disease progresses, chorea and other symptoms of Huntington’s disease worsen as well and the individual’s functional abilities deteriorate. The rate of progression and the duration varies from person to person, and the life expectancy lies somewhere between 10 to 30 years from the inception of this condition.

Moreover, along with chorea and other prominent symptoms, people with Huntington’s disease are diagnosed with clinical depression as well and this increases their risk of suicide. This is often associated with the advancing chorea and the loss of function which forces them to depend on caregivers for performing their daily activities. As the condition progresses, they lose control over their movements and are usually confined to the bed and lose their ability to communicate as they can no longer speak.

Chorea is one of the prominent symptoms of Huntington’s disease, which like all its other symptoms worsens gradually and contributes to the individual’s deteriorating health.