An Overview of Huntington's Disease (Huntington Chorea)

Huntington's disease (sometimes called Huntington's chorea) is a health condition in which definite nerve cells in the brain degenerate. This condition is the result of an inherent imperfection in chromosome 4. The imperfection causes part of the DNA, named CAG to replicate many more times than it is supposed to. In normal conditions that part of the DNA replicates 10 to 35 times. But for individuals having Huntington's disease, it is recurring 36 -120 times. The gene is passed from one generation to another. The greater the gene replications, the greater are the chances to develop symptoms of Huntington's disease at an earlier age.

Types of Huntington's disease

There are two main types of Huntington's disease or Huntington's chorea; they are adult onset and early onset Huntington's disease. The word chorea refers to involuntary movements.

Adult onset Huntington's disease - Adult onset Huntington's disease is the most common form of the disease. The symptoms of this form of the disease usually develop in one's mid 30s and 40s.

Early onset Huntington's disease - Early onset Huntington's disease can begin in childhood. The symptoms of this form of the disease may look similar to Parkinson's disease; the child would have stiffness, tremors and have move very slowly.

If one of the parents has the gene for Huntington's disease, there is a 50 percent chance that the children of that parent will inherit the same gene. If both parents carry the gene, it is almost certain that the children will develop the symptoms of Huntington's disease at some time in their lives.

Symptoms of Huntington's disease

The symptoms of Huntington's disease include:

-- Antisocial behaviors
-- Anxiety, tension and stress
-- Confusion or disorientation
-- Difficulty in swallowing
-- Facial movements consisting of grimaces
-- Fidgeting or Restlessness
-- Hallucinations
-- Irritability
-- Loss of judgment
-- Loss of memory
-- Moodiness
-- Paranoia
-- Personality changes
-- Psychosis
-- Slow and uncontrolled movements
-- Speech changes
-- Speech impairment
-- Unsteady gait
-- Rigidity
-- Tremors

Diagnosis for Huntington's disease

The patient will need to be examined by the doctor. The medical professional will ask questions regarding the patient's symptoms and family history. A neurological exam will also be done. The doctor will check for these signs:

-- Abnormal movements
-- Abnormal reflexes
-- Dementia
-- Prancing with wide walk
-- Poor enunciation or hesitant speech
-- DNA marker studies to determine if have the gene of Huntington's disease.
-- Head CT scan
-- Head MRI scan
-- PET (isotope) scan for brain

Treatment for Huntington's disease

Unfortunately, there is no treatment available to treat Huntington's disease. The manifestations of the disease may start out fairly mild, but later the symptoms may become progressively worse. The treatment objective is to reduce the speed of the disease and to help the person to function as long as possible.

The medications used to treat Huntington's disease are aimed to minimize the symptoms. Dopamine blockers can help to reduce the abnormal movements and behaviors. Drugs such as amantadine (Symmetrel), haloperidol (Haldol) and tetrabenazine (Xenazine) are used to control tremors and abnormal movements and behaviors.

Suicide and depression are common in people who suffer from Huntington's disease. It is very important to monitor the symptoms and treat according the patient's symptoms. To prevent thoughts and actions of suicide, the person should be treated with medications that will minimize or eliminate those thoughts.

Huntington's disease is a heart breaking condition. As the disease progresses, the patients will need assistance and supervision. People suffering from Huntington's disease may finally require 24-hour care. The possible complications that arise from Huntington's disease is depression, increased risk for infection, injury, loss of the ability to care for him/herself, and loss of the ability to interact with others. Death is the final outcome of Huntington's disease, because there is no cure for the disease. Families with a history of Huntington's disease may be advised to seek genetic counseling, if thoughts of children are in the future.

Sources:

Medline Plus

Mayo Clinic