Psychology Facts

▲ Huntington's Disease ▲

》 An inherited condition in which nerve cells in the brain break down over time.

》 Huntington's disease is a progressive brain disorder caused by a single defective gene on chromosome 4 — one of the 23 human chromosomes that carry a person's entire genetic code.

》 Symptoms of Huntington's disease usually develop between ages 30 and 50, but they can appear as early as age 2 or as late as 80.

》 The symptoms of HD can vary a lot from person to person, but they usually include:

Personality changes, mood swings & depression

Forgetfulness & impaired judgment

Unsteady gait & involuntary movements (chorea)

Slurred speech, difficulty in swallowing & significant weight loss

irritability

hallucinations

psychosis

difficulty understanding new information

trouble making decisions

》 Symptoms that may occur as the disease progresses include:

uncontrolled twitching movements, called chorea

difficulty walking

trouble swallowing and speaking

confusion

memory loss

personality changes

speech changes

decline in cognitive abilities

》 Symptoms usually worsen over the course of 10 to 25 years and affect the ability to reason, walk, and talk.

》 Early on, a person with HD or their friends and family may notice difficulties with planning, remembering, and staying on task. They may develop mood changes like depression, anxiety, irritability, and anger.

》 Most people with HD become "fidgety" and develop movements of the face and limbs known as chorea, which they are not able to control.

》 Scientists identified the defective gene that causes Huntington's disease in 1993. A diagnostic genetic test is now available.

》 The Huntington gene defect involves extra repeats of one specific chemical code in one small section of chromosome 4.
The normal huntingtin gene includes 17 to 20 repetitions of this code among its total of more than 3,100 codes.
The defect that causes Huntington's disease includes 40 or more repeats.

》 Family history plays a major role in the diagnosis of Huntington's disease. However, a variety of clinical and laboratory testing can be done to assist in diagnosing the problem.

》 Some tests that might/can be performed are as follows:

If you have several symptoms associated with Huntington's disease, your doctor might recommend genetic testing. A genetic test can definitively diagnose this condition.

A neurologist will do tests to check your:

reflexes

coordination

balance

muscle tone

strength

sense of touch

hearing

vision

》Occupational therapy can be used to evaluate your daily activities and recommend devices that help with:

movement

eating and drinking

bathing

getting dressed

》Medications can provide relief from some of your physical and psychiatric symptoms. -

Involuntary movements may be treated with tetrabenazine and antipsychotic drugs.

Muscle rigidity and involuntary muscle contractions can be treated with diazepam.

Depression and other psychiatric symptoms can be treated with antidepressants and mood-stabilizing drugs.

》 People with the adult-onset form of Huntington's disease usually live for 15 to 20 years after symptoms begin to appear.

》 Causes of death among people with Huntington's disease include:

infections, such as pneumonia

suicide

injuries from falling

complications from being unable to swallow