Huntington's disease is a degenerative disease that destroys the neurons of the brain affecting cognitive ability and motor function in some parts of the body. The disease is hereditary so that children born with one parent having Huntington's disease have a 50 percent chance of developing the disease. The first symptoms of Huntington's usually appear in midlife, but in rare occasions, infants may already show symptoms. Usually, the progress of the disease is fast in people showing symptoms before 20 years old.
Causes of Huntington's disease
Huntington's disease is genetic. It's enough for a single defective gene from one parent to cause Huntington's disease to his or her children. However, one child developing the disease has no bearing on whether or not his or her siblings will develop the disease, too. But all children who inherited the defective gene will develop Huntington's some time in their life.
Pathophysiology of Huntington's disease
Humans have Huntingtin gene that produces huntingtin protein. Sometimes this gene goes haywire so that it produces defective huntingtin protein that interferes with other proteins disturbing proper cholesterol distribution in the brain. In order for the brain to properly function and live, the amount and distribution of cholesterol in it must be proper and constant. Any disturbances will result to the appearance of the Huntington's disease symptoms.
Symptoms of Huntington's disease
The symptoms of Huntington's disease and progress vary from patient to patient: it all depends on the amount of neurons destroyed by the disease. People who develop Huntington's before the age of 20 tends to progress faster than those who develop the disease in their midlife. On average, however, patients die 10 to 30 years since the symptoms appear.
Huntington's disease can manifest as personality changes: patients becomes irritable or they suddenly lose interest. The next thing to go is their cognitive ability so that they may have memory problem and making decisions and learning new things is difficult. As the disease progress further, patients will have severe balance and coordination problem, slurred speech, dementia, and they will have involuntary rapid movements or chorea.
Treatment of Huntington's disease
There is no medicine to stop the progress of neuron destruction due to Huntington's disease. Patients can only be given supportive treatment for the symptoms. Example, patients are given medication to increase the dopamine levels in the brain to reduce chorea. Antipsychotic and antidepressant drugs are also given to counter psychotic and depression symptoms. Patients are also advised to undergo speech, physical, and occupational therapy to augment the remaining cognitive ability.
Support for people with Huntington's disease
Huntington's disease diagnosis is very difficult. However, people diagnosed with Huntington's must prepare for the time when they are no longer capable of handling themselves to live the remainder of their lives. In this case, they should prepare legal matters like wills and testaments and if possible, prepare for their future nursing care. Patients are also advised to get support groups and counseling. In America, the number to call for Huntington's disease support is 800-345-4372.