Huntington’s disease is a progressive, degenerative disorder of brain cells. It is an inherited disease. Huntington’s disease causes physical, mental and behavioral disturbances in the body. The disease is named after George Huntington who first described the symptoms. It was previously called Huntington’s chorea because of the involuntary jerky movement just like a dance that occurs in later stage of the disease. Chorea in Greek means ‘dance’.
In most patients the onset of symptoms is in fourth or fifth decade of life. Patient gradually looses the ability to think, walk, and reason out.Ultimately it has a serious effect on his daily activities. He has to rely on others. A person or a child inheriting the faulty gene will develop this disease usually after the age of 40 years.
Huntington’s disease cannot be cured as there is no definitive treatment available. Chorea and other symptoms may respond to drugs and physical or psychotherapy.
What Causes Huntington’s Disease?
Huntington’s disease is a genetic disorder. The child inherits a faulty gene from his father or mother or from both parents. It is an autosomal dominant disorder. The child of a parent carrying Huntington’s disease gene has 50 percent chances of inheriting the gene.The faulty gene is located in the short arm of chromosome 4.
In normal case the gene produces a protein called huntingtin. But a faulty gene in this case is large and produces larger size of protein. The larger form of huntingtin gradually destroys certain brain cells giving rise to various manifestations of Huntington’s disease.
The disease is not as common as other inherited neurological disorders, but the incidence of this disease is remarkably high in the Lake Maracaibo region of Venezuela. Men and women are equally affected with this disease.
Signs And Symptoms Of Huntington Disease
Although a person carries faulty gene from birth, the symptoms of this disease are manifested at middle age.These symptoms gradually become worse in 15 to 20 years of the onset of disease. Symptom manifestation may vary from one patient to another. Some patients may have depression and anxiety as first symptom while few patients may present with abnormal movement or chorea. This disease affects body, mind and emotions.
Early symptoms of this disease are:
- Involuntary movement of fingers, toes, hands, legs, face and neck.
- Sudden mood changes such as anger, frustration, irritation, depression etc.
- Poor memory.
- Unable to make decision.
- Muscle twitching.
- Twitching of eyelids.
- Difficulty in speaking, swallowing etc.
- Loosing balance while walking.
- Patient often stumbles and falls while walking.
- Reduced concentration.
- Constant contraction of muscles.
- Irritable, stubborn.
- Loss of interest in life as patient has no emotions left for joy, pleasure or sadness.
- Weight loss at later stage of disease.
At later stage of the disease patient may exhibit psychiatric symptoms such as delusion, hallucination, and even a state of paranoia. Patient may need round the clock monitoring and care as he gets bed ridden, unable to walk, talk, and even eat. He may understand all that is said and can recognize relatives and friends.
Treatment Options For Huntington’s Disease
Huntington’s disease is incurable condition. However, all effort with multidisciplinary approach is focused to make life of the patient more meaningful and versatile.
Certain medicines will help in relieving physical and psychiatric symptoms. Doctors often prescribe antidepressants, and anti-psychotic drugs to control abnormal mood changes. Patient is also recommended physical therapy, speech therapy which helps him to speak and swallow properly.
As the intake of patient decreases gradually, diet and supplements are essential for improving overall health and immunity. Foods that help to improve memory and overall immunity are beneficial for improving his health. Vegetables, dried fruits, fruit juices which are good source of vitamins and minerals should be consumed by the patient.