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Huntington’s Disease

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Huntington’s disease,  or HD is an hereditary disorder that affects the central nervous system. It is also called as Huntington’s  Chorea (HC). The symptoms are noticed in mid-life and affects both men and women equally. If one parent has huntington’s disease, there is a 50% chance one child will have Huntington’s.

What is a Huntington’s Disease?

Huntington’s disease (HD) is an hereditary and incurable brain disorder. The nerve cells of the brain get damaged, causing different parts of the brain to breakdown. Thus, it starts affecting behavior, movement, cognition, etc. The person has difficulties walking, thinking, reasoning as well as talking. As the disease progresses, the person becomes more and more dependent on a caregiver. This disease causes a major difference in the lives of the patient and their family, emotionally, socially, mentally as well as economically.

Why Was Huntington’s  Disease Called as Huntington’s Chorea?

Huntington’s disease was known as Huntington’s Chorea because of the involuntary writing or jerking or dancing movements made by the patients. The word chorea in Greek means ‘dancing’. Thus, the disease came to be known as Huntington’s chorea.

Who Are Affected By Huntington’s  Disease?

Men and women who are well into their middle age are affected by huntington’s disease. About 1 in every 10,000 Americans suffer from this disease. It is very common in people of Caucasian origin than people with Asian or African inheritance.

Not all parents with Huntington’s disease pass the genes to their children. The passing of genes is a random process. In some, one child or all children may be affected, whereas some parents do not pass the gene to any of their children. Every person who inherits the gene develops Huntington’s disease.

What Are the Symptoms of Huntington’s Disease?

The signs and symptoms of Huntington’s disease vary person to person. The severity of the cognitive and communication problems differ in every individual. The signs may be similar in all, but no two people have similar problems or show similar progress of the disease. The symptoms include movement disorders, cognitive problems, and psychiatric disorders. The symptoms usually appear in people between 30-50 years. But people between the age group of 2-80 years can develop the symptoms.

Symptoms Affecting Movement

  • Involuntary writhing, shaking, jerking and uncontrolled movements (chorea)
  • Lack of coordination, small unintentional motions, slow saccadic eye movements
  • Rigidity, abnormal posturing
  • Abnormal facial expressions, difficulty chewing, swallowing and speaking
  • Malnutrition and weight loss due to swallowing problems

Cognitive Disorders

  • Problems with planning, abstract thinking, initiation of appropriate action, etc.
  • Impairment with short-term memory and deficiency in long-term memory
  • One may forget a complete episode of their life (episodic loss) or forget how to perform a task (procedural) or have problems with their working memory
  • Dementia

Neuropsychiatric Problems 

  • Anxiety
  • Depression
  • Loss of display of emotions
  • Aggression
  • Egocentrism
  • Compulsive disorder
  • One may become an alcoholic, gambler, etc.
  • Suicidal thoughts

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Causes of Huntington’s Disease

Huntington’s disease is an autosomal dominant disorder. This means, just one copy of the defective gene is enough to develop the disease. The faulty gene is in this case is present on the chromosome 4. This gene produces a protein called Huntingtin. The faulty gene, whose mechanism is not yet clear, causes the nerve cells to get damaged. These nerves of the brain include those present in the basal ganglia and the cerebral cortex. Thus, one experiences physical, cognitive, emotional and mental changes.

Every family with one parent with Huntington has a 50-50 chance of children inheriting the disease.

Diagnosis of Huntington’s Disease

Diagnosis is based on the physical symptoms, family medical history, and psychiatric examinations. Certain tests for assessing the structure and function of the brain are carried out. These include brain-imaging test and Electroencephalogram (EEG).

Treatment for Huntington’s Disease

There is no cure for Huntington’s disease. The medications available only help reduce the severity of the symptoms related to movement and psychiatric disorders.

Tetrabenazine,antipsychotic drugs, etc. help with the movements. Medications for psychiatric disorders include antidepressants, mood-stabilizing drugs, etc. The patient may be given physical therapy, occupational therapy, etc. to help with their coordination, balance and movement problems.

Huntington’s disease causes a lot of stress and emotional upheaval within the family. One may have seen other’s suffer from the disease or may develop a fear that other’s in the family may be at risk. There are many support groups that help one cope up with the situation and more. Speak to your doctor regarding the disease and undergo genetic counseling for Huntington’s disease, if you have someone in the family or yourself diagnosed with this dreaded condition.

Written by: healthplus24.com team

Date last updated: december 29, 2014