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Huntington'Chorea

 

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Huntington's Chorea is a genetic disorder characterised by the appearance of dementia and involuntary jerky movements of the whole body, beginning in mid adult life. The disease slowly progresses, and death often takes place within 10 years of onset, usually due to an intercurrent infection. Occurs in two forms, the classic choreic form and the rigid form. About 150,000 Americans may be at risk of inheriting Huntington's disease from a parent. The onset is usually gradual and slow to notice, facial movements and involuntary movements progress and can be very disabling. Medications are available to help manage the signs and symptoms of Huntington's disease, but treatments can't prevent the physical and mental decline associated with this condition.

Huntington disease (HD), also known as Huntington chorea (HC), is an inherited disease characterized by choreiform movements and progressive dementia. As a result, you may experience uncontrolled movements, emotional disturbances and mental deterioration. HD causes bizarre involuntary movements (chorea) and loss of intellectual abilities ( dementia ). This degeneration causes chorea movements, called Huntington's chorea, which are uncontrollable and irregular muscle movements, especially of the arms, legs, and face. The risk of developing Huntington's chorea decreases rapidly after age 50 for each year that passes without developing the disease. This leads to increasing chorea and loss of mental abilities. Younger people with Huntington's disease often have a more severe case, and their symptoms may progress more quickly. Rarely, children may develop this condition. Huntington's Chorea is a progressive degenerative disease characterized by a wide-based, bouncy gait, abnormal speech patterns, jerky movements of the limbs, body, and face. Depression or psychosis, however, can be temporarily alleviated by antidepressant and antipsychotic medications The posture is abnormal. Muscle movements become very violent and finally in later stage decrease to akinesia.

Huntington's disease (Huntington's chorea) is a progressive, degenerative disease that causes certain nerve cells in your brain to waste away. Huntington's disease causes the brain to slowly decay. This leads to increasing chorea and loss of mental abilities. Other names in usage include Huntington chorea and Huntington's chorea. Symptoms of Huntington's chorea usually develop between 35 and 40 years of age. Men and women are both as likely to develop the disease. The name "chorea" comes from the Greek word for "dance" and refers to the incessant quick, jerky, involuntary movements that are characteristic of this condition. The disorder is named after a little-known American family doctor by the name of George S. Huntington (1850-1916) who wrote about an illness he saw as "an heirloom from generations away back in the dim past." The onset is usually gradual and slow to notice, facial movements and involuntary movements progress and can be very disabling.

Causes of Huntington'Chorea

The common Causes of Huntington'Chorea :

  • Common medical opinion holds that nearly all cases are inherited and that appearance of the disease through new mutations is extremely rare.
  • The cause of sydenham chorea is only certain types of streptococci, called "Lancefield Group A beta-hemolytic."
  • When a patient presents with HD, identification of a parent with an intermediate allele who lacks the full clinical manifestations of the disease is often possible.
  • A small number of patients exist who have DNA-proven HD but a completely negative family history.
  • The gene encodes a protein known as huntingtin. Gradually, this protein accumulates within brain cells.
  • Chorea is an abnormal voluntary movement disorder, one of a group of neurological disorders called dyskinesias, which are caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement.
  • Huntington's disease causes damage to brain cells in the areas of the brain involved in the control of movement, planning and motivation .

Symptoms of Huntington'Chorea

Some common Symptoms of Huntington'Chorea :

  • A wide, prancing gait .
  • Facial movements, grimaces.
  • Loss of memory.
  • Severe problems with balance and coordination.
  • Need to turn head to shift the gaze.
  • Involuntary movements.
  • Difficulty shifting your gaze without moving your head.
  • Loss of judgment.
  • Hesitant, halting or slurred speech.
  • Lack of concentration.
  • Difficulty swallowing.
  • Abnormal posture and.

Treatment of Huntington'Chorea

Here is the list of the methods for treating Huntington'Chorea :

  • All the other manifestations of rheumatic fever, including Sydenham's chorea and excluding heart valve damage, remit with the acute disease and do not require treatment.
  • Dopamine receptor-blocking agents (eg, phenothiazines, haloperidol) may be helpful in reducing some of the involuntary movements, but such therapy always should be coordinated with the primary care provider.
  • Various medications, including fluoxetine (Prozac, Sarafem), sertraline (Zoloft) and nortriptyline (Aventyl, Pamelor), can help control depression and the obsessive-compulsive rituals that some people with Huntington's disease develop.
  • Treatment for Huntington's disease is supportive, while treatment for Syndenham's chorea usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence.
  • Symptomatic treatment for the dementia is similar to that used for any organic brain syndrome .
  • The initial pharmacologic approach to treatment is similar to that of other patients.
  • There is no known treatment for the mental degeneration that accompanies the physical symptoms.
  • Drug-resistant depression may yield to electroconvulsive therapy.