Huntingtons chorea

Huntingtons chorea

Last Reviewed : 12/24/2020
Huntingtons chorea


Chronic Progressive Chorea

  • Degenerative Chorea
  • HD
  • Hereditary Chorea
  • Hereditary Chronic Progressive Chorea
  • Huntington's Chorea
  • Very Early Onset Huntington's Disease
  • Woody Guthrie's Disease

Huntington's disease (HD), also known as Huntington's chorea, is an inherited disorder that results in death of brain cells. The earliest symptoms are often subtle problems with mood or mental abilities.A general lack of coordination and an unsteady gait often follow.As the disease advances, uncoordinated, jerky body movements become more apparent.Physical abilities gradually worsen until coordinated movement becomes difficult and the person is unable to talk.Mental abilities generally decline into dementia. The specific symptoms vary somewhat between people.Symptoms usually begin between 30 and 50 years of age, but can start at any age.The disease may develop earlier in life in each successive generation.About 8% of cases start before the age of 20 years and typically present with symptoms more similar toParkinson's disease.People with HD often underestimate the degree of their problems.

HD is typically inherited from a person's parents with 10% of cases due to a new mutation. The disease is caused by anautosomal dominant mutation in either of an individual's two copies of a gene called Huntingtin. This means a child of an affected person typically has a 50% chance of inheriting the disease.The Huntingtin gene provides the genetic information for a protein that is also called "huntingtin".Expansion of CAG (cytosine-adenine-guanine) triplet repeats in the gene coding for the Huntingtin protein results in an abnormal protein, which gradually damages cells in the brain, through mechanisms that are not fully understood.Diagnosis is by genetic testing, which can occur at any point in time regardless of whether or not symptoms are present.This fact raises several ethical debates: the age at which an individual is considered mature enough to choose testing; whether parents have the right to have their children tested; and managing confidentiality and disclosure of test results.

There is no cure for HD.Full-time care is required in the later stages of the disease.Treatments can relieve some symptoms and in some improve quality of life. The best evidence for treatment of the movement problems is withtetrabenazine. HD affects about 4 to 15 in 100,000 people of European descent. It is rare among Japanese and occurs at an unknown rate in Africa. The disease affects men and women equally.Complications such as pneumonia, heart disease, and physical injury from falls reduce life expectancy. Suicide is the cause of death in about 9% of cases.Death typically occurs fifteen to twenty years from when the disease was first detected.


We researched this topic for you and found the following best online resources. They are categorized into basic, advanced, and research level based on the extent of information you need. You will be taken to the respective websites by pressing on the links below.


Basic information:

Http:// mayoclinic huntington's disease

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Http:// web md huntington's disease: symptoms and treatment

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Https://,-spinal-cord,-and-nerve-disorders/movement-disorders/huntington-disease merck manuals huntington's disease

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Http:// of maryland medical center huntington's disease

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Https:// very well huntington's disease: symptoms, causes, prognosis and treatment


Advanced information:

Http:// medical news today huntington's disease: causes, symptoms and treatments

Https:// genetics home reference huntington disease

Http:// medicine net huntington's disease

Http:// medscape huntington's disease

Http:// patient huntington's disease

Http:// patient huntington's disease

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Http:// understanding evolution huntington's chorea: evolution and genetic disease

Https:// national organisation for rare diseases huntington's disease

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Https:// radiopedia huntington's disease



Http:// association of huntington's disease and schizophrenia-like psychosis in a huntington's disease pedigree by bernardo barahona corrêa et al., linical practice and epidemiology in mental health20062:1 doi: 10.1186/1745-0179-2-1

Http:// arq. Neuro-psiquiatr. Vol.69 no.3 são paulo june 2011 article:clinical and genetic analysis of 29 brazilian patients with huntington's disease-like phenotype by guilherme riccioppo rodrigues et al.,

Http:// arq. Neuro-psiquiatr. Vol.65 no.2b são paulo june 2007 non-choreic movement disorders as initial manifestations of huntington's disease by nilson becker et al.,

Http:// todd d, gowers i, dowler sj, wall md, mcallister g, et al. (2014) a monoclonal antibody trkb receptor agonist as a potential therapeutic for huntington’s disease. Plos one 9(2): e87923. Doi:10.1371/journal.pone.0087923

Http:// dose-dependent improvement of myoclonic hyperkinesia due to valproic acid in eight huntington's disease patients: a case series by carsten saft et al.,bmc neurology20066:11 doi: 10.1186/1471-2377-6-11

Https:// a. Ciammola, j. Sassone, b. Poletti, n. Mencacci, r. Benti, and v. Silani, “atypical parkinsonism revealing a late onset, rigid and akinetic form of huntington's disease,” case reports in neurological medicine, vol. 2011, article id 696953, 3 pages, 2011. Doi:10.1155/2011/696953

Http:// huntington's disease: a clinical review by raymund ac roos orphanet journal of rare diseases20105:40 doi: 10.1186/1750-1172-5-40

Http:// review article:front. Neuroanat., 23 august 2011 | huntington’s disease and striatal signaling by emmanuel roze et al.,


Other helpful resources(support groups): internat ional parkinson andmovement disorder society

Http:// huntingtons disease society of america

Http:// huntingtons disease society of canada


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Presentations/quiz/newspaper articles:

Https:// theguardian huntington’s disease: the new gene therapy that sufferers cannot afford

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