Neurology Book

Huntington's Chorea

Aka: Huntington's Chorea, Progressive Hereditary Chorea, Huntingtons Chorea, Huntington Chorea
  1. Epidemiology
    1. Autosomal Dominant inherited disease
    2. Onset of symptoms in middle life
  2. Symptoms
    1. Gradual onset and progression of symptoms
      1. Failing memory
      2. Restlessness
      3. Lack of initiative
  3. Signs
    1. Choreiform movements
    2. Shuffling gait
    3. Mental deterioration
    4. Deep Tendon Reflexes
      1. Normal or increased

Huntington Disease (C0020179)

Definition (MEDLINEPLUS)

Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste away. People are born with the defective gene, but symptoms usually don't appear until middle age. Early symptoms of HD may include uncontrolled movements, clumsiness, and balance problems. Later, HD can take away the ability to walk, talk, and swallow. Some people stop recognizing family members. Others are aware of their environment and are able to express emotions.

If one of your parents has Huntington's disease, you have a 50 percent chance of getting it. A blood test can tell you if have the HD gene and will develop the disease. Genetic counseling can help you weigh the risks and benefits of taking the test.

There is no cure. Medicines can help manage some of the symptoms, but cannot slow down or stop the disease.

NIH: National Institute of Neurological Disorders and Stroke

Definition (NCI) A progressive hereditary neurodegenerative disorder inherited in an autosomal dominant fashion. Symptoms may appear at any age and include uncontrolled movements, clumsiness, balance problems, difficulty walking, talking, or swallowing. The disease has a progressive course with a decline in mental abilities, and the development of psychiatric problems.
Definition (MSH) A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
Definition (CSP) chronic progressive chorea and mental deterioration terminating in dementia; the age of onset usually is in the fourth decade of life.
Concepts Disease or Syndrome (T047)
MSH D006816
ICD9 333.4
ICD10 G10
SnomedCT 58756001, 155006000
English Chorea, Huntington, Huntington Chorea, Huntington's Chorea, Chorea, Huntington's, Huntington Disease, Huntington's Disease, HUNTINGTON DISEASE, HUNTINGTON CHOREA, HD, HUNTINGTONS DIS, HUNTINGTON DIS, Huntington's chorea (diagnosis), Chorea, Chronic Progressive Hereditary (Huntington), Chronic Progressive Hereditary Chorea (Huntington), Huntington Chronic Progressive Hereditary Chorea, Progressive Chorea, Chronic Hereditary (Huntington), Progressive Chorea, Hereditary, Chronic (Huntington), Huntington chorea, Huntington Disease [Disease/Finding], Huntington's disease, huntingtons chorea, Chorea;Huntingtons, Huntington disease, huntington's chorea, huntingtons disease, huntington's disease, huntington chorea, Huntington's disease (disorder), Chronic progressive hereditary chorea, Huntington's chorea, Chronic progressive chorea, HC - Huntington chorea, HD - Huntington chorea, Huntington's chorea (disorder), Huntington; chorea, Huntington, chorea; Huntington, Huntingtons Chorea, Huntingtons Disease, Huntingtons chorea
Swedish Huntingtons sjukdom
Czech Huntingtonova chorea, Huntingtonova nemoc, Huntingtonova choroba
Finnish Huntingtonin tauti
Italian Corea progressiva ereditaria (Huntington), Malattia di Huntington, Corea cronica progressiva (Huntington), Corea di Huntington
Japanese ハンチントン病-若年性, 若年性ハンチントン病, ハンチントン病, 舞踏病-Huntington, 舞踏病-ハンチントン, Huntington病, Huntington舞踏病, ハンチントン舞踏病, ハンティングトン舞踏病, ハンティントン病, ハンチントンビョウ, ハンチントンブトウビョウ
Korean 헌팅톤병
Dutch ziekte van Huntington, Huntington-chorea, Huntington; chorea, chorea; Huntington, Chorea van Huntington, Huntington, chorea van, Ziekte van Huntington
German Huntington Krankheit, Chorea Huntington, Chorea hereditiva, Huntington-Krankheit, Chorea progressiva hereditaria
Polish Pląsawica Huntingtona, Pląsawica przewlekła, Choroba Huntingtona
Hungarian Huntington-chorea, Huntington betegség
Norwegian Huntingtons Chorea, Setesdalsrykka, Huntingtons sykdom, Lund-Huntingtons Chorea, St.Veitsdans, Huntingtons lidelse
Portuguese Coreia de Huntington, Doença de Huntington
Spanish corea de Huntington (trastorno), corea de Huntington, corea hereditaria progresiva crónica, corea progresiva crónica, Corea de Huntington, Enfermedad de Huntington
French Chorée de Huntington, Maladie de Huntington, Chorée chronique progressive de Huntington, Chorée chronique progressive héréditaire de Huntington
Derived from the NIH UMLS (Unified Medical Language System)

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