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Huntingtonova choroba

  1. Subject h.Huntingtonova choroba
    Subject h.Huntington Disease
    Entry termsHuntingtonova chorea
    Huntingtonova choroba juvenilná
    Huntingtonova choroba, akineticko-rigidný variant
    chorea progresívna hereditárna chronická (Huntington)
    chorea progresívna dedičná chronická (Huntington)
    Huntingtonova progresívna hereditárna chronická chorea
    Huntingtonova progresívna dedičná chronická chorea
    Huntingtonova choroba, juvenilná
    Huntingtonova choroba, s nástupom v ranom veku
    Huntingtonova choroba, s nástupom v neskoršom veku
    Huntingtonova choroba, s neskorším nástupom
    English X referencesHuntington Chorea
    Juvenile Huntington Disease
    Scope note in EnglishA 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)
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