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  1. purple says:

    Basically, symptoms occur after the brain starts to waste away. Damage occurs to the brain’s nerve tissue or cells, usually around the age of 40, but can vary. If you’re asking if a particular activity can trigger the symptoms, the answer is no. However, a person with Huntingtons can become extremely frustrated with the progressive inability to perform day to day tasks; and this can exacerbate the symptoms… DrP

  2. Linda says:

    Background: Huntington disease (HD), also known as Huntington chorea (HC), is an inherited disease characterized by choreiform movements and progressive dementia.

    In adults, HD most often causes involuntary movements, but rigidity can also be a feature of the disease.

    The initial diagnosis is rarely established in the emergency department, but patients with established disease may present to the ED because of worsening symptoms.

    Pathophysiology: HD is inherited as an autosomal dominant disorder with complete penetrance. An HD gene has been identified with an abnormal protein product (huntingtin) that can be identified in the brain. The link between this protein and the selective loss of neuronal groups in the CNS remains to be established. HD has now been identified genetically as a trinucleotide CAG-repeat mutation on chromosome 4. The CAG repeat length may be important in determining the age of onset and the rate of disease progression.

    Frequency:

    In the US: Prevalence of HD in the United States is 5.15 cases per 100,000 persons.
    Internationally: HD is encountered throughout the world; however, localized geographic clusters of disease exist. Countries that have been settled by western Europeans have an incidence of the disease similar to the incidence in the United States.
    Mortality/Morbidity: HD is a progressive neurological disorder usually leading to death 15-20 years after onset of neurological or psychological impairment.

    Race: HD is found in all ethnic groups.

    Sex: Males and females are diagnosed in equal numbers.

    Age:

    Symptoms arising from a typical presentation of HD usually do not develop until a person is aged 35 years or older. By the time of diagnosis, many patients already have had children and have passed the gene to another generation.
    As many as 10% of patients with HD have a juvenile form of the disease in which the onset of symptoms may occur when the patient is younger than 20 years.
    Muscular rigidity is more common with juvenile-onset illness.

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