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Huntington's chorea in Norway.

L Saugstad, O Odegård

    Psychological Medicine
    |February 1, 1986
    PubMed
    Summary
    This summary is machine-generated.

    Huntington's chorea patients in Norway showed better social adjustment than expected, with normal marriage and reproduction rates. Predictive testing for this neurodegenerative disease may increase carrier burden without effective treatments.

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    Inbreeding and schizophrenia.

    Clinical genetics·1986

    Area of Science:

    • Neurology
    • Psychiatry
    • Epidemiology

    Background:

    • Huntington's chorea is a progressive neurodegenerative disorder.
    • Understanding the long-term social and clinical course is crucial for patient care and genetic counseling.

    Purpose of the Study:

    • To investigate the social adjustment, reproductive patterns, and clinical course of Huntington's chorea patients.
    • To assess the impact of predictive testing in the context of disease manifestation and treatment availability.

    Main Methods:

    • Utilized the Norwegian National Case Register for psychiatric hospital admissions.
    • Analyzed case histories of 199 patients diagnosed with Huntington's chorea between 1916 and 1975.
    • Examined prevalence rates, social adjustment, marriage, reproduction, occupation, migration, and psychiatric symptoms.

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    Main Results:

    • Prevalence rates were 6-7 per 100,000 population during 1930-50.
    • Patients exhibited favorable social adjustment until severe neurological/psychiatric symptoms appeared.
    • Marriage and reproduction rates were not reduced; depression and suicide were rare.
    • Age of onset was earlier in single patients, suggesting marriage selection.
    • Late disease manifestation and high marriage rates complicate predictive testing without treatment.

    Conclusions:

    • Huntington's chorea patients can maintain good social functioning for a significant period.
    • Predictive genetic testing poses ethical challenges due to late onset and lack of curative treatments.
    • Further research is needed to balance the benefits and burdens of genetic testing for Huntington's chorea.