Skip to main content Accessibility help

Huntington's chorea in Norway

  • Letten Saugstad (a1) and Ørnulv Ødegård (a1)


By means of a National Case Register of patients admitted to psychiatric hospitals in Norway it was possible to study the case-histories of the 199 patients admitted from 1916 to 1975 with a diagnosis of Huntington's chorea. Ascertainment is likely to have been most complete for the years 1930–50, when the prevalence rates ranged between 6 and 7 per 100000 population. The social adjustment of the patients was found to be more favourable than expected up to the onset of serious psychiatric and neurological symptoms. The marriage rate and the rate of reproduction were not lowered. Occupational distribution and the pattern of migration were normal. Depression and suicide were remarkably rare. The age of onset among single patients was nearly 10 years lower than among the married, and more than 13 years lower on admission to hospital, indicating selection for marriage. With a marriage rate of about 70% and the late manifestation of the disease, predictive tests without prospects of treatment are apt to increase significantly the burden on the carriers.


Corresponding author

1Address for correspondence: Dr L. Saugstad, National Case Register of Serious Mental Disorder, Gaustad Hospital, Oslo 3, Norway.


Hide All
Bird, S. J. (1983) Effective genetic counselling for Huntington's disease. Lancet i, 872873.
Bruyn, G. W. (1968) Huntington's chorea. In Handbook of Clinical Neurology, Vol. 6, pp. 298378. North-Holland: Amsterdam.
Burke, W. (1976). Age of onset in Huntington's disease: lack of parental effect. Journal of Medical Genetics 13, 462465.
Folstein, S. E., Franz, M. L., Jensen, B. A., Chase, G. A. & Folstein, M. F. (1983). Conduct disorder and affective disorder among the offspring of patients with Huntington's disease Psychological Medicine 13, 4552.
Gjertsen, T. (1932). Chorea Huntington. Tidskrift Norske Lægeforening 52, 332335.
Hans, M. B. & Gilmore, T. H. (1968). Social aspects of Huntington's chorea. British Journal of Psychiatry 114, 9398.
Hansen, O. (1926). Nouvelles recherches sur la Chorée chronique du Setesdal. Acta Medico. Scandinavica Suppl. 16, 86.
Harper, P. S. (1983). A genetic marker for Huntington's chorea. British Medical Journal 287, 15671568.
Haugerud, S. (1968). Chronisch progressive Chorea bei zwei Schwestern. Schweizerische Medizinische Wochenschrift 98, 20162020.
Hughes, E. M. (1925). Social significance of Huntington's chorea. American Journal of Psychiatry 4, 537557.
Kenen, R. H. & Schmidt, R. M. (1978) Stigmatization of carrier status. American Journal of Public Health 68, 11161120.
Lund, J. C. (1860). Chorea Sti Viti i Setesdalen. Beretning om sundhetstilstanden og medisinske forhold. Oslo.
Magnus, V. (1916). Totilfeller av Huntington's chorea. Norsk Magasin for Lagevidenskapen 77, 14331436.
Mattson, B. (1974). Huntington's chorea in Sweden. Acta Psychiatrica Scandinavica Suppl. 255, 211235.
Minski, L. & Guttmann, E. (1938). Huntington's chorea. A study of 34 families. Journal of Mental Science 84, 2196.
Refsum, S. (1938). Et tilfelle av Huntington's chorea. Norsk Magasin for Lagevidenskapen. 99, 12011210.
Sæthre, G. (1958). Lund–Huntington–s chorea en nyopdaget slekt I Østfold. Tidskrift Norske Lægeforening 78, 642643.
Saugstad, L. (1977 a.) Inbreeding in Norway. Annals of Human Genetics (London) 40, 481491.
Saugstad, L. (1977 b). The relationship between inbreeding, migration and population density in Norway. Annals of Human Genetics (London) 40, 331341.
Saugstad, L. (1983). The contribution of increased male susceptibility perinatally to the later differences between the sexes.13th International Summer School of Brain Research,AmsterdamAugust. 22–26: Sex differences in the brain, relation between structure and function.
Saugstad, L. & ødegård, Ø. (1979). Mortality in psychiatric hospitals in Norway 1950–74. Acta Psychiatrica Scandinavica 59, 431447.
Saugstad, L. & Øhdegård, ø. (1983). Persistent discrepancy in international diagnostic practice since 1970. Acta Psychiatrica Scandinavica 68, 501510;
Saugstad, L. & ødeg˚rd, ø (1986). Inbreeding and functional psychosis (schizophrenia). Clinical Genetics (in the press).
Seip, T. (1928). Ein Fall von Chorea Huntington und einige Bemerkungen zu dieser Krankheit. Acta Psychiatrica et Neurologica 3, 139145.
Shaw, M. & Caro, A. (1982). The mutation rate to Huntington's chorea. Journal of Medical Genetics 19, 161167.
Standnes, B. (1979). Juvenil chorea Huntington. Tidskrift Norske Lægeforening 99, 1113.
ødegård, Ø. (1946). Marriage and mental disease: a study in social psychopathology. Journal of Mental Science 92, 3559.
Ødegård, Ø. (1953). New data on marriage and mental disease: the incidence of psychoses in the widowed and the divorced. Journal of Mental Science 98, 778785.
Ørbech, A L & Quelprud, T. (1954). Setesdalsrykka (Chorea progressiva hereditaria), p. 126. J. Dybwad: Oslo.
Vegter-van der Vlis, M., Volkers, W. S. & Went, L. N. (1976). Ages at death of children with Huntington's chorea and of their affected parents. Annals of Human Genetics (London) 39, 329334.
Went, L. N. & Vegter-van der Vlis, M. (1984). Parental transmission in Huntington's disease. Lancet i, 11001102.

Huntington's chorea in Norway

  • Letten Saugstad (a1) and Ørnulv Ødegård (a1)


Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed