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J Med Genet. 1992 April; 29(4): 239–242.
PMCID: PMC1015921

Age at onset and life table risks in genetic counselling for Huntington's disease.


Age related genetic risk data for carrying the gene for Huntington's disease in relatives at risk, based on a previously documented life table approach, are presented in a form allowing ready access for use in genetic counselling. Figures are given for first degree relatives and for second degree relatives showing varying age combinations of consultand and intervening parent at risk. Data are also given for the risk of developing Huntington's disease over varying finite periods in relation to age. The availability of this information in tabular form should help those involved in genetic counselling for this disorder in providing accurate risk estimates to relatives; the data are also of importance for combination with genotype information in predictive testing.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Newcombe RG. A life table for onset of Huntington's chorea. Ann Hum Genet. 1981 Oct;45(Pt 4):375–385. [PubMed]
  • Newcombe RG, Walker DA, Harper PS. Factors influencing age at onset and duration of survival in Huntington's chorea. Ann Hum Genet. 1981 Oct;45(Pt 4):387–396. [PubMed]
  • Adams P, Falek A, Arnold J. Huntington disease in Georgia: age at onset. Am J Hum Genet. 1988 Nov;43(5):695–704. [PubMed]
  • O'Brien T, Newcombe RG, Harper PS. Outlook for a clinically normal child in a sibship with congenital myotonic dystrophy. J Pediatr. 1983 Nov;103(5):762–763. [PubMed]
  • Morris MJ, Tyler A, Lazarou L, Meredith L, Harper PS. Problems in genetic prediction for Huntington's disease. Lancet. 1989 Sep 9;2(8663):601–603. [PubMed]
  • Walker DA, Harper PS, Wells CE, Tyler A, Davies K, Newcombe RG. Huntington's Chorea in South Wales. A genetic and epidemiological study. Clin Genet. 1981 Apr;19(4):213–221. [PubMed]
  • Walker DA, Harper PS, Newcombe RG, Davies K. Huntington's chorea in South Wales: mutation, fertility, and genetic fitness. J Med Genet. 1983 Feb;20(1):12–17. [PMC free article] [PubMed]
  • Conneally PM. Huntington disease: genetics and epidemiology. Am J Hum Genet. 1984 May;36(3):506–526. [PubMed]
  • Went LN, Vegter-van der Vlis M, Bruyn GW, Volkers WS. Huntington's Chorea in the Netherlands. The problem of genetic heterogeneity. Ann Hum Genet. 1983 Jul;47(Pt 3):205–214. [PubMed]
  • Hayden MR, Soles JA, Ward RH. Age of onset in siblings of persons with juvenile Huntington disease. Clin Genet. 1985 Aug;28(2):100–105. [PubMed]
  • Clarke DJ, Bundey S. Very early onset Huntington's disease: genetic mechanism and risk to siblings. Clin Genet. 1990 Sep;38(3):180–186. [PubMed]
  • Penney JB, Jr, Young AB, Shoulson I, Starosta-Rubenstein S, Snodgrass SR, Sanchez-Ramos J, Ramos-Arroyo M, Gomez F, Penchaszadeh G, Alvir J, et al. Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals. Mov Disord. 1990;5(2):93–99. [PubMed]

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