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Age of onset in siblings of persons with juvenile Huntinqton disease
Author(s) -
Hayden M. R.,
Soles J. A.,
Ward D R. H.
Publication year - 1985
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1985.tb00367.x
Subject(s) - juvenile , age of onset , sibling , proband , population , disease , medicine , pediatrics , biology , demography , genetics , psychology , developmental psychology , mutation , environmental health , sociology , gene
The age of onset distribution of Huntington disease (HD) has been defined for siblings of patients who exhibit the disease before age 20. The mean age of onset for the siblings of juvenile onset cases is 26.8 years, which is significantly less than the mean age of onset (39.8) observed in siblings of non‐juvenile cases. We have shown that the curve for age of onset in the general affected population is significantly different from that of the juvenile sibships. Furthermore, the significant regression equation suggests that the ‘expected age of onset’ of a sibling can be predicted from a knowledge of the age of onset of the juvenile proband. This information can be used to predict a range of age of onset for those sibs of juvenile patients who are likelv to be asymtomatic heterozygotes with DNA polymorphism studies.