Clin Genet. 1985 Aug;28(2):100-5.

Age of onset in siblings of persons with juvenile Huntington disease.

Abstract

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 likely to be asymptomatic heterozygotes with DNA polymorphism studies.

PMID:: 2931216; [PubMed - indexed for MEDLINE]

Publication Types, MeSH Terms

Publication Types

Research Support, Non-U.S. Gov't

MeSH Terms

LinkOut - more resources

Other Literature Sources

COS Scholar Universe

Medical

Supplemental Content

Save items

Related citations in PubMed

Factors related to onset age of Huntington disease. [Am J Hum Genet. 1982]
Factors related to onset age of Huntington disease.
Myers RH, Madden JJ, Teague JL, Falek A. Am J Hum Genet. 1982 May; 34(3):481-8.
Juvenile Huntington disease. [Hum Genet. 1986]
Juvenile Huntington disease.
van Dijk JG, van der Velde EA, Roos RA, Bruyn GW. Hum Genet. 1986 Jul; 73(3):235-9.
Inverse relationship between age at onset of Huntington disease and paternal age suggests involvement of genetic imprinting. [Am J Hum Genet. 1992]
Inverse relationship between age at onset of Huntington disease and paternal age suggests involvement of genetic imprinting.
Farrer LA, Cupples LA, Kiely DK, Conneally PM, Myers RH. Am J Hum Genet. 1992 Mar; 50(3):528-35.
Review Huntington's disease of early onset or juvenile Huntington's disease. [Hosp Med. 2003]
Review Huntington's disease of early onset or juvenile Huntington's disease.
Gordon N. Hosp Med. 2003 Oct; 64(10):576-80.
Review Juvenile onset Huntington's disease--clinical and research perspectives. [Ment Retard Dev Disabil Res Re...]
Review Juvenile onset Huntington's disease--clinical and research perspectives.
Nance MA, Myers RH. Ment Retard Dev Disabil Res Rev. 2001; 7(3):153-7.

See reviews... See all...

Cited by 4 PubMed Central articles

Age at onset in Huntington's disease and methylation at D4S95. [J Med Genet. 1993]
Age at onset in Huntington's disease and methylation at D4S95.
Reik W, Maher ER, Morrison PJ, Harding AE, Simpson SA. J Med Genet. 1993 Mar; 30(3):185-8.
Huntington disease in Georgia: age at onset. [Am J Hum Genet. 1988]
Huntington disease in Georgia: age at onset.
Adams P, Falek A, Arnold J. Am J Hum Genet. 1988 Nov; 43(5):695-704.
Patterns of inheritance of the symptoms of Huntington's disease suggestive of an effect of genomic imprinting. [J Med Genet. 1991]
Patterns of inheritance of the symptoms of Huntington's disease suggestive of an effect of genomic imprinting.
Ridley RM, Frith CD, Farrer LA, Conneally PM. J Med Genet. 1991 Apr; 28(4):224-31.

See all...

Related information

Related Citations
Calculated set of PubMed citations closely related to the selected article(s) retrieved using a word weight algorithm. Related articles are displayed in ranked order from most to least relevant, with the “linked from” citation displayed first.
Cited in PMC
Full-text articles in the PubMed Central Database that cite the current articles.

Recent activity

Clear Turn Off Turn On

Age of onset in siblings of persons with juvenile Huntington disease.
Age of onset in siblings of persons with juvenile Huntington disease.
Clin Genet. 1985 Aug ;28(2):100-5.

PubMed
Health hazards of firefighters: acute pulmonary effects after toxic exposures.
Health hazards of firefighters: acute pulmonary effects after toxic exposures.
Br J Ind Med. 1989 Mar ;46(3):209-11.

PubMed
Preoperative assessment as a predictor of mortality and morbidity after lung res...
Preoperative assessment as a predictor of mortality and morbidity after lung resection.
Am Rev Respir Dis. 1989 Apr ;139(4):902-10.

PubMed
Increased intraoperative cardiovascular morbidity in diabetics with autonomic ne...
Increased intraoperative cardiovascular morbidity in diabetics with autonomic neuropathy.
Anesthesiology. 1989 Apr ;70(4):591-7.

PubMed
Benzodiazepine dependence and withdrawal in elderly patients.
Benzodiazepine dependence and withdrawal in elderly patients.
Am J Psychiatry. 1989 Apr ;146(4):529-31.

PubMed

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

PubMed

Result Filters

Display Settings:

Send to: