Literature DB >> 1833547

Age at onset in Huntington's disease: effect of line of inheritance and patient's sex.

R A Roos1, M Vegter-van der Vlis, J Hermans, H M Elshove, A C Moll, J J van de Kamp, G W Bruyn.   

Abstract

The Leiden Roster for Huntington's disease (HD) contained data on 2617 cases up to July 1988. The age at onset (AO) was known in 1084 cases and in 1020 of these both their AO and the sex of the affected parent was known. The mean AO was higher for females than for males and higher for maternal than for paternal cases. However, in the group born before 1925 only females with maternal inheritance had a higher mean AO. Data on influence of sex and line of inheritance were present for the grandparents as well as for the great grandparents. Influence of the line of inheritance from the grandparents was particularly present for the grandmother-father (MP) lineage; regarding the great grandparents a significant difference was found between the MPM and PMP lineage. The results obtained for juvenile HD cases were comparable to those previously published. In late onset cases (over 50 years) no maternal preponderance in inheritance was found.

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Year:  1991        PMID： 1833547      PMCID： PMC1016978          DOI： 10.1136/jmg.28.8.515

Source DB:  PubMed          Journal:  J Med Genet        ISSN： 0022-2593            Impact factor:   6.318


  14 in total

1.  A genetic and statistical study of some sex-related factors in Huntington's disease.

Authors:  C J Brackenridge
Journal:  Clin Genet       Date:  1971       Impact factor: 4.438

2.  Socio-psychiatric consequences of Huntington's disease.

Authors:  K Dewhurst; J E Oliver; A L McKnight
Journal:  Br J Psychiatry       Date:  1970-03       Impact factor: 9.319

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Authors:  R G Newcombe; D A Walker; P S Harper
Journal:  Ann Hum Genet       Date:  1981-10       Impact factor: 1.670

4.  Parental transmission in Huntington's disease.

Authors:  L N Went; M Vegter-van der Vlis; G W Bruyn
Journal:  Lancet       Date:  1984-05-19       Impact factor: 79.321

5.  Maternal transmission in Huntington's disease.

Authors:  R H Myers; D Goldman; E D Bird; D S Sax; C R Merril; M Schoenfeld; P A Wolf
Journal:  Lancet       Date:  1983-01-29       Impact factor: 79.321

6.  Two models for a maternal factor in the inheritance of Huntington disease.

Authors:  M Boehnke; P M Conneally; K Lange
Journal:  Am J Hum Genet       Date:  1983-09       Impact factor: 11.025

7.  A genetic model for age at onset in Huntington disease.

Authors:  L A Farrer; P M Conneally
Journal:  Am J Hum Genet       Date:  1985-03       Impact factor: 11.025

8.  Late onset of Huntington's disease.

Authors:  R H Myers; D S Sax; M Schoenfeld; E D Bird; P A Wolf; J P Vonsattel; R F White; J B Martin
Journal:  J Neurol Neurosurg Psychiatry       Date:  1985-06       Impact factor: 10.154

9.  Factors related to onset age of Huntington disease.

Authors:  R H Myers; J J Madden; J L Teague; A Falek
Journal:  Am J Hum Genet       Date:  1982-05       Impact factor: 11.025

10.  Ages of death of children with Huntington's chorea and of their affected parents.

Authors:  M Vegter-van der Vlis; W S Volkers; L N Went
Journal:  Ann Hum Genet       Date:  1976-01       Impact factor: 1.670
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  23 in total

1.  Intelligence indices in people with a high/low risk for developing Huntington's disease.

Authors:  G M de Boo; A Tibben; J B Lanser; A Jennekens-Schinkel; J Hermans; M Vegter-van der Vlis; R A Roos
Journal:  J Med Genet       Date:  1997-07       Impact factor: 6.318

2.  Preparing for presymptomatic DNA testing for early onset Alzheimer's disease/cerebral haemorrhage and hereditary Pick disease.

Authors:  A Tibben; M Stevens; G M de Wert; M F Niermeijer; C M van Duijn; J C van Swieten
Journal:  J Med Genet       Date:  1997-01       Impact factor: 6.318

3.  The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice.

Authors:  A Reiner; D C Lafferty; H B Wang; N Del Mar; Y P Deng
Journal:  Neurobiol Dis       Date:  2012-03-27       Impact factor: 5.996

4.  Duration of illness in Huntington's disease is not related to age at onset.

Authors:  R A Roos; J Hermans; M Vegter-van der Vlis; G J van Ommen; G W Bruyn
Journal:  J Neurol Neurosurg Psychiatry       Date:  1993-01       Impact factor: 10.154

5.  Psychological distress in applicants for predictive DNA testing for autosomal dominant, heritable, late onset disorders. The Rotterdam/Leiden Genetics Workgroup.

Authors:  A C DudokdeWit; A Tibben; H J Duivenvoorden; P G Frets; M W Zoeteweij; M Losekoot; A van Haeringen; M F Niermeijer; J Passchier
Journal:  J Med Genet       Date:  1997-05       Impact factor: 6.318

6.  Huntington's disease: confirmation of diagnosis and presymptomatic testing in Spanish families by genetic analysis.

Authors:  A Sánchez; S Castellví-Bel; M Milà; D Genis; M Calopa; D Jiménez; X Estivill
Journal:  J Neurol Neurosurg Psychiatry       Date:  1996-12       Impact factor: 10.154

7.  Estimating decreased risks for Huntington disease without a test.

Authors:  Reinier Timman; Benno Bonke; Theo Stijnen; Aad Tibben; Anneke Maat-Kievit
Journal:  Eur J Epidemiol       Date:  2008-01-30       Impact factor: 8.082

8.  Predictive testing for Huntington disease: nonparticipants compared with participants in the Dutch program.

Authors:  I M van der Steenstraten; A Tibben; R A Roos; J J van de Kamp; M F Niermeijer
Journal:  Am J Hum Genet       Date:  1994-10       Impact factor: 11.025

9.  Neocortical expression of mutant huntingtin is not required for alterations in striatal gene expression or motor dysfunction in a transgenic mouse.

Authors:  Timothy B Brown; Alexey I Bogush; Michelle E Ehrlich
Journal:  Hum Mol Genet       Date:  2008-07-16       Impact factor: 6.150

10.  Dynamic mutation in Dutch Huntington's disease patients: increased paternal repeat instability extending to within the normal size range.

Authors:  K E De Rooij; P A De Koning Gans; M I Skraastad; R D Belfroid; M Vegter-Van Der Vlis; R A Roos; E Bakker; G J Van Ommen; J T Den Dunnen; M Losekoot
Journal:  J Med Genet       Date:  1993-12       Impact factor: 6.318
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