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. 1997 Jul;34(7):564–568. doi: 10.1136/jmg.34.7.564

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

G M de Boo 1, A Tibben 1, J B Lanser 1, A Jennekens-Schinkel 1, J Hermans 1, M Vegter-van der Vlis 1, R A Roos 1
PMCID: PMC1050996  PMID: 9222964

Abstract

Intelligence in 20 presymptomatic subjects with an increased risk (> 95%) for carrying the gene for Huntington's disease (HD) was studied in a prospective, case-control, single blind study. No significant differences between the groups were detected for intelligence indices and subtest scores (Wechsler Adult Intelligence Scale). The high level of the performance IQ and the significant discrepancy between performance IQ and verbal IQ found in both the high risk and the low risk groups contrasted with our expectations based on anamnestic information, general clinical opinion, and the results of previously conducted studies. We propose that psychosocial circumstances could explain the test results and discuss the consequences of our findings for clinical genetics practice.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Blackmore L., Simpson S. A., Crawford J. R. Cognitive performance in UK sample of presymptomatic people carrying the gene for Huntington's disease. J Med Genet. 1995 May;32(5):358–362. doi: 10.1136/jmg.32.5.358. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Caine E. D., Shoulson I. Psychiatric syndromes in Huntington's disease. Am J Psychiatry. 1983 Jun;140(6):728–733. doi: 10.1176/ajp.140.6.728. [DOI] [PubMed] [Google Scholar]
  3. Codori A. M., Brandt J. Psychological costs and benefits of predictive testing for Huntington's disease. Am J Med Genet. 1994 Sep 15;54(3):174–184. doi: 10.1002/ajmg.1320540304. [DOI] [PubMed] [Google Scholar]
  4. Diamond R., White R. F., Myers R. H., Mastromauro C., Koroshetz W. J., Butters N., Rothstein D. M., Moss M. B., Vasterling J. Evidence of presymptomatic cognitive decline in Huntington's disease. J Clin Exp Neuropsychol. 1992 Nov;14(6):961–975. doi: 10.1080/01688639208402547. [DOI] [PubMed] [Google Scholar]
  5. Evers-Kiebooms G., Cassiman J. J., van den Berghe H. Attitudes towards predictive testing in Huntington's disease: a recent survey in Belgium. J Med Genet. 1987 May;24(5):275–279. doi: 10.1136/jmg.24.5.275. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Giordani B., Berent S., Boivin M. J., Penney J. B., Lehtinen S., Markel D. S., Hollingsworth Z., Butterbaugh G., Hichwa R. D., Gusella J. F. Longitudinal neuropsychological and genetic linkage analysis of persons at risk for Huntington's disease. Arch Neurol. 1995 Jan;52(1):59–64. doi: 10.1001/archneur.1995.00540250063014. [DOI] [PubMed] [Google Scholar]
  7. Gusella J. F., Wexler N. S., Conneally P. M., Naylor S. L., Anderson M. A., Tanzi R. E., Watkins P. C., Ottina K., Wallace M. R., Sakaguchi A. Y. A polymorphic DNA marker genetically linked to Huntington's disease. Nature. 1983 Nov 17;306(5940):234–238. doi: 10.1038/306234a0. [DOI] [PubMed] [Google Scholar]
  8. Jason G. W., Pajurkova E. M., Suchowersky O., Hewitt J., Hilbert C., Reed J., Hayden M. R. Presymptomatic neuropsychological impairment in Huntington's disease. Arch Neurol. 1988 Jul;45(7):769–773. doi: 10.1001/archneur.1988.00520310079021. [DOI] [PubMed] [Google Scholar]
  9. Kessler S., Field T., Worth L., Mosbarger H. Attitudes of persons at risk for Huntington disease toward predictive testing. Am J Med Genet. 1987 Feb;26(2):259–270. doi: 10.1002/ajmg.1320260204. [DOI] [PubMed] [Google Scholar]
  10. Lyle O. E., Gottesman I. I. Premorbid psychometric indicators of the gene for Huntington's disease. J Consult Clin Psychol. 1977 Dec;45(6):1011–1022. doi: 10.1037//0022-006x.45.6.1011. [DOI] [PubMed] [Google Scholar]
  11. Mastromauro C., Myers R. H., Berkman B. Attitudes toward presymptomatic testing in Huntington disease. Am J Med Genet. 1987 Feb;26(2):271–282. doi: 10.1002/ajmg.1320260205. [DOI] [PubMed] [Google Scholar]
  12. Meissen G. J., Berchek R. L. Intended use of predictive testing by those at risk for Huntington disease. Am J Med Genet. 1987 Feb;26(2):283–293. doi: 10.1002/ajmg.1320260206. [DOI] [PubMed] [Google Scholar]
  13. Mohr E., Brouwers P., Claus J. J., Mann U. M., Fedio P., Chase T. N. Visuospatial cognition in Huntington's disease. Mov Disord. 1991;6(2):127–132. doi: 10.1002/mds.870060207. [DOI] [PubMed] [Google Scholar]
  14. Podoll K., Caspary P., Lange H. W., Noth J. Language functions in Huntington's disease. Brain. 1988 Dec;111(Pt 6):1475–1503. doi: 10.1093/brain/111.6.1475. [DOI] [PubMed] [Google Scholar]
  15. Roos R. A., Vegter-van der Vlis M., Hermans J., Elshove H. M., Moll A. C., van de Kamp J. J., Bruyn G. W. Age at onset in Huntington's disease: effect of line of inheritance and patient's sex. J Med Genet. 1991 Aug;28(8):515–519. doi: 10.1136/jmg.28.8.515. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Rosenberg N. K., Sørensen S. A., Christensen A. L. Neuropsychological characteristics of Huntington's disease carriers: a double blind study. J Med Genet. 1995 Aug;32(8):600–604. doi: 10.1136/jmg.32.8.600. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Skraastad M. I., Verwest A., Bakker E., Vegter-van der Vlis M., van Leeuwen-Cornelisse I., Roos R. A., Pearson P. L., van Ommen G. J. Presymptomatic, prenatal, and exclusion testing for Huntington disease using seven closely linked DNA markers. Am J Med Genet. 1991 May 1;39(2):217–222. doi: 10.1002/ajmg.1320390221. [DOI] [PubMed] [Google Scholar]
  18. Strauss M. E., Brandt J. Are there neuropsychologic manifestations of the gene for Huntington's disease in asymptomatic, at-risk individuals? Arch Neurol. 1990 Aug;47(8):905–908. doi: 10.1001/archneur.1990.00530080093016. [DOI] [PubMed] [Google Scholar]
  19. Tibben A., Duivenvoorden H. J., Niermeijer M. F., Vegter-van der Vlis M., Roos R. A., Verhage F. Psychological effects of presymptomatic DNA testing for Huntington's disease in the Dutch program. Psychosom Med. 1994 Nov-Dec;56(6):526–532. doi: 10.1097/00006842-199411000-00008. [DOI] [PubMed] [Google Scholar]
  20. Tibben A., Frets P. G., van de Kamp J. J., Niermeijer M. F., Vegtervan der Vlis M., Roos R. A., Rooymans H. G., van Ommen G. J., Verhage F. On attitudes and appreciation 6 months after predictive DNA testing for Huntington disease in the Dutch program. Am J Med Genet. 1993 Jul 15;48(2):103–111. doi: 10.1002/ajmg.1320480209. [DOI] [PubMed] [Google Scholar]
  21. Wiggins S., Whyte P., Huggins M., Adam S., Theilmann J., Bloch M., Sheps S. B., Schechter M. T., Hayden M. R. The psychological consequences of predictive testing for Huntington's disease. Canadian Collaborative Study of Predictive Testing. N Engl J Med. 1992 Nov 12;327(20):1401–1405. doi: 10.1056/NEJM199211123272001. [DOI] [PubMed] [Google Scholar]

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