J Med Genet 34:564-568 doi:10.1136/jmg.34.7.564
  • Research Article

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

  1. G M de Boo,
  2. A Tibben,
  3. J B Lanser,
  4. A Jennekens-Schinkel,
  5. J Hermans,
  6. M Vegter-van der Vlis,
  7. R A Roos
  1. Department of Neurology, Leiden University Hospital, The Netherlands.


      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.