Letters to the editor
Psychological studies in Huntington's disease: making up the balance
Magdalena Duisterhofa, Rutger W Trijsburga, Martinus F Niermeijerb, Raymund A C Roosc, Aad Tibbena b ca Department
of Medical Psychology and Psychotherapy, Erasmus University Medical
Centre Rotterdam, Dr Molewaterplein 50, Room CF 226, 3015 GE Rotterdam,
The Netherlands, b Department
of Clinical Genetics, Erasmus University Medical Centre Rotterdam,
Rotterdam, The Netherlands, c Department of Neurology, Leiden University
Medical Centre, Leiden, The Netherlands
Correspondence to: Dr Duisterhof, duisterhof@mpp.fgg.eur.nl
| The first 150 words of the full text of this article appear below. |
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Introduction |
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EDITOR
Huntington's disease (HD) is an incurable
neurodegenerative disease, characterised by involuntary movements,
changes in behaviour and personality, and cognitive impairment, leading to death 15 to 20 years after its onset.1 HD is an
autosomal dominantly inherited disorder, the gene for which is
localised on the short arm of chromosome 4.2 Subjects
carrying the gene will develop the disease in the absence of other
causes of death. The mean age of onset is 40 years, by which time gene
carriers may have passed on the gene to their offspring. The age of
onset ranges from 2 to 75 years3 so that those at risk
(that is, risk carriers at 50% or 25% genetic risk) can never be sure
of having escaped HD.
Since 1986, presymptomatic DNA testing using genetic linkage analysis
has made it possible for risk carriers to have their risk modified
to approximately 98% or 2%. After identification of the HD gene
mutation
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This article has been cited by other articles:
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Tibben, A.
(2009). Genetic discrimination in Huntington's disease. BMJ
338: b1281-b1281
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Timman, R, Stijnen, T, Tibben, A
(2004). Methodology in longitudinal studies on psychological effects of predictive DNA testing: a review. J. Med. Genet.
41: e100-e100
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