Changes in striatal dopamine d2 receptor binding in pre‐clinical huntington's disease

JCH Van Oostrom, M Dekker… - European Journal of …, 2009 - Wiley Online Library
JCH Van Oostrom, M Dekker, ATM Willemsen, BM De Jong, RAC Roos, KL Leenders
European Journal of Neurology, 2009Wiley Online Library
Background: Carriers of the Huntington disease (HD) mutation develop a progressive
neurodegenerative disorder after a pre‐clinical phase. We examined the value of 11C‐
raclopride PET (RAC) as a biomarker for pre‐clinical HD pathophysiology. Methods: In a
prospective cohort study with clinical and neuropsychological assessment we collected
complete RAC data in 18 pre‐clinical mutation carriers (HD‐PMC) and 11 controls. Follow‐
up was 2 years. We calculated striatal RAC binding potential (BP) to measure dopamine D2 …
Background:  Carriers of the Huntington disease (HD) mutation develop a progressive neurodegenerative disorder after a pre‐clinical phase. We examined the value of 11C‐raclopride PET (RAC) as a biomarker for pre‐clinical HD pathophysiology.
Methods:  In a prospective cohort study with clinical and neuropsychological assessment we collected complete RAC data in 18 pre‐clinical mutation carriers (HD‐PMC) and 11 controls. Follow‐up was 2 years. We calculated striatal RAC binding potential (BP) to measure dopamine D2 receptor availability.
Results:  No HD‐PMC had overt neuropsychological dysfunction. RAC‐BP in putamen was abnormal in up to 44% of HD‐PMC. The rate of RAC‐BP decline (2.6% per year) was not significantly higher than in controls. Follow‐up putaminal BP correlated weakly with predicted distance to onset of clinical HD (P = 0.034), but the rate of decline did not. Three HD‐PMC developed motor abnormalities suspect for HD but did not show an increased rate of decline of putaminal BP.
Conclusions:  Many HD‐PMC have striatal abnormalities but we found no clearly increased rate of D2 receptor changes around the onset of clinical HD. A longer follow‐up of the present study cohort is needed to establish the value of RAC‐BP in assessing the risk of clinical conversion from striatal D2 binding data.
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