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Does arterial hypertension influence the onset of Huntington's disease?

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  • REGISTRY investigators of the European Huntington’s Disease Network (EHDN)
  • Leire Valcárcel-Ocete
  • Asier Fullaondo
  • Gorka Alkorta-Aranburu
  • María García-Barcina
  • Raymund A C Roos
  • Lena E Hjermind
  • Carsten Saft
  • Marina Frontali
  • Ralf Reilmann
  • Hugh Rickards
  • Ana M Zubiaga
  • Ana Aguirre
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Huntington's disease (HD) age of onset (AO) is mainly determined by the length of the CAG repeat expansion in the huntingtin gene. The remaining AO variability has been attributed to other little-known factors. A factor that has been associated with other neurodegenerative diseases is arterial hypertension (AHT). The aim of this study is to evaluate the contribution of AHT to the AO of HD. We used data from a cohort of 630 European HD patients with adult onset collected by the REGISTRY project of the European Huntington's Disease Network. Multiple linear regression and ANOVA, controlling for the CAG repeat number of the expanded allele (CAGexp) of each patient, were performed to assess the association between the AHT condition and the AO of the motor symptoms (mAO). The results showed a significant association between AHT and mAO, especially when we only considered the patients diagnosed with AHT prior to manifesting any HD signs (pre-HD AHT). Remarkably, despite the low number of cases, those patients developed motor symptoms 5-8 years later than normotensive patients in the most frequent CAGexp range (40-44). AHT is an age-related condition and consequently, the age of the patient at the time of data collection could be a confounder variable. However, given that most pre-HD AHT patients included in our study had started treatment with antihypertensive drugs prior to the onset of HD, and that antihypertensive drugs have been suggested to confer a neuroprotective effect in other neurodegenerative diseases, raises the interest in elucidating the impact of AHT and/or AHT treatment in HD age of onset in further studies. A confirmation of our results in a larger sample set would open the possibility to significantly improve HD management.

Original languageEnglish
JournalPLoS One
Volume13
Issue number5
Pages (from-to)e0197975
ISSN1932-6203
DOIs
Publication statusPublished - 23 May 2018

ID: 55495095