Aguirre, A.

Alkorta-Aranburu, G.

Frontali, M.

Fullaondo, A.

Garcia-Barcina, M.

Hjermind, L.E.

REGISTRY Invest European Huntingto

Reilmann, R.

Rickards, H.

Roos, R.A.C.

Saft, C.

Valcarcel-Ocete, L.

Zubiaga, A.M.

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

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

REGISTRY investigators of the European Huntington’s Disease Network (EHDN)

Ana M Zubiaga

Ana Aguirre

Directory of Open Access Journals

PLoS ONE

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

Leiden University Scholary Publications

Age: patient’s age at the time of data collection; Sex: M (male) and F (female); CAGexp: CAG number repeat in expanded allele; mAO: motor onset, age of the first motor symptoms; AHT condition: normotensive (patient without arterial hypertension record), with AHT (patient with arterial hypertension records) and pre-HD AHT (patient with arterial hypertension records who manifested hypertension before HD symptoms). (PDF)</p

Leire Valcárcel-Ocete (765355)

Asier Fullaondo (765356)

Gorka Alkorta-Aranburu (113905)

María García-Barcina (22356)

Raymund A. C. Roos (435493)

Lena E. Hjermind (5264375)

Carsten Saft (42954)

Marina Frontali (352662)

Ralf Reilmann (728313)

Hugh Rickards (5264378)

Ana M. Zubiaga (429975)

Ana Aguirre (765363)

The Francis Crick Institute

630 European adult-onset HD patient dataset.

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.This study was funded by Basque Government Department of Industry grants (Saiotek PE08UN78 and University-Company Program 09+ UEGV096/C01), by the Basque Government Department of Education (IT634-13) and by the University of the Basque Country UPV/EHU (UFI11/20). No funding bodies had any role in study design, data collection and analysis, decision to publish, or preparation of the manuscript

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

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