Abstract
BackgroundHypertension is a modifiable cardiovascular risk factor implicated in neurodegeneration and dementia risk. In Huntington's disease, a monogenic neurodegenerative disease, autonomic and vascular abnormalities have been reported. This study's objective was to examine the relationship between hypertension and disease severity and progression in Huntington's disease.MethodsUsing longitudinal data from the largest worldwide observational study of Huntington's disease (n = 14,534), we assessed the relationship between hypertension, disease severity, and rate of clinical progression in Huntington's disease mutation carriers. Propensity score matching was used to statistically match normotensive and hypertensive participants for age, sex, body mass index, ethnicity, and CAG length.ResultsHuntington's disease patients had a lower prevalence of hypertension compared with age‐matched gene‐negative controls. Huntington's disease patients with hypertension had worse cognitive function, a higher depression score, and more marked motor progression over time compared with Huntington's disease patients without hypertension. However, hypertensive patients taking antihypertensive medication had less motor, cognitive, and functional impairment than Huntington's disease patients with untreated hypertension and a later age of clinical onset compared with untreated hypertensive patients and normotensive individuals with Huntington's disease.ConclusionsWe report the novel finding that hypertension and antihypertensive medication use are associated with altered disease severity, progression, and clinical onset in patients with Huntington's disease. These findings have implications for the management of hypertension in Huntington's disease and suggest that prospective studies of the symptomatic or disease‐modifying potential of antihypertensives in neurodegenerative diseases are warranted. © 2020 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.
Highlights
Manifest Huntington’s disease (HD) participants were older and had a lower body mass index (BMI) compared with premanifest HD and control participants, and premanifest HD participants were younger with a lower BMI than control participants, justifying the use of propensity matching to account for confounding variables
Using rich longitudinal data from the largest observation study in HD, we present novel insights into the association between hypertension — a cardiovascular risk factor implicated in neurodegeneration — and HD disease severity and progression
These observed differences between normotensive and hypertensive HD patients appear to be driven by antihypertensive medication use; hypertensive HD patients not receiving antihypertensive treatment had worse motor, cognitive, and functional capacity and more marked symptom progression over time compared with normotensive HD patients and hypertensive patients treated with antihypertensive medication, with potential implications for the clinical management of hypertension in HD
Summary
Enroll-HD is a multicenter longitudinal observational study designed to facilitate clinical research in HD. Performance on motor, functional, behavioral, and cognitive subdomains of the Enroll-HD assessment were used as a measure of disease severity and progression. The measures of interest were total motor score (TMS), depression and anxiety score on the Hospital Anxiety and Depression Scale, total functional capacity (TFC), and all the Unified Huntington’s Disease Rating Scale cognitive subdomains.[36] TFC is a measure of capacity to work, handle finances, perform chores and self-care, and live independently, and the scale ranges from 13 (normal) to 0 (severe disability). BMI, sex, and ethnicity were identified as a priori confounding variables.[35,37,38] Propensity score matching with nearest neighbor matching was used to match (2:1 ratio) the 2 groups of interest and adjust for confounds.[39,40] Propensity scores were calculated using age, BMI, sex, and ethnicity, along with CAG length for HD-specific analysis; the resulting sample size and demographics of included patients for each analysis are shown in Table
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