Key result
Huntington's disease mutation is linked to pathological cardiovascular values in ~45% of early carriers.
Why the study?
Cardiovascular events are a major cause of early death in Huntington's disease, and early dysautonomia and circadian rhythm deterioration may affect cardiac health, but cardiovascular risk in pre-manifest and early-stage HD patients compared to controls was unclear.
Cross-Sectional (n=76)
Yes
Answers are grounded in this paper and cite their sources.
BACKGROUND AND PURPOSE: Cardiovascular events are a major cause of early death in the Huntington's disease (HD) population. Dysautonomia as well as deterioration of circadian rhythms can be detected early in the disease progression and can have profound effects on cardiac health. The aim of the present study was to determine if patients with HD and pre-manifest mutation carriers present a higher risk of cardiovascular disease than non-mutation-carrying controls. METHODS: This was a prospective, cross-sectional, multicentre study of 38 HD mutation carriers (23 pre-manifest and 15 early-stage patients) compared with 38 age- and gender-matched healthy controls. Clinical and epidemiological variables, including the main haematological vascular risk factors, were recorded. Ambulatory blood-pressure monitoring and carotid intima-media thickness (CIMT) measurement were performed to assess autonomic function and as target-organ damage markers. RESULTS: percentile in 46.7% and 43.5%, respectively, of the early-stage and pre-manifest patients, whereas none of the controls presented pathological values (P = 0.001 and P = 0.006, respectively). Nocturnal non-dipping was significantly associated with CIMT values in patients (P = 0.002) but not in controls. CONCLUSIONS: These results suggest that higher cardiovascular risks and target-organ damage are present even in pre-manifest patients. Although larger studies are needed to confirm these findings, clinicians should consider these results in the cardiovascular management of patients with HD.
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Diago et al. (2018) conducted a cross-sectional in Huntington's disease (n=76). Huntington's disease mutation status vs. Healthy non-mutation-carrying controls was evaluated on Pathological cardiovascular values (e.g., nocturnal blood pressure non-dipping >95th percentile) (p=0.001). Pathological cardiovascular values were present in 46.7% of early-stage and 43.5% of pre-manifest Huntington's disease mutation carriers compared to 0% of healthy controls.
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