Abstract

Diabetes mellitus and hypercholesterolemia are known risk factors of stroke, and altered glucose and cholesterol metabolism has been reported in patients with Huntington's disease (HD). We investigated the incidence and risk factors of stroke in this population. National registries were used to identify a cohort of 192 patients with HD. Data on stroke, silent cerebral infarcts and risk factors were obtained from the patient records. Five patients with an ischemic stroke (IS) were found suggesting a crude incidence of 42/100,000 person years. Silent brain infarcts were found in 13 patients and a hemorrhagic stroke in two patients, while none were found with a transient ischemic attack (TIA). The cumulative incidence of IS was 2.7% and that of silent cerebral infarct 6.7% by age of 65 years. The CAG age product (CAP) score, an estimate of genetic burden, was 495 ± 117 for the patients with IS or silent cerebral infarct and 568 ± 126 for the patients without ischemic events (P = 0.025 for difference). The frequency of diagnoses of stroke risk factors was at least twofold higher among the patients with IS or silent infarcts than among those without. Cerebrovascular disease is as common in patients with HD as in the general population, but minor cerebrovascular events and vascular risk factors may remain unrecognized. Genetic burden of the HTT mutation does not appear to increase the risk of stroke.

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