Functional impairment of precerebral arteries in Huntington disease
Jan Kobal1, Ksenija Cankar2, Janja Pretnar3
1University Medical Centre Ljubljana, Division of Neurology, Ljubljana, Slovenia.
Insights
Huntington disease (HD) shows early functional vascular changes in precerebral arteries, even before symptoms appear. These arterial stiffness and compliance issues suggest early cardiovascular involvement in HD progression.
Area of Science:
- Neurology
- Cardiology
- Vascular Biology
Background:
- Cardiovascular pathology in Huntington disease (HD) is complex, with early microvascular dysfunction and later risks of cardiomyopathy and stroke.
- The progression of cardiovascular complications in HD warrants further investigation into early-stage vascular changes.
Purpose of the Study:
- To evaluate coronary heart disease (CHD) risk factors and precerebral artery structure/function in Huntington disease (HD) patients.
- To assess changes in carotid artery intima-media thickness (IMT) and distensibility across different stages of HD.
Main Methods:
- The study included 41 HD subjects (categorized as presymptomatic-PHD, early-EHD, midstage-MHD, late-LHD) and 41 matched controls.
- Coronary heart disease (CHD) risk factors were assessed, alongside Doppler examinations of precerebral arteries, including IMT and distensibility parameters.
Main Results:
- No significant differences in non-obstructive or obstructive plaques were found between HD patients and controls.
- Midstage HD (MHD) patients exhibited significantly increased carotid artery intima-media thickness (IMT).
- Presymptomatic-PHD and early-EHD groups showed significantly higher arterial stiffness and lower carotid artery compliance.
Conclusions:
- Functional vascular pathology is evident in presymptomatic-PHD, early-EHD, and midstage-MHD patients.
- Precerebral artery dysfunction in HD appears primarily functional, correlating with known autonomic nervous system changes in the disease.
Background:
Cardiovascular pathology of Huntington disease (HD) appears to be complex; while microvascular dysfunction seems to appear early, deaths from cardiomyopathy and stroke might occur in the late phase of HD.
Methods:
Our study evaluated global risk factors for coronary heart disease (CHD), structure and function of precerebral arteries in 41 HD subjects and 41 matched controls. HD subjects were divided into groups by the United Huntington disease rating scale (presymptomatic-PHD, early-EHD, midstage-MHD and late-LHD). CHD risk factors assessment and Doppler examination of precerebral arteries were performed, including measurements of the carotid artery intima-media thickness (IMT), and parameters indicating local carotid artery distensibility (stiffness index β, pulse wave velocity, pressure strain elasticity module and carotid artery compliance).
Results:
In the HD and controls we identified a comparable number of non-obstructive plaques (<50% lumen narrowing). No obstructive plaques (>50% lumen narrowing) were found. There was significantly increased IMT in MHD. In PHD and EHD the parameters of arterial stiffness were significantly higher and the carotid artery compliance was significantly lower.
Conclusions:
Our results reveal functional vascular pathology in PHD, EHD, and MHD. Precerebral arteries dysfunction in HD therefore appears to be mostly functional and in agreement with recently described autonomic nervous system changes in HD.
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