Functional impairment of precerebral arteries in Huntington 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.
University Medical Centre Ljubljana, Division of Neurology, Ljubljana, Slovenia. Electronic address: jan.kobal@gmail.com.
Jan
J
Kobal
University of Ljubljana, Faculty of Medicine, Institute of Physiology, Ljubljana, Slovenia.
Ksenija
K
Cankar
University Medical Centre Ljubljana, Division of Neurology, Ljubljana, Slovenia; University of Ljubljana, Faculty of Medicine, Department of Neurology, Ljubljana, Slovenia.
Janja
J
Pretnar
University Medical Centre Ljubljana, Division of Neurology, Ljubljana, Slovenia.
Marjan
M
Zaletel
University of Ljubljana, Faculty of Medicine, Department of Neurology, Ljubljana, Slovenia.
Lucijan
L
Kobal
University Medical Centre Ljubljana, Division of Gynaecology and Obstetrics, Ljubljana, Slovenia.
Natasa
N
Teran
University of Ljubljana, Faculty of Medicine, Institute of Physiology, Ljubljana, Slovenia.
Ziva
Z
Melik
D000328:Adult; D001783:Blood Flow Velocity / Q000502:physiology; D001794:Blood Pressure / Q000502:physiology; D059168:Carotid Intima-Media Thickness; D016022:Case-Control Studies; D003327:Coronary Disease / Q000000981:diagnostic imaging / Q000209:etiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications* / Q000000981:diagnostic imaging / Q000235:genetics; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D014463:Ultrasonography; D059289:Vascular Stiffness / Q000502:physiology; D055815:Young Adult
Arterial stiffness parameters; Autonomic nervous system activity; Huntington disease; Intima-media thickness; Ultrasonography