P182 ARTERIES IN PATIENTS WITH HUNTINGTON’S DISEASE

Abstract

Background: Huntington’s disease (HD) is a neurodegenerative disorder leading to the progressive death of neurons in various brain regions. Although it is a disease of the central nervous system (CNS), mortality surveys indicate that heart disease is one of the mayor causes of death in HD patients. The mechanisms of cardiac pathophysiology of the disease remain unknown. It might be a consequence of altered activity of autonomic nervous system as part of the CNS. 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 in patients or controls. There was significantly increased IMT in MHD patients. 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 autonomic nervous system dysfunction in HD.