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ABSTRACT: Background
Huntington's disease (HD) patients often present with abnormal modulation of blood pressure and heart rate. We investigated whether cardiac autonomic innervation assessed by 123I-metaiodobenzylguanidine (MIBG) imaging is impaired in HD patients, in comparison with controls (Ctrl).Methods
Fifteen patients (6 F and 9 M) were assessed by the motor section of the Unified HD Rating Scale, the Total Function Capacity, and the scale for outcomes in Parkinson's disease-autonomic (SCOPA-AUT) questionnaire. All patients and 10 Ctrl (5 F and 5 M) underwent 123I-MIBG imaging. From planar images, the early and late heart-to-mediastinum (H/M) ratios and myocardial washout rates (WR) were calculated.Results
We did not find significant differences in early and late H/M ratios and WR between the two groups. At individual level, three patients showed reduced early and/or late H/M ratios. The most common autonomic complaints were gastrointestinal and genitourinary disorders. SCOPA-AUT questionnaire score results positively correlated with the disease duration and WR.Conclusions
Our study indicates that myocardial postganglionic sympathetic innervation is essentially preserved or only minimally involved in HD. These findings suggest that the cardiovascular dysfunction might be mainly due to the impairment of brain areas associated with the regulation and modulation of the heart function.
SUBMITTER: Assante R
PROVIDER: S-EPMC8993714 | biostudies-literature | 2022 Apr
REPOSITORIES: biostudies-literature
Assante Roberta R Salvatore Elena E Nappi Carmela C Peluso Silvio S De Simini Giovanni G Di Maio Luigi L Palmieri Gianluigi Rosario GR Ferrara Isabella Pia IP Roca Alessandro A De Michele Giuseppe G Cuocolo Alberto A Pappatà Sabina S De Rosa Anna A
Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology 20200816 2
<h4>Background</h4>Huntington's disease (HD) patients often present with abnormal modulation of blood pressure and heart rate. We investigated whether cardiac autonomic innervation assessed by 123I-metaiodobenzylguanidine (MIBG) imaging is impaired in HD patients, in comparison with controls (Ctrl).<h4>Methods</h4>Fifteen patients (6 F and 9 M) were assessed by the motor section of the Unified HD Rating Scale, the Total Function Capacity, and the scale for outcomes in Parkinson's disease-autonom ...[more]