Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Apr;29(2):642-648.
doi: 10.1007/s12350-020-02299-7. Epub 2020 Aug 16.

Autonomic disorders and myocardial 123I-metaiodobenzylguanidine scintigraphy in Huntington's disease

Roberta Assante  1 , Elena Salvatore  2 , Carmela Nappi  1 , Silvio Peluso  2 , Giovanni De Simini  1 , Luigi Di Maio  2 , Gianluigi Rosario Palmieri  2 , Isabella Pia Ferrara  2 , Alessandro Roca  2 , Giuseppe De Michele  2 , Alberto Cuocolo  1 , Sabina Pappatà  3 , Anna De Rosa  4
Affiliations

Autonomic disorders and myocardial 123I-metaiodobenzylguanidine scintigraphy in Huntington's disease

Roberta Assante et al. J Nucl Cardiol. 2022 Apr.

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.

Keywords: Huntington’s disease; MIBG; autonomic disorders; scintigraphy.

PubMed Disclaimer

Conflict of interest statement

Anna De Rosa, Roberta Assante, Elena Salvatore, Carmela Nappi, Silvio Peluso, Giovanni De Simini, Luigi Di Maio, Gianluigi Rosario Palmieri, Alessandro Roca, Giuseppe De Michele, Alberto Cuocolo and Sabina Pappatà declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
Early H/M (A) and late H/M ratio (B) results in patients (Pt) and controls (Ctrl). The central boxes represent the values from the lower to upper quartile (25° to 75° percentile). The middle lines represent the mean. The vertical lines extend from the minimum to the maximum value; pathological values are displayed as separate points
Figure 2
Figure 2
Relationship between SCOPA-AUT score and washout rate (Spearman’s correlation; rho 0.631; P = 0.015)
See this image and copyright information in PMC

Comment in

References

    1. McColgan P, Tabrizi SJ. Huntington’s disease: A clinical review. Eur J Neurol. 2018;25(1):24–34. doi: 10.1111/ene.13413. - DOI - PubMed
    1. Roos RA. Huntington’s disease: a clinical review. Orphanet J Rare Dis. 2010;20(5):40. doi: 10.1186/1750-1172-5-40. - DOI - PMC - PubMed
    1. Petersén A, Björkqvist M. Hypothalamic–endocrine aspects in Huntington’s disease. Eur J Neurosci. 2006;24(4):961–967. doi: 10.1111/j.1460-9568.2006.04985.x. - DOI - PubMed
    1. Aziz NA, Anguelova GV, Marinus J, van Dijk JG, Roos RA. Autonomic symptoms in patients and pre-manifest mutation carriers of Huntington’s disease. Eur J Neurol. 2010;17(8):1068–1074. doi: 10.1111/j.1468-1331.2010.02973.x. - DOI - PubMed
    1. Abildtrup M, Shattock M. Cardiac dysautonomia in Huntington’s disease. J Huntingtons Dis. 2013;2(3):251–261. doi: 10.3233/JHD-130054. - DOI - PubMed

MeSH terms