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
. 2024 Jan;39(1):197-203.
doi: 10.1002/mds.29655. Epub 2023 Dec 26.

Baseline Large-Scale Network Dynamics Associated with Disease Progression in Huntington's Disease

Ignacio Aracil-Bolaños  1   2   3   4 , Jesús Pérez-Pérez  1   2   3   4 , Saül Martínez-Horta  1   2   3   4 , Andrea Horta-Barba  1   2   3   4 , Arnau Puig-Davi  1   2   3   4 , Júlia García-Cornet  1   2   3 , Gonzalo Olmedo-Saura  1   2   3   4 , Antonia Campolongo  1   2   3   4 , Javier Pagonabarraga  1   2   3   4 , Jaime Kulisevsky  1   2   3   4
Affiliations

Baseline Large-Scale Network Dynamics Associated with Disease Progression in Huntington's Disease

Ignacio Aracil-Bolaños et al. Mov Disord. 2024 Jan.

Abstract

Background: Huntington's disease (HD) is a genetically determined disease with motor, cognitive, and neuropsychiatric disorders. However, the links between clinical progression and disruptions to dynamics in motor and cognitive large-scale networks are not well established.

Objective: To investigate changes in dynamic and static large-scale networks using an established tool of disease progression in Huntington's disease, the composite Unified Huntington's Disease Rating Scale (cUHDRS).

Methods: Sixty-four mutation carriers were included. Static and dynamic baseline functional connectivity as well as topological features were correlated to 2-year follow-up clinical assessments using the cUHDRS.

Results: Decline in cUHDRS scores was associated with higher connectivity between frontal default-mode and motor networks, whereas higher connectivity in posterior, mainly visuospatial regions was associated with a smaller decline in cUHDRS scores.

Conclusions: Structural disruptions in HD were evident both in posterior parietal/occipital and frontal motor regions, with reciprocal increases in functional connectivity. However, although higher visuospatial network connectivity was tied to a smaller cUHDRS decline, increased motor and frontal default-mode connections were linked to a larger cUHDRS decreases. Therefore, divergent functional compensation mechanisms might be at play in the clinical evolution of HD.

Keywords: Huntington's disease; functional MRI.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Ross CA, Aylward EH, Wild EJ, Langbehn DR, Long JD, Warner JH, et al. Huntington disease: natural history, biomarkers and prospects for therapeutics. Nat Rev Neurol 2014;10(4):204-216.
    1. Tabrizi SJ, Langbehn DR, Leavitt BR, Roos RA, Durr A, Craufurd D, et al. Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol 2009;8(9):791-801.
    1. Peavy GM, Jacobson MW, Goldstein JL, Hamilton JM, Kane A, Gamst AC, et al. Cognitive and functional decline in Huntington's disease: dementia criteria revisited. Mov Disord 2010;25(9):1163-1169.
    1. Schobel SA, Palermo G, Auinger P, Long JD, Ma S, Khwaja OS, et al. Motor, cognitive, and functional declines contribute to a single progressive factor in early HD. Neurology 2017;89(24):2495-2502.
    1. Estevez-Fraga C, Scahill RI, Durr A, Leavitt BR, Roos RAC, Langbehn DR, et al. Composite UHDRS correlates with progression of imaging biomarkers in Huntington's disease. Mov Disord 2021;36(5):1259-1264.

LinkOut - more resources