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Brain networks in Huntington disease
David Eidelberg, D. James Surmeier
David Eidelberg, D. James Surmeier
Published February 1, 2011
Citation Information: J Clin Invest. 2011;121(2):484-492. https://doi.org/10.1172/JCI45646.
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Review Series Article has an altmetric score of 6

Brain networks in Huntington disease

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Abstract

Recent studies have focused on understanding the neural mechanisms underlying the emergence of clinical signs and symptoms in early stage Huntington disease (HD). Although cell-based assays have focused on cell autonomous effects of mutant huntingtin, animal HD models have revealed alterations in the function of neuronal networks, particularly those linking the cerebral cortex and striatum. These findings are complemented by metabolic imaging studies of disease progression in premanifest subjects. Quantifying metabolic progression at the systems level may identify network biomarkers to aid in the objective assessment of new disease-modifying therapies and identify new regions that merit mechanistic study in HD models.

Authors

David Eidelberg, D. James Surmeier

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Figure 2

HDRP.

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HDRP.
(A) This pattern was identified by spatial covariance analysis of ...
(A) This pattern was identified by spatial covariance analysis of 18F-Fluorodeoxyglucose (FDG) PET scans at baseline from 12 premanifest HD gene carriers and 12 age-matched healthy control subjects (115). This pattern is characterized by metabolic decreases in the striatum and cingulate cortex, with relative increases in the ventral thalamus, primary motor cortex (Brodmann areas [BA] 4), and occipital cortex (BA 17, 18). The covariance map was overlaid on T1-weighted magnetic resonance–template images. Voxel weights for the pattern were thresholded at P = 0.005. Voxels with positive region weights (increases) are shown in red, and voxels with negative region weights (decreases) are shown in blue. (B) Time course of HDRP expression in the 12 premanifest gene carriers scanned at baseline and at 18 and 44 months. Mean subject scores are displayed for the 8 premanifest subjects who subsequently phenoconverted (red), the 4 subjects who did not phenoconvert during the period of follow-up (blue), and all 12 subjects (black). Subject scores, reflecting the expression of this pattern, separate the premanifest from the control groups (P < 0.01). (The mean value [± 1 SD] for the healthy control group is represented by dotted lines. Error bars represent 1 SEM at each time point). Reprinted with permission from Brain (88).

Copyright © 2025 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

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