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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

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 4

Hypothesized time course of changes in striatal D2 binding, pattern expression, and regional metabolism in premanifest HD.

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Hypothesized time course of changes in striatal D2 binding, pattern expr...
The individual growth curve model (114) was applied to the longitudinal imaging data (88) to estimate the time course of these imaging measures in the period prior to clinical diagnosis. The decline in striatal D2 receptor binding (black) begins approximately 25 years before diagnosis (year 0). About 5 years later, striatal metabolism (green) begins to decline. Thalamic metabolic activity (blue) is likely elevated early on (exactly when is not known), but declines to normal levels as clinical signs (Unified HD Rating Scale [UHDRS] score, purple) become evident. HDRP activity is estimated to increase beginning approximately 15 years before diagnosis. This network measure likely increases for a number of years, then declines prior to symptom onset (see text). The horizontal dashed line represents the normal mean. Reprinted with permission from Brain (88).

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