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Updated: May 21, 2026
09:06
Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
Published on: June 9, 2018
Brain metabolite alterations and cognitive dysfunction in early Huntington's disease
Paul G Unschuld1, Richard A E Edden, Aaron Carass
1Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA. unschuld@jhmi.edu
Summary
Huntington
Area of Science:
- Neuroscience
- Biochemistry
- Medical Imaging
Background:
- Huntington's disease (HD) is a genetic neurodegenerative disorder.
- It is characterized by progressive cognitive decline, dementia, and movement disorders.
- The disease stems from a mutation in the Huntingtin gene.
Purpose of the Study:
- To investigate the link between brain metabolites and cognitive function in HD mutation carriers.
- To utilize high-field-strength magnetic resonance spectroscopy (MRS) for metabolite analysis.
- To explore potential biomarkers for early HD stages.
Main Methods:
- 1H magnetic resonance spectroscopy (MRS) at 7 Tesla was performed on 12 HD mutation carriers and 12 healthy controls.
- MRS targeted the posterior cingulate cortex.
- Cognitive function was assessed using the Montreal Cognitive Assessment (MoCA).
Main Results:
- HD mutation carriers showed significantly lower N-acetylaspartate (NAA) and glutamate levels in the posterior cingulate cortex compared to controls.
- No significant differences in brain morphology (striatal and ventricle volumes) were observed between groups.
- NAA and glutamate levels strongly correlated with MoCA scores in HD subjects.
Conclusions:
- Reduced NAA and glutamate in the posterior cingulate cortex are associated with cognitive decline in early-stage HD.
- MRS metabolite signals may serve as potential biomarkers for HD progression.
- These biomarkers could be valuable in tracking treatment outcomes, especially when structural changes are minimal.
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