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Updated: May 22, 2026
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Published on: March 11, 2020
Selective Cingulum Degeneration in Huntington's Disease: A Clinically Relevant Event
Fedal Saini1,2,3,4, Ali Demir1,2, H Diana Rosas1,2
1Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.
Summary
Huntington
Area of Science:
- Neuroimaging
- Neurodegenerative Diseases
- Diffusion MRI
Background:
- Huntington's disease (HD) is characterized by brain abnormalities, particularly in the cingulum bundle, which affects executive and emotional functions.
- The cingulum bundle's vulnerability in HD warrants detailed investigation.
Purpose of the Study:
- To analyze cross-sectional and longitudinal diffusion MRI changes in the cingulum bundle in HD.
- To assess the clinical significance of these diffusion MRI changes in Huntington's disease.
Main Methods:
- Longitudinal diffusion MRI study with 66 participants (HD, premanifest HD, controls) over ~4.8 years.
- Analysis using free water elimination and neurite orientation dispersion and density imaging (NDI).
- Examined cingulate (CCG) and angular (CAB) subdivisions, correlating with UHDRS Total Functional Capacity (TFC).
Main Results:
- Baseline HD showed widespread CCG diffusion alterations; CAB changes were limited.
- Longitudinally, only the CCG exhibited progressive changes: reduced fractional anisotropy (FAt) and NDI, increased diffusivity and dispersion.
- Declines in UHDRS TFC correlated with CCG NDI and FAt reduction.
Conclusions:
- The posterior cingulate cortex (CCG) shows more extensive, progressive changes than the angular (CAB) in symptomatic HD.
- Diffusion changes suggest axonal loss and demyelination in fronto-cingulate pathways.
- Cingulate NDI and FAt are potential markers for tracking HD progression.
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