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The topographic distribution of brain atrophy in Huntington's disease and progressive supranuclear palsy
D M Mann1, R Oliver, J S Snowden
1Department of Pathological Sciences, University of Manchester, UK.
Insights
Brain atrophy patterns differ between Huntington's disease (HD) and progressive supranuclear palsy (PSP). HD causes maximal atrophy in basal ganglia and cortex, while PSP affects cortex and basal ganglia differently.
Area of Science:
- Neuroscience
- Neuropathology
- Neuroimaging
Background:
- Huntington's disease (HD) and progressive supranuclear palsy (PSP) are neurodegenerative disorders.
- Understanding their distinct patterns of brain atrophy is crucial for diagnosis and research.
Purpose of the Study:
- To compare the topographic distribution of brain atrophy in Huntington's disease (HD) and progressive supranuclear palsy (PSP).
Main Methods:
- Image analysis of fixed coronal brain slices from patients with HD and PSP.
- Quantification of brain atrophy in specific brain regions.
Main Results:
- In HD, maximal atrophy occurred in the caudate nucleus, putamen, and globus pallidus, with cortical and subcortical structure involvement.
- In PSP, cortical atrophy was less severe than in HD, primarily affecting white matter, with reductions in the globus pallidus and thalamus but not caudate nucleus or putamen.
- Both diseases showed a greater loss of white matter than grey matter, elevating the grey/white matter ratio.
Conclusions:
- Distinct patterns of brain atrophy characterize Huntington's disease and progressive supranuclear palsy.
- These findings aid in differentiating the two conditions neuropathologically.
Abstract:
The topographic distribution of brain atrophy was quantified by image analysis of fixed coronal brain slices from 12 patients dying with Huntington's disease (HD) and from 4 other patients dying with progressive supranuclear palsy (PSP). In HD, atrophy was maximal within the caudate nucleus, putamen and globus pallidus. However, the cerebral cortex was also atrophied with reductions in cross-sectional area within frontal, temporal and parietal lobes. In general, more white matter than grey matter was lost leading to an elevation in the grey/white matter ratio. The amygdala and thalamus were reduced in area. In PSP, lesser reductions in cortical area than those of HD were seen, these again being mostly due to a loss of white matter, resulting in an elevation of the grey/white ratio. The globus pallidus and thalamus were decreased in area, but no changes in the caudate nucleus and putamen were measured.