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Alzheimer-type lesions in Huntington's disease
1Ludwig Boltzmann Institute of Clinical Neurobiology, Vienna, Austria.
Journal of Neural Transmission (Vienna, Austria : 1996)
|December 30, 1998
Summary
Huntington's disease (HD) patients show early Alzheimer-like lesions, but these progress slower than in typical Alzheimer's disease (AD). This suggests a complex interplay between HD and AD pathologies affecting cognitive decline.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
- Cognitive Neurology
Background:
- Cognitive impairment in Huntington's disease (HD) is linked to striato-frontal and limbic circuit disruption.
- Emerging evidence suggests a higher prevalence of Alzheimer-like lesions in HD brains.
- Understanding the co-occurrence and progression of these pathologies is crucial.
Purpose of the Study:
- To compare the prevalence and characteristics of Alzheimer-type lesions in Huntington's disease (HD) brains versus non-demented controls.
- To investigate the early onset and progression patterns of tau and amyloid pathologies in HD.
- To clarify the relationship between HD, Alzheimer-like lesions, and cognitive decline.
Main Methods:
- A comparative morphological study was conducted on autopsy brain samples.
- 27 cases of Huntington's disease (HD) were analyzed.
- 26 age- and sex-matched non-demented controls were used for comparison.
Main Results:
- 33% of HD brains lacked Alzheimer-type lesions; 48% showed early tau pathology (Braak stages I-II) without amyloid, some as early as age 34.
- 19% of HD brains exhibited Braak stages II-III with amyloid plaques, with the youngest case at 42 years.
- Controls showed similar tau pathology onset later (age 45) and amyloid plaques in 26% (all >60 years).
- No definitive Alzheimer's disease (AD) cases were found in either cohort by CERAD criteria.
Conclusions:
- While the coexistence of HD and AD is rare, initial stages of Alzheimer-like lesions appear early in HD.
- These lesions exhibit slower progression in HD patients compared to typical AD, even in advanced age.
- The mechanisms behind the early onset and mild progression of Alzheimer-like lesions in HD, and their impact on cognition, require further research.