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Hippocampal sclerosis dementia with tauopathy
Thomas G Beach1, Lucia Sue, Sarah Scott
1W. H. Civin Laboratory for Neuropathology, Sun Health Research Institute, Sun City, Ariz 85372, USA. thomas.beach@sunhealth.org
Brain Pathology (Zurich, Switzerland)
|August 30, 2003
Summary
Hippocampal sclerosis dementia (HSD) is often linked to tauopathy, a brain disease affecting neurons and glial cells. This study identifies HSD as a sporadic multisystem tauopathy, proposing the term "hippocampal sclerosis dementia with tauopathy" (HSDT).
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
- Geriatric Medicine
Background:
- Hippocampal sclerosis (HS) is an autopsy finding in some elderly dementia patients, with unclear causes.
- Known causes like seizures or hypoxia are infrequent in these HS cases.
- This study investigates the underlying pathology in dementia associated with HS.
Purpose of the Study:
- To determine the cause of hippocampal sclerosis (HS) in elderly individuals with dementia.
- To characterize the neuropathological features of HS cases, particularly tau protein aggregation.
- To propose a new classification for HS-related dementia based on findings.
Main Methods:
- Analysis of 14 consecutive cases of hippocampal sclerosis (HS).
- Clinical diagnosis review, including dementia, Alzheimer's disease (AD), and parkinsonism.
- Gallyas silver staining and AT8 immunohistochemistry for tau pathology; screening for tau mutations; Western blot analysis of tau protein.
Main Results:
- 12 of 14 HS cases showed widespread neuronal and/or glial tauopathy.
- Tau pathology was observed in various brain regions, including the neocortex, basal ganglia, thalamus, and limbic regions.
- 8 cases also exhibited argyrophilic grains; tau mutation screening was negative, and Western blots revealed mixed 3- and 4-repeat tau forms.
Conclusions:
- Most cases of hippocampal sclerosis dementia are sporadic multisystem tauopathies.
- The findings support a distinct clinicopathological entity.
- The term "hippocampal sclerosis dementia with tauopathy" (HSDT) is proposed for these cases.