Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy
Abstract
Hippocampal sclerosis is defined as selective neuronal loss and gliosis of the hippocampus with heterogeneous etiologies, including neurodegenerative tauopathies. We report a 78-year-old woman who presented with depression, in whom postmortem examination revealed almost complete loss of neurons with gliosis in the subiculum and CA1-3 regions of the hippocampus and abundant neuronal cytoplasmic inclusions in the dentate gyrus. The inclusions were round, slightly basophilic and argyrophilic, resembling Pick bodies. However, they were Gallyas- and 4-repeat tau-positive, and 3-repeat tau- and ubiquitin-negative. To our knowledge, the histopathological features in this case were different from those in hippocampal sclerosis or 4-repeat tauopathies reported previously. It is likely that this case is a new variant of 4-repeat tauopathy presenting with hippocampal sclerosis.
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TDP-43 immunoreactive inclusions are observed in the majority of cases of HS.
“TDP-43 immunoreactive inclusions are observed in the majority of cases of HS (Figure 6) [8,28,40,64], about one-half of cases with FTLD and ubiquitin inclusions with or without motor neuron disease, most sporadic cases of amyotrophic lateral sclerosis, and commonly in cases with AD neuropathologic change [8,9] or with LBD [43], as well as other neurodegenerative diseases [48].”
From National Institute on Aging–Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease: a practical approach (2012) · cites this paper as [40]
TDP-43 immunoreactive inclusions are observed in about one-half of cases with FTLD and ubiquitin inclusions with or without motor neuron disease.
“TDP-43 immunoreactive inclusions are observed in the majority of cases of HS (Figure 6) [8,28,40,64], about one-half of cases with FTLD and ubiquitin inclusions with or without motor neuron disease, most sporadic cases of amyotrophic lateral sclerosis, and commonly in cases with AD neuropathologic change [8,9] or with LBD [43], as well as other neurodegenerative diseases [48].”
From National Institute on Aging–Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease: a practical approach (2012) · cites this paper as [40]
TDP-43 immunoreactive inclusions are observed in most sporadic cases of amyotrophic lateral sclerosis.
“TDP-43 immunoreactive inclusions are observed in the majority of cases of HS (Figure 6) [8,28,40,64], about one-half of cases with FTLD and ubiquitin inclusions with or without motor neuron disease, most sporadic cases of amyotrophic lateral sclerosis, and commonly in cases with AD neuropathologic change [8,9] or with LBD [43], as well as other neurodegenerative diseases [48].”
From National Institute on Aging–Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease: a practical approach (2012) · cites this paper as [40]
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References 41 · Cited by 29
- Authors
- Miki, Y., Mori, F., Hori, E., Kaimori, M., & Wakabayashi, K.
- Affiliations
- Hirosaki University (JP)
- Aomori Prefectural Central Hospital (JP)
- Corresponding author
- Miki, Yasuo
- Venue
- Acta Neuropathologica
- Published
- 2009-04-10
- Volume
- 117
- Issue
- 6
- Pages
- 713-718
- ISSN
- 0001-6322 (ISSN-L)
- 1432-0533
- OpenAlex
- W2054669920
- Cited by
- 29
- References
- 41
- Citations by year
- 2025: 1 · 2024: 1 · 2022: 2 · 2021: 2 · 2019: 1 · 2018: 2 · 2017: 1 · 2016: 3 · 2015: 1 · 2014: 4 · 2013: 4 · 2012: 2
- FWCI
- 1.55
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Acta Neuropathologica (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Hirosaki University
- Topics
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- hippocampal sclerosis, dentate gyrus, Pick bodies, 4-repeat tau, neuronal loss, gliosis, subiculum, depression, neurodegeneration
- MeSH terms
- Aged, Inclusion Bodies, Diagnosis, Differential, Female, Hippocampus, Humans, Immunohistochemistry, Microscopy, Electron, Neurons, Sclerosis, Neurofibrillary Tangles, tau Proteins, Cell Death, Pyramidal Cells, Dentate Gyrus, Protein Isoforms, Tauopathies, Ubiquitin
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