Most cases of dementia with hippocampal sclerosis may represent frontotemporal dementia
Abstract
Hippocampal sclerosis dementia (HSD) is a disease of unknown etiology and pathogenesis. To determine whether HSD cases could be reclassified as variants of frontotemporal dementia (FTD), a heterogeneous group of disorders, 18 brain autopsy cases previously diagnosed as HSD were re-evaluated. In 11 cases, ubiquitinated neuronal inclusions, similar to those of motor neuron disease inclusion dementia (MNDID), were found. Brain levels of soluble and insoluble tau were normal. Most patients with pathologic findings of HSD may actually have the MNDID variant of FTD.
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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 [28]
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 [28]
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 [28]
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References 14 · Cited by 86
- Authors
- Hatanpaa, K. J., Blass, D. M., Pletnikova, O., Crain, B. J., Bigio, E. H., Hedreen, J. C., White, C. L., & Troncoso, J. C.
- Affiliations
- Johns Hopkins University (US)
- From the Department of Pathology (Drs. Hatanpaa, Bigio, and White), University of Texas Southwestern Medical School, Dallas, and Departments of Psychiatry and Behavioral Sciences (Dr. Blass), Pathology (Drs. Pletnikova, Crain, Hedreen, and Troncoso), and Neurology (Drs. Crain and Troncoso), Johns Hopkins University School of Medicine, Baltimore, MD.
- Venue
- Neurology
- Published
- 2004-08-10
- Volume
- 63
- Issue
- 3
- Pages
- 538-542
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W1927378289
- Cited by
- 86
- References
- 14
- Citations by year
- 2024: 2 · 2023: 3 · 2022: 3 · 2020: 1 · 2019: 2 · 2018: 2 · 2017: 2 · 2016: 1 · 2015: 4 · 2014: 2 · 2013: 10 · 2012: 3
- FWCI
- 5.39
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Johns Hopkins University
- National Institute on Aging (P30AG012300, P50AG005146, AG 05146, AG 12300)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- frontotemporal dementia, tau protein, differential diagnosis, autopsy findings
- MeSH terms
- Adult, Aged, Aged, 80 and over, Dementia, Female, Frontal Lobe, Hippocampus, Humans, Male, Middle Aged, Phenotype, Sclerosis, Case-Control Studies, tau Proteins, Dentate Gyrus, Myoclonic Epilepsies, Progressive, Tauopathies
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