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limbic-predominant age-related TDP-43 encephalopathy (LATE); limbic-predominant amnestic neurodegenerative syndrome (LANS)
Epidemiology:
- 20% of people older than > 80 years
Pathology:
- proteinopathy of advanced age, especially > 80 years
- involves TAR DNA-binding protein 43 (TDP-43)
- limbic-predominant age-related encephalopathy
- disproportionate hippocampal atrophy
- limbic hypometabolism
- absence of neocortical degeneration
- low likelihood of neocortical tau pathology
Clinical manifestations:
- older age at evaluation
- mild clinical syndrome, slow decline
- disease-specific cognitive impairment ... of the Alzheimer's disease type
- memory impairment dominates symptoms
- impaired semantic memory
Radiology:
- MRI neuroimaging
- FDG-PET: limbic hypometabolism, absence of neocortical degeneration
Differential diagnosis:
- Alzheimer's disease
- LANS symptoms are restricted to memory loss
- other cognitive domains are not involved
Related
Alzheimer's disease (AD)
transactive response (TAR) DNA-binding protein 43; TDP-43 (TARDBP TDP43)
General
encephalopathy
neurodegenerative disease
References
- Nelson PT, Dickson DW, Trojanowksi JQ et al
Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working
group report.
Brain. April 30, 2019
PMID: 31039256
https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awz099/5481202
- Abbasi J
Debate Sparks Over LATE, a Recently Recognized Dementia.
JAMA. Published online August 21, 2019.
PMID: 31433447
https://jamanetwork.com/journals/jama/fullarticle/2748914
- Corriveau-Lecavalier N, Botha H, Graff-Radford J et al
Clinical criteria for a limbic-predominant amnestic neurodegenerative syndrome.
Brain Communications. 2024, 6($): July 17
PMID: 39021510 Free PMC article.
https://academic.oup.com/braincomms/article/6/4/fcae183/7712717