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

More general terms

• encephalopathy
• neurodegenerative disease

Additional terms

• Alzheimer's disease (AD)
• transactive response (TAR) DNA-binding protein 43; TDP-43 (TARDBP TDP43)