Frontotemporal dementia pathology

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FTD pathology organized by clinical syndrome

(Ljubenkov and Miller, 2016)


FTD organized by underlying pathology

(Ljubenkov and Miller, 2016

  • FTLD-tau: (MAPT)
    • 3R tau (Pick’s disease)
      • bvFTD > nfvPPA, svPPA, CBS
    • 4R tau, CBD
      • Motor dysexecutive (including CBS) > PSP-S, nfvPPA, bvFTD
    • 4R tau, PSP
      • PSP-S > CBS, nfvPPA, bvFTD
  • FTLD-TDP
    • TDP type A (GRN)
      • bvFTD > CBS, nfsvPPA > FTD-MND
    • TDP type B (C9ORF72)
      • bvFTD, FTD-MND, ALS
    • TDP type C
      • svPPA
    • TDP type D (always VCP mutation)
      • bvFTD, ALS, Paget’s disease of the bone, inclusion body myositis
  • FTLD-FUS (FUS gene)
    • atypical FTLD with ubiquitin inclusions, basophilic inclusion body disease; neuronal intermediate filament inclusion disease
      • bvFTD if sporadic (w/ early age of onset (22-46 years) versus ALS if genetic


References

Ljubenkov, P. A. & Miller, B. L. A Clinical Guide to Frontotemporal Dementias. FOCUS 14, 448–464 (2016). https://pubmed.ncbi.nlm.nih.gov/31975825/