disease 3,759 words KG: ent-dise-7446954b 2026-03-23
kind:diseasesection:diseasesstate:published
Contents

Frontotemporal Dementia

Disease Info
Typical OnsetTypically 45-65 years, though ranges from 20-80 years
Behavioral Variant FTD (bvFTD)Characterized by disinhibition, apathy, loss of empathy, compulsivity, and dietary changes
Logopenic Variant PPA (lvPPA)Word-finding pauses and memory lapses
Age-specific10-20 per 100,000 in individuals aged 45-64 years
Gender distributionSlight male predominance (1.5:1) in bvFTD
SurvivalMedian survival 6-11 years from symptom onset
Type ANeuronal intranuclear inclusions (NII) and neuronal cytoplasmic inclusions (NCI); associated with **GRN** mutations
Type BDiffuse NCI without NII; associated with **C9orf72** expansions
Type CNCI predominant; typical of semantic variant PPA
VCP-associatedInclusion body myopathy with Paget disease of bone (IBMPFD) and FTD; **VCP** mutations cause TDP-43 pathology
Pick's Disease3R tau filaments, Pick bodies, focal frontal/temporal atrophy
Argyrophilic Grain Disease (AGD)Argyrophilic grain disease, 4R tau, mild cognitive impairment
DatabasesOMIMOrphanetClinicalTrialsPubMed

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