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

Knowledge Graph

Related Hypotheses (30)

Gamma entrainment therapy to restore hippocampal-cortical sy
Score: 0.68
Hippocampal CA3-CA1 circuit rescue via neurogenesis and syna
Score: 0.68
Circadian Glymphatic Entrainment via Targeted Orexin Recepto
Score: 0.62
p38α Inhibitor and PRMT1 Activator Combination to Restore Ph
Score: 0.61
Digital Twin-Guided Metabolic Reprogramming
Score: 0.61

Related Analyses (21)

Astrocyte reactivity subtypes in neurodegeneration
neurodegeneration · archived
Microglia-astrocyte crosstalk amplification loops in neurode
neurodegeneration · archived
APOE4 structural biology and therapeutic targeting strategie
neurodegeneration · archived
Autophagy-lysosome pathway convergence across neurodegenerat
neurodegeneration · archived
Digital biomarkers and AI-driven early detection of neurodeg
neurodegeneration · archived

Related Experiments (22)

s:** - Temporal analysis showing mitochondrial defects prece
falsification · proposed · Score: 0.40
s:** - Compare tau strain spreading in EXT1/EXT2 conditional
falsification · proposed · Score: 0.40
Autophagy Enhancement Drug Screening for Neurodegeneration
clinical · proposed · Score: 0.40
C9orf72 Phenotype Divergence: ALS vs FTD Mechanism Study
clinical · proposed · Score: 0.40
Circadian-Vascular-Metabolic Syndrome (CVMS) Intervention Tr
clinical · proposed · Score: 0.40