| Typical Onset | Typically 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-specific | 10-20 per 100,000 in individuals aged 45-64 years |
| Gender distribution | Slight male predominance (1.5:1) in bvFTD |
| Survival | Median survival 6-11 years from symptom onset |
| Type A | Neuronal intranuclear inclusions (NII) and neuronal cytoplasmic inclusions (NCI); associated with **GRN** mutations |
| Type B | Diffuse NCI without NII; associated with **C9orf72** expansions |
| Type C | NCI predominant; typical of semantic variant PPA |
| VCP-associated | Inclusion body myopathy with Paget disease of bone (IBMPFD) and FTD; **VCP** mutations cause TDP-43 pathology |
| Pick's Disease | 3R tau filaments, Pick bodies, focal frontal/temporal atrophy |
| Argyrophilic Grain Disease (AGD) | Argyrophilic grain disease, 4R tau, mild cognitive impairment |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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