| Prevalence | 15-22 per 100,000 in the 45-65 age group; a 2025 meta-analysis reported a pooled estimate of 9.17 per 100,000[@connectomebased] |
| Incidence | 2.7-4.1 per 100,000 person-years |
| Age at onset | Typically 45-65 years (range 21-85); mean onset age approximately 58 years |
| Sex | Male predominance with a ratio of 1.3-1.6:1 |
| Diagnostic delay | Average 3-6 years from symptom onset; approximately 50% receive an initial psychiatric diagnosis[^6] |
| Proportion of FTD | Approximately 60% of all FTD cases |
| Familial proportion | Approximately 40% have a positive family history |
| Salience network | Centered on the anterior cingulate cortex and frontoinsular cortex; its disruption explains disinhibition, impaired social cognition, and emotional blunting |
| Default mode network | Reciprocal dysregulation with the salience network; paradoxically preserved or increased early in bvFTD |
| Reward and motivation circuits | Ventromedial prefrontal and orbitofrontal cortex dysfunction explains apathy and dietary changes |
| Theory of mind network | Medial prefrontal and temporal pole disruption underlies loss of empathy |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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