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- Tau protein or progranulin mutation
- Language and behavioral symptoms
- 5-10% of all dementia
- Autosomal dominant, Chr. 17
Lund-Manchester criteria (3 main subtypes)
- Behavioral variant frontotemporal dementia
- Progressive non-fluent aphasia
- Semantic dementia
Imaging
- Asymmetrical atrophy of frontal and temporal lobes
- Semantic dementia: marked left temporal lobe atrophy (temporal pole > hippocampus)
- Behavioral variant: frontal lobe atrophy is often mild at time of diagnosis