Frontotemporal lobar degeneration

  • 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
  • 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
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Möller, Christiane, et al. “Alzheimer disease and behavioral variant frontotemporal dementia: automatic classification based on cortical atrophy for single-subject diagnosis.” Radiology 279.3 (2016): 838-848.

Matsuo, Koushun, et al. “Cerebral white matter damage in frontotemporal dementia assessed by diffusion tensor tractography.” Neuroradiology 50.7 (2008): 605-611.