Neurosurgery notes/Other specialities/Neurology/Dementia/Frontotemporal lobar degeneration (FTLD)

Frontotemporal lobar degeneration (FTLD)

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Numbers

  • 3rd most common type of cortical dementia after Alzheimer’s and Lewy body dementia.

Classification

  • Frontotemporal dementia (Pick’s disease).
    • Most common type
    • Clinical features
      • Personality change and impaired social conduct.
      • Hyperorality, disinhibition, increased appetite, and perseveration behaviors.
    • Investigation
      • Focal gyral atrophy (“knife-blade” atrophy
      • Localized to frontal and temporal lobes only.
      • Microscopic findings include
        • Pick bodies
        • Gliosis
        • Neurofibrillary tangles
        • Senile plaques
  • Progressive non-fluent aphasia (chronic progressive aphasia).
    • Patients have non-fluent speech, they make short utterances that are agrammatic but comprehension is relatively preserved.
  • Semantic dementia
    • Here the patient has a progressive fluent aphasia but speech lacks content and conveys little meaning.
    • Unlike in Alzheimer’s memory is better for recent rather than remote events.

Clinical features

  • Onset < 65 years,
  • Insidious onset,
  • Relatively preserved memory and visuospatial skills,
  • Personality change and social conduct problems.

Investigation

  • CT shows cortical loss in the frontal and temporal lobes,
  • FDG-PET/CT shows hypometabolism.