Frontotemporal Dementia

Summary

Frontotemporal dementia is a type of cognitive decline, or dementia, caused by the degeneration of the brain’s frontal and temporal lobes. Patients can present with compulsive or inappropriate behavior as well as altered speech. The diagnosis is usually clinical, but after death, autopsy and microscopy of the brain can reveal the presence of pick bodies. These pick bodies are round bodies filled with long tangles of hyperphosphorylated tau proteins.

Key Points

• Frontotemporal Dementia
  • Formerly called Pick disease
  • Pathophysiology
    • Neurodegeneration that begins in the frontal and progresses to temporal lobes
    • Different variants
      • Behavioral
      • Primary progressive aphasia
  • Presentation
    • Compulsive/Inappropriate Behaviors
      • Personality and behavioral changes are common and include disinhibition (compulsive, inappropriate behaviors) and hyperorality
      • Due to loss of frontal lobe function
    • Altered Speech (aphasia)
      • Reduced speech production and repeated phrases
  • Diagnosis
    • Brain Imaging (MRI)
      • Symmetrical atrophy of the frontal and/or anterior temporal lobes
    • Neuropathology/Brain Biopsy
      • Pick Bodies
        • Round intracellular aggregates of hyperphosphorylated tau protein
        • Visualized on silver stain
  • Treatment/Prognosis
    • No effective medications
    • Treatment based on symptoms
      • Eg. SSRIs for behavioral symptoms
    • Progressive symptom deterioration        
      • Worsening disinhibition and personality changes
      • Impairment in activities in daily living