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Updated: Jul 27 2020

Frontotemporal Dementia (Pick Disease)

  • Snapshot
    • A 58-year-old male is brought to the physician by his daughter for "strange" behavior. He has been innapropriately making sexual remarks, invading people's personal space, and urinating in public. She has noticed that he has been binge eating sweets as of late. His manners and social abilities have drastically worsened. On physical exam, there is a deficit in executive functioning and working memory. Visuospatial function is intact.
  • Introduction
    • Neurodegenerative disorder affecting the frontal and/or temporal lobes
      • dementia
      • behavioral decline and changes in personality
      • aphasia
    • There are different variants
      • behavioral variant
      • primary progressive aphasia
    • Pick bodies
      • hyperphosphorylated tau inclusion bodies
        • ballooned neurons
  • Presentation
    • Behavioral variant
      • disinhibition
        • presents early
      • hyperorality
      • apathy
      • compulsiveness
    • Primary progressive aphasia
      • language deficit (aphasia) early in disease
    • Memory deficits present later
    • Visuospatial function is usually intact
    • Primitive reflexes
      • relatively early than other dementias
  • Evaluation
    • Clinical diagnosis
    • Histologic findings
      • Pick bodies
        • round intracellular aggregates of hyperphosphorylated tau proteins
          • same protein seen in Alzheimer's disease but results in tangles NOT round aggregates
    • MRI
      • atrophy of the frontal and/or anterior temporal lobes
  • Differential
    • Alzheimer's disease
    • Parkinson's disease
    • Huntington's disease
  • Treatment
    • No medications have been shown to be disease modifying
      • treatment is based on symptoms
        • e.g., SSRIs for behavioral symptoms
  • Prognosis, Prevention, Complications
    • Prognosis
      • progressive symptom deterioration
        • worsening disinhibition and personality changes
        • impairment in activities in daily living
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