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Snapshot
  • A 59-year-old male is brought to the physician by his wife due to "odd behavior." She reports that he would attempt to kiss strangers, or would urinate in public. At times, he would grow distant and show no interest in maintaining a conversation. She has also noticed that he has been frequently binge eating sweets. He does not have insight into his symptoms. On exam, the patient displays disinhibition. There is impairment in executive functioning with intact visuospatial functioning.
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
    • hyperorality
    • apathy
    • compulsiveness
  • Primary progressive aphasia
    • language deficit (aphasia) early in disease
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
Differential
  • Alzheimer's disease
  • Parkinson's disease
  • Huntington's disease
Treatment
  • Symptomatic management
Prognosis, Prevention, Complications
  • Prognosis
    • progressive symptom deterioration
      • worsening disinhibition and personality changes
      • impairment in activities in daily living
 

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