Frontotemporal Dementia (Pick Disease)

MSnapshot

• 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
  • behavioral decline and changes in personality
  • aphasia
• Average onset: 50’s
• There are different variants
  • behavioral variant
  • primary progressive aphasia
• Pick bodies  
  • hyperphosphorylated tau inclusion bodies
• ballooned neurons

Presentation

• Behavioral variant (most common) 
  • behavioral disinhibition
  • hyperorality (putting objects in mouth, excessive eating)
  • apathy
  • compulsive behaviors
  • perseverative behaviors 
• Primary progressive aphasia
  • language deficit (aphasia) early in disease
• Memory and visuospatial function are relatively preserved (unlike many other forms of dementia)

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