diagnostic criteria for frontotemporal dementia

Introduction

Essential features:

• insidious onset & gradual progression
• early decline in social interpersonal conduct
• early impairment in regulation of personal conduct
• early emotional blunting
• early loss of insight

Supportive features:

• behavior
  • decline in personal hygiene & grooming
  • mental rigidity & inflexibility
  • distractability & impersistence
  • hyperoral behavior & dietary changes
  • perseveration & stereotypy
• speech & language
  • lack of spontaniety & economy of speech
  • echolalia
  • mutism
• signs
  • primitive reflexes (frontal release signs)
  • incontinence
  • akinesia, rigidity & tremor
  • low & labile blood pressure
• neuropsychiatric testing
  • impairment on frontal lobe tests
  • absence of:
    • severe amnesia
    • aphasia
    • perceptuospatial disorder
• normal conventional EEG despite dementia
• MRI or PET show predominant frontal &/or anterior temporal lobe abnormality
• onset before age 65
• positive family history in a 1st degree relative
• associated motor neuron disease (minority of patients)
  • bulbar palsy
  • muscle atrophy
  • fasciculations

Notes

• performance of MMSE may be near normal

More general terms

• criteria

Additional terms

• frontotemporal dementia; frontotemporal lobar degeneration; frontotemporal neurocognitive disorder (FTD, FTLD)