Frontotemporal Dementia

Frontotemporal Dementia Introduction

  • Frontotemporal dementia is the most common form of dementia in patients less than 60 years old
  • Frontotemporal dementia can run in families in approximately 30% of patients
  • On average, it takes three years to make the diagnosis
  • There are no therapies to reverse or stop the progression of the illness
  • Symptoms and behavior can be effectively medically managed
  • The rate of progression of the illness is rapid, but can vary from 2 to 10 years

Frontotemporal Dementia Clinical Features

  • Frontotemporal dementia affects social behavior and ability to think and communicate, but in a small proportion of patients, movement and walking can also be affected
  • There are three clinical subtypes
  • Brain imaging can be helpful in characterizing which subtype of frontotemporal dementia is most likely

Behavioral variant frontotemporal dementia

  • Most common subtype
  • Characterized by changes in behavior
    • Apathy, or lack of engagement, which may look like boredom or laziness
    • Problems with setting and achieving goals
    • Difficulty with making decisions
    • Socially inappropriate behavior like laughing when hearing a sad story or making sexual remarks in public
    • Loss of manners
    • Lack of emotional connection; in other words, loss of a deep emotional understanding of one’s family and friends
    • Lack of empathy which may look like a loss of compassion or inability to be supportive to others
    • Excessively repeating words during conversations
    • Exactly repeating what someone else is saying
    • Repetitive simple behaviors like finger tapping, picking at objects, or pacing
    • Changes in diet including increased appetite or binge eating, especially of sugary foods

Semantic variant primary progressive aphasia

  • Characterized by difficulty with speaking and writing
    • Not knowing the meaning of words
    • Inability to get the point across
    • Not knowing the names of everyday objects like a pen, phone, or pants
    • Normal speech, not stop and go speech
    • Grammatically correct sentences

Non-fluent variant primary progressive aphasia

  • Also characterized by difficulty with speaking and writing, but:
    • Speech is not grammatically correct, like in the following example:
      “Barbara and Joe decide go on a cruises and was a couple.”
  • Stop and go (halting) speech

Frontotemporal Dementia Further Reading

Large study explores age of onset of frontotemporal dementia by genetics, family history | National Institute on Aging (nih.gov)

Frontotemporal dementia | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program (nih.gov)