Frontotemporal

• Frontotemporal dementia:

  • Less common but responsible for a significant number of diagnoses of dementia in those under 65
  • Tends to affect both sexes equally
  • Great variation in progression but the average life expectancy is 8 years post-diagnosis
  • 3 subtypes: behavioural (pick's disease), semantic and non-fluent
• Aetiology:

  • Neuron damage and death occur in the frontal and temporal lobes
  • Atrophy occurs due to the deposition of abnormal proteins - often tau protein
  • Thought to be a genetic component in about a quarter of cases
• Behavioural presentation (Pick's):

  • Altered emotional responsiveness, apathy, disinhibition, impulsivity
  • Progressive decline noted in interpersonal skills
  • Changes in food preference, more childlike amusements
  • Obsessions and rituals may also be noted
• Semantic presentation:

  • Progressive decline in the understanding of word meanings
  • Speech may be fluent but there is difficulty in the name retrieval and the use of less precise terms
  • Unable to determine the meanings of common words when asked
  • Tends to develop into the inability to recognise objects, or familiar faces (prosopagnosia)
• Non-fluent presentation:

  • Progressive breakdown in the output of language
  • Speech takes effort and is not fluent
  • Speech apraxia (poor articulation) or disorders of speech sound
  • Impaired comprehension of sentences and an impact on literacy skills