Frontotemporal dementia

• What is frontotemporal dementia?

  Heterogeneous group of neurodegenerative disorders

  Characterised by progressive atrophy of frontal and/or temporal lobes of the brain -> impairments in behaviour, personality, language & motor functions
• Frontotemporal dementia is the 2nd most common cause of dementia in pts under 65.
• What are the RFs for frontotemporal dementia?

  Genetic mutations

  • MAPT → tau inclusion bodies
  • PGRN → TDP-43 inclusion bodies
  • C9orf72
• What is the classification of frontotemporal dementia?

  3 primary clinical syndromes

  Behavioural variant frontotemporal dementia (bvFTD)

  Semantic dementia (SD)

  Progressive nonfluent aphasia (PNFA)
• What is behavioural variant FTD?

  Most common form of frontotemporal dementia

  Changes in personality & behaviour (inc. disinhibition, apathy, loss of empathy or compulsive behaviours)

  Imaging = frontal & anterior temporal lobe atrophy
• What is semantic dementia?

  Also known as semantic variant primary progressive aphasia

  Presentation = loss of semantic knowledge (difficulty understanding words or recognising familiar people/objects)

  Imaging = selective left anterior temporal lobe atrophy
• What is progressive nonfluent aphasia?

  Progressive language disorder with non-fluent speech & grammatical errors

  May also be signs of agrammatism or apraxia of speech

  Imaging = left perisylvian or insular atrophy
• What are the signs & symptoms of frontotemporal dementia?

  Behavioural changes

  • early apathy
  • disinhibition
  • loss of empathy
  • decline in hygiene
  • primitive reflexes

  Difficulties with speech (predominant feature)

  • aphasia
  • decrease in speed of speech
  • difficulties with comprehension

  Decline in executive functioning & cognition

  • difficulty planning
  • poor conc

  Memory problems

  • short term memory
  • difficulty with recall

  Some pts may develop features of motor neuron disease (muscle weakness & dysarthria)
• What are the DDx of frontotemporal dementia?

  Alzheimer's disease

  Vascular dementia

  Primary progressive aphasia

  Dementia with Lewy bodies

  Depression
• What are the Inx for frontotemporal dementia?

  Full Hx (inc. collateral history)

  Formal cognitive screen (MMSE)

  Full neuro exam

  Urinalysis

  BM

  Bloods (cognitive screen (FBC, U&Es, LFTs, CRP/ESR, TFTs, B12, folate, syphilis, HIV))

  MRI/CT
• What is the management of frontotemporal dementia?

  Patient, family & carer education

  Physio & occupational therapy

  SALT input

  Group therapy & support groups

  Meds

  • SSRIs
  • antipsychotics