Definition

[1]
Fronto-temporal Dementia (also Frontotemporal Dementia) is a disease causing cognitive decline that is characterised by early changes in behaviour, personality, mood and language.

Prevalence

[2]
2nd most common dementia. 15 per 100 000 people

Age

55-65

Gender

No difference between male and female

Risk Factors

[3]
  • Family history of Fronto-temporal dementia

Signs and Symptoms

[4]
  • Changes in behaviour, mood and personality
    • Disinhibition
    • Impulsivity
    • Apathy
    • OCD traits
  • Agitation
  • Confusion
  • Memory loss
  • Self neglect

Pathophysiology

[5]
Fronto-temporal Dementia on histological inspection oftens hows intra-cellular inclusions composed of the microtubule protein tau. Other brains have intra-nuclear inclusions that are immunoreactive to ubiquitin but are tau negative. The underlying pathogenesis of the disease is poorly understood but thought to result from mutations in the MAPT or PGRN genes.

Investigations

[6]

Bloods

Delirium screen

Imaging

  • Brain CT - focal frontal and temporal lobe atrophy
  • Brain MRI - focal frontal and temporal lobe atrophy
  • 18-FDG PET - hypometabolism in the frontal and/or anterior temporal lobes

Other

  • Neuropsychological assessment - Frontal Assessment Battery
  • Genetic testing - may show MAPT or PGRN mutation.

Diagnosis

[7]
Diagnosis of Fronto-temporal Dementia should be made following longitudinal assessment by a neuropsychiatrist or neurologist.

Differentials to rule out

Treatment

[8]

Non-Pharmacological

  • Supportive care

Pharmacological

Management

[9]
Behavioural evaluations should be made every 6-12 months. It is also important to assess carer burden and discuss full time care at nursing home facilities.

Complications

[10]
  • Behavioural changes (may affect driving, adherence to laws and finances)

Prognosis

[11]
There is no cure and the median survival time following onset of symptoms is about 7 years.

See Also

Dementia
BMJ Best Practice - Frontotemporal Dementia
  1. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  2. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  3. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  4. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  5. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  6. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  7. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  8. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  9. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  10. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html
  11. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/968.html