Frontotemporal Dementia

Question 1

Outline the clinical features of frontotemporal dementia

Answer 1

1) Earliest deficit personality change attributable to loss in social cognition
2) Late deficit in executive function, judgement, problem solving
3) There are 4 variants of frontotemporal dementia; 1 behavioural variant and 3 primary progressive aphasic (PPA) variants
1. behavioural variant FTD (bvFTD) [most common]
2. nonfluent variant PPA
3. semantic variant PPA
4. logopenic variant

Question 2

List the 6 diagnostic features of bvFTD

Answer 2

FTD is a diagnosis by exclusion of other -non-neurodegenrative conditions after which 3/6 of the following will be diagnostic for bvFTD

1) Disinhibition
2) Apathy/inertia
3) Loss of sympathy/empathy
4) Perservative/compulsive behaviours
5) Hyperorality
6) Dysexecutive neuropsychological profile

MMSE

Question 3

Outline the imaging features of frontotemporal dementia

Answer 3

1) CT/MRI brain
- early: unilateral focal frontal and temporal atrophy
- late: bilateral frontal and temporal atrophy

2) PET scan
- hypometabolism and hypoperfusion in frontal and temporal areas

Question 4

Outline the management for frontotemporal dementia

Answer 4

1) No pharmacological treatment is available; as the pathology does not include low ACh levels, cholinesterase inhibitors are not of benefit in FTD
2) Behavioural therapy to address behavioural changes should be provided