Frontotemporal lobar degeneration (FTLD)

Question 1

What is frontotemporal lobar degeneration (FTLD)?

Answer 1

FTLD is the third most common type of cortical dementia, after Alzheimer’s and Lewy body dementia. It includes three recognised types:
- Frontotemporal dementia (Pick’s disease)
- Progressive non-fluent aphasia (chronic progressive aphasia, CPA)
- Semantic dementia

Question 2

What are common features of frontotemporal lobar dementias (FTLD)?

Answer 2

• Onset before 65
• Insidious onset
• Relatively preserved memory and visuospatial skills
• Personality change and social conduct problems

Question 3

What are the characteristics of Pick’s disease?

Answer 3

• Personality change and impaired social conduct
• Common features include hyperorality, disinhibition, increased appetite, and perseveration behaviors
• Focal gyral atrophy with a knife-blade appearance

Question 4

What are the macroscopic and microscopic changes seen in Pick’s disease?

Answer 4

Macroscopic changes:
Atrophy of the frontal and temporal lobes

Microscopic changes:
- Pick bodies (spherical aggregations of tau protein, silver-staining)
- Gliosis
- Neurofibrillary tangles
- Senile plaques

Question 5

How is Pick’s disease managed?

Answer 5

NICE do not recommend using acetylcholinesterase inhibitors (AChE inhibitors) or memantine in people with frontotemporal dementia.

Question 6

What is characteristic of Progressive Non-Fluent Aphasia (CPA)?

Answer 6

The chief feature of CPA is non-fluent speech. Patients make short, agrammatic utterances. Comprehension is relatively preserved.

Question 7

What is semantic dementia?

Answer 7

Semantic dementia is characterized by fluent but empty speech that conveys little meaning. Unlike Alzheimer’s, memory for recent events is better than for remote events.