Which of the following is not consistent with a diagnosis of frontotemporal lobar degeneration?
In frontotemporal lobar degeneration there is selective atrophy of the frontal and temporal lobes.
Neurofibrillary tangles5%Pick bodies10%Pronounced parietal lobe atrophy64%Gliosis11%Senile plaques9%
In frontotemporal lobar degeneration there is selective atrophy of the frontal and temporal lobes.
Frontotemporal lobar degeneration
Frontotemporal lobar degeneration is the third most common type of cortical dementia after Alzheimer's and Lewy body dementia.
There are three recognised types of FTLD
- Frontotemporal dementia (Pick's)
- Progressive non fluent aphasia (chronic progressive aphasia, CPA)
- Semantic dementia
| Common features of frontotemporal lobar dementias |
|---|
| Onset before 65 |
| Insidious onset |
| Relatively preserved memory and visuospatial skills |
| Personality change and social conduct problems |
Pick's
This is the most common type and is characterised by personality change and impaired social conduct. Other common features include hyperorality, disinhibition, increased appetite, and perseveration behaviours.
Focal gyral atrophy with a knife-blade appearance is characteristic of Pick's disease. The term kinfe-blade is used to describe the gyri that are thin and narrow and resemble knives.
CPA
Here the chief factor is non fluent speech. They make short utterances that are agrammatic. Comprehension is relatively preserved.
Semantic dementia
Here the patient has a fluent progressive aphasia. The speech is fluent but empty and conveys little meaning. Unlike in Alzheimer's memory is better for recent rather than remote events.
Pathological changes seen in FTLD
Macroscopic changes seen in Frontotemporal lobar degeneration include:-
- Atrophy of the frontal and temporal lobes
Microscopic changes include:-
- Pick bodies - spherical aggregations of tau protein
- Gliosis
- Neurofibrillary tangles
- Senile plaques
