Frontotemporal Degeneration

Question 1

definition of frontotemporal dementia

Answer 1

characterised by progressive degeneration of the frontal and/or temporal lobe

• sparing of parietal and occipital lobe
• degeneration of the striatum

Question 2

three main types of Frontotemporal lobar degeneration

Answer 2

1. Frontotemporal dementia - pick’s disease
2. Progressive non fluent aphasia - slow onset of language deficit overtime / difficulty with speech production
3. Semantic dementia - progressive neurological disorder characterized by loss of semantic memory (

Question 3

common features of frontotemporal dementia (pick’s disease)

Answer 3

1. onset before 65
2. insidious onset
3. relatively preserved memory and visuaspatial skills ( cognitive features preserved )
4. personality change and social conduction problems - apathy, social inhibition and sexual inhibition
5. increased appetite

Question 4

macroscopic changes of Pick’s disease

Answer 4

1. atrophy of frontal and temporal lobes

Question 5

microscopic changes of Pick’s disease

Answer 5

1. Pick bodies - spherical aggregations of tau protein
2. Gliosis - non specific reactive change of glial cells in response to cns damage
3. Neurofibrillary tangles
4. Senile plaques - microscopic mass of fragmented and decaying nerve terminals

Question 6

management of frontotemporal dementia

Answer 6

NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia

Question 7

CPA

Answer 7

Here the chief factor is non fluent speech. They make short utterances that are agrammatic. Comprehension is relatively preserved.

Question 8

Semantic dementia

Answer 8

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