Frontotemporal Degeneration Flashcards
definition of frontotemporal dementia
characterised by progressive degeneration of the frontal and/or temporal lobe
- sparing of parietal and occipital lobe
- degeneration of t
he striatum
three main types of Frontotemporal lobar degeneration
- Frontotemporal dementia - pick’s disease
- Progressive non fluent aphasia - slow onset of language deficit overtime / difficulty with speech production
- Semantic dementia - progressive neurological disorder characterized by loss of semantic memory (
Presenting picture in Pick’s disease?
- personality change and social conduction problems - apathy, social inhibition and sexual inhibition
- increased appetite
macroscopic changes of Pick’s disease
- atrophy of frontal and temporal lobes
microscopic changes of Pick’s disease
- Pick bodies - spherical aggregations of tau protein
- Gliosis - non specific reactive change of glial cells in response to cns damage
- Neurofibrillary tangles
- Senile plaques - microscopic mass of fragmented and decaying nerve terminals
management of frontotemporal dementia
NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia
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
What is preserved in frontotemporal lobar dementia?
Relatively preserved memory and visuospatial skills
Describe the onset of frontotemporal lobar dementia
Insidious onset
Age of onset of fronto temporal dementia?
Onset before 65
Main feature of frontotemporal lobar dementia
Personality change and social conduct problems
What are pick’s bodies
spherical aggregations of tau protein (silver-staining)