Protein pathology FTD

Question 1

Neurological damage

Answer 1

Damage caused by abnormal aggregation of proteins:

• Tau
• TARDNA binding protein (TDP-43)
• fused in sarcoma (FUS) protein

To diagnose any variant of FTD, there must be;
- atrophy in the frontal and anterior temporal regions
- presence of abnormal protein aggregation in neurons (only possible post-mortem)

Question 2

Link between variant and protein

Answer 2

TDP-43
- ALS
- FTD-MND
- bvFTD
- nfvFTD

FUS
- bvFTD

TAU
- bvFTD
- nfvFTD
- CBS
- PSP

lvPPA – linked with AD pathology (TAU)

Question 3

Later stages of FTD

Answer 3

Differences between variants becomes less clear
- Greater range of symptoms as the disease spreads to other brain areas

• bvFTD may also develop language problems
• language variants may develop changes in behaviour and personality.
• Existing symptoms become worse
• symptoms become similar to the later stages of other types of dementia, e.g. AD

Question 4

Life expectancy after diagnosis

Answer 4

Meta analysis:

Mean survival duration in years
 bvFTD 8.17
 nfvFTD 8.11
 svFTD 7.4
 PSP 6.38
 CBD 6.40
 AD 6.86
 FTD-ALS 2.50

Question 5

Assessment bvFTD

Answer 5

Main difference w AD is episodic memory (relatively spared in bvFTD)

Quite often bvFTD is misdiagnosed;
- AD; symptom and lesion location overlap
- psych disorder (depression/schizo)

Also bc the neuropsychological profile varies a lot, might be due to lack of motivation/behavioural changes as well)