Fronto-Temporal Dementias Flashcards Preview

NMH: Module 2 > Fronto-Temporal Dementias > Flashcards

Flashcards in Fronto-Temporal Dementias Deck (19)
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1

Key clinical features of FTDs?

- semantic dementia
- behavioural variant
- progressive non-fluent aphasia

2

Neuropathological features of Pick's Disease?

- Frontal symptoms
- sporadic tauopathy (no MAPT mutation)
- F-T atrophy ("knife edge atrophy")
- tau positive PIck bodies
- marked gliosis and neuronal loss
- balloon neurons

3

No. of isoforms of tau protein?

6 isoforms

4

Isoforms of tau antibodies?

PHF1 antibody ]- general
4R-Tau antibody
3R-Tau antibody]- Pick's bodies

5

Clinical correlate of 3R/4R tauopathy?

AD

and
FTLD w/ 3R and 4R Tau (V337M, R406W)

6

Clinical correlate of 4R tauopathy?

CBD
PSP

and
FLTLD w/ 4R Tau (P301L)

7

Clinical correlate of 3R tauopathy?

Pick's Disease

and
FTLD w/ 3R Tau (K257T, G389R)

8

Spectrum of Tau FTD pathology (+neuropath features): Pick's, CBD and PSP?

Pick's Disease: Pick bodies

CBD: astrocytic plaques

PSP: coiled bodies, tufted astrocytes

9

On what chromosome is the tau gene located

Chr 17

10

Tau -ve, TDP+ve, ubiquitin +ve, neuronal inclusions in FTD?

Progranulin (function currently unknown)

Mutated progranulin (adjacent to MAPT Tau gene)

-> instracytoplasmic and intranuclear inclusions

11

Molecular subtypes of FTD?

- Tauopathies: 3R/4R, 4R, 3R

- Progranulin

- TDP-43

- FUS

- C9orf72`

12

MRI imaging changes in FTDs?

Assymetrical atrophy

13

TDP-43 subtypes?

A- semantic dementia
B- C9orf72
C- progranulin, FTD
D

14

What is FUS regading FTD?

"Fused in Sarcoma"

Characteristic inclusion accounting for some atypical FTD

Ubiquitin +ve

15

What is C9orf72 regarding FTD?

Commonly associated w/ ALS

Ubiquitin +ve
TDP-43 +ve
C9orf72 +ve inclusion in cerebellum (other parts TDP43 -ve)

16

Current classification of FTLD

Tau +ve FTLD
3R tauopathy
4R tauopathy
3R/4R tauopathy

Tau -ve FTLD
TDP-43 +ve, ubiquitin +ve]- FTLD-TDP (type A-D)
TDP-43 -ve, ubiquitin +ve (FTLD-FUS, FTLD-UPS)
TDP-43 -ve, ubiquitin -ve (DLDH)

17

What is DLDH?

Dementia lacking distinctive histology

18

What mutation causes FTLD-UPS?

Mutation in CHMP2B gene

19

Tauopathies and FTLD generally?

Many tauopathies are grouped under the term FTLD (even if they are not strictly FTLD)