Frontotemporal Dementia

Question 1

What are the three major symptoms of FTD?

Answer 1

1. ) Behavioural
2. ) Cognitive i.e language + exec. Memory spared
3. ) Motor i.e subgroup of parkinsonism

Question 2

What are the two variants of FTD?

Answer 2

Behavioural vs language

Question 3

What sort of behavioural symptoms are there?

Answer 3

Disinhibition, stereotypy, apathy, hallucinations, sweet cravings

Question 4

What is associated with disinhibition?

Answer 4

Increased creativity

Question 5

What is disinhibiton?

Answer 5

Disregard for social convention i.e say inappropriate things

Question 6

How is the behavioural variant of FTD diagnosed?

Answer 6

1. ) progressive deterioration of behaviour/cognition
2. ) 3/6 of the following at least: disinhibition, apathy, loss of empathy, stereotypy, hyperorality, executive dysfunction
3. )Imaging: frontal/temporal atrophy
4. ) Exclusion: psychiatric/AD/other non-degenerative

Question 7

What is stereotypy?

Answer 7

Persistent repetition of an act

Question 8

When does FTD onset?

Answer 8

<65

Question 9

Whats FTD incidence?

Answer 9

15/100000 (45-65)

Question 10

What happens to FTD eventually?

Answer 10

Progresses to a general dementia

Question 11

Whats the survival time of FTD

Answer 11

1-10years variable

Question 12

Whats FTD commonly misdiagnosed as?

Answer 12

Bipolar
Depression
Schizophrenia

Question 13

Whats the pathology to the gross anatomy?

Answer 13

Progressive Atrophy of the frontal and/or anterior temporal atrophy +/- other regions (variable)

Question 14

On the microscopic level what are the pathologies?

Answer 14

Abnormaly protein inclusions inside neurons and glia including Tau (40%) and TDP43

Question 15

Is FTD genetic?

Answer 15

Yes 25-50% familial

Autosomal dominant complete penetrance

Question 16

What are common mutations resulting in FTD?

Answer 16

MAPT, GRN, C9orf72 (80%)

Question 17

What protein accumulations are cused by which genes?

Answer 17

Tau = MAPT

TDP43 = GRN, C9orf72

Question 18

Whats a problem of clinical diagnosis of FTD?

Answer 18

usually significant atrophy has occurred when diagnosis can be made

Question 19

What do the current treatments resolve?

Answer 19

Symptoms only

Do not halt disease progression

Question 20

What are the sorts of current treatment?

Answer 20

• Movement: parkinsonism
  
  • Psychiatric e.g. depression, anxiety
  • NMDA antag/AchE inhib: not helpful

Question 21

Whats needed to FTD?

Answer 21

The identification of FTD

Question 22

Why biomarkers important?

Answer 22

Measurable
Indicate presence/absence of disease
Preferably non-invasive
For example: genes, molecules, characteristics, brain activity

Question 23

Whats a biomarker currently used?

Answer 23

IVS 10+16 (gene)
Biomarkers in:
- blood
-Neuropsych
-olfaciton
-MRI

Question 24

How many types of clumped protein will individuals have?

Answer 24

Only one type