bvFTD

Question 1

What are the main pathologies of bvFTD?

Answer 1

1. Tauopathy
2. TDP-43
3. FUS/FET

Question 2

What are the diagnostic criteria for bvFTD?

Answer 2

Must have progressive deterioration in behaviour +/- cognition

Possible 3/6 of:
1. Early behavioural disinhibition
2. Early apathy/inertia
3. Early loss of sympathy/empathy
4. Early perseveration, stereotyped or compulsive/ritualistic behaviours
5. Hyperorality/dietary changes
6. Neuropsych - deficit in executive, sparing of episodic memory and visuospatial skills

Probable:
1. Meet criteria for possible
2. Significant functional decline
3. Imaging shows - frontal +/- anterior temporal atrophy/hypoperfusion/hypometabolism on MRI/CT/PET/SPECT

Question 3

What motor symptoms are associated with bvFTD?

Answer 3

1. Parkinsonsim
2. UMN or LMN signs

Question 4

What are pharmacologic tx options for bvFTD?

Answer 4

1. Disinhibition - SSRI, trazodone, atypical antipsychotics
2. Apathy - none
3. Sympathy - none
4. Perseveration - SSRI, trazodone
5. Hyperorality - SSRI, trazodone
6. Neuropsych - none, no role for cholinesterase inhibitor

Question 5

What neurotransmitters are involved in FTD?

Answer 5

Serotonin
Dopamine
Glutamate
GABA

Question 6

What are limitations to screening tools in FTD?

Answer 6

1. Screening tools lack test of frontal/executive function and are superficial for language (esp MMSE)
2. No component of personality/behavioural changes
3. Tools not validated in FTD
4. FAB only evaluates executive function
5. Some exec dysfunction in normal aging - overcalled on screening

Question 7

How to differentiate AD with frontal features vs. FTD?

Answer 7

1. AD older age onset, FTD 45-65
2. AD includes memory and visuospatial impairment
3. AD normal neuro exam
4. MRI global/diffuse atrophy in AD, frontotemporal for FTD
5. AD biomarker presence

Question 8

What are assessments for executive function?

Answer 8

1. Trails A/B
2. Abstraction
3. Attention - digit span, serial 7s
4. Proverbs
5. Verbal fluency > animal
6. FAB
7. Clock draw

Question 9

What are pharmacologic tx for sexual disinhibition in FTD and sfx?

Answer 9

1. SSRI - nausea, somnolence, diarrhea, headache
2. Trazodone - sedation, OH, falls, delirium, priapism
3. TCA - dry mouth, blurry vision, urinary retention, CI
4. Antiandrogens (cyproterone, medroxyprogesterone, finasteride) - gynecomastia, galactorrhea, inc blood glucose
5. Estrogens - weight gain, VTE, CV, fluid retention

Question 10

List one gene that is most common in familial FTD and has abnormal movements and late onset psychosis

Answer 10

C9orf72 expansion

Question 10

What 3 diseases are associated with FTD?

Answer 10

FTD-MND (motor neuron disease)
Corticobasal syndrome
PSP