Frontotemporal Dementia

Question 1

What’s the percentage of FTD’s been caused by abnormal accumulation of tau protein

Answer 1

40%

Question 2

Are all FTD cases tau positive?

Answer 2

No, more than half of the cases are TAU negative

Question 3

For cases that are TAUnegative, FTD is caused by abnormal accumulation of what

Answer 3

TDP 43 protein

Question 4

Name two contributions that lead to frontal temporal dementia

Answer 4

TAU abnormal accumulation

TDP 43 Abnormal accumulation

Question 5

Percentage of people with frontal temporal dementia having a family history of dementia

Answer 5

40%

Question 6

What age does cognitive and behavioral symptoms start emerging in frontotemporal dementia

Answer 6

40 to 50

Question 7

How many variants are there in frontal temporal dementia

Answer 7

Behavioral variant
Language variant
Motor variant

Question 8

What is another term for frontal temporal dementia behavior variant

Answer 8

Picks disease

Question 9

What happens in behavioral variant of FTD

Answer 9

Initial changes in social behavior, personality.

Progressive for focal de generation of frontal temporal brain regions

Presence of argyrophilic globular Inclusions (picks bodies)
Swollen acrobatic cells known as picks cells

Question 10

Common locations in the brain that are affected by picks disease

Answer 10

Frontal temporal neo cortex
Amygdala
Dentate gyrus
Piramidal cells of CA one section of hippocampus
Hypothalamic lateral to pronucleus
Dorsal medial region of putamen
Globus pallidus
Lucas cerulus

Question 11

50% of patients with picks present with greater left or right hemisphere involvement

Answer 11

Left Hemisphere involvement is greater than right hemisphere involvement

Right hemisphere in five minutes about 20%

Question 12

Other neurological contributions for picks disease

Answer 12

Neuro inflammation
Non thyroid Spectrum auto immune disorders
Altered metabolism of serotonin and lower levels of CSF dopamine

Question 13

Most common variant of FTD

Answer 13

Behavior variant accounts for half

Question 14

Age and gender

Answer 14

Average age 54 years,

onset between 40 to 65,

Onset is very rare after age 75

Median survival rate from 3 to 8 years

Question 15

Onset of FTD behavior variant

Answer 15

Insidious onset
2 subtypes

Apathy type
Disinhibited type

Question 16

Other conditions to be considered

Answer 16

OCD, ritualistic behaviors, psychosis could be mistaken for schizophrenia. Hallucinations and delusions are not common in behavior FTD.

Question 17

Diagnostic criteria using what language for probabilistic diagnosis

Answer 17

Possible probable definite

Question 18

Probable diagnosis requires confirmed neural imaging, functional decline, and all criteria met for possible

Question 19

Definite diagnosis of FTD behavior in variant

Answer 19

Probable diagnosis also with histopathological evidence on biopsy or post Mortem exam or non-genetic mutation

Question 20

Behavioral variant frontal temporal dementia phenocopy syndrome

Answer 20

Does not progressed to dementia
Nonprogressive neural imaging results
No atrophy, normal scans

Question 21

Neuropsychological findings with behavior variant FTD

Answer 21

Mostly everything is preserved early in the disease process except some deficits in executive functions in episodic memory.

Cognitive screening is typically insensitive to deficits early in the course.

Question 22

Medication for behavior variant frontal temporal dementia

Answer 22

Acetylcholinesterase inhibitor’s typically prescribed for Alzheimer’s, do not work for behavior variant FTD

SSRI, SNRI are used for emotional symptoms

Question 23

Frontal temporal language variant

Answer 23

Also known as primary progressive aphasia PPA
Three subtypes

Nonfluent agrammatic variant

Semantic dementia

Logopenic variant

Question 24

PPA Characteristics

Answer 24

Initial and prominent language deterioration with relative preservation of other cognitive abilities until late

Marked word finding difficulties
Decreased performance on worthless generation
Problems with verbal comprehension
Dysarthria
Reading and writing abilities typically remain preserved longer than speech
Swallowing problems

Question 25

Semantic dementia characteristics

Answer 25

Naming deficits with loss of single word knowledge and comprehension

loss of person recognition
Surface dyslexia or dysgraphia

Repetition OK, speech production OK

Question 26

PPA nonfluent agrramatic variant

Answer 26

Wrong grammar in language production
effortful halting speech
impaired comprehension of complex sentences

Single word comprehension OK
object knowledge OK

Question 27

Logo penic Variant

Answer 27

Impaired single word retrieval in speech and naming
Impaired repetition

Motor speech OK grammar OK

Question 28

Neuropathology of PPA non fluent type

Answer 28

Deterioration in the left posterior frontal and insular regions associated with nonfluent forms of the disease

Strongly associated with TAU pathology
Predominant left posterior frontal insular pathology

Question 29

Neuropathology of PPA, semantic dementia

Answer 29

Bilateral anterior temporal region
Most present with left hemisphere atrophy, 25% have right atrophy

ALL semantic dementia cases are caused by abnormal accumulation of TDP 43

 Least likely to be inherited

Question 30

Neuropathology of PPA- logopenic variant

Answer 30

Left temporal parietal

Question 31

Epidemiology of PPA

Answer 31

20 to 25% of people have semantic dementia

males more likely to have semantic dementia,

females more likely to have non-fluent in variant

There is more non-right handedness in semantic dementia

Question 32

Average length of time for months at a death in language variant FTD

Answer 32

12 years

Question 33

How to differentiate between nonfluent PPA and semantic dementia

Answer 33

Nonfluent PPA are known to have more problems with word list generation

Semantic dementia patients have extremely poor confrontation naming. Deficits in object knowledge begin with loss of the ability to distinguish between types of object and progressed to losing knowledge of the word