Frontotemporal Dementia (FTD)

Question 1

How common

Answer 1

4th most common dementia

Question 2

Onset

Answer 2

Between 50-60 years old
Mean onset is 59

Question 3

Risk factors

Answer 3

Genetic factors
Presence of ubiquitin

Question 4

Pathology

Answer 4

Inclusion bodies
Regional cortical atrophy
Degeneration of some aspect of the frontal/temporal lobes
Behavioral Variant FTD (bvFTD)
Primary progressive aphasia (PPA) or language variant FTD

Question 5

Inclusion bodies

Answer 5

Accumulations of protein inside cells that are markers of disease
Tau inclusions
Ubiquitin inclusions

Question 6

Regional cortical atrophy

Answer 6

Predominantly in frontal and/or temporal lobes

Question 7

Behavioral Variant FTD (bvFTD)

Answer 7

Atrophy, neuronal loss of frontal cortex
Tauopathy with tau-positive inclusion

Question 8

Primary progressive aphasia (PPA) or language variant FTD

Answer 8

Nonfluent/agrammatic PPA
Semantic variant PPA
Logopenic PPA

Question 9

Nonfluent/agrammatic PPA

Answer 9

Frontal and basal ganglia atrophy (tau)
Characterized by speech and grammar difficulties
Knowledge of word meaning are intact

Question 10

Semantic variant PPA

Answer 10

Temporal atrophy (ubiquitin positive)
Loss of comprehension with fluent speech
Difficulties understanding what others are saying and recalling the meaning of words

Question 11

Logopenic PPA

Answer 11

Temporal and parietal atrophy (usually AD pathology; plaques and tangles)
Word-finding difficulties

Question 12

Frontal/Behavioral Variant FTD - Behavioral presentation

Answer 12

Personality changes consistent with frontal systems dysfunction
Loss of social awareness, apathy, social withdrawal, decreased personal hygiene, social disinhibition, impulsivity, perseveration
Decreased insight

Question 13

Frontal/Behavioral Variant FTD - Neuropsychological performance

Answer 13

Executive dysfunction greater than memory dysfunction
Language deficits (fluency, naming)
Intact visuospatial skills and orientation