CHAPTER 20 - SPECTRUM FRONTOTEMPORAL DEMENTIA

What is the most prevalent form of early-onset dementia

Frontotemporal dementia

what is FTD characterized by

changes in behaviour, language, or motor function, but there is a lot of variation

behavioural variant frontotemporal dementia (bvFTD)

changes in personality, social behaviour, specific cognitive impairments and language impairments

there are three levels diagnostic certainty and 6 clusters of behavioural and cognitive symtpoms.

1) first level: possible bvFTD

2) second level: probably bvFTD

3) third level: defintie bvFTD

name the symptoms necessary for first level

3 of the following

→ early behavioural disinhibition manifesting in socially inappropriate behaviour, loss of manners, or impulsive actions

→ early apathy or inertia

→ early loss of sympathy or empathy

→ early perseverative, stereotyped or compulsive behaviour

→ dietary chanegs or hyperorality

→ impairments in executive function

name the symptoms necessary for second level

→ exhibitis significant functional decline

→ imaging results show frontotemporal atrophy or hypoperfusion or hypometabolism

→ absence of biomarkers indicating AD or another neurodegenerative process

name the symptoms of the third level

→ histopathological of evidence of FTD on biopsy or post-mortem

→ presence of a known pathogenic mutation

the diagnosis of PPA also has three levels of certainty. Diagnosis is a two-step process. Name the two steps

step 1: meets basic criteria for PPA

step 2: being classified into one of the three subtypes

explain the first step for PPA

→ difficulty wiht language

→ impaired activities of daily living

→ aphasia

explain the three subtypes in step 2 of PPA

1. semantic variant (svPPA); difficulty in word understanding and finding

2. non-fluent variant (nfvPPA); speech apraxia, error in speech sounds, and difficulty forming complex sentences

3. logopenic variant (lvPPA); non0fluent speech

facts on FTD

• % with a form of FTD, and % of early-onset FTD

• mean age

• prevalence, incidence

• what is the shortest survival and what is the longest

• men or women?

• 10-20% have a form of FTD, and 75% have early-onset FTD

• mean age is 53 years

• prevalence is 2-3.1 in 100000, incidence is 2.7-4.1 per 100000

• shortest survival is FTD-ALS (3.5 years), and longest is nfvPPA and svPPA (9-12 years)

• equal in men and women

how many patients have a family history of dementia

40-60%

what are the three most common caues of genetic FTD

1) Mutation in MAPT

2) Mutation in GRN genes

3) repeat expansion in the Cgorf72 gene

most common locatons of atrophy in bvFTD

PFC, OFC, ACC, anterior insula and subcortical-limbic structures

most common locatons of atrophy in PPA

left hemisphere

most common locatons of atrophy in svPPA

left anterior and inferior temporal lobe

most common locatons of atrophy in nfvPPA

left inferior frontal gyrus, insular cortex, and premotor and supplementary motor areas

most common locatons of atrophy in lvPPA

posterior, temporal and parietal areas

what are the cognitive impairment in bvFTD overlooked by

1) executive functioning

2) response inhibition, initiation, planning, strategizing and abstraction

3) language

4) social cognition

PPA has some cognitive imapirments that can occur as the disease develops. Name them of svPPA.

deterioration of semantic knowledge, verbal episodic memory, behavioural changes and social cognition

PPA has some cognitive imapirments that can occur as the disease develops. Name them of nfvPPA

cogntive impairments, behavioural problems and impairments in social cognition

PPA has some cognitive imapirments that can occur as the disease develops. Name them of lvPPA

impairments in phonological loop, memory, executive functioning and visuo-construction