Frontotemporal Dementia Flashcards
1
Q
What is FTD
A
- Group of disorders characterized by progressive loss of cells in the frontal and temporal lobes
- Causes deterioration in behaviour, personality, and/or difficulty producing or comprehending language
- It’s the leading type of early onset dementia
- Diagnosis is difficult due to similarity in behavioural changes between FTD and psychiatric disorders
- Takes about 6 years to get a diagnosis
- No treatments just medication that can reduce symptoms
2
Q
Most common age range for FTD
A
- 60% of those diagnosed are diagnosed between 45 and 64
3
Q
What brain regions are involved with FTD and what cognitive functions are involved
A
- Frontal and temporal lobes are involved
- Impacting behaviour, movement, language, and emotion
4
Q
BV-FTD causes problems with what
A
- Changes in personality, behaviour, and judgement
5
Q
What is Primary progressive aphasia (PPA) - Language variant of FTD
A
- Type of frontal temporal disorder that makes it difficult to use language, read, write, and understanding
- Language dysfuntion is the main symptom for the first 2 years of the illness
- deficits include language production, object naming, syntax, or word comprehension
- Takes two forms
- Non fluent PPA: Speaking is hesistant, labored, or ungrammatical
- Semantic variant: lose ability to understand or formulate words
6
Q
What causes FTD
A
- Build up of proteins called tau and TDP-43 in nerve cells in the brain
7
Q
FTD Progression
A
- Primary symptoms are often speech and movement
- Continually progresses, but rate of decline varies between people
- People often show progressive muscle weakness and coordination problems
- Average life expectancy is 7-13 years after the start of symptoms
8
Q
Risk factors for FTD
A
- 1/3 of cases are assocaited with genetics but there are no known risk factors for FTD
- At least 8 genes including some with rare mutations
- The behavioural variant of FTD is most often inherited; semantic FTD is only rarely inherited
9
Q
Tau Protein
A
- Protein that is normally present and stabilizes neurons
- Forms tangles inside neurons and leads to destruction of brain cells
10
Q
TDP-43
A
- Involved with gene expression and RNA/DNA processing and metabolism
- Become mislocated from nucleus to cytoplasm and forms inclusions interfering with cell functions
11
Q
Causes of FTD
A
- Loss of neurons and abnormal amounts of proteins called Tau and TDP-43
- Proteins are different then the ones involved in AD, but function in a similar way
12
Q
Behavioural Variant FTD
A
- Progressive atrophy of cell loss in frontal and anterior temporal lobes
- The most common variant of FTD and includes changes in personality, behaviour, and judgement
- Includes: lack of motivation, disinterested in activities/people, disinhibition/impulsivity/problems planning
- Problems with cognition may present but memory stays intact
- Over time language and or movement problems may occur
13
Q
Brain changes with FTD
A
- Typical changes include atrophy of frontal lobes and anterior temporal (temporal tends to be to a lesser degree)
- Degree can be asymmetric (typically worse on right)
14
Q
Brain changes in non-fluent PPAt (nfvPPA)
A
- Atrophy is more variable, typically in posterior frontal, and temporal lobe
- Generally left hemisphere is thought to be most affected but not universal
- The brain has a hard time creating speech and understanding sentences
- Posterior frontal and temporal lobes are impacted
15
Q
Brain changes in semantic variant PPA (svPPA)
A
- Left hemisphere (anterior) temporal lobe atrophy
- More anterior than posterior
- Includes many areas including, amygdala, hippocampus, fusiform gyrus
- The more it spreads the more complicated it gets
16
Q
Motor Disturbances in FTD
A
- About 1/10 people with FTD develop a motor neuron disease, though more common in behavioural variant and less common in non-fluent PPA
- Three main movement/muscle function disorders in both types of FTD
1. ALS: Muscle weakness/wasting
2. Corticobasal syndrome: Arms and legs become uncoordinated and/or stiff
3. Palsy (PSP): Muscle stiffness, difficulty walking, changes in posture, with changes in eye movements also observed
17
Q
Three subtypes of FTD and where do they begin
A
- Behavioural varient frontotemporal dementia (bvFTD): starts in frontotemporal lobes and causes behavioural changes
- Semantic variant primary progressive aphasia (svPPA): starts in temporal lobes and causes changes in using and understanding words
- Nonfluent primary progressive aphasia (nfvPPA): starts in left posterior frontal lobe and surronding brain areas and causes troubles with pronouncing words
