3b. Frontal Lobe Dementia (cortical dementia) Flashcards
What is FTD? And what other name does it have?
Frontal Lobe Dementia / Pick’s disease = a form of dementia with behavioral changes, more often in younger people.
Which 2 types of FTD can be distinguished?
And in the 2nd one, which 3 types can be distinguished?
- Frontotemporal dementia - behavioral variant (FTDbv)
- Primary progressive aphasia (PPA)
- Progressive nonfluent/agrammatic aphasia (naPPA)
- Semantic dementia (SD)
- Logopenic progressive aphasia (LPA)
FTDbv
- Distrubution men/women
- Which gene is mutated
- Link with which disease?
- Evenly distributed aoround men and women
- Mutation on the FTDP gene
- Link between FTD and ALS
FTDbv
What is the most prominent feature of criteria?
There has to be progressive deterioration of behavior and/or cognition.
Possible FTDbv
3 symptoms needed of 6 symptoms…
- Early behavioral disinhibition
- Early apathy or inertia
- Early loss of sympathy or empathy
- Early perseverative, stereotyped, or compulsive/rituatlistic behavior
- Hyperorality and/or dietary changes
- NPA: EF deficits with sparing of other domains
Probable FTDbv
We are more sure when… (2x)
- There is a significant functional decline
- If there are imaging results consistent with FTDbv
Which 2?
- Frontal and/or anterior temporal atrophy (MRI/CT)
- Frontal/anterior temporal hypoperfusion or hypometabolism (PET/SPECT)
Definite FTDbv
We are most sure when… (2x)
- There is also histopathological evidence (biopsy or postmortem)
- There is a pathogenic mutation
FTDbv diagnostics
Which 2 domains are mostly affected in a NPA?
And which 1 domain is relatively intact?
- EF: disinhibition, deficits in planning, perseveration
- Visuoconstruction: perseveration
- Memory: relatively intact
FTDbv diagnostics
What can be seen on MRI?
Especially tissue loss in the frontal and temporal parts of the brain. The brain almost looks like a walnut (gyri are much smaller)
FTDbv diagnostics
What can be seen on SPECT?
In AD there is less activity (less red) in the back of the brain, in FTDbv there is especially less activity in the front of the brain.
FTDbv diagnostics
What are 4 histopathologic changes in FTDbv?
- Microvacuolation in the upper layers: white looking compartments filled with water-containing molecules.
- Ubiquitin inclusions: also abnormal material in the brain cells.
- Pick’s bodies: accumulation of tau proteins (hallmark of FTD).
- Neurofibrillary tangles: also from the tau protein.
What are 3 criteria’s of PPA?
- Difficulty in language as most prominent clinical feature.
- It causes impairment in daily functioning.
- Aphasia is the most prominent deficit at onset and initial phase.
PPA-non fluent
Which 3 symptoms?
And which 2 domains are spared?
Symptoms
- Agrammatism: difficulties with grammer
- Effortful, halting speech: slow and with a lot of hesitation
- Impaired comprehension of complex sentences
Spared
- Single-word comprehension
- Spared object knowledge
Semantic dementia
Which 4 symptoms?
And which 2 domains are spared?
Symptoms
- Difficulties with confrontation naming
- Difficulties with single-word comprehension
- Impaired object knowledge: memory of objects is gone
- Surface dyslexia/dysgraphia: problems with reading/writing
Spared
- Repetition: they can repeat things
- Speech production: grammar and motor speech is in touch
Logopenic PA
Which 3 symptoms?
And which 3 domains are spared?
(impairments in naming and sentence repetition)
Symptoms
- Impaired single-word retrieval in spontaneous speech, and naming
- Impaired repetition of sentences and names
- Phonologic speech errors (=klanken)
Spared
- Single-word comprehension
- Object knowledge
- Motor speech
