Frontotemporal dementia Flashcards
FTD
- Pick’s disease
- Primary progressive aphasia
- semantic dementia
- corticobasal degeneration
Ages
- FTD affects younger populations
- onset between 40-75
- 20% presenile cases of dementia
Genetics
-linked to chromosome 17
Early clinical features
- changes in personality
- changes in behaviour
- behaviour rigidity, impulsivity, emotional lability, fatuousness, executive dysfunction and hyperorality
Memory
- affected later
- less severely affected
Spatial orientation
-well preserved
Insight
-lost early
Brain changes
- asymmetircal focal atrophy of the frontotemporal regions
- underlying neuronal loss, gliosis and subsequent spongiform change in the affected cortices
FTD and MND
-10% of patients with MND show features of dementia
Treatment
- SSRIs may help with behaviour
- acetylcholinesterase inhibitors are unlikely to be beneficial
Diagnosis investigations
- bilateral asymmetrical abnormalities of the frontal and temporal lobes
- seen on CT/MRI
SPECT
-regional cerebral blood flow studies have demonstrated disproportionate decrease in blood flow, radio tracer uptake and glucose metabolism in the frontal lobe in patients with FTF
Neuropsychology
- impaired frontal lobe function such as deficiency in abstract thinking, attentional shifting or set formation
- spared memory, speech and perceptuospatial functions
Pick’s disease
- onset is slow with steady deterioration
- predominance of frontal lobe involvement
- emotional blounting
- coarsening of social behaviour
- disinhibiton
- apathy
- restlessness
- aphasia
Early stages of PIcks
- memory and parietal lobe functions are preserved
- personality change is the first thing to go
Onset of Picks disease
- between 45-65 years
- men more affected than women
- average duration of illness is 8 years
- positive family history in 50%
Genetics of Picks
-autosomal dominant mutation in the Tau gene (Ch 17q21-22) with complete penetration
Picks cells
- swollen cells
- stain pink on H&E stain
- pathognomic
- demyleination and fibrous gliosiss of the frontal lobe white matter may be seen
- senile plaques and NFTs are absent
CT/MRI in Picks
- mild generalised atrophy
- marked atrophy of frontal and temporal lobes
- sparing of the posterior third of the superior temporal gyrus
- Knife blade atrophy
Primary progressive aphasia
- progressive decline in language
- cognitive sparing
- speech is non-fluent and effortful
- patient may become mute in later stages
- MRI shows atrophy in perisylvian region
Semantic dementia
- speech is fluent but impaired understanding of word meaning, naming difficulties and use of substitute words
- behaviour features are later in the disease
- MRI shows asymmetric atrophy of the temporal lobe (left more than right)
- atrophy of the anterior temporal lobe is more pronounced than the posterior temporal lobe
