Frontotemporal dementia

Question 1

FTD

Answer 1

• Pick’s disease
• Primary progressive aphasia
• semantic dementia
• corticobasal degeneration

Question 2

Ages

Answer 2

• FTD affects younger populations
• onset between 40-75
• 20% presenile cases of dementia

Question 3

Genetics

Answer 3

-linked to chromosome 17

Question 4

Early clinical features

Answer 4

• changes in personality
• changes in behaviour
• behaviour rigidity, impulsivity, emotional lability, fatuousness, executive dysfunction and hyperorality

Question 5

Memory

Answer 5

• affected later

- less severely affected

Question 6

Spatial orientation

Answer 6

-well preserved

Question 7

Insight

Answer 7

-lost early

Question 8

Brain changes

Answer 8

• asymmetircal focal atrophy of the frontotemporal regions

- underlying neuronal loss, gliosis and subsequent spongiform change in the affected cortices

Question 9

FTD and MND

Answer 9

-10% of patients with MND show features of dementia

Question 10

Treatment

Answer 10

• SSRIs may help with behaviour

- acetylcholinesterase inhibitors are unlikely to be beneficial

Question 11

Diagnosis investigations

Answer 11

• bilateral asymmetrical abnormalities of the frontal and temporal lobes
• seen on CT/MRI

Question 12

SPECT

Answer 12

-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

Question 13

Neuropsychology

Answer 13

• impaired frontal lobe function such as deficiency in abstract thinking, attentional shifting or set formation
• spared memory, speech and perceptuospatial functions

Question 14

Pick’s disease

Answer 14

• onset is slow with steady deterioration
• predominance of frontal lobe involvement
• emotional blounting
• coarsening of social behaviour
• disinhibiton
• apathy
• restlessness
• aphasia

Question 15

Early stages of PIcks

Answer 15

• memory and parietal lobe functions are preserved

- personality change is the first thing to go

Question 16

Onset of Picks disease

Answer 16

• between 45-65 years
• men more affected than women
• average duration of illness is 8 years
• positive family history in 50%

Question 17

Genetics of Picks

Answer 17

-autosomal dominant mutation in the Tau gene (Ch 17q21-22) with complete penetration

Question 18

Picks cells

Answer 18

• 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

Question 19

CT/MRI in Picks

Answer 19

• mild generalised atrophy
• marked atrophy of frontal and temporal lobes
• sparing of the posterior third of the superior temporal gyrus
• Knife blade atrophy

Question 20

Primary progressive aphasia

Answer 20

• 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

Question 21

Semantic dementia

Answer 21

• 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