Pick’s disease (fronto-temporal dementia)

Question 1

Onset

Answer 1

early
<65
FHx

Question 2

Pathophysiology macro and micro (3 types)

Answer 2

• Macroscopic → bilateral atrophy of frontal lobe + anterior temporal lobe; degeneration of striatum
• Microscopic → 3 subtypes

1. Common type: loss of large cortical nerve cells, spongiform degeneration, minimal gliosis
2. Pick type (Pick’s disease): loss of large cortical nerve cells, widespread gliosis, no spongiform change, Pick cells (swollen neurones) and Pick bodies (inclusions +ve for tau and ubiquitin)
3. Associated w/ MND

Question 3

CFs

Answer 3

Opposite to AD: early on in course there are personality changes → then later manifestations are cognitive changes (memory loss)

Distinctive features
• Early personality changes (disinhibition, apathy/restlessness)
• Insight lost early (that something is wrong w/ them)
• Worsening of social behaviour
• Repetitive behaviour
• Memory preserved until later
• Language problems: difficult to find words, problems naming/understanding words
• Overeating + preference for sweet foods
• Cognition (frontal lobe): Poor organisation, impaired attention, ineffective retrieval strategies…etc

Question 4

5 core features

Answer 4

a. Insidious onset + gradual progression
b. Early decline in social interpersonal conduct
c. Early impairment in regulation of personal conduct
d. Early emotional blunting
e. Early loss of insight

Question 5

3 supportive features

Answer 5

a. Behavioural disorder: decline in personal hygiene…etc
b. Speech + language
c. Physical signs: primitive reflexes, incontinence, akinesia, rigidity, tremor

Question 6

Ix

Answer 6

1. frontal lobe tests
2. EEG → normal
3. CT/MRI → bilateral atrophy of frontal and temporal regions
4. SPECT → frontal and temporal lobe abnormalities

Question 7

Rx

Answer 7

Rx = non-specific (need to use the dementia principles for management)