Frontotemporal lobar degeneration

Question 1

3 commonly recognised types of frontotemporal lobar degeneration

Answer 1

1. Frontotemporal dementia (Pick’s disease)
2. Progressive non fluent aphasia (AKA chronic progressive aphasia)
3. Semantic dementia

Question 2

Common features of frontotemporal lobar dementias

Answer 2

• Onset <65
• Insidious onset
• Relatively preserved memory and visuospatial skills
• Personality change and social conduct problems

Question 3

Macroscopic appearance of Picks disease on imaging

Answer 3

Frontotemporal atrophy

Focal gyral atrophy with a
“knife-blade appearance” is characteristic

Question 4

Microscopic changes seen in Picks disease

Answer 4

Pick bodies - spherical aggregations of tau protein

Gliosis

Neurofibrillary tangles

Senile plaques

Question 5

Main symptom in Progressive non fluent aphasia (AKA chronic progressive aphasia)

Answer 5

• non fluent speech
  => short utterances that are agrammatic
• Comprehension is relatively preserved.

Question 6

Main features in semantic dementia

Answer 6

• speech is fluent but empty and conveys little meaning
• short term memory better than long term