Fronto-temporal Dementias

Question 1

What is the most common frontotemporal lobar dementia?

Answer 1

Pick’s disease

Question 2

State 5 pathological features of Pick’s disease

Answer 2

Fronto-temporal atrophy, marked gliosis and neuronal loss, balloon neurons, tau-positive Pick bodies, and no glial inclusions (unlike other tau-opathies)

Question 3

Is Pick’s disease a 3-repeat, 4-repeat, or 3 and 4-repeat tauopathy?

Answer 3

3-repeat

Question 4

How does Pick’s disease typically present clinically?

Answer 4

Difficulties in executive function, disinhibition, more specific amnestic features than other dementias

Question 5

Which syndromes have given most knowledge on frontotemporal lobar dementias?

Answer 5

Autosomal dominant frontotemporal dementia with parkinsonism (FTDP-17) syndromes

Question 6

How many isoforms does tau have?

Answer 6

6

Question 7

How many tau isoforms are involved in Alzheimer’s disease?

Answer 7

6

Question 8

How many tau isoforms are involved in progressive supranuclear palsy and corticobasal degeneration?

Answer 8

3 - the 4-repeat tau isoforms

Question 9

Name the classical sign of frontotemporal dementias - especially Pick’s disease - macroscopically on pathology

Answer 9

Knife-edge atrophy

Question 10

Name a gene which can cause frontotemporal dementia without involvement of tau

Answer 10

Progranulin, on chromosome 17; FUS; C9orf72

Question 11

State the pathological characteristic of frontotemporal dementia caused by progranulin mutations

Answer 11

Ubiquitin-tagged nuclear inclusions known as catseye inclusions

Question 12

How long before clinical symptoms develop are changes apparent on MRI in individuals with progranulin mutations?

Answer 12

7-9 years

Question 13

State some symptoms of frontotemporal dementia caused by progranulin mutations

Answer 13

Word-finding difficulty, progressive language impairment, progressive personality change, limb apraxia, facial agnosia, anomia, dyscalculia

Question 14

Is atrophy usually symmetrical or asymmetical in frontotemporal dementia caused by progranulin mutations?

Answer 14

Asymmetrical

Question 15

How many alleles of TDP-43 are there?

Answer 15

4

Question 16

State the term given to clinical dementia with no obvious causative pathology

Answer 16

Dementia lacking distinctive histology (DLDH)