Frontotemporal dementia

Question 1

FTD

Answer 1

Aka frontotempolar lobar degeneration (FTLD), which refers to the underlying disease
(FTD refers to symptoms)

A heterogeneous group of clinical syndrome associated with degeneration of the frontal and temporal lobe (progressive)

Varies depending on the protein pathology

Question 2

Symptoms

Answer 2

Personality changes
Behavioural changes
Impaired cognition
Language
Motor functioning

Which of the symptoms occur depends on the neuropathology

Question 3

Arnold Pick

Answer 3

First observed it when a patient with paraphasia showed much bigger gyri of the frontal and temporal lobes in the autopsy; atrophy of the brain

First called Picks disease; paraphasia and personality changes and frontotemporal atrophy

Question 4

Etiology

Answer 4

2nd most prevalent dementia under the age of 65 (after AD). Early onset is typical (45-65 yrs)

Roughly 5% of dementia cases

Prev in women and men = equal

Strong genetic factor; 30-50% of cases have family history with FTD. Relatives have a 3.5 higher risk to get it compared to general population

Question 5

Classifications of varieties 1st

Answer 5

Behavioural/dysexecutive variant
- Right temporal or frontal variant of FTD

Language variant (primarily progressive aphasia; PPA) further divided into
- Semantic dementia (fluent aphasia, AKA Left temporal FTD)
- Progressive non-fluent aphasia (PNFA)

Question 6

Classifications language

Answer 6

Language variant;
- Semantic variant (comparable to SD_)
- Agrammatic variant (Overlap PPA)
- Logopenic variant (word finding difficulties, no comprehension impairments)

Question 7

Classifications motor

Answer 7

Motor variants;
- Motor neuron disease (MND)
- Progressive supranuclear palsy (PSP)
- Corticobasal syndrome (CBS)