Other Dementias

Question 1

what are the distinct brain changes in FTD

Answer 1

relatively focal frontal and temporal lobe atrophy

Question 2

onset of fronto-temporal dementia

Answer 2

earlier, <65

Question 3

what determines which variant FTD pt will have, and which is the most common

Answer 3

• depends which area of teh brainis affected first
• frontal presentation (behavioural variation) is the most common
• language variants - temporal lobe involvement

Question 4

how does frontal presentation FTD present

Answer 4

• Coarsening of personality, social behaviour, habits
• Eating habits changed
• Out of character, rude, compulsive

Question 5

how is memory affected in FTD

Answer 5

it tends to be unaffected in the early stages of all variants

Question 6

how do semantic dementia and progressive non fluent aphasia present

Answer 6

semantic - speech is fluent but they lose understanding of the language, might have difficulty finding teh right word (eg say animal not cat) and ask things like what is bread

progressive non-fluent aphasia - loss of verbal fluency, speech is slow and hesitant and often telegraphic - dont say inbetween words like “if”

Question 7

do teh 3 types remain clinically distinct throughout disease progression?

Answer 7

no, as the disease progresses patients develop both language and behavioural symptoms

Question 8

which part of teh brain is affected in semantic variant of FTD

Answer 8

left temporal lobe

Question 9

what is the basic underlying pathology of fronto-temporal dementia

Answer 9

abnormal protein inclusions in neurons and glial cells

Question 10

what are the 3 groups of protein inclusions seen in FTD

Answer 10

• FTD-tau - tau positive inclusions
• FTD-TDP - tau negative, contain TAR DNA binding protein 43 conjugated with uniquitin
• FTD-FUS - contains fused sarcoma protein

Question 11

what are Pick bodies and cells

Answer 11

Pick bodies are accumulations of tau proteins, cells are ballooned neurons with dissolution of chromatin

Question 12

which disease does FTD overlap with

Answer 12

there is an overlap between TDP43 proteinopathies and motor neuron disease

Question 13

which chromosome is important in TDP 43

Answer 13

chromosome 9

Question 14

what are the 3 clinical subtypes of FTD

Answer 14

behavioural variant, semantic and progressive non fluent aphasia

Question 15

what does MRI of FTD show

Answer 15

frontal and anterior temporal lobe atrophy - normally asymmetric

Question 16

what feature is consistent across all clinical subtypes of FTD on formal cognitive testing

Answer 16

poor emotional processing

Question 17

what is seen on CSF of FTD

Answer 17

increased tau and normal amyloid

Question 18

aetiology of vascular dementia

Answer 18

represents the cumulataive effects of small strokes

Question 19

onset of VD

Answer 19

often sudden

Question 20

how does VD progress

Answer 20

often stepwise progression ( small strokes)

Question 21

what is predominantly effected in VD

Answer 21

executive functions (eg planning, following a recipe, concentrating for a period of time)

Question 22

what should be looked for in a history of VD

Answer 22

history of stroke or cerebrovascular event

Question 23

what is the most useful investiagation for VD

Answer 23

SPECT scan

or MRI

Question 24

how does a SPECT scan work

Answer 24

combined MRI and radioactive tracer to visualise blood vessels

Question 25

what is seen on imaging of VD

Answer 25

widespread small vessel disease

Question 26

what is the mainstay of VD management

Answer 26

management of risk factors to prevent further disease

Question 27

are drugs used in VD management?

Answer 27

yes, antiplatlet therapy

there is not much role for cholinesterase inhibitors or memantine

Question 28

how does DLB present

Answer 28

• Typically, with fluctuating cognitive impairment, detailed visual hallucinations and later, parkinsonism
• REM sleep behaviour disorder

Question 29

are cognitive or motor features (parkinsonism) more prominent in DLB

Answer 29

cognitive features dominate, Parkinsonism may devlop later but it is typically mild

Question 30

pathophysiology of DLB

Answer 30

there are lewy body inclusions inthe brain which contain protein alpha synuclein

these disrupt cholinergic and dopamine pathways

Question 31

which drugs do patients with DLB show marked sensitivty to and must avoid

Answer 31

antipsychotics (neuroleptics) - severe worsenin of motor symptoms and change in consciousness

Question 32

what is parkinson’s dementia

Answer 32

cognitive problems that develop late in the progression of Parkinson’s disease (at least 1 year after onset, but pt is usually >75)

Question 33

management of PD and LBD

Answer 33

small dose levodopa and cholinen

sterase inhibitors

remember to avoid APs!!