Other Dementias Flashcards
what are the distinct brain changes in FTD
relatively focal frontal and temporal lobe atrophy
onset of fronto-temporal dementia
earlier, <65
what determines which variant FTD pt will have, and which is the most common
- depends which area of teh brainis affected first
- frontal presentation (behavioural variation) is the most common
- language variants - temporal lobe involvement
how does frontal presentation FTD present
- Coarsening of personality, social behaviour, habits
- Eating habits changed
- Out of character, rude, compulsive
how is memory affected in FTD
it tends to be unaffected in the early stages of all variants
how do semantic dementia and progressive non fluent aphasia present
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”
do teh 3 types remain clinically distinct throughout disease progression?
no, as the disease progresses patients develop both language and behavioural symptoms
which part of teh brain is affected in semantic variant of FTD
left temporal lobe
what is the basic underlying pathology of fronto-temporal dementia
abnormal protein inclusions in neurons and glial cells
what are the 3 groups of protein inclusions seen in FTD
- FTD-tau - tau positive inclusions
- FTD-TDP - tau negative, contain TAR DNA binding protein 43 conjugated with uniquitin
- FTD-FUS - contains fused sarcoma protein
what are Pick bodies and cells
Pick bodies are accumulations of tau proteins, cells are ballooned neurons with dissolution of chromatin
which disease does FTD overlap with
there is an overlap between TDP43 proteinopathies and motor neuron disease
which chromosome is important in TDP 43
chromosome 9
what are the 3 clinical subtypes of FTD
behavioural variant, semantic and progressive non fluent aphasia
what does MRI of FTD show
frontal and anterior temporal lobe atrophy - normally asymmetric
what feature is consistent across all clinical subtypes of FTD on formal cognitive testing
poor emotional processing
what is seen on CSF of FTD
increased tau and normal amyloid
aetiology of vascular dementia
represents the cumulataive effects of small strokes
onset of VD
often sudden
how does VD progress
often stepwise progression ( small strokes)
what is predominantly effected in VD
executive functions (eg planning, following a recipe, concentrating for a period of time)
what should be looked for in a history of VD
history of stroke or cerebrovascular event
what is the most useful investiagation for VD
SPECT scan
or MRI
how does a SPECT scan work
combined MRI and radioactive tracer to visualise blood vessels
what is seen on imaging of VD
widespread small vessel disease
what is the mainstay of VD management
management of risk factors to prevent further disease
are drugs used in VD management?
yes, antiplatlet therapy
there is not much role for cholinesterase inhibitors or memantine
how does DLB present
- Typically, with fluctuating cognitive impairment, detailed visual hallucinations and later, parkinsonism
- REM sleep behaviour disorder
are cognitive or motor features (parkinsonism) more prominent in DLB
cognitive features dominate, Parkinsonism may devlop later but it is typically mild
pathophysiology of DLB
there are lewy body inclusions inthe brain which contain protein alpha synuclein
these disrupt cholinergic and dopamine pathways
which drugs do patients with DLB show marked sensitivty to and must avoid
antipsychotics (neuroleptics) - severe worsenin of motor symptoms and change in consciousness
what is parkinson’s dementia
cognitive problems that develop late in the progression of Parkinson’s disease (at least 1 year after onset, but pt is usually >75)
management of PD and LBD
small dose levodopa and cholinen
sterase inhibitors
remember to avoid APs!!
