Dementia

Question 1

what is cognition?

Answer 1

mental action of acquiring knowledge and understanding through thought, experience and senses

Question 2

what 5 processes is cognition often broken into?

Answer 2

attention 
language 
memory 
executive function (problem solving etc)
perceptuo-motor 
social functioning

Question 3

what is the DSMV criteria for diagnosing dementia?

Answer 3

evidence of significant cognitive decline in at least 1 cognitive domain

plus cognitive deficits interfere with independence in every day activities

plus they are not better explained by another process / do not occur exclusively in delirium

Question 4

dementias are generally due to what?

Answer 4

neurodegenerative proteinopathies

Question 5

what symptoms do those with viral encephalitis usually present with?

Answer 5

frontal temporal problems - memory problems, behaviour change and language problems

Question 6

what symptoms do those with head injury often present with?

Answer 6

global problems - attention, memory, executive dysfunction

Question 7

what are the clinical features of transient global amnesia?

Answer 7

abrupt onset antegrade > retrograde amnesia (repetitive)

preserved knowledge of self

lasts 4-6 hours and generally once off

Question 8

what is thought to be triggering factors of transient global amnesia?

Answer 8

emotion

change in temperature

Question 9

the pathophysiology is transient global amnesia is uncertain but what is thought to happen?

Answer 9

transient changes in hippocampus

Question 10

what are the clinical features of transient epileptic amnesia?

Answer 10

forgetful / repetitive questioning

carry out complex activities with no recollection

short (20-30 mins)

Question 11

transient epileptic amnesia is associated with what kind of seizures?

Answer 11

temporal lobe

Question 12

how is transient epileptic amnesia treated?

Answer 12

anti-epileptic

Question 13

what are the clinical features of functional / subjective cognitive impairment?

Answer 13

everyday forgetfulness impacting on function

fluctuation of symptoms

mismatch between symptoms + reported function

Question 14

what are symptoms of generalised functional disorder?

Answer 14

decreased concentration / attention / reaction time

subsequent memory difficulties as brain working too hard to correct things above

Question 15

what is the treatment for functional cognitive impairment?

Answer 15

exclude a mood disorder

refer to neuropsychology for treatment

Question 16

what is the most common human prion (type of neurodegenerative proteinopathy) disease?

Answer 16

creutzfeldt-jakob disease

Question 17

what is age of onset and clinical features sporadic of CJD?

Answer 17

60s

rapid onset dementia + neurological signs + myoclonus which lasts 4 months

Question 18

what is age of onset and clinical features of variant CJD?

Answer 18

20s

painful sensory disturbance + neuropsychotic decline which lasts 14 months

Question 19

what causes variant CJD?

Answer 19

exposure to BSE (or contaminated blood)

MAD COW DISEASE

Question 20

what is age of onset and clinical features of iatrogenic CJD?

Answer 20

30s

cerebellar / visual onset. Multifocal neurological decline which lasts <2 years

Question 21

what causes iatrogenic CJD?

Answer 21

99% HGH + dura mater

Question 22

what is age of onset and clinical features of genetic CJD?

Answer 22

may mimic sporadic

specific subtypes:
GGS (prolonged ataxic syndrome)
FFI (insomnia)

variable duration, often <2 years

Question 23

what causes genetic CJD?

Answer 23

mutation of PRNP

Question 24

what investigations should take place in CJD?

Answer 24

EEG
MRI
CSF

Question 25

what are the two most common types of dementia?

Answer 25

alzheimers | vascular dementia

Question 26

what is alzheimers disease?

Answer 26

neurodegenerative proteinopathy (amyloid)

Question 27

what is the pathophysiology of alzheimers?

Answer 27

disruption of cholinergic pathways in brain + synaptic loss extracellular amyloid plaques intracellular neurofibrillary tangles

Question 28

what parts of brain are degenerating in alzheimers?

Answer 28

medial hippocampus + later parietal lobes

Question 29

what is the progress of alzheimers symptoms?

Answer 29

forgetfulness -> apraxia / visuospatial difficulties

Question 30

what age is characterised as early onset alzheimers?

Answer 30

<65

Question 31

what are two atypical variants of alzheimers disease?

Answer 31

posterior cortical atrophy progressive primary aphasia

Question 32

what are symptoms of posterior cortical atrophy?

Answer 32

visuospatial disturbance starts here but then spreads out to forgetfulness etc

Question 33

what do those with progressive primary aphasia have problems with?

Answer 33

semantic (naming) logopenic aphasia (repeating) non-fluent aphasia (effortful)

Question 34

what investigations should take place in alzheimers?

Answer 34

MRI - atrophy of temporal / parietal lobe SECT - temporoparietal decreased metabolism CSF - decreased amyloid, increased tau ratio

Question 35

what treatments can be given for alzheimer disease?

Answer 35

address vascular risk factors acetylcholine boosting

Question 36

what kinds of acetylcholine boosters can be given in alzheimers?

Answer 36

cholinesterase inhibitors (rivastigmine / galantamine) NMDA receptor blocker (memantine)

Question 37

what is frontotemporal dementia?

Answer 37

early onset dementia (majority <65 years old)

Question 38

what is pathophysiology of frontotemporal dementia?

Answer 38

neurodegenerative proteinopathy Tau > TDP-43 > ubiquitin protein aggregation = cell damage

Question 39

what are the symptoms of frontotemporal dementia?

Answer 39

early frontal features (disinhibition, apathy, loss of empathy, stereotyped or compulsive behaviours, hyperorality) early loss of insight (collateral history vital)

Question 40

what investigations should take place in frontotemporal dementia?

Answer 40

MRI - atrophy of frontotemporal lobes SPECT - frontotemporal decreased metabolism CSF - increased tau / normal amyloid

Question 41

what can be used to treat frontotemporal dementia?

Answer 41

trazadone / antipsychotics safety - control food, money etc CPN

Question 42

what is vascular dementia?

Answer 42

late onset dementia (majority >65 years old)

Question 43

what is the core criteria of vascular dementia diagnosis?

Answer 43

presence of cerebrovascular disease plus a clear temporal relationship between the onset of dementia and cerebrovascular disease

Question 44

what is the presentation of vascular dementia?

Answer 44

subcortical (small vessel disease) - decreased attention, executive dysfunction and slowed processing

Question 45

what medical event is a significant risk factor for vascular dementia?

Answer 45

stroke

Question 46

how is vascular dementia managed?

Answer 46

vascular risk factors +/- cholinesterase inhibitor CPN

Question 47

what is dementia with lewy bodies?

Answer 47

another type of late onset dementia (>65)

Question 48

what is the pathophysiology of dementia with lewy bodies?

Answer 48

neurodegenerative proteinopathy (a-synuclein) a-synuclein aggregates = insoluble -> cell dysfunction -> cell damage leads to disruption of cholinergic and dopaminergic pathways

Question 49

what is the core criteria of dementia with lewy bodies diagnosis?

Answer 49

fluctuating cognition plus recurrent well formed visual hallucinations +/- presence of extrapyramidal features

Question 50

what additional feature can be seen in dementia with lewy bodies?

Answer 50

neuroleptic sensitivity

Question 51

what investigations should take place in dementia with lewy bodies?

Answer 51

DaT (dopamine transporter imagine) new: a-synuclein ligand imaging / in CSF

Question 52

how can dementia with lewy bodies be treated?

Answer 52

small dose levodopa (decreases acetylcholine) trial cholinesterase inhibitors

Question 53

what is parkinsons disease dementia?

Answer 53

late onset dementia (>65) which 80% of PD sufferers develop after 20 years of disease

Question 54

what other type of dementia does PDD overlap with?

Answer 54

DLB

Question 55

how do you differentiate PDD and DLB?

Answer 55

DLB = <1 year of presentation PDD = >1 year of presentation

Question 56

what are symptoms of PDD?

Answer 56

mix of parkinsons + dementia symptoms

Question 57

how is PDD managed?

Answer 57

same as DLB: small dose levodopa (decreases acetylcholine) trial cholinesterase inhibitors

Question 58

what are the clinical features of huntington's disease?

Answer 58

dysexecutive syndrome + slowed speech of processing eventual involvement of memory associated changes in mood / personality and chorea +/- later psychosis

Question 59

what investigations should take place in huntington's disease?

Answer 59

genetic testing MRI (loss of caudate heads)

Question 60

who should you refer to if patient is >65 with gradual onset dementia / no additional neurology?

Answer 60

old age psychiatry

Question 61

who should you refer to if patient is <65 / any unusual features (inc onset speed) / additional neurology?

Answer 61

neurology

Question 62

what is treatment for huntington disease?

Answer 62

mood stabiliser rx for chorea HD nurse specialist

Question 63

what are the psychiatric symptoms in HD?

Answer 63

``` depression anxiety psychosis blunted affect aggression suicidality compulsions ```

Question 64

what are the cognitive symptoms in HD?

Answer 64

decline in executive function (planning, abstract thinking) short and long term memory deficits dementia - decline in global cognition

Question 65

what are motor symptoms in HD?

Answer 65

choreiform movements rigidity writhing movements gait disturbance problems chewing / speaking / swallowing all actions requiring muscle control become impaired

Question 66

what happens when father passes HD on to daughter?

Answer 66

even more repeats so presents earlier when mother passes on to son = same number