Dementia Flashcards
what is cognition?
mental action of acquiring knowledge and understanding through thought, experience and senses
what 5 processes is cognition often broken into?
attention language memory executive function (problem solving etc) perceptuo-motor social functioning
what is the DSMV criteria for diagnosing dementia?
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
dementias are generally due to what?
neurodegenerative proteinopathies
what symptoms do those with viral encephalitis usually present with?
frontal temporal problems - memory problems, behaviour change and language problems
what symptoms do those with head injury often present with?
global problems - attention, memory, executive dysfunction
what are the clinical features of transient global amnesia?
abrupt onset antegrade > retrograde amnesia (repetitive)
preserved knowledge of self
lasts 4-6 hours and generally once off
what is thought to be triggering factors of transient global amnesia?
emotion
change in temperature
the pathophysiology is transient global amnesia is uncertain but what is thought to happen?
transient changes in hippocampus
what are the clinical features of transient epileptic amnesia?
forgetful / repetitive questioning
carry out complex activities with no recollection
short (20-30 mins)
transient epileptic amnesia is associated with what kind of seizures?
temporal lobe
how is transient epileptic amnesia treated?
anti-epileptic
what are the clinical features of functional / subjective cognitive impairment?
everyday forgetfulness impacting on function
fluctuation of symptoms
mismatch between symptoms + reported function
what are symptoms of generalised functional disorder?
decreased concentration / attention / reaction time
subsequent memory difficulties as brain working too hard to correct things above
what is the treatment for functional cognitive impairment?
exclude a mood disorder
refer to neuropsychology for treatment
what is the most common human prion (type of neurodegenerative proteinopathy) disease?
creutzfeldt-jakob disease
what is age of onset and clinical features sporadic of CJD?
60s
rapid onset dementia + neurological signs + myoclonus which lasts 4 months
what is age of onset and clinical features of variant CJD?
20s
painful sensory disturbance + neuropsychotic decline which lasts 14 months
what causes variant CJD?
exposure to BSE (or contaminated blood)
MAD COW DISEASE
what is age of onset and clinical features of iatrogenic CJD?
30s
cerebellar / visual onset. Multifocal neurological decline which lasts <2 years
what causes iatrogenic CJD?
99% HGH + dura mater
what is age of onset and clinical features of genetic CJD?
may mimic sporadic
specific subtypes:
GGS (prolonged ataxic syndrome)
FFI (insomnia)
variable duration, often <2 years
what causes genetic CJD?
mutation of PRNP
what investigations should take place in CJD?
EEG
MRI
CSF
what are the two most common types of dementia?
alzheimers
vascular dementia
what is alzheimers disease?
neurodegenerative proteinopathy (amyloid)
what is the pathophysiology of alzheimers?
disruption of cholinergic pathways in brain + synaptic loss
extracellular amyloid plaques
intracellular neurofibrillary tangles
what parts of brain are degenerating in alzheimers?
medial hippocampus + later parietal lobes
what is the progress of alzheimers symptoms?
forgetfulness -> apraxia / visuospatial difficulties
what age is characterised as early onset alzheimers?
<65
what are two atypical variants of alzheimers disease?
posterior cortical atrophy
progressive primary aphasia
what are symptoms of posterior cortical atrophy?
visuospatial disturbance
starts here but then spreads out to forgetfulness etc
what do those with progressive primary aphasia have problems with?
semantic (naming)
logopenic aphasia (repeating)
non-fluent aphasia (effortful)
what investigations should take place in alzheimers?
MRI - atrophy of temporal / parietal lobe
SECT - temporoparietal decreased metabolism
CSF - decreased amyloid, increased tau ratio
what treatments can be given for alzheimer disease?
address vascular risk factors
acetylcholine boosting
what kinds of acetylcholine boosters can be given in alzheimers?
cholinesterase inhibitors (rivastigmine / galantamine)
NMDA receptor blocker (memantine)
what is frontotemporal dementia?
early onset dementia (majority <65 years old)
what is pathophysiology of frontotemporal dementia?
neurodegenerative proteinopathy
Tau > TDP-43 > ubiquitin
protein aggregation = cell damage
what are the symptoms of frontotemporal dementia?
early frontal features (disinhibition, apathy, loss of empathy, stereotyped or compulsive behaviours, hyperorality)
early loss of insight (collateral history vital)
what investigations should take place in frontotemporal dementia?
MRI - atrophy of frontotemporal lobes
SPECT - frontotemporal decreased metabolism
CSF - increased tau / normal amyloid
what can be used to treat frontotemporal dementia?
trazadone / antipsychotics
safety - control food, money etc
CPN
what is vascular dementia?
late onset dementia (majority >65 years old)
what is the core criteria of vascular dementia diagnosis?
presence of cerebrovascular disease plus
a clear temporal relationship between the onset of dementia and cerebrovascular disease
what is the presentation of vascular dementia?
subcortical (small vessel disease) - decreased attention, executive dysfunction and slowed processing
what medical event is a significant risk factor for vascular dementia?
stroke
how is vascular dementia managed?
vascular risk factors +/- cholinesterase inhibitor
CPN
what is dementia with lewy bodies?
another type of late onset dementia (>65)
what is the pathophysiology of dementia with lewy bodies?
neurodegenerative proteinopathy (a-synuclein)
a-synuclein aggregates = insoluble -> cell dysfunction -> cell damage
leads to disruption of cholinergic and dopaminergic pathways
what is the core criteria of dementia with lewy bodies diagnosis?
fluctuating cognition plus
recurrent well formed visual hallucinations +/-
presence of extrapyramidal features
what additional feature can be seen in dementia with lewy bodies?
neuroleptic sensitivity
what investigations should take place in dementia with lewy bodies?
DaT (dopamine transporter imagine)
new: a-synuclein ligand imaging / in CSF
how can dementia with lewy bodies be treated?
small dose levodopa (decreases acetylcholine)
trial cholinesterase inhibitors
what is parkinsons disease dementia?
late onset dementia (>65) which 80% of PD sufferers develop after 20 years of disease
what other type of dementia does PDD overlap with?
DLB
how do you differentiate PDD and DLB?
DLB = <1 year of presentation
PDD = >1 year of presentation
what are symptoms of PDD?
mix of parkinsons + dementia symptoms
how is PDD managed?
same as DLB:
small dose levodopa (decreases acetylcholine)
trial cholinesterase inhibitors
what are the clinical features of huntington’s disease?
dysexecutive syndrome + slowed speech of processing
eventual involvement of memory
associated changes in mood / personality and chorea +/- later psychosis
what investigations should take place in huntington’s disease?
genetic testing
MRI (loss of caudate heads)
who should you refer to if patient is >65 with gradual onset dementia / no additional neurology?
old age psychiatry
who should you refer to if patient is <65 / any unusual features (inc onset speed) / additional neurology?
neurology
what is treatment for huntington disease?
mood stabiliser
rx for chorea
HD nurse specialist
what are the psychiatric symptoms in HD?
depression anxiety psychosis blunted affect aggression suicidality compulsions
what are the cognitive symptoms in HD?
decline in executive function (planning, abstract thinking)
short and long term memory deficits
dementia - decline in global cognition
what are motor symptoms in HD?
choreiform movements
rigidity
writhing movements
gait disturbance
problems chewing / speaking / swallowing
all actions requiring muscle control become impaired
what happens when father passes HD on to daughter?
even more repeats so presents earlier
when mother passes on to son = same number
