Flashcards in Cognitive Neurology Deck (43):
1
what generally causes dementias
neurodegenerative proteinopathies
2
what is cognition
the mental action of acquiring knowledge and understanding through thought, experience ans the scenes
encompases
- formation of knowledge and memory
-executive function (problem solving/ decision making)
-language (comprehension and production of language)
-social functioning (judgement, evaluation, reasoning)
-attention
3
what is dementia
significant cognitive decline in at least 1 cognitive domain (attention, executive, learning and memory, language, perceptuo-motor (ie praxis) or social cognition
generally progressive/ pervasive and generally associated with neurodegeneration
4
what cognitive problems arise from viral encephalitis
frontal temporal problems
-memory, behaviour change, language
5
what cognitive problems can arise from head injury
attention
memory
executive dysfunction
6
what is transient global amnesia
an acute cognitive disorder
abrupt onset antegrade>retrograde amnesia that is repetitive
will have preserved knowledge of self
lasts 4-6 hours (always <24hrs)
7
what causes a transient global amnesia
triggering factors inc emotion/ changes in temperature
transient changes in the hippocampus
8
what are the clinical features of a transient epileptic amnesia
forgetful/ repetitive questioning
can carry out complex activities with no recollection of events
short lived(20-30 mins)
recurrent problems
9
what is transient epileptic amnesia associated with
temporal lobe seizures
10
what can cause sub acute cognitive disorders
toxins- alcohol, CO
neurodegeneration (CJD)
metabolic- B12, calciulm, thyroid etc
inflammatory- limbic encephalitis
mood disorders
functional
infection- HIV, syphilis
11
what are the clinical features of functional/ subjective cognitive impairment
everyday forgetfulness impacting on functioning
fluctuation of symptoms
mismatch of symptoms (symptoms vs reported symptoms/ function, or symptoms vs symptoms of known neurodegenerative disorders)
(will think symptoms are worse than they actually are)
12
what can a functional/ subjective cognitive impairment be a part of
generalised functional disorder (decreased concentration/ attention/ reaction time and memory problems)
13
what is the treatment for a functional cognitive impairment
exclude a mood disorder
neuropsychology
14
what is creutzfeldt-jakob disease
the most common human prion disease
15
what causes prion disease
neurodegenerative proteinopathy (prion)
prion protein in misfolded
16
what are the types of CJD
sporadic (60s, rapid onset dementia + neuro signs + myoclonus, lasts 4 months)
variant (20s, painful sensory disturbance + neuropsychiatric decline, lasts 14 months)
iatrogenic
genetic (any age onset, may mimic sporadic, duration <2 years)
17
what is seen pathologically in prion disease
spongiform change
18
what is the pathology of alzheimers
is a neurodegenerative proteinopathy (amyloid)
disruption of cholinergic pathways in the brain + synaptic loss
-extracellular amyloid plaques
-intracellular neurofibrillary tangles
19
what parts of brain are affected in alzheimers
degeneration of medial hippocampus + later parietal lobes
(forgetfullness -> apraxia/ visuospatial difficulties)
20
what are the symptoms of posterior cortical atrophy (atypical presentation of AD)
visuospatial disturbance
problems recognising death
21
what are the features of progressive primary aphasia (an atypical presentation of AD)
semantic (naming difficulties)
logopenic aphasia (repeating)
non fluent aphasia (effortful speech)
22
what is seen on investigations in AD
MRI- atrophy of temporal/ parietal lobes
SPECT - temporoparietal decreased metabolism
CSF- decreased amyloid : increased tau ratio
23
what is the treatment for alzheimers
address vascular risk factors
acetylcholine boosting treatments:
- cholinesterase inhibitors (rivastigmine/ galantamine)
-NMDA receptor blockers (memantine)
24
what are the features of frontotemporal dementia
early onset dementia (<65y/o)
Early frontal features (disinhibition, apathy, loss of empathy, stereotyped or compulsive behaviours, hyperorality)
Early loss of insight (collateral history vital)
25
what causes frontotemporal dementia
Neurodegenerative proteinopathy
protein aggregation -> cell damage
26
what is seen in investigations in FTD
MRI: Atrophy of frontotemporal lobes
SPECT: Frontotemporal ↓metabolism
CSF: ↑ tau / normal amyloid
27
what is the management for FTD
Trial of Trazadone / antipsychotics to help behavioural features
Safety management
Controlled access to food / money / internet
Structured activities
Power of attorney
Support: MND nurse specialist if co-existent MND / CPN
28
what are the features of vascular dementia
late onset dementia (>65)
presence of cerebrovascular disease (vascular changes on MRI)
co existent amyloid pathology
slow progression
prognosis similar to AD
29
what is the presentation of vascular dementia
subcortical (small vessel disease)= decreased attention, executive dysfunction, slowed processing
post stroke dementia
30
what is the management for vascular dementia
vascular risk factors +/- cholinesterase inhibitor
31
what are the features of lewy bodies dementia
late onset dementia (>65)
fluctuating cognition
recurrent well formed visual hallucinations +/-
presence of extrapyramidal features
neuroleptic sensitive (gets worse when you give halopuridol)
32
what causes lewy body dementia
Neurodegenerative proteinopathy (α-synuclein)
α-synuclein aggregates = insoluble → cell dysfunction → cell damage
Leads to disruption of cholinergic and dopaminergic pathways
33
what is seen on investigations in lewy body dementia
dopamine transporter imagine
alpha-synuclein ligand imaging
alpha synuclein in CSF
34
what is the treatment for dementia with lewy bodies
small dose levodopa
cholinesterase inhibitors
35
what are the features of parkinsons disease dementia
late onset >65
clinically and pathologically overlap with LBD (LBD has quicker onset of dementia after presentation< PD usually dementia 20 years after presentation)
36
what is the management for parkinsons disease dementia
small dose levodopa
cholinesterase inhibitors
37
what are the symptoms of parkinsons disease dementia
Parkinson’s disease (bradykinesia + rigidity + tremor) + dementia (↓attention + slowness of processing, impaired visuospatial functions + memory) +/- hallucinations
38
what are the features of huntingtons disease dementia
early onset 30-50
Dementia: Dysexecutive syndrome + slowed speed of processing. Eventual involvement of memory
Associated changes in mood / personality, and chorea +/- later psychosis
39
what causes huntingtons
Expansion of the CAG trinucleotide repeat on the huntingtin gene
produces neurodegenerative protein (Huntingtin)
40
what is seen in investigations in huntingtons
CAG trinucleotide repeat
MRI- loss of caudate heads
41
what is the treatment for huntingtons
mood stabilisers
Tx for chorea
nurse specialist
42
what is the apo34 gene associated
amyloid deposition with age- alzheimers
43