Cognitive Neurology

Question 1

what generally causes dementias

Answer 1

neurodegenerative proteinopathies

Question 2

what is cognition

Answer 2

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

Question 3

what is dementia

Answer 3

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

Question 4

what cognitive problems arise from viral encephalitis

Answer 4

frontal temporal problems

-memory, behaviour change, language

Question 5

what cognitive problems can arise from head injury

Answer 5

attention
memory
executive dysfunction

Question 6

what is transient global amnesia

Answer 6

an acute cognitive disorder
abrupt onset antegrade>retrograde amnesia that is repetitive
will have preserved knowledge of self
lasts 4-6 hours (always <24hrs)

Question 7

what causes a transient global amnesia

Answer 7

triggering factors inc emotion/ changes in temperature

transient changes in the hippocampus

Question 8

what are the clinical features of a transient epileptic amnesia

Answer 8

forgetful/ repetitive questioning
can carry out complex activities with no recollection of events
short lived(20-30 mins)
recurrent problems

Question 9

what is transient epileptic amnesia associated with

Answer 9

temporal lobe seizures

Question 10

what can cause sub acute cognitive disorders

Answer 10

toxins- alcohol, CO 
neurodegeneration (CJD)
metabolic- B12, calciulm, thyroid etc 
inflammatory- limbic encephalitis 
mood disorders 
functional 
infection- HIV, syphilis

Question 11

what are the clinical features of functional/ subjective cognitive impairment

Answer 11

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)

Question 12

what can a functional/ subjective cognitive impairment be a part of

Answer 12

generalised functional disorder (decreased concentration/ attention/ reaction time and memory problems)

Question 13

what is the treatment for a functional cognitive impairment

Answer 13

exclude a mood disorder

neuropsychology

Question 14

what is creutzfeldt-jakob disease

Answer 14

the most common human prion disease

Question 15

what causes prion disease

Answer 15

neurodegenerative proteinopathy (prion) 
prion protein in misfolded

Question 16

what are the types of CJD

Answer 16

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)

Question 17

what is seen pathologically in prion disease

Answer 17

spongiform change

Question 18

what is the pathology of alzheimers

Answer 18

is a neurodegenerative proteinopathy (amyloid)
disruption of cholinergic pathways in the brain + synaptic loss
-extracellular amyloid plaques
-intracellular neurofibrillary tangles

Question 19

what parts of brain are affected in alzheimers

Answer 19

degeneration of medial hippocampus + later parietal lobes

forgetfullness -> apraxia/ visuospatial difficulties

Question 20

what are the symptoms of posterior cortical atrophy (atypical presentation of AD)

Answer 20

visuospatial disturbance

problems recognising death

Question 21

what are the features of progressive primary aphasia (an atypical presentation of AD)

Answer 21

semantic (naming difficulties)
logopenic aphasia (repeating)
non fluent aphasia (effortful speech)

Question 22

what is seen on investigations in AD

Answer 22

MRI- atrophy of temporal/ parietal lobes
SPECT - temporoparietal decreased metabolism
CSF- decreased amyloid : increased tau ratio

Question 23

what is the treatment for alzheimers

Answer 23

address vascular risk factors
acetylcholine boosting treatments:
- cholinesterase inhibitors (rivastigmine/ galantamine)
-NMDA receptor blockers (memantine)

Question 24

what are the features of frontotemporal dementia

Answer 24

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)

Question 25

what causes frontotemporal dementia

Answer 25

Neurodegenerative proteinopathy protein aggregation -> cell damage

Question 26

what is seen in investigations in FTD

Answer 26

MRI: Atrophy of frontotemporal lobes SPECT: Frontotemporal ↓metabolism CSF: ↑ tau / normal amyloid

Question 27

what is the management for FTD

Answer 27

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

Question 28

what are the features of vascular dementia

Answer 28

``` late onset dementia (>65) presence of cerebrovascular disease (vascular changes on MRI) co existent amyloid pathology slow progression prognosis similar to AD ```

Question 29

what is the presentation of vascular dementia

Answer 29

subcortical (small vessel disease)= decreased attention, executive dysfunction, slowed processing post stroke dementia

Question 30

what is the management for vascular dementia

Answer 30

vascular risk factors +/- cholinesterase inhibitor

Question 31

what are the features of lewy bodies dementia

Answer 31

late onset dementia (>65) fluctuating cognition recurrent well formed visual hallucinations +/- presence of extrapyramidal features neuroleptic sensitive (gets worse when you give halopuridol)

Question 32

what causes lewy body dementia

Answer 32

``` Neurodegenerative proteinopathy (α-synuclein) α-synuclein aggregates = insoluble → cell dysfunction → cell damage Leads to disruption of cholinergic and dopaminergic pathways ```

Question 33

what is seen on investigations in lewy body dementia

Answer 33

dopamine transporter imagine alpha-synuclein ligand imaging alpha synuclein in CSF

Question 34

what is the treatment for dementia with lewy bodies

Answer 34

small dose levodopa | cholinesterase inhibitors

Question 35

what are the features of parkinsons disease dementia

Answer 35

late onset >65 clinically and pathologically overlap with LBD (LBD has quicker onset of dementia after presentation< PD usually dementia 20 years after presentation)

Question 36

what is the management for parkinsons disease dementia

Answer 36

small dose levodopa | cholinesterase inhibitors

Question 37

what are the symptoms of parkinsons disease dementia

Answer 37

Parkinson’s disease (bradykinesia + rigidity + tremor) + dementia (↓attention + slowness of processing, impaired visuospatial functions + memory) +/- hallucinations

Question 38

what are the features of huntingtons disease dementia

Answer 38

early onset 30-50 Dementia: Dysexecutive syndrome + slowed speed of processing. Eventual involvement of memory Associated changes in mood / personality, and chorea +/- later psychosis

Question 39

what causes huntingtons

Answer 39

Expansion of the CAG trinucleotide repeat on the huntingtin gene produces neurodegenerative protein (Huntingtin)

Question 40

what is seen in investigations in huntingtons

Answer 40

CAG trinucleotide repeat | MRI- loss of caudate heads

Question 41

what is the treatment for huntingtons

Answer 41

mood stabilisers Tx for chorea nurse specialist

Question 42

what is the apo34 gene associated

Answer 42

amyloid deposition with age- alzheimers

Question 43

what proteins are associated with the different types of dementias IMPORTANT

Answer 43

``` AD +/- VaD – amyloid, PDD / DLB – α-synuclein, CJD – prion, FTD – tau, HD – huntingtin ```