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cognitive neurology Flashcards

1
Q

what pathophysio are dementias usually due to?

A

neurodegenerative proteinopathies

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2
Q

what domains does cognition encompass?

A

o Memory
o Executive function – problem solving, decision making
o Social functioning – judgement, evaluation
o Attention
o Visuospatial- perception, spatial awareness
o Language – comprehension + production of language

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3
Q

proteinopathy assoc with Alzheimer’s +/- vascular dementia

A

amyloid

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4
Q

proteinopathy assoc with Parkinsons / Lewy body dementia

A

alpha-synuclein

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5
Q

proteinopathy assoc with Creutzfeldt-Jakob Disease (CJD)

A

prion

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6
Q

proteinopathy assoc with frontotemporal dementia

A

tau

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7
Q

proteinopathy assoc with Huntingtons dementia

A

huntingtin

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8
Q

dementia

A

Evidence of significant cognitive decline in at least 1 cognitive domain, plus both –
- Cognitive deficits interfere with independence in everyday activities
- They are not better explained by another process/do not occur exclusively in the context of delirium

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9
Q

stuttering vs gradual onset of cognitive decline

A

stuttering - vascualr

gradual - proteinopathy

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10
Q

dementia investigation

A

blood screen to exclude reversible causes
- glucose, TFTs, vit B12, folate, Ca, LFTs, ESR/CRP, FBC + U&Es

imaging - subdural haematoma, normal pressure hydroceph, paterns of atrophy

MRI- if young, fast progression
SPECT - most usueful for frontotempotal/clarifying alzheimers

DAT - for DLB/parkinsons if not enough supported features

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11
Q

who to refer to?

A
  • > 65 years old gradual onset dementias / no additional neurology = old age psychiatry
  • <65 yrs / any unusual features (including speed of onset) / additional neurology = neurology
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12
Q

brain insults

A

Deficits depend on area of brain affected
Viral encephalitis
- E.g. herpes simplex encephalitis
o Loves temporal lobes
o Memory, behaviour, language changes

Head injury
- Subcortical problems
- Attention, memory, executive dysfunction

stroke - depends

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13
Q

Transient global amnesia (TGA)

A

sudden episode of memory loss, during which they cannot form new memories + have difficulty recalling recent memories (can remember old memories, antegrade >retrograde)
- asks repetitive questions

transient - 4-6hrs (always <24hrs)
generally a one off
rare, >50s (most 70)

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14
Q

triggers and pathophysio of Transient global amnesia (TGA)

A

triggers = emotion, changes in temp

pathophysio = transient changes in hippocampus

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15
Q

transient epileptic amnesia (TEA)

A

usually diagnosed with TGA but then have another episode -> TEA
antegrade memory - forgetful, repetitive questions

can carry out complex activities with no recollection of events

short lived - 20-30mins

Assoc with temporal lobe seizures
o 30% seizures not witnessed
*response to anti-epileptic medication should be seen
* Want to be certain be start medication – bit of watching/waiting

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16
Q

functional/subjective cognitive impairment

A

Everyday forgetfulness impacting on function
o Went upstairs + forgot why I was there
o Lost track of conversation

  • Fluctuation of symptoms

Mismatch between symptoms + reported function
 can’t remember a thing but are able to run a busy household, can work etc

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17
Q

Creutzfeldt-Jakob disease

A

rapidly progressive neurological condition caused by prion proteins

these protein induce the formation of amyloid fold resulting in tightly packed beta-pleated sheets resistant to proteases

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18
Q

prion protein in CJD

A

prion protein important for brain health

dodgy protein has domino effect on healthy proteins

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19
Q

subtypes of CJD

A

sporadic - 60s, rapid onet dementia, myoclonus, 4months duration

variant - 20s, painful sensory dissturbance, psychiatric decline, 14mnths

iatrogenic - 30s, cerebellar/visual onset, <2yrs

genetic - any age, may mimic sCJD, mutation of PRNP

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20
Q

sporadic CJD

A
  • 85% of cases
  • 10-15% of cases are familial
  • Mean age of onset is 65yrs

duration of illness = 4months

rapid onset dementia + neuro signs + myoclonus

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21
Q

hallmark histological sign of CJD

A

spongiform change (all subtypes)

small round or oval empty spaces in the neuropil. When confluent, they merge to form “morula-like” structures.

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22
Q

CJD presentation

A
  • Dementia – rapid onset
  • Myoclonus = sudden, brief involuntary twitching/jerking of muscle(s)
  • New variant CJD
    o Psychological symptoms
     Anxiety
     Withdrawals
     Dysphonia
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23
Q

new variant CJD

A

younger -25yrs

psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features

median survival 13months

24
Q

CJD investigations

A
  • CSF is usually normal
  • EEG – biphasic, high amplitude sharp waves (only in sporadic CJD)
  • MRI – hyperintense signals in the basal ganglia + thalamus
25
Q

who needs a scan?

A

CT - standard
- No if over 80 with typical Alzheimer’s history
- Helpful excluding tumour/bleed/large stroke, vascular or structural changes

MRI
o If young, fast progression or other atypical features

SPECT
o Most useful for frontotemporal, may also be used if trying to clarify Alzheimers diagnosis

DAT
o For suspected DLB/DPD when patient doesn’t have enough supporting features to be sure of a diagnosis

26
Q

Alzheimer’s disease

A

Progressive degenerative disease of the brain accounting for most dementia in UK

<65yrs = early onset
o Genetic influences
o May be atypical presentation

> 65yrs = sporadic
o Environmental > genetic influences
o Usually typical forgetful

27
Q

risk factors for alzheimers

A

increasing age
more common in women
fam history
5% inherited as autosomal dominant
caucasion

downs - onset in 3rd or 4th decade

28
Q

atypical presentation of alzheimers (15% of cases)

A

younger more likely

posterior cortical atrophy - visuospatial disturbance, commonly referred from ophth

progressive primary aphasia
- semantic(naming) - can describe all around word but not word
- logophenic (repeating)
- non-fluent (effortful)

29
Q

pathophysio of Alzheimers

A
  1. loss of cortical neurones -> cortical atrophy, widening of sulci, compensatory dilation of ventricles -> 2nd degree hydroceph
  2. neurofibrillary tangles
  3. senile plaques - extracellular protein deposits containing amyloid beta-protein

degeneration of medial hippocampus + lateral parietal lobes -> forgetfulness -> apraxia/visuospatial

30
Q

genetic influences in Alzheimers

A

autosomal dominant traits

Mutations in the –
- amyloid precursor protein (chromosome 21) – downs

  • presenilin 1 (chromosome 14)
  • presenilin 2 (chromosome 1)
    ->presenilin 1+2 are components of gamma-secretase, an enzyme that normally cleaves the APP protein

apoprotein E allele E4 – encodes for cholesterol transport protein
o boxer hit on head with more likely to get if have this allelle

31
Q

alzheimers on imaging

A

MRI – atrophy of temporal / parietal lobes

SPECT – temporoparietal decreased metabolism

CSF – decrease amyloid:increased tau ratio !!!!!!!!!!***

Research – amyloid ligand imaging

Shrinkage of hippocampus + cerebral cortex
Severely enlarged ventricles
wide sulci, narrow gyri

32
Q

alzheimers presentation

A
  • Gradual onset, decline of particularly short-term memory
  • Autobiographical and political memory often well preserved
  • Poor concentration, poor sleep, low mood
  • Personality change - disinhibited, aggression, lack of self-care
  • In end stages - hallucinations, poor dentition, skin ulcers, loss of verbal communication
  • Atypical presentations:
    • Posterior cortical atrophy - visuospatial disturbance
    • Progressive primary aphasia
33
Q

alzheimers management

A

Address vascular risk factors

ACh boosting treatments
- Cholinersterase inhibitors - rivastigmine, donepezil, galantamine
—(**not with active peptic ulcer or severe asthma/COPD)
- Mematine - NMDA receptor blocker used in moderate or severe AD or where cholinesterase inhibitors are not tolerated

34
Q

amyloid angiopathy

A

extracellular eosinophilic accumulation
polymerized beta pleated sheets formed from A-beta

stain CONGO RED

disrupts blood brain barrier - serum leaking, oedema, local hypoxia

35
Q

vascular dementia presentation

A

majority >65yrs
post-stroke
Hx of hypertension or stroke

sudden stepwise deterioration of cognitive function and not regained
insight preserved**

can include visual, sensory/motor, seizures, concentration

36
Q

vascular dementia investigation + management

A

CT - vascular changes
- white matter change, white fluffy patches in subcortical

manage vascular RF
potential cholinesterase inhibitors

CPN(community psychiatric nurse)

37
Q

core criteria of vascular dementia

A
  1. Prescence of cerebrovascular disease plus
  2. A clear temporal relationship between the onset of dementia + cerebrovascular disease

Multi-infarct
o Abrupt onset
o Stepwise progression
o History of hypertension/stroke
o Evidence of stroke on CT or MRI

38
Q

dementia with Lewy bodies

A

progressive dementia, along with hallucinations + fluctuation levels of attention/cognition

late onset - most >65yrs
assoc with parkinsons

39
Q

dementia with Lewy bodies pathophysio

A

alpha-synuclein cytoplasmic inclusions (Lewy bodies) in substantia nigra, paralimbic + neocortical areas
- pallor in substantia nigra where pigmented dopaminergic neurons run

reactive gliosis
remaining neurons may show Lewy bodies -> eosinophilic, elongated bodes that have dense core + surrounding pale halo

spread of Lewy bodies from brainstem to cortex critical

40
Q

dementia lewy bodies core criteria

A
  1. Fluctuating cognition plus
  2. Recurrent well-formed visual hallucinations
    a. Feeling someone’s behind you
  3. +/- presence of extrapyramidal features (seen in 75%)
    a. Motor features (parkinsons vibes)
41
Q

dementia with Lewy bodies presentation

A

visual hallucinations
fluctuating cognition - delirium like
REM sleep behavioural disorder
parkinsonism - not more than 1yr prior to onset of dementia

42
Q

neuroleptic association in dementia with Lewy bodies

A

Neuroleptic sensitivity
– give them haloperidol (for delirium) will make them much worse

-> history of a patient who has deteriorated following the introduction of an antipsychotic agent

43
Q

investigation dementia with lewy bodies

A

clinical diagnosis but

DaT -> reduced dopaminergic activity within the basal ganglia

44
Q

dementia with lewy bodies management

A

small dose levodopa
acetylcholinesterase inhibitors - donepsil, rivatigimine

*avoid neuroleptics (antipsychotics)

45
Q

frontotemporal dementia

A

characterised by progressive changes in character + social deterioration leading to impairment of intellect, memory + language
early onset dementia - most <65yrs

early onset dementa - most <65yrs
25% genetic cause

rapidly progressive -> mean length of illness = 7yrs (between 2-10)

assoc with MND

46
Q

frontotemporal dementia pathophysio

A

neurodegen proteinopathy
tau > TDP-43 > ubiquitin
protein aggregation ->cell damage

extreme atrophy in cerebral cortex in frontal + later in temporal lobes
brain weight <1kg

neuronal loss + gliosis

47
Q

histological hallmarks of frontotemporal dementia

A

Pick’s cells -> swollen neurons

intracytoplasmic filamentous inclusion (Pick’s bodies)

48
Q

frontotemporl dementia presentation

A

behavioral variant (60%) > primary progressive aphasia

disinhibition (impulsivity), apathy, compulsive behaviour
hyperorality - put things in mouth, may gain weight
decreased attention

early loss of insight -> collateral history important

symptoms related to damage to frontal + temporal lobes

49
Q

frontotemporal dementia investigation

A

MRI - atrophy of frontotemporal lobes

SPECT - decreased frontotemporal metabolism

CSF - INCREASED tau:normal amyloid

50
Q

management of frontotemporal dementia

A

trazadone
antipsychotics to help behavioural features

power of attorney
controlled access to food/money

MND support?

51
Q

differentiation between parkinsons + Lewy body dementia

A

DLB dementia <1yr of presentation

PDD >1yr presentation

52
Q

alcohol related dementia

A

Damage secondary to long term, excessive alcohol consumption
- Predominantly affects the frontal lobes

  • Severe = alcohol related brain injuries (ARBI)
    o If remain abstinent from 9-12 months then some degree of damage may be reversed
    -> Allow this period of abstinence before considering cognitive impairments
53
Q

huntingtons disease

A

early onset dementia 30-50yrs
autosomal dominant trait

progression to severe dependency + death over 15-20yrs

  • Genetic anticipation = the phenomenon in which each generation develops a genetic disease at an earlier age
54
Q

huntingtons pathophysio

A

expasion of the CAG trinucleotide repeat on huntingtin gene
- produces neurodegenerative protein
- CAG codes for glutamine, expansion means a long string of glutamine that is toxic to soe cells within the brain
- age of onset is assoc with repeat size

55
Q

huntingtons presentation

A

involuntary movements - gait, writhing movements, probs chewing/swallowing/speaking

depression, blunted affect, compulsions, aggresion

Dementia (later)
o Dysexecutive syndrome – difficulty planning
o Slowed speed of processing
o Eventual involvement of memory
o Associated changes in mood/personality
o Chorea = abnormal involuntary movement
o +/- later psychosis

Later clinical signs
o Rigidity
o Bradykinesia – difficulty initiating + continuing movements
o Severe chorea
o Weight loss
o Inability to walk, speak

56
Q

huntingtons investigation + management

A

genetic testing
MRI - atrophy of basal ganglia, loss of caudate heads -> butterfly appearance

mood stabilisers
drugs for chorea
HD nurse specialist

neuro (26 decks)

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