Cognitive Neurology (dementias) Flashcards

1
Q

What is the definition of dementia?

A

Decline in at least 1 area of cognitive function (e.g. attention, language, memory) plus interference in ADLs plus not better explained by another cause and not in context of delirium

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2
Q

Patients with viral encephalitis will be pyrexial and have cognitive dysfunction symptoms. What are 3 cognitive problems they may have?

A

memory,
behaviour change,
language

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3
Q

Approximately how long does transient global amnesia last?

A

4-6 hrs - never longer than 24 hours

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4
Q

Transient global amnesia is an acute cognitive disorder in which patients experience short period of memory loss. The patient still knows themselves but tend to be confused and repetitive. It occurs > ___ yrs old and can be triggered by ___ or ____.

A
  • > 50yrs old

- emotion or changes in temperature

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5
Q

What acute cognitive disorder presents as <30min episodes of confusion and repetitive questioning & temporal lobe seizures?

A

Transient epileptic amnesia

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6
Q

How do subacute cognitive disorders such as functional/subjective cognitive impairment tend to present? (3)

A

everyday forgetfulness,
fluctuation in symptoms ,
mismatch with symptoms & reported functioning e.g. work in high level job

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7
Q

What is a prion disease?

A

Change of prion protein in the brain and so then can’t be broken down

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8
Q

What is the most common human prion disease?

A

Creutzfeldt-Jakob disease

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9
Q

What are the 4 types of CJD in order of most common to least? Which is the quickest killer (approx. 4 months)?

A

sporadic (quickest killer!),
genetic,
iatrogenic (human growth hormone from diseased cadaver),
variant (bovine spongiform encephalopathy)

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10
Q

What type of disorder is limbic encephalitis and what are the 2 antibodies associated with it?

A

inflammatory sub-acute cognitive disorder,

VGKC (cell-surface) or anti-Hu (intraneuronal)

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11
Q

What are 2 investigations that will indicate disease in limbic encephalitis?

A

MRI/CSF inflammatory changes,

CSF/serum antibodies

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12
Q

Limbic encephalitis may be autoimmune or may be associated with paraneoplastic syndrome. What are the treatments?

A

tumour treatment if paraneoplastic

and immunsuppressive treatment

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13
Q

What is the abnormal protein in Alzheimer’s disease?

A

Amyloid and so build up amyloid plaques

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14
Q

In Alzheimer’s disease usual initial presentation is forgetfulness due to degeneration of parietal lobes first then the medial hippocampus. True/false?

A

False - first medial hippocampus followed by parietal lobes

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15
Q

Name two atypical presentations of Alzheimer’s and how they present?

A
  • primary cortical atrophy presents with visuospatial disturbances
  • progressive primary aphasia presents with language disturbances e.g. semantic (naming)
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16
Q

What investigations would you carry out in alzheimers?

A

MRI for temporal/parietal atrophy,
SPECT for decreased temporoparietal metabolism,
CSF for decreased amyloid:increased tau ratio (marker of degeneration),
Amyloid ligand imaging

17
Q

Acetylcholine boosting drugs may be used in Alzheimers. What are the 2 main types of these and name one of each?

A

cholinesterase inhibitors e.g. donepezil, rivastigmine,

NDMA receptor blocker e.g. memantine

18
Q

What is the most common protein to change in Frontotemporal dementia?

A

Tau

19
Q

Which type of dementia usually develops <65 years old and initially with early frontal features e.g. loss of empathy, disinhibition, hyperorality and with early loss of insight?

A

frontotemporal demetia

20
Q

Approx. what percentage of frontotemporal dementia is genetic and what other condition is it associated with?

A
  • 25%

- MND

21
Q

What are the investigations carried out in frontotemporal dementia? (3)

A
  • MRI for frontotemporal atrophy
  • SPECT for decreased frontotemporal metabolism
  • CSF for increased tau:normal amyloid
22
Q

What are treatment options for frontotemporal dementia?

A
  • trial of trazadone/antipsychotics
  • safety management
  • power of attorney
  • family support
23
Q

What are the 2 core criteria in diagnosing vascular dementia?

A
  • presence of cerebrovascular disease

- clear temporal relationship between onset of dementia and cerebrovascular disease

24
Q

How does subcortical vascular dementia present?

A
  • decreased attention
  • executive dysfunction
  • slowed processing
25
Q

Strokes increase the risk of getting vascular dementia by 9 times. True/false?

A

true

26
Q

What is management for vascular dementia?

A
  • vascular risk factors
  • cholinesterase inhibitors if amyloid pathology present
  • community psych nurse
27
Q

What type of gradual onset dementia is caused by alpha-synuclein aggregates building up and disrupting cholinergic and dopaminergic pathways?

A

Lewy body dementia

28
Q

What are the 3 core criteria for diagnosing Lewy body dementia?

A

fluctuating cognition,
recurrent visual hallucinations,
presence of extra-pyramidal signs

29
Q

What treatment can be offered in Lew body dementia?

A
  • small dose levodopa

- cholinesterase inhibitor

30
Q

What other type of dementia overlaps clinically and pathologically with Parkinson’s Disease dementia?

A

Dementia with Lewy Bodies

31
Q

What are the two types of early onset dementia?

A
  • frontotemporal dementia

- Huntington’s disease

32
Q

How does Huntington disease present?

A
  • dementia mostly frontotemporal signs first
  • changes in mood/personality
  • chorea
  • later psychosis
33
Q

What occurs on the Huntington gene to cause Huntington’s disease?

A

repeats of the CAG trinucleotide

34
Q

Huntington disease is diagnosed with the finding of loss of caudate heads on MRI. True/false?

A

False. Diagnosis is clinical and genetic testing, MRI is additional but not essential

35
Q

What is Addenbrooke’s Cognitive Assessment used for and what disease is it not useful for?

A
  • cognitive assessment

- doesn’t show frontotemporal dementias

36
Q

Score of __ or less on ACE III score highlights cognitive impairment.
Score of __ or less on FAB score highlights cognitive impairment.

A
  • 88 or less for ACE III

- 12 or less for FAB