Cognitive Neurology Flashcards

1
Q

Define cognition

A

The mental action of acquiring knowledge and understanding through thought, experience and senses

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2
Q

Define dementia

A

The un-doing of the mind - generally progressive and neurodegenerative

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3
Q

What is the criteria for ‘dementia’?

A
  1. evidence of a significant decline in at least one domain
    + deficits interfere with independence
    + not explained by another progress/exclusively delirium
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4
Q

Name three acute cognitive disorders

A
  • focal injury
  • transient global amnesia
  • transient epileptic amnesia
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5
Q

Name three focal injuries that can cause cognitive disorders

A

Viral encephalitis - memory, behaviour, pyrexic -frontal lobe
Head injury - attention, memory, executive dysfunction - subcortical or frontal lobe
Stroke - depends on area

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6
Q

What is transient global amnesia?

A

Abrupt onset antegrade amnesia - difficulty laying down new memories but preserved knowledge of self

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7
Q

Who is usually affected by transient global amnesia?

A

> 50 years old usually in 70s, lasts less than 24 hours but triggers can be change in temperature or emotion

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8
Q

What is transient epileptic amnesia?

A

Forgetful with repetitive questioning - can carry out complex activities with no recollection of the event

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9
Q

How long does transient epileptic amnesia last?

A

20 minutes with associated temporal lobe seizures

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10
Q

Name three sub-acute cognitive disorders

A
  • functional impairment
  • prion disease
  • limbic encephalitis
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11
Q

What is functional impairment?

A

Everyday forgetfulness impacting on functioning, can lead to a fugue state - usually has fluctuation of symptoms with a mismatch of symptoms and reported function/neurodegenerative disorders

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12
Q

What is the most common prion disease?

A

Creutzfeldt Jakob Disease

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13
Q

Describe the pathology of prion disease

A

Change of prion protein in the brain, misfolded protein cannot be broken down so builds up leading to neurodegeneration

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14
Q

What are the four types of prion disease?

A
  • sporadic
  • variant
  • iatrogenic
  • genetic
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15
Q

Describe sporadic prion disease

A

60 years old, rapid onset dementia with neurological signs, myoclonus. 4 month duration.

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16
Q

Describe variant prion disease

A

20 years old, painful sensory disturbance and neuropsychiatric decline. 14 months duration due to exposure to BSE (mad cow disease) or contaminated blood

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17
Q

Describe iatrogenic prion disease

A

30 years old, cerebellar/visual onset with multifocal neurological decline. <2 years duration occurs due to CJD infection from human growth hormone or dura mater from infected cadavers

18
Q

Describe genetic prion disease

A

Can present at any age, mutation in the gene that produces prion protein has a variable duration. Associated with fatal familial insomnia

19
Q

How is prion disease diagnosed?

A

EEG, MRI, biomarkers - all will show spongiform changes

20
Q

What is limbic encephalitis?

A

Inflammatory autoimmune disease

  • short term deficits
  • seizures
  • behavioural changes
21
Q

What antibodies can be involved in limbic encephalitis?

A

Cell surface - VGKC

Intraneuronal - anti HU

22
Q

What can cause limbic encephalitis and how is it treated?

A

Paraneoplastic syndrome - treat tumour

Autoimmune - immunosuppression

23
Q

What investigations are done in limbic encephalitis?

A

MRI - changes in temporal lobe

CSF - protein and WCC

24
Q

Name six gradual onset disorders

A
  • Alzheimer’s
  • Fronto-temporal dementia
  • Vascular dementia
  • Lewy body dementia
  • Parkinson’s disease dementia
  • Huntington’s disease
25
Q

What is the initial symptom usually in Alzheimer’s?

A

Forgetfulness

26
Q

Where does degeneration occur in alzheimer’s?

A

Hippocampus and parietal lobes

27
Q

What is classed as early onset alzheimers?

A

<65 years old, often with atypical presentations

28
Q

Name two atypical presentations of alzheimers

A

Posterior cortical atrophy - visuospatial disturbance

Primary progressive aphasia - language dominant

29
Q

How is alzheimer’s investigated?

A

MRI for atrophy
CSF for increased TAU and decreased amyloid
SPECT/PET reduced metabolism

30
Q

When does frontotemporal dementia usually present?

A

<65 years old, early onset

31
Q

How does frontotemporal dementia present?

A
  • behavioural changes
  • primary progressive aphasia
    Early frontal features - disinhibition, apathy, compulsive behaviours
32
Q

What investigations are required in frontotemporal dementia?

A

MRI - frontal atrophy
SPECT - decreased metabolism
CSF - increased TAU

33
Q

How can frontotemporal dementia be treated?

A

Trazadone, antipsychotics, safety management, family support

34
Q

What is the criteria for vascular dementia diagnosis?

A
  1. presence of cerebrovascular disease

2. clear temporal relationship between dementia and vascular disease

35
Q

What are the two types of vascular dementia?

A
  • subcortical (small vessel disease)

- stroke

36
Q

How is vascular dementia managed?

A

Manage risk factors and may need a cholinesterase inhibitor

37
Q

When does Lewy Body dementia present?

A

Late onset

38
Q

What is the criteria for Lewy Body dementia?

A
  1. Fluctuating cognition
  2. Recurrent well formed visual hallucinations
  3. Presence of extrapyramidal features - 75% (TRAP)
39
Q

How is lewy body dementia treated?

A

Small dose levodopa

Cholinesterase inhibitor

40
Q

What is the difference between Parkinson’s and lewy body dementia?

A

> 1 year of pre-existing motor symptoms

41
Q

What age does huntington’s present?

A

30-50 years old

42
Q

What are the symptoms for Huntington’s?

A

Dysexecutive syndrome - frontal lobe type, slowed speed of processing and eventually memory involvement
Mood and personality changes, chorea and psychosis