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Gwen - Neurology > Degenerative diseases - Alzheimer's, Dementia > Flashcards

Degenerative diseases - Alzheimer's, Dementia Flashcards

1
Q

What is dementia?

A

A description of a set of symptoms which show an acquired decline in memory and other cognitive functions in an alert person sufficiently severe to cause functional impairment and present for more than 6 months.

  • An acquired loss in multiple domains of higher mental function
  • A progressive decline
  • Occurs in clear consciousness
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2
Q

What are the major causes of dementia?

A
  • Alzheimer’s disease
  • Vacular dementia
  • Dementia with lewy bodies
  • Fronto-temporal dementia
  • Parkinson’s disease + dementia
  • Reversible dementias
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3
Q

Name some rare causes of dementia

A

Vit B12 deficiency,

hypothyroidism, infective (HIV, syphillis, CJD), intracranial mass (subdural haematoma, hydrocephalus, tumour), Huntington’s disease, alcohol/drug use

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4
Q

Major causes of late onset demntia (>65)

A

Alzeimer’s (55%), vascular (20%), lewy body (20%), others (5%).

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5
Q

Major causes of early onset dementia

A

Alzeimer’s (33%), vascular (15%), fronto-temporal (15%), Others (35%) - huntington’s, infection (HIV), infalmmatory (MS), toxic (alcohol)

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6
Q

What is Alzheimer’s disease?

A

A chronic neurodegenerative disease that usually starts slowly and worsens over time. It involes the build up of amyloid plaques and neurofibrillary tangles within brain tissue.

It is a primary degenerative cerebral disease

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7
Q

Pathology of alzheimer’s

A

Tempero-parietal dementia

  • Neruofibrillary tangles
  • Senile plaques (amyloid B protein)
  • Apoliprotein E4
  • Cerebral atrophy
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8
Q

What is the pathogenesis of amyloid plaques in Alzheimer’s disease?

A

Abnormal breakdown of membrane Amyloid precursor proteins (involed in neuronal growth and repair), meaning that previously soluble breakdwon products are no longer soluble (Amyloid-beta). Clumps (plaques) of beta-amyloid form in between neurons, which disrupt conduction mechanisms. Plaques also illicit an inflammatory response, and cause amyloid angiopathy, which weakens vessel walls and increase risk of haemorrhage

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9
Q

What is the pathogenesis of neurofibullary tangles in Alzheimer’s disease?

A

The presence of amyloid plaques leads to the activation of kinase, which adds phophate groups to Tau proteins in the microtubules inside the neuronal cells. This causes a conformational change in Tau protein structure, which causes them to stop supporting microtubules, break away and form neurofibrillary tangles

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10
Q

What macroscopic changes occur due to neuronal cell death in alzheimer’s disease?

A
  • Atrophy
    • Narrowing gyri
    • Widening sulci
    • Increased ventricular volume
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11
Q

What are the symptoms of alzheimer’s disease?

A

Progresses slowly over years

  • Memory disturbance - short term memory first
  • Global cognitive decline with intact personality
    • Decline in launguage,
    • Decline in executive function (planning)
    • Disorientation - temporal = visuospatial skills
  • Progresses to broad, often global cognitive dysfunction, behavioural change (eg agitation, aggression or apathy) and functional impairment (BPSD)

O/E May be increased tone

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12
Q

What is vascular dementia?

A

Dementia caused by problems in the supply of blood to the brain, typically a series of minor strokes, leading to worsening cognitive decline that occurs step by step

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13
Q

What are risk factors for vascular dementia?

A
  • DM
  • Hypertension
  • Smoking
  • Other vascular disease
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14
Q

How does vascular dementia present?

A

Problems occur in a stepwise fashion

  • Frontal lobe, extrapyramidal, pseudobulber and emotional lability is common
  • Urinary incontinence and falls without other explanation
  • Executive dysfunction may predominate
  • Gait abnormalities
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15
Q

What would you see in neuroimaging in someone with vascular dementia?

A
  • Multiple large vessel infarcts
  • White matter infarcts/periventricular white matter changes
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16
Q

What is dementia with lewy bodies?

A

A dementia syndrome that is characterized by the development of abnormal collections of (alpha-synuclein) protein within the cytoplasm of neurons (known as Lewy bodies)

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17
Q

How does dementia with lewy bodies present?

A

General

  • Deficits of attention, frontal executive, visuospatial.

Of the following, Two = probable, One = possible:

  • Fluctuations in cognitive function and alertness
  • Prominent auditory and visual hallucinations - often with paranoia and delusions
  • Parkinsonism

Commonly presents with falls due to visuospatial impairment

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18
Q

What drugs can worsen confusion in dementia with lewy bodies?

A
  • Typical antipsychotics - haloperidol
  • Levodopa
  • Dopamine
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19
Q

How could you distinguish dementia with lewy bodies from delerium?

A
  • Insidious onset
  • No underlying illness found
  • Complex hallucinations - not misrepresentation of stimuli
  • Persistent delusions
  • Antipsychotics worsen status
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20
Q

How would Parkinson’s disease with dementia present?

A
  • Typical parkinsonian motor features
  • Presentation variable - may resemble vascular, alzheimer’s or lewy body
  • Often preceded by parkinson’s
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21
Q

What is frontotemporal dementia?

A

A group of disorders caused by progressive nerve cell loss in the brain’s frontal lobes or its temporal lobes. This invariably cause deterioration in behavior and personality, language disturbances, or alterations in muscle or motor functions.

There is frontal and temporal atrophy wihtout alzeimer’s histology.

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22
Q

What are the features of fronto-temporal dementia?

A

Early onset - insidious/slow

  • Behavioural – personality change, often changes in eating habits (and eraly dysphasia)
  • Speech disorder - altered output, stereotypy, echolalia, perseveration, mutism
  • Neuropsychology - frontal dysexecutive syndrome. Memory, praxis and visuospatial function not severely impaired. Disinhibition.
  • Lack of Insight - early on

Memory and visulo-spatial is relatively preserved

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23
Q

What are the core features of dementia syndrome?

A

A - activities of daily living

B - Behavioural and Psychiatric Symptoms of dementia

C - cognitive impairment

D - Decline

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24
Q

What are the cognitive features of dementia?

A

Memory

  • Dysmnesia (memory impairment), plus one of the following:
    • Dysphasia - expressive/receptive
    • Dyspraxia - inability to carry out motor tasks
    • Dysgnosia - difficulty recognising objects
    • Dysexecutive function

Functional

  • ADL’s
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25
Q

What are the behavioural and psychiatric symptoms of dementia?

A
  • Psychosis (hallucinations and delusions)
  • Depression (and other mood disturbances) - in 30%
  • Altered circadian rhythms
  • Agitation/irritability
  • Anxiety
  • Agnosia

Personality change in 75%

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26
Q

What physical signs can be seen in dementia?

A
  • Signs of vascular disease
  • Signs of late dementia
    • Primitive reflexes
    • Global hyperreflexia
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27
Q

When performing a mental state exam for someone with suspected dementia, what would you want to exclude as differential diagnoses?

A
  • Delerium - Agitation, restlessness, poor attention, fluctuating consicousness
  • Depression - low affect, poor motivation
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28
Q

What investigations would you do in someone with dementia to look for potetnially reversible causes?

A
  • Bloods - FBC, U+E’s, B12, Folate, ESR, Calcium, LFT’s, TSH, CRP
  • ECG
  • CXR
  • Consider EEG, Neuroimaging (vascular damage, haemorrhage or structural pathology?) or LP
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29
Q

When is neuroimaging indicated in the context of a presentation of dementia?

A
  • Age < 60
  • Sudden onset/brisk decline
  • High risk of structural pathology
  • Focal CNS signs
30
Q

How does delerium differ from dementia in terms of mode of onset?

A

Delerium is acute

31
Q

How does delerium differ from dementia in terms of fluctuation?

A

Diurnal/hourly fluctuation common in delerium

Can have deterioration in dementia in the evening

32
Q

How does delerium differ from dementia in terms of hallucinations?

A

Common in delerium, whereas late sign in dementia (except lewy body dementia)

33
Q

How does delerium differ from dementia in terms of fear, agitation and aggression?

A

This is common in delerium, and is uncommon in the early stages of dementia

34
Q

How does delerium differ from dementia in terms of motor signs?

A

Tremor, myoclonus and asterixis common in delerium

Motor signs late in delerium

35
Q

How does delerium affect speech?

A
  • Dysarthric - slurred or slow speech that can be difficult to understand
  • Dynomic - fluent type of aphasia where an individual has word retrieval failures and cannot express the words they want to say
36
Q

How does delerium differ from dementia in terms of memory?

A

Short and long term affected in delerium, whereas long term memory is often spared until later on in dementia

37
Q

How would you manage someone with dementia?

A

Modify reversible factors

Medications

  • Cholinesterase inhibitors - mild to moderate
  • Memantine - moderate to severe
  • Antipsychotropics

Adequate care at home

  • Support caregivers
  • Provide extra care for ADLs
  • Carer education
38
Q

What is important to remember when managing someone with dementia?

A

Notify DVLA at diagnosis

If early dementia license may be yearly

“those with poor short term memory, disorientation or lack of insight should almost certainly not drive”

39
Q

What are risks associated with dementia?

A
  • Falls
  • Wandering
  • Aggression towards carers/family
  • Self neglect
  • Abuse towards patient
  • Financial abuse
40
Q

Name some of the acetylcholinesterase inhibitors used in dementia?

A
  • Donepezil
  • Rivastigmine
  • Galantamine
41
Q

What sort of drug is Memantine?

A

NMDA receptor blocker - reduces cholinergic neuron destruction

42
Q

How would you manage agitation/aggression in a demented patient?

A

Non medical interventions

  • Familiar environemtn
  • Avoid precipitants

Medical interventions

  • Benzodiazepines
  • SSRI’s - if depression prominent
  • Antipsychotics
43
Q

How would you assess dementia severity?

A
  • MMSE
  • Montreal Cognitive assessment
44
Q

What are classed as reversible causes of dementia?

A
  • Hypothyroidism
  • Intracerebral bleeds/tumours
  • B12 deficiency
  • Hypercalcaemia
  • Normal pressure hydrocephalus
  • Depression
45
Q

Where are changes in alzheimer’s disease normally found in the brain?

A

Neocortex and hippocampus

46
Q

What cholinesterase inhibitor is used to treat dementia with lewy bodies?

A

Rivastigmine

47
Q

What cholinesterase inhibitor can be used to treat alzheimer’s disease?

A

Donepezil

48
Q

What can galantamine be used for?

A

Mixed dementia

49
Q

What is the triad of symptoms commonly seen in normal pressure hydrocephalus?

A
  • Dementia
  • Incontinence
  • Gait disturbance (similar to parkinsons)
50
Q

What is normal pressure hydrocephalus?

A

Reversible cause of demential seen in elderly patients. Secondary to reduced CSF absorption at the arachnoid villi. Could be caused by head injury, subarachnoid haemorrage or meningitis.

Symptoms typically develop over 3 months.

Management is ventriculoperitoneal shunting.

51
Q

What is always important to remember to screen for when assessing someone with suspected dementia?

A

DEPRESSION

52
Q

What is the defintion of capacity?

A

Ability to understand information relevant to a decision or action and to appreciate the reasonably forseeable consequences of taking or not taking that action or decison

53
Q

What are the steps to assesing capacity?

A
  1. Presume capacity - always assume unless there is evidence to the contrary
  2. Maximise capacity - if lacking capcaity
  3. Bad decisions not necessarily incapable
  4. Mental disorder or severe communication disorder
54
Q

What classes of mental disorders can lack capacity?

A
  • Dementia
  • Delerium
  • Schizophrenia
  • Aphasia
  • Sensory impairment
55
Q

What are the 5 aspects of decision making that need to be present in order for someone to be deemed to have capacity?

A
  • Acting
  • Making decision
  • Understanding decisions
  • Communicating decisions
  • Retaining memory of decisions
56
Q

What is power of attorney?

A

Power granted by person (when they have the capacity to do so) to make sure that their future decisions are safeguarded, even when they lode the ability to make these decisions themselves

57
Q

What are the different types of power of attorney?

A
  • Continuing - financial only
  • Welfare - decisions about health and wellbeing
  • Combined - both financial and decisions
58
Q

What is guardianship?

A

Legal authority for someone to make decisions and act on behalf of a person who has lost capacity to make certain decisions.

May cover money, property, welfare and health

59
Q

What are the 4 main areas of cognition tested in the MMSE?

A
  • Orientation
  • Memory/Registration and Recall
  • Attention and calculation
  • Language
60
Q

What are disadvantages to using the MMSE?

A
  • Biased against people with poor education due to elements of language and mathematical testing
  • Bias against visually impaired
  • Limited examination of visuospatial cognitive ability
  • Poor sensitivity at detected mild/early dementia
61
Q

Name some of the domains of cognition

A

Memory, attention/concentration, language, visuospacial, behaviour, emotion, executive functioning, problem solving, personality

62
Q

Name 2 screening tests used for diagnosis of dementia

A

MMSE - orientation, registration, attention and calculation, recall, language, copying

MOCA

63
Q

Speed of progression clues to diagnosis

A

Rapid - CJD, tumour, infection

Stepwise - vascular

64
Q

Additional neruological signs linked to dementia

A

Abnormal movement - huntington’s

Parkinsonism - lewy body

Myoclonus - CJD

65
Q

Differentials from presentation of dementia

A

Hydrocephalus

Tumour

Depression (pseudodementia)

66
Q
A
67
Q

What is agnosia?

A

Failure to recognise places, people etc

68
Q

What is anosagnosia?

A

Lack of insight into the problems caused by the disease eg missed appointments, misunderstood plots, conversations, films, mishandling of money and clerical work

69
Q

What is apraxia?

A

Inability to carry out skilled tasks

70
Q
A

Gwen - Neurology (38 decks)

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