Week 6 Flashcards

1
Q

What are the 3 ways to divide progressive dementia?

A
  1. cortical dementia
  2. subcortical dementia
  3. mixed dementia
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2
Q

What is the most prevalent dementia in the population?

A

AD

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3
Q

Who is clinical evaluation of dementia usually done by?

A

Geriatrician, neurologist, medical practitioner. Clinical neuropsychologist opinions is essential.

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4
Q

___ diagnosis is important in dementia

A

Early

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5
Q

Information obtained to make a dementia diagnosis includes:

A
  • present history
  • past/social history
  • cognitive assessment
  • brain imaging
  • other medical testing
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6
Q

What is one advantage of early diagnosis in dementing cognitions

A
  • provide a diagnostic answer and education for the patient and family
  • provide strategies
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7
Q

Instead of ‘dementia’, what is usually diagnosed according to the DSM 5?

A

neurocognitive disorder

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8
Q

What is the first diagnostic criteria in major neurocognitive disorder?

A

evidence of significant cognitive decline from a previous level of performance

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9
Q

What is the second diagnostic criteria in major neurocognitive disorder?

A

Impairment needs to interfere with independence in everyday activities

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10
Q

What is the third diagnostic criteria in major neurocognitive disorder?

A

Cognitive deficits do not occur exclusively in the context of a delirium

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11
Q

What is the fourth diagnostic criteria in major neurocognitive disorder?

A

Cognitive deficits are not better explained by another rental disorder (e.g., MDD or schizophrenia)

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12
Q

What is the next step in diagnosing major neurocognitive disorder?

A
  1. specify type of dementia
  2. specify with or without behavioural disturbance
  3. specify if it produces milk difficulties with instrumental activities of daily living
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13
Q

What is cortical dementia?

A

Characterised by preferential neuronal loss to cortical regions of the brain

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14
Q

Involvement of the subcortical regions in dementia is rare, or if it does occur it occurs in:

A

late stages of the disease

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15
Q

What are some risk factors for dementia in early life?

A

less education (7%)

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16
Q

What are some risk factors for dementia in midlife?

A
  • hearing loss
  • hypertension
  • alcohol
  • obesity
  • TBI
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17
Q

What are some risk factors for dementia in later life?

A
  • smoking
  • depression
  • social isolation
  • diabetes
  • physical inactivity
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18
Q

Because of associated risk factors with dementia throughout life, what percentage of dementia is potentially modifiable?

A

40%

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19
Q

What percentage accounts for the risk unknown in dementia onset?

A

60%

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20
Q

Who first described Alzheimers disease?

A

Alois Alzheimer

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21
Q

How do we refer to AD as today?

A

an irreversible cortical progressive dementia

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22
Q

What were the symptoms of the first ever AD patient?

A
  • hiding things
  • severe behaviour change
  • suspicious of husband
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23
Q

There is no single identifiable cause for AD. However:

A
  1. incidence increases with age (strongest predictor)

2. twice as many women develop AD

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24
Q

Average duration of AD is what?

A

8-10 years

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25
Q

What is the mean age of sporadic AD?

A

mean age of onset of 80 years

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26
Q

Familial Alzheimer’s is rare, but early onset AD at:

A

More than 50 years of age

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27
Q

What are the two key features of AD?

A
  1. the disease target specific regions of the brain

2. the disease targets structures which then sustain massive cell loss

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28
Q

Cortical atrophy in AD patients are more evident in:

A

frontal, temporal and parietal lobes

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29
Q

Cortical ___ results in ____ in AD patients:

A

thinning

ventricular enlargement

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30
Q

What are two histological markers of AD in the brain:

A
  1. amyloid/senile plaques

2. neurofibrillary tangles (TAU protein)

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31
Q

Neurofribillary tangles (as associated with AD) appear throughout the brain, but are disproportionately concentrated in which areas?

A

temporal parietal areas and hippocampal complex

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32
Q

Where are senile plaques (as involved in AD) mainly located?

A

most likely to concentrate in frontal and temporal regions of the brain and in the hippocampal complex

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33
Q

What are some signs and symptoms with the onset of AD?

A
  • failing recent memory
  • depression and irritability
  • occasionally a seizure
  • one or more language problems
  • confusion when sudden disruption to work or social life occurs
34
Q

As AD progresses, what happens to various cognitive symptoms?

A
  • apraxias become increasingly prominent
  • agnosias become increasingly evident
  • disfluency becomes more apparent in speech
  • in end stage, neuropsychological functions can no longer be measured. Primitive reflexes reemerge.
35
Q

The progression of AD resembles a ___ of development from ____. With __ being lost first and a progression to a ___ state.

A

reversal
infancy to childhood
higher level skills
reflexive

36
Q

In combination with cognitive changes, there are a range of ___ and ___ changes in AD patients

A

behavioural

personality

37
Q

What are some behavioural and personality changes associated with AD?

A
  • clinging to caregiver
  • poor self care
  • depression
  • wandering
  • agitation
  • suspiciousness and paranoia
38
Q

Frontal lobe dementia typically affects which areas of the brain?

A

atrophy in the frontal and temporal lobes

39
Q

The cerebellum and ___ are unaffected in frontal lobe demential patients.

A

brainstem

40
Q

What are the two subtypes of frontotemporal dementia?

A
  1. behavioural variant frontotemporal dementia

2. primary progressive aphasia

41
Q

What are the 2 different types of PPA?

A
  • semantic dementia
  • logopenic aphasia
  • progressive non fluent aphasia
42
Q

What are one of the first signs of frontal lobe dementia

A

silliness, socially disinhibited behaviour, poor judgemnet

43
Q

What are the middle stages of frontal lobe dementia characterised by?

A

blunted affect, progressive apathy, significant cognitive function

44
Q

What can we expect to see in the late stages of frontal lobe dementia?

A

mute and display motor rigidity. Typically ends in terminal vegetative state.

45
Q

What is the typical disease duration of frontal lobe dementia?

A

2-17 years

46
Q

Frontal lobe dementia affects what percentage of all cases of dementia?

A

12%

47
Q

Huntingtons disease is a ____ dementia.

A

progressive subcortical

48
Q

HD affects ___ people in 100, 000

A

5-10

49
Q

Is there a known treatment or cure for HD?

A

no

50
Q

What gene is HD linked to?

A

ITI5 on chromosome 4

51
Q

Onset of HD symptoms usually when what age?

A

30-40

52
Q

Survival is how many years following onset of symptoms?

A

10-20

53
Q

HD is always what in terms of outcome?

A

fatal

54
Q

Who first described HG?

A

George Huntington aged 22 years

55
Q

HD is a hereditary condition, the only known autosomal dominant condition. What does this mean?

A

Affects 50% of children with one parent with the disorder

56
Q

What is Huntingtons disease also called, Huntingtons Chorea?

A

symptoms of involuntary, spasmodic, often tortuous movements that ultimately become disabling.

57
Q

On average, if one parent has the Huntingtons disease and one doesn’t:

A

2 out of 4 children will get the disease

58
Q

If one has the HD illele:

A

they WILL develop HD

59
Q

Regarding the neuropathology of HD, primary neuropathological change is bilateral deterioration of the ___

A

caudate nucleus - deterioration of multiple systems involving white matter structures and areas of the cerebellum

60
Q

The caudate nucleus is a component of the ____. It is involved in:

A

basal ganglia

timing, ordering, sequencing of movement patters

61
Q

Caudate sends projections to and from the ___ and ___ areas of the brain.

A

Limbic

prefrontal

62
Q

By the end of HD, what percentage of frontal lobe shrinkage volume is there?

A

20-30%

63
Q

HD may also affect other areas of the basal ganglia such as:

A

putamen, areas of the striatum and other limbic structures.

64
Q

What are some aspects of the cognitive profile in HD?

A
  • eye movements
  • decreased attention span
  • memory deficits
  • deteriorating speech production
  • impaired visuo-spatial orientation
  • impaired behaviour regulation
  • planning and organisation deficits
65
Q

What is the second largest type of dementia?

A

multi-infarct (vascular) dementia

66
Q

Multi-infarct dementia results from:

A

Multiple infarction of brain tissue, from repeated strokes or blockages to blood vessels.

67
Q

There are multiple ways that multi infarct dementia differs from AD. What are some of these?

A
  • onset is acute and rapid
  • history of risk factors for stroke
  • better memory sometimes than AD
  • reduced verbal production
  • cognitive deficits precede personality changes
  • frontal compromise earlier
68
Q

Vascular dementia is heterogenous. what does this mean?

A

The deficits depend on the location of damage

69
Q

What is Creutzfeldt Jakob Disease?

A
  • extremely rare
  • rapid and progressive
  • brain turns into a spongelike consistency, holes appear
  • present in sheep, cows, humans
70
Q

In the Fore people of New Guinea (cannibals), how is Creutzfeldt Jakob disease transmitted?

A

tradition of eating their dead. Females and children typically eat the brains, injecting infectious diseases

71
Q

What is sporadic CJD?

A

most common, occurs despite no risk factors being apparent

72
Q

What is variant CJD (mad cow disease)

A

infection via tainted meat containing neural tissue

73
Q

What is familial CJD?

A

extremely rare, only found in a handful of families, results in fatal insomnia

74
Q

What is iatrogenic CJD?

A

transmitted via neural transplants, or other medical procedures

75
Q

Why is CJD hard to identify?

A
  • doesn’t produce unusual symptoms
  • slow virus
  • camouflaged by cells
76
Q

CJD targets the:

A

cerebellum as well as the cerebrum

77
Q

Astrogliosis occurs as a ____ of the CJD not the ___

A

result

cause

78
Q

Mertz identified CJD in sheep and found:

A

twisted sticklike fibres in cells using electron microscope. Unique to CJD

79
Q

What are the hallmarks of CJD?

A
  • uncoordinated movements
  • druken stagger
  • speech is slurred and inarticulate
  • victim cannot swallow and may die of starvation
  • visual function alters
80
Q

Emotional and cognitive symptoms are seen in CJD are:

A
  • mood disorders
  • fatigue
  • sleeping difficulties
  • attention and concentration problems