Dementia Flashcards

1
Q

Degeneration in the cerebral cortex leads to what?

A

Alzheimers
Picks disease
Creutzfeldt jakob disease

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2
Q

Degeneration in the Basal Ganglia and brain stem leads to what?

A

Parkinsons
Multi System atrophy
Huntingtons

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3
Q

Degeneration in the spinocerebellar region leads to what?

A

Spinocerebellar ataxia

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4
Q

Degeneration in the motor neurones leads to what?

A

Motor Neurone Disease

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5
Q

What is dementia?

A

Pathological acquired and persistent generalised disturbance in higher mental function.

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6
Q

List some primary causes of dementia.

A

Alzheimers
Huntingtons
Picks disease

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7
Q

List some secondary causes of dementia.

A
Multi infarct dementia
Infection
Trauma
Drugs
Toxins
Vitamin deficiencies
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8
Q

Epidemiology of Alzheimer’s

A

Most common dementia

F:M 2:1

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9
Q

What are some genetic risk causes of Alzhiemer’s?

A

Amyloid Precursor Protein

Presenilin 1 and 2

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10
Q

In what condition is there an increased risk of Alzhiemer’s?

A

Trisomy 21 - Down syndrome

Presence of Amyloid Precursor Protein

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11
Q

What is the cause of death in most Alzheimer’s cases?

A

Secondary cause due to insidious impairment of higher function

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12
Q

What does the brain of someone with Alzheimer’s look like?

A
Decreased size and weight 
Cortical Atrophy - Frontotemporal and Parietal 
Widened Sulci 
Narrowed gyro
Compensatory ventricle dilation
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13
Q

Compensated ventricular dilation is a cause of what in Alzheimers?

A

Secondary Hydrocephalus

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14
Q

On a microscopic level what does the brain tissue of someone with Alzheimer’s show?

A

Simple neuronal atrophy
Gliosis
Neurofibrillary tangles
Neuritic plaques

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15
Q

What are neurofibrillary tangles?

A

Microtubules within the cytoplasm contain Tau proteins

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16
Q

What make neuritic plaques?

A

Aβ amyloid plaques

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17
Q

What produces Aβ amyloid?

A

Cleavage of the Amyloid precursor protein

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18
Q

Describe the clinical presentation and history of someone with levy body dementia.

A

Fluctuating levels of attention/cognition
Hallucinations
Late onset memory loss
Motor features of Parkinsonism

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19
Q

Conditions affecting where in the brain will result in Parkinsonism?

A

Nigro-striatal dopaminergic pathways

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20
Q

What are lewy bodies?

A

Eosinophilic dense body surrounded by a halo of radiating fibrils

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21
Q

What causes lewy body dementia?

A

Degeneration of substantia nigra

Dopaminergic pathways are broken down

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22
Q

The brain of someone with lewy body dementia will show.

A

Pallor where pigmented substantia nigra was located.

Reactive gliosis

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23
Q

What is hunitingtons disease?

A

Inherited Autosomal dominant disease leading to rapidly progressive motor and cognitive disturbances.

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24
Q

What are the main symptoms of Huntington’s?

A
Chorea
Myoclonus
Clumsiness
Slurred speech
Depression
Irritability
Apathy
Dementia later on
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25
Q

How many CAG repeats are present in normal population?

A

<28

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26
Q

How many CAG repeats are present when the disease is expressed?

A

> 35

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27
Q

What regions of the brain undergo atrophy? HD

A

Basal Ganglia
Caudate nucleus
Putamen
Later fronto-parietal

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28
Q

On a microscopic level how are the tissues of the brain affected by Huntingtons?

A

Neuronal atrophy of striatal neurones within Basal ganglia

Astrocytic gliosis

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29
Q

Loss of striatal neurones within basal ganglia results in what?

A

Loss of motor inhibition

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30
Q

Frontotemporal dementia is also known as….

A

Picks disease

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31
Q

What is Picks disease?

A

Progressive dementia with onset in middle life around 50-60 years

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32
Q

What are the main symptoms of Picks disease?

A

Personality and behavioural changes
Speech and communication issues
Eating habits change
Reduced attention span

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33
Q

What is the mean time from onset of symptoms to death in Picks disease?

A

7 years

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34
Q

What does the brain of someone with Picks disease look like?

A

Extreme atrophy of cerebral cortex
Neuronal loss and gliosis
Picks cells

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35
Q

What are pick cells?

A

Swollen neurones

Intracytoplasmic filaments inclusion (Picks bodies)

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36
Q

Describe someone who classical presents with Multi Infarct Dementia.

A

> 60 male with history of hypertension

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37
Q

What is the pathology of Multi Infarct Dementia?

A

Succesive cerebral infarction lead to a growing area of cell death and damage leading to cognitive dysfunction.

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38
Q

At what volume of brain destruction does Multi Infarct Dementia present?

A

50-100mls of brain tissue

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39
Q

Why are people suffering from of Multi Infarct dementia more prone to suffer depression and anxiety?

A

Due to stepwise progression of the disease they are far more aware of their own cognitive deficits.

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40
Q

How can you differentiate Multi Infarct Dementia from Alzheimer’s?

A

Abrupt onset with a stepwise progression
History of hypertension of strokes
Evidence of a stroke on CT or MRI

41
Q

A large vessel infarct will usually affect..

A

A whole hemisphere

42
Q

A small vessel infarct will usually affect…..

A

Central subcortical distribution

43
Q

What is the usual cause of a small vessel infarct?

A

Longstanding hypertension

Arteriosclerosis

44
Q

What is the usual cause of large vessel infarcts?

A

Atheroma

45
Q

What is Prion disease an example of?

A

Creutzfeldt jakob Disease

46
Q

Creutzfeldt Jakob Disease are examples of what?

A

Neurodegenerative Proteinopathy

47
Q

What are the four subtypes of Prion disease?

A

Sporadic
Varient
Iatrogenic
Genetic

48
Q

Describe Sporadic Prion disease.

A

Onset in 60’s
Rapid onset dementia
4 months to death

49
Q

Describe Varient Prion disease/

A

Onset in the 20’s
Painful sensory disturbances
Neuropsychiatric decile
14 months to death

50
Q

What are some causes of Variant prion Disease?

A

Exposure to BSE

Bovin Spongiform Encephalopathy

51
Q

Describe Iatrogenic Prion disease.

A

Onset in the 30’s
Cerebellar or visual onset
Multifocal neurological decline
>2 years to death

52
Q

What are some causes of Iatrogenic Prion disease.

A

Exposure to hGH (Human Growth Hormone)

less common now

53
Q

Why is Iatrogenic Prion disease caused by hGH less common now?

A

hGH is no longer extracted from cadavers but produced in a lab.

54
Q

What is Limbic Encephalitis ?

A

Inflammatory antibody mediated encephalitis

55
Q

What are the symptoms of Limbic Encephalitis?

A

Short term memory deficits
+/- seizures
+/- behavioural changes

56
Q

A CSF sample of someone with limbic encephalitis will show?

A

Antibodies

57
Q

What can limbic encephalopathy be linked to?

A

Underlying malignancy or autoimmune condition

58
Q

What is the treatment for Limbic Encephalopathy?

A

Treatment of tumour if present

Immunosuppression

59
Q

What causes Alzhiemer’s?

A

Disruption of cholinergic pathway and synaptic loss

60
Q

How do extracellular Amyloid plaques cause Alzheimer’s?

A

Disrupt normal cell function

Induce Apoptosis

61
Q

How do intracellular neurofibrillary tangles cause the symptoms in Alzheimer’s?

A

Inhibit the cytoskeleton

Cell death

62
Q

If someone presents with Alzheimer’s before 65 what is its likely aetiology?

A

Genetic

63
Q

How does a genetic Alzheimers present?

A

Atypical presentation

64
Q

What is an atypical presentation of Alzheimers?

A

Visuospatial disturbances

Primary progressive aphasia

65
Q

What causes visuospatial disturbances?

A

Posterior Cortical Atrophy

66
Q

What types of Primary Progressive Aphasia are there?

A

Lopogenic - Difficuilty thinking of words
Semantic - Naming
Non fluent - effortful speaking

67
Q

If someone presents with Alzheimers after 65 what is its likely aetiology?

A

Sporadic

Environmental > Genetic

68
Q

How does a sporadic Alzheimers present?

A

Usual pattern off forgetfulness.

69
Q

An MRI of Alzheimers shows….

A

Atrophy of temporal and parietal lobes

70
Q

A SPECT of an Alzheimers patient will show…

A

Reduced temperoparietal metabolism

71
Q

What does the CSF of an Alzheimers patient show?

A

Decreased amyloid

Increase TAU ratio

72
Q

What investigations are undertaken in a suspected Alzheimers case?

A

MRI
SPECT scan
CSF sample
Amyloid ligand imaging

73
Q

What is the treatment for Alzheimers?

A

Acetylcholine boosters - Cholinesterase inhibitor

NMDA blocker

74
Q

Give an example of an NMDA blocker used in Alzheimers?

A

Memantine

75
Q

Give an example of a Cholinesterase inhibitor used in Alzheimers?

A

Rivastigmine

Donepezil

76
Q

In an Alzheimers patient what else should be treated?

A

Address any vascular risk factors

77
Q

When does Frontotemporal dementia usually present?

A

Early <65

78
Q

What is the usual cause of Frontotemporal dementia?

A

Neurodegenerative proteinopathy

Aggregation of protein leads to cell death

79
Q

List three proteins in order of occurrence that can cause a frontotemporal dementia.

A

TAU
TDD-43
Ubiquitin

80
Q

What are the three presentations of a frontotemporal dementia?

A

Behavioural variant
Early frontal features
Early loss of insight

81
Q

What behavioural variant is common in frontotemporal dementia?

A

Primary progressive aphasia

82
Q

What are the frontal features that present in frontotemporal dementia?

A

Disinhibition
Lack of apathy
Loss of empathy
Compulsive behaviours

83
Q

What is needed in order to assess whether a patient has lost their insight into oneself?

A

A collateral history

84
Q

What investigations are undertaken in a suspected Frontotemporal dementia?

A

MRI
CSF
SPECT

85
Q

What is to be seen on an MRI with frontotemporal dementia?

A

Atrophy of frontal lobes

86
Q

What is to be found within the CSF of a patient with frontotemporal dementia?

A

raised TAU

Normal amyloid

87
Q

What is to be seen on a SPECT with frontotemporal dementia?

A

Reduced frontal and temporal lobe metabolism

88
Q

What is the management for Frontal Temporal Dementia?

A

Trazodone / Antipsychotics for behavioural features
Safety Management - access to money internet etc
Structured activities
Power of attorney
Attached a specialist nurse

89
Q

A subcortical presentation of Vascular dementia due to small vessel infarction will present with?

A

Reduced attention
Executive dysfunction
Slowed processing

90
Q

What is the management of Vascular Dementia?

A

Vascular risk factors are treated.

+/- cholinesterase inhibitors

91
Q

What investigations are undertaken in Lewy body dementia?

A

DaT- Dopamine transport imaging
Alpha synuclein ligand imaging
CSF - alpha synuclein in CSF

92
Q

What is the treatment for lewy body dementia?

A

Low dose Levodopa

Cholinesterase inhibitors

93
Q

Someone with Parkinson’s Disease Dementia presents with these physical symptoms.

A

Bradykinesia, Rigidity, Tremor

94
Q

Someone with parkinsons disease dementia present with these cognitive symptoms.

A
Dementi
Reduced attention
Slowness of processing 
Impaired visuospatial function
Hallucinations
95
Q

What is the management for Parkinsons Disease Dementia?

A

Small does Levodopa

Cholinesterase inhibitors

96
Q

What is the management for Huntingtons?

A

No curative options
Mood stabilisers
Nurse specialist

97
Q

If someone is over 65 with no additional neurology where are they referred?

A

old age psychiatry

98
Q

If someone is under 65 with unusual features where are they referred?

A

Neurology