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Neurology Year 3 > Dementia > Flashcards

Dementia Flashcards

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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
How many CAG repeats are present in normal population?
<28
26
How many CAG repeats are present when the disease is expressed?
>35
27
What regions of the brain undergo atrophy? HD
Basal Ganglia Caudate nucleus Putamen Later fronto-parietal
28
On a microscopic level how are the tissues of the brain affected by Huntingtons?
Neuronal atrophy of striatal neurones within Basal ganglia | Astrocytic gliosis
29
Loss of striatal neurones within basal ganglia results in what?
Loss of motor inhibition
30
Frontotemporal dementia is also known as....
Picks disease
31
What is Picks disease?
Progressive dementia with onset in middle life around 50-60 years
32
What are the main symptoms of Picks disease?
Personality and behavioural changes Speech and communication issues Eating habits change Reduced attention span
33
What is the mean time from onset of symptoms to death in Picks disease?
7 years
34
What does the brain of someone with Picks disease look like?
Extreme atrophy of cerebral cortex Neuronal loss and gliosis Picks cells
35
What are pick cells?
Swollen neurones | Intracytoplasmic filaments inclusion (Picks bodies)
36
Describe someone who classical presents with Multi Infarct Dementia.
>60 male with history of hypertension
37
What is the pathology of Multi Infarct Dementia?
Succesive cerebral infarction lead to a growing area of cell death and damage leading to cognitive dysfunction.
38
At what volume of brain destruction does Multi Infarct Dementia present?
50-100mls of brain tissue
39
Why are people suffering from of Multi Infarct dementia more prone to suffer depression and anxiety?
Due to stepwise progression of the disease they are far more aware of their own cognitive deficits.
40
How can you differentiate Multi Infarct Dementia from Alzheimer's?
Abrupt onset with a stepwise progression History of hypertension of strokes Evidence of a stroke on CT or MRI
41
A large vessel infarct will usually affect..
A whole hemisphere
42
A small vessel infarct will usually affect.....
Central subcortical distribution
43
What is the usual cause of a small vessel infarct?
Longstanding hypertension | Arteriosclerosis
44
What is the usual cause of large vessel infarcts?
Atheroma
45
What is Prion disease an example of?
Creutzfeldt jakob Disease
46
Creutzfeldt Jakob Disease are examples of what?
Neurodegenerative Proteinopathy
47
What are the four subtypes of Prion disease?
Sporadic Varient Iatrogenic Genetic
48
Describe Sporadic Prion disease.
Onset in 60's Rapid onset dementia 4 months to death
49
Describe Varient Prion disease/
Onset in the 20's Painful sensory disturbances Neuropsychiatric decile 14 months to death
50
What are some causes of Variant prion Disease?
Exposure to BSE | Bovin Spongiform Encephalopathy
51
Describe Iatrogenic Prion disease.
Onset in the 30's Cerebellar or visual onset Multifocal neurological decline >2 years to death
52
What are some causes of Iatrogenic Prion disease.
Exposure to hGH (Human Growth Hormone) | less common now
53
Why is Iatrogenic Prion disease caused by hGH less common now?
hGH is no longer extracted from cadavers but produced in a lab.
54
What is Limbic Encephalitis ?
Inflammatory antibody mediated encephalitis
55
What are the symptoms of Limbic Encephalitis?
Short term memory deficits +/- seizures +/- behavioural changes
56
A CSF sample of someone with limbic encephalitis will show?
Antibodies
57
What can limbic encephalopathy be linked to?
Underlying malignancy or autoimmune condition
58
What is the treatment for Limbic Encephalopathy?
Treatment of tumour if present | Immunosuppression
59
What causes Alzhiemer's?
Disruption of cholinergic pathway and synaptic loss
60
How do extracellular Amyloid plaques cause Alzheimer's?
Disrupt normal cell function | Induce Apoptosis
61
How do intracellular neurofibrillary tangles cause the symptoms in Alzheimer's?
Inhibit the cytoskeleton | Cell death
62
If someone presents with Alzheimer's before 65 what is its likely aetiology?
Genetic
63
How does a genetic Alzheimers present?
Atypical presentation
64
What is an atypical presentation of Alzheimers?
Visuospatial disturbances | Primary progressive aphasia
65
What causes visuospatial disturbances?
Posterior Cortical Atrophy
66
What types of Primary Progressive Aphasia are there?
Lopogenic - Difficuilty thinking of words Semantic - Naming Non fluent - effortful speaking
67
If someone presents with Alzheimers after 65 what is its likely aetiology?
Sporadic | Environmental > Genetic
68
How does a sporadic Alzheimers present?
Usual pattern off forgetfulness.
69
An MRI of Alzheimers shows....
Atrophy of temporal and parietal lobes
70
A SPECT of an Alzheimers patient will show...
Reduced temperoparietal metabolism
71
What does the CSF of an Alzheimers patient show?
Decreased amyloid | Increase TAU ratio
72
What investigations are undertaken in a suspected Alzheimers case?
MRI SPECT scan CSF sample Amyloid ligand imaging
73
What is the treatment for Alzheimers?
Acetylcholine boosters - Cholinesterase inhibitor | NMDA blocker
74
Give an example of an NMDA blocker used in Alzheimers?
Memantine
75
Give an example of a Cholinesterase inhibitor used in Alzheimers?
Rivastigmine | Donepezil
76
In an Alzheimers patient what else should be treated?
Address any vascular risk factors
77
When does Frontotemporal dementia usually present?
Early <65
78
What is the usual cause of Frontotemporal dementia?
Neurodegenerative proteinopathy | Aggregation of protein leads to cell death
79
List three proteins in order of occurrence that can cause a frontotemporal dementia.
TAU TDD-43 Ubiquitin
80
What are the three presentations of a frontotemporal dementia?
Behavioural variant Early frontal features Early loss of insight
81
What behavioural variant is common in frontotemporal dementia?
Primary progressive aphasia
82
What are the frontal features that present in frontotemporal dementia?
Disinhibition Lack of apathy Loss of empathy Compulsive behaviours
83
What is needed in order to assess whether a patient has lost their insight into oneself?
A collateral history
84
What investigations are undertaken in a suspected Frontotemporal dementia?
MRI CSF SPECT
85
What is to be seen on an MRI with frontotemporal dementia?
Atrophy of frontal lobes
86
What is to be found within the CSF of a patient with frontotemporal dementia?
raised TAU | Normal amyloid
87
What is to be seen on a SPECT with frontotemporal dementia?
Reduced frontal and temporal lobe metabolism
88
What is the management for Frontal Temporal Dementia?
Trazodone / Antipsychotics for behavioural features Safety Management - access to money internet etc Structured activities Power of attorney Attached a specialist nurse
89
A subcortical presentation of Vascular dementia due to small vessel infarction will present with?
Reduced attention Executive dysfunction Slowed processing
90
What is the management of Vascular Dementia?
Vascular risk factors are treated. | +/- cholinesterase inhibitors
91
What investigations are undertaken in Lewy body dementia?
DaT- Dopamine transport imaging Alpha synuclein ligand imaging CSF - alpha synuclein in CSF
92
What is the treatment for lewy body dementia?
Low dose Levodopa | Cholinesterase inhibitors
93
Someone with Parkinson's Disease Dementia presents with these physical symptoms.
Bradykinesia, Rigidity, Tremor
94
Someone with parkinsons disease dementia present with these cognitive symptoms.
``` Dementi Reduced attention Slowness of processing Impaired visuospatial function Hallucinations ```
95
What is the management for Parkinsons Disease Dementia?
Small does Levodopa | Cholinesterase inhibitors
96
What is the management for Huntingtons?
No curative options Mood stabilisers Nurse specialist
97
If someone is over 65 with no additional neurology where are they referred?
old age psychiatry
98
If someone is under 65 with unusual features where are they referred?
Neurology

Neurology Year 3 (29 decks)

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