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Dementia Flashcards

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1
Q

What is dementia

A

An acquired and persistent generalised disturbance of higher mental functions in an otherwise fully alert person
Neurodegenerative condition - loss of neurons

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2
Q

Is dementia always pathological?

A

YES

It is not part of the ageing process

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3
Q

What are the primary dementias

A

Alzheimer’s
Lewy body dementia
Pick’s disease - fronto-temporal dementia
Huntington’s

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4
Q

What are the secondary dementias

A 
Multi-infarct - vascular 
Infection - HIV, syphilis 
Trauma
Drugs and toxins - alcohol 
Vitamin deficiencies - thiamine 
SOL's and paraneoplastic
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5
Q

What is the most common types of dementia

A

Alzheimer’s

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6
Q

Describe the trends in prevalence of Alzheimer’s

A

Get more common with age

Later the onset, the more rapid and severe the changed tend to be

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7
Q

Is Alzheimer’s genetic

A

Yes and No
Usually sporadic
Some cases are familial - APP, Presenilin 1&2
Increased risk in Down’s syndrome

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8
Q

Why is Alzheimer’s more common in trisomy 21

A

The amyloid precursor protein gene is found on chromosome 21
This is implicated in the disease and leads to early onset

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9
Q

How does Alzheimer’s present

A

Insidious impairment of higher intellectual function, with alterations in mood and behaviour
General forgetfulness is common initially
Also have visuospatial difficulties

Later – progressive disorientation, memory loss and aphasia indicate severe cortical dysfunction

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10
Q

Does Alzheimer’s usually cause death

A

It is usually due to a secondary cause such as bronchopneumonia

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11
Q

What is the macroscopic pathology of Alzheimer’s

A

Cortical atrophy
Widening of the sulci
Narrowing of the gyri
Compensatory dilation of ventricles

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12
Q

Which areas of the brain are usually spared in Alzheimer’s

A

Occipital lobe
Brainstem
Cerebellum

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13
Q

What are the microscopic features of Alzheimer’s

A

Neuronal loss - atrophy and gliosis
Disruption of the cholinergic pathways
Neurofibrillary tangles - insoluble tau protein which disrupts neurons
Neuritic plaques - amyloid plaques extracellularly
Amyloid angiopathy

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14
Q

What is amyloid angiopathy

A

Extracellular accumulation of amyloid protein

Disrupts the BBB - oedema, local hypoxia and serum leaking

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15
Q

How does Lewy body dementia present

A

Progressive dementia alongside visual hallucinations and fluctuating attention
Memory is affected later
Fluctuation in severity of condition on a day‐to‐day basis
Features of Parkinsonism present at onset, or emerge shortly after
Symmetrical tremor
Visual spatial defect

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16
Q

Does everyone with Parkinson’s get Lewy body dementia

A

NOPE

However, everyone with Lewy body dementia will have features of parkinsonism

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17
Q

What are the features of Parkinsonism

A 
Loss of facial expression 
Stooping 
Shuffling gait 
Slow initiation of movement 
Pill rolling tremor
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18
Q

What are the pathological features of Lewy body dementia

A

Degeneration of the substantia nigra
Disrupts the cholinergic and dopaminergic pathway
Will appear pale - loss of pigmented neurons
Reactive gliosis and accumulation of microglia
Lewy bodies present

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19
Q

How is Huntington’s inherited

A

Autosomal dominant
Mutation on the Huntington’s gene causes CAG repeats
Disease occurs when more than 35 repeats
Produces a neurodegenerative protein

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20
Q

What are the clinical features of Huntington’s

A

Triad of emotional, cognitive and motor disturbance

Chorea (dance‐like movements), myoclonus, clumsiness, slurred speech, depression, irritability and apathy.
Develop dementia later

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21
Q

Huntington’s has a long disease process - true or false

A

True

Often around 15 years from symptoms starting until death

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22
Q

Describe the pathology seen in Huntington’ s

A

Atrophy of the basal ganglia (caudate nucleus and putamen)

Cortical atrophy occurs later and corresponds to the onset of dementia

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23
Q

What is the other name for Pick’s disease

A

Fronto-temporal dementia

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24
Q

How does Pick’s disease/FTD present

A

Personality and behavioural change - including social deterioration
Speech and communication problems
Impairment of intellect, memory and language
Changes in eating habits
Reduced attention span
Fast loss of insight - no idea their behaviour is abnormal

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25
Describe the disease pattern of Pick's disease/FTD
Starts in middle life (50-60) which is younger than most dementias Rapidly progressing - lasts between 2-10 years
26
Describe the underlying pathology of Pick's disease
Extreme atrophy of the frontal lobes and later in the temporal lobes Tau protein problem, aggregate within neuronal cells which causes death and neurodegeneration Neuronal loss and gliosis Will see Picks cells and Picks bodies on histology
27
What causes multi-infarct dementia
Successive, multiple cerebral infarctions cause increasingly larger areas of cell death and damage Damage is cumulative and caused by hypoxia as a result of infarcts
28
Who gets multi-infarct dementia
More common in men Usually seen after age 60 Also seen in younger hypertensives Post-stroke
29
What characterises multi-infarct dementia
Insight | Sufferers aware of their mental deficits and are prone to depression and anxiety.
30
How does mutli-infarct dementia present
Similar to Alzheimer's symptom wise Abrupt onset with a stepwise progression Progresses slowly History of hypertension or stroke (evidence on CT) Patient has insight into deficits Depression is common
31
What are the risk factors for multi-infarct dementia
Same as CVD Hypertension, smoking etc Previous stroke
32
Describe the morphological appearance of multi-infarct dementia
See large vessel infarcts scattered through the hemispheres | May have smaller lacunar infarcts
33
What is cognition
The mental action of acquiring knowledge and understanding through thought, experience, and the senses Split into: Memory Attention (ability to be alert enough to take info in) Social functioning; how to evaluate and reason Language: ability to produce, and understand language Executive function; problem solving, decision making
34
What are the criteria for a diagnosis of dementia
Evidence of significant cognitive decline in at least 1 domain Must interfere with everyday activities and independence Must not be diagnosable as something else
35
Is forgetfulness always a sign of dementia
NO | Everyone has days of poor cognitive function such as forgetting names
36
What is the greatest risk factor for dementia
Ageing | Most occur above the age of 65
37
List acute cognitive disorders
Brain insults/injury Transient global ischaemia Transient epileptic amnesia
38
Describe the effects of brain insults
Deficits depend on the area of brain affected Includes: Viral encephalitis (memory, behaviour change, and language) Head injury (memory, executive dysfunction) Stroke
39
How does transient global amnesia
``` Abrupt onset More commonly anterograde amnesia Preserved knowledge of self - name, age etc Transient - 4-6hours Generally a once off ```
40
What triggers transient global amnesia
Emotion Changes in temperature Uncertain pathophysiology
41
Who gets transient global ischaemia
The over 50s | usually in 70s
42
What is anterograde amnesia
Can't lay down new memories
43
What is retrograde amnesia
Cannot remember things from the past
44
What are the clinical features of transient epileptic amnesia
Forgetful / repetitive questioning Can carry out complex activities with no recollection of events Short lived (20 – 30 minutes) Recurrent problem
45
What is transient epileptic amnesia associated with
Temporal lobe seizures
46
How can you treat transient epileptic amnesia
Should respond to anti-epileptic medication
47
List some common sub-acute cognitive disorders
``` Functional disorders Mood disorders Metabolic - B12, thyroid etc Inflammatory - encephalitis Toxins - alcohol CJD Infection - HIV etc ```
48
What is functional/subjective cognitive impairment
Everyday forgetfulness that starts to impact upon ability to function Losing keys, forgetting names etc Fluctuating symptoms - good and bad days Mismatch between symptoms + reported function (feel they should remember more than they do, even if normal and no one has reported concern)
49
How can you treat functional/subjective impairment
Exclude a mood disorder and Alzheimer's | Neuropsychology
50
What is the most common human prion disease
Creutzfeldt-Jakob Disease Still very rare Leads to neurodegenerative conditions due to prion abnormality in the brain
51
What are the 4 types of CJD
Sporadic Variant - from BSE in cows Iatrogenic Genetic
52
What are the symptoms of sporadic CJD
Rapid onset dementia, neurological signs and myoclonus Lasts around 4 months Often fatal
53
What are the symptoms of variant CJD
Painful sensory disturbance Neuropsychiatric decline - personality changes Duration of around 14 months before death
54
How does iatrogenic CJD present
Cerebellar or visual onset Multifocal neurological decline Illness duration of less than 2 years before death
55
How does genetic CJD present
Similar to sporadic May have ataxia or insomnia Variable duration but usually less than 2 years
56
How do you diagnose CJD
EEG and MRI | Gold standard is a brain biopsy - becomes spongiform
57
What is posterior cortical atrophy
Unusual form of Alzheimer's Part of the visual field deteriorates Cannot recognise faces and has difficulty going down stairs Memory symptoms appear later
58
What is progressive primary aphasia
Unusual for of Alzheimer's Can't name something correctly, can't repeat something back, very difficult to talk memory and behavioural symptoms come later
59
What investigations would you do for Alzheimer's
MRI - shows atrophy SPECT - shows decreases in metabolism CSF will show reduced amyloid
60
How can you treat Alzheimer's
Address vascular risk factors - reduced risk of progression | ACh boosting treatment - cholinesterase inhibitors or NMDA blockers
61
How would frontotemporal dementia appear on investigations
MRI would show atrophy of frontotemporal lobes (later sign) SPECT would show decreased metabolism in the frontotemporal areas CSF will have increased tau
62
How can you manage frontotemporal dementia
Trials of anti-psychotics to help behavioural symptoms SSRIs may help behaviour Mainly safety management - controlled access to food, money, internet Support for family - power of attorney
63
How can you manage vascular dementia
Reduce CVS risk factors- stop smoking, reduce cholesterol, | Give cholesterase inhibitors if clear also have AD
64
How can you investigate Lewy body dementia
DaT scan - dopamine transporter imaging Will show reduced uptake in the basal ganglia Will look like dots instead of commas
65
How can you treat Lewy body dementia
Small dose of levodopa - will help motor but not cognitive symptoms Community and nursing support
66
What generally causes dementias
Neurodegenerative proteinopathies
67
What is Parkinson's disease dementia
Dementia that present in someone who already has Parkinson's The dementia will present over one year after the appearance of Parkinson's symptoms
68
How does dementia present in Huntington's
Early onset dementia (30-50) Slow processing Eventual involvement of memory Changes in mood and personality
69
How can you manage Huntington's
Mood stabilisers Treat chorea Specialist Huntington's nurse
70
Who do you refer dementia patients to
If over 65 and no additional features - old age psychiatry | If under 65, unusual features or additional neurology - neurology
71
What tests are found in a dementia blood screen
``` FBC, LFT and U&E B12 and folate Thyroid function HIV and syphilis Calcium +/- genetic panel ```
72
When is SPECT imaging used
Done if MRI normal but clear cognitive changes
73
What is the most common type of vascular dementia
Multi-infarct dementia
74
What comes first in Lewy body dementia - cognitive decline or Parkinsonism
The cognitive decline starts first | Parkinsonism will appear shortly after
75
What is the issue in cortical dementia
The problem is with storing memories | Alzheimer's is a type of cortical dementia
76
What is the issue in subcortical dementia
The problem is with retrieving memories - may remember with a prompt Lewy body and vascular dementia are in this category
77
Which drugs increase mortality in Lewy body dementia
Anti-psychotics
78
What is a REM sleep disorder
Shouting out or purposeful movement when asleep | Seen in Lewy body dementia and Parkinson's
79
Which other disease can frontotemporal dementia be seen in
Motor neurone disease
80
How is memantine used in dementia
Its a glutamate inhibitor used in moderate to severe dementia May help with behavioural issues
81
What is the contraindication to using cholinesterase inhibitors
Can slow the heart rate so need to check pulse first | Do an ECG if you suspect bradycardia or heart block

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