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Dementia Flashcards

1
Q

Define dementia?

A

Dementia is a progressive and largely irreversible syndrome that is characterised by a widespread impairment of mental function.

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2
Q

Can dementia have different affects on diffrent people?

A

Yes depending what part of the brain is affected determiens the symptoms

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3
Q

What are the conditions that mimic dementia?

A 
Depression
Iatrogenic (anticholinergics, sedatives, narcotics, H2 blockers, multiple meds)
Hypothyroidism
B12 deficiency
Neurosyphilis
Normal pressure hydrocephalus  need to number of lumbar punctures to reduce pressure 
Subdural hematoma
Encephalitis
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4
Q

what is the classification of dementia?

A

Based on site
Anterior/posterior
or
Subcortical/cortical

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5
Q

Give examples MOVEMENT DISORDERS?

A

Parkinson’s Disease

Huntington’s Disease
Motor Neuron Disease

Parkinson plus syndrome
Progressive Supranuclear Palsy
Multiple System Atrophy
Corticobasal Degeneration

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6
Q

What are symptoms of Frontotemporal Dementia?

A

Behavioural/frontal variant
Non-fluent progressive aphasia
Semantic Dementia

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7
Q

What are the current molecular - genetic classification of neurodegenerative diseases. Example of each

A

Tauopathies
Corticobasal Degeneration*
Alzheimer’s Disease

Synucleinopathies
Parkinson’s Disease
Dementia with Lewy Bodies
MSA

Ubiquinopathies
Motor neuron disease and MND/Dementia*
Semantic Dementia*

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8
Q

What is the triad of symptoms of Normal Pressure Hydrocephalus?

A

Dementia
Gait disturbance

Urinary incontinence
occuring in conjunction with hydrocephalus and normal CSF pressure (possibly, intermittently raised a better term).

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9
Q

What are the two types of normal pressure hydrocephalus?

A

NPH with a preceding cause (SAH, meningitis, trauma, radiation-induced).

NPH with no known preceding cause – idiopathic (50%).

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10
Q

What will be seen in MRI of a person with Varient CJD?

A

Characteristic abnormality seen in the posterior thalamic region (the so
called “pulvinar sign”) which is highly sensitive and specific for vCJD

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11
Q

What will be seen in MRI of a person with sporadic CJD?

A

Non-specific
changes basal
ganglia

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12
Q

What are common symptoms of a person with Cortical basal degeneration ?

A

Gain difficulty with one side of the arm mainly right –> hard to control
Then over a couple of months develop similar problems in the leg.
Speech becomes sparse
Bradykinetic on right, no tremore and midl rigidity

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13
Q

Key facts of Limbic Encephalitis due to Voltage Gated Potassium Channel ?

A

Subacute memory loss
Psychiatric/behavioural disturbance
Seizures, usually partial
Hyponatraemia common
60 % show MTL high signal on MRI
Most cases serum and CSF antibodies to LGI1 subunit of the K channel, others CASPR2
Median age 65 years, 2:1 male:female
Thymoma/SCLC and other tumour may be present but are not commonly associated (more so if CASPR2 ab.)
If no tumour prognosis very good with immuno-modulation
Blood test is used

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14
Q

What discrimination occurs with dementia?

A

Significant misunderstanding about and stigma attached to dementia

Manifests itself in widespread discriminatory attitudes.

Age discrimination.

Risk of discrimination and infringements of their human rights because they may not have the capacity to challenge abuses of their human rights or to report what has occurred.

Fear of the diagnosis

Misunderstandings about dementia: that it is a natural part of ageing, that it only affects older people and that nothing can be done to help people live well with it

Poor quality care and failure to deliver services

Avoidance of people with dementia

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15
Q

What is the limbic system involved in? If there is disorder what happens?

A

Limbic system: arousal, emotion, motivation, attention, and memory

Lack of emotion, lack of motivation and memory. The people are always sad and disinterested

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16
Q

What is the action of parietal lobe?

A

Executive functions: motor, planning,, strategic thinking

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17
Q

What is the role of hippocampus?

A

Major memory sight –> where new memory is formed and stored

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18
Q

What sight in the brain is damaged mainly in alzheimer’s disease?

A

Hippocampus

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19
Q

What is cognition?

A

Is the sum of brain functions which allows us to integrate in the environment.

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20
Q

What skills are invovled in cognition?

A 
Learning and Memory 
Language
visuospatial skills 
Emotion
Personality
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21
Q

What is the route of the microcircuit of the brain?

A

Neuron > Synapse > neurotransmitter > action potential

If the neurone dies it cannot be regenerated –> depending where it is that function will be lost.

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22
Q

What are the differenet classifications of Neurodegenerative disorders?

Give examples of each

A

Functional
Cognitive disorders, e.g. Alzheimer’s Disease (AD)
Movement disorders, e.g. Parkinson disease (PD

Anatomical –> Frontotemporal dementia (FTD), Corticobasal degeneration (CBD

Etiological, vascular dementia (VaD), Prion disease

Proteinopathy,Taupathy (AD), α-Synucleinopathy (DLB)

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23
Q

What cognitive disorder effects Temporal, parietal and frontal degeneration?

A

Alzheimers

24
Q

What cognitive disorder effects Cognitive and movement?

A

Dementia with Lewy body (DLB)

25
What cognitive disorder caues Multifocal degeneration?
Corticobasal degeneration (CBD
26
Is alzheimers usually inherited?
No usually sporadic
27
What are teh 4 main proteins invovled in cognitive defect?
Β-amyloid Tau α-synclein Ubiquitin
28
What are Amyloid proteins?
Insoluble fibrous proteins aggregates sharing specific structural traits
29
How do amyloiud proteins arise and what is the consequense?
They arise from at least from 18 inappropriately folded versions of proteins and polypeptides present naturally in the body. These misfolded structures alter their proper configuration such that they erroneously interact with one another or other cell components forming insoluble fibrils.
30
What is the conseuqence of the accumulation of amyloid proteins?
Abnormal accumulation of amyloid fibrils in organs may lead to amyloidosis, and may play a role in various neurodegenerative disorders (β-amyloid
31
What is Tau proteins?
A group of proteins that stabilize microtubules in neurons. | There are 6 isoforms
32
What happens when Tua proteins are defective?
When Tau proteins are defective the microtubules become instable and dysfunctioning. This happens in Alzheimer's
33
What is the relationship between Tau and amyloid B? Which one is intraceullar and which is extracellular
The two together are seen as a cause of AD Tau is intracellular Amyloid B is extracellular
34
What disease have the presence of α-Synuclein?
See in Parkinson disease, Lew body dementia
35
What is Ubiquitin?
A small regulatory protein that has been found in almost all cells (ubiquitously) of eukaryotic organisms. It directs proteins to compartments in the cell, including the proteasome which destroy and recycle proteins. It can be attached to proteins and label them for destruction.
36
What happens if you have a mutation in Ubiquitin?
Mean that unwanted substanes in the cell accumulate and not got rid off which can damage the cells
37
What are the different pathogenesis caues of neurodegenerative disease?
Oxidative stress: free radicals Excitotoxicity: glutamine stimulation Induction of programmed cell death (apoptosis) Cytokines: inflammatory response? Genetic factors: single gene mutations, multiple genes Aging: age-related decline in the efficiency of some metabolic pathways (e.g RNA synthesis) Unknown aetiology
38
Which proteins that accumulate and cause cognitive disease are intracellular?
Tau (AD) α-Synuclein (DLB) Polyglutamine (HD) Ubiquitin (Pi D)
39
Which proteins that accumulate and cause cognitive disease are extracellular?
Amyloid
40
What is the normal weight of the brain and what is the weight when person have cognitive disease?
Brain weight 900-1100 grams (normal 1200-1400)
41
What neuropathological findings are there with Macroscopiacl examination?
``` Atrophy in cerebral gyri: Hippocampus  imp in AZ Temporal lobe Parietal lobe Frontal lobe Cingulate gyrus ``` Atrophy in white matter: thin corpus callosum Atrophy in deep white matter Ventricular dilatation Atrophy in brain stem and cerebellum Pale substantia nigra and locus ceruleus --> should be darker
42
What neuropathological findings are there with Microscopiacl examination?
Neuron loss from hippocampus, cerebral cortex Due to neurone loss you have Microvacuolation in cerebral neocortex Just absence of any substances Attenuation in white matter Wide perivascular spaces in white matter Accumulation of abnoraml proteins
43
What is the cause of accumulaion of abnormal proteins?
Amyloid: plaques Tau: neurofibrillary tangles α-Synuclein: Lewy bodies Ubiquitin: Pick’s bodies
44
Describe the difference between a normal brain and one that has AD?
A lot more atrophy in brain with AD and a lot smaller.
45
What is the relationship between alzheimers and hippocampus?
Hippocampus is the first hit of AD --> difficulty in learning new skills due to damage to hippocampus. Then later on start losing facts which they used to know Correlation between the level of neuron loss and loss of memory
46
What is Braak staging?
Braak staging refers to two methods used to classify the degree of pathology in Parkinson's disease and Alzheimer's disease
47
Name different types of staging systems?
Braak staging | CERAD plaque densities
48
What is the Macroscopical findings in Alzheimers?
Brain weight: 900 –1200 (Normal: 1200 – 1400) Atrophy of gyri and widening of sulci: frontal, temporal, parietal, and hippocampus Ventricular dilatation
49
What is the Microscopical findings in Alzheimers?
Neuronal loss Neurofibrillary tangle and neuropil threads
50
What is the Macroscopical findings in Dementia with Lewy body ?
Pale Substantia nigra and Locus coeruleus Atrophy in amygdala, cingulate gyrus, temporal, parietal and frontal lobes
51
What is the Microscopical findings in Dementia with Lewy body ?
Neuronal loss from the SN and LC Accumulation of α – Synuclein +ve bodies in the neurons of the SN, amygdala and later in the cerebral cortex
52
What is locus coeruleus
The locus coeruleus is a nucleus in the pons involved with physiological responses to stress and panic
53
What is substantia nigra?
The substantia nigra is one of the brainstem nuclei  It is an important relay station in the motor system
54
Give 3 examples of vascular dementia and explain what happens in each one
Multi-infarct dementia The proteins are fine and there is no tangles or plaques The blood vessels supplying the brain start to close to areas of the brain such as hippocampus -->loosing of memory Binswanger’s disease Mainly in the white matter Blood vessels causing damage to the white matter --> means that all the axons transmitting info is lost Arteriolosclerosis No longer smooth muscle cells --> get collagen fibers Infarction around the arteries Difficulty supplying the tissue
55
What is another name for Frontotemporal Dementia (FTD) and where in the brain is effected?
Mainly in the frontal and temporal area is damaged | Also called Picks diseae

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