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Clinical presentations of Dementia Flashcards

1
Q

Define Dementia

A

Dementia is a clinical syndrome of progressive cognitive decline with impaired social or occupational functioning

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2
Q

In people over the age of 80 how many people will have dementia?

A

1 in 5 people

Much less common in younger people

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3
Q

What is the commonest in people over age of 65?

A

Alzheimer’s

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4
Q

Beyond Alzheimer;s what does the balance of disease change with?

A

Age of onset

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5
Q

What is extremely rare in younger people?

A

Lewy bodies

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6
Q

What is very important clinically?

A

Frontal temporal dementia

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7
Q

What does Dementia refer to?

A

Particular pattern of changes

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8
Q

What is the common pattern of cognitive impairment for AD?

A
  1. Episodic (and topographical) memory initially
  2. Early parietal deficits visuo-perceptual, visuo-spatial, calculation,praxis
  3. Later global cognitive impairment
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9
Q

What is the common pattern of cognitive impairment for DLB?

A
  1. Executive dysfunction
  2. Visual hallucinations
  3. Parietal lobe deficits/episodic memory
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10
Q

What is the common pattern of cognitive impairment for FTLD?

A
  1. Behavioural/personality changes
  2. Executive dysfunction
  3. Language-specific subtypes
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11
Q

What is the common pattern of cognitive impairment for VaD?

A
  1. Cognitive slowing

2. Executive dysfunction + can affect other lobes

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12
Q

What are the profiles seen on the neuropsychological tests mapped onto?

A

Particular hallmarks of atrophy

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13
Q

What does Alzheimer’s produce problems within?

A

Selective early hippocampi

- The medial temporal lobe

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14
Q

What do Semantic dementia produce problems within?

A

Understanding of speech

- The left temporal lobe

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15
Q

What does Primary progressive aphasia (PPA) affect?

A

The frontal lobe of the brain

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16
Q

What do profile on the scans go with?

A

Profile we see neuropsychologically

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17
Q

What does Neuroimaging contribute?

A
  1. Exclusion of surgically remediable lesion
  2. Diagnostic positive predictive value
  3. Detection and quantification of change
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18
Q

What is a framework for understanding (degenerative) dementias?

A
  1. Genetic/ other risk factors
  2. Abnormal protein deposited in the brain
  3. Loss of brain cells (brain atrophy)
  4. Altered neuro-transmitter function
  5. Symptoms and signs of the disease
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19
Q

What is a risk factor for the acceleration of the early onset of Alzheimer’s?

A

APOE4

not used clinically but mentioned dermatologically

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20
Q

What is the genetics of Alzheimer’s disease?

A
  1. APP
  2. PS1
  3. PS2
  4. ApoE4
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21
Q

What is the abnormal protein deposited in the brain for Alzheimer’s disease?

A
  1. Amyloid

2. Tau

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22
Q

What is the brain atrophy for Alzheimer’s disease?

A

Hippocampal atrophy initially then whole brain

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23
Q

What is the altered neuro-transmitter function of Alzheimer’s disease?

A

Cholinergic

24
Q

What are the symptoms and signs of Alzheimer’s disease?

A

Episodic memory problems

later global deficits

25
What is serial scan used for?
Repeated Imaging | Look at brain shrinkage
26
What is the process of a serial scan?
Register the scans onto a common space and measure whether there has been a change in brain volume Quantify the change by putting a colour on it
27
What is Alzheimer's disease summary?
* The most common disease-causing dementia and most closely associated in the public mind * Insidious onset, usually early memory impairment * Later global cognitive deficits * Neurological examination normal early in disease * Amyloid plaques & (tau-positive) neurofibrillary tangles * Deficiency of acetylcholine biochemically * Initially hippocampal atrophy, later global cerebral atrophy
28
What does posterior cortical atrophy have (PCA)?
1. Visual disorientation 2. Led by visuospatial awareness 3. Early prominent involvement of parietal cortex & occipital cortex
29
What is the abnormal protein deposited in the brain for dementia with Lewy bodies?
Alpha-synuclein
30
What is the brain atrophy for dementia with Lewy bodies?
Posterior or generalised
31
What is the altered neuro-transmitter function?
1. Dopaminergic | 2. Cholinergic
32
What are the symptoms and signs of Dementia with Lewy bodies?
1. Visual hallucinations 2. Cognitive decline 3. Fluctuations 4. Parkinsonism
33
Dementia with Lewy Bodies summary
• Extensive overlap (? continuum) with Parkinson’s disease • Cognitive decline often includes prominent attentional, executive as well as posterior cortical deficits, memory variable • Other key features: - Visual hallucinations - Fluctuations in cognition - REM sleep disorder - Parkinsonism • Relative ‘absence’ of disproportionate hippocampal atrophy on MRI
34
What is the genetics for FTLD?
1. MAPT 2. Progranulin 3. C9ORF72 4. CHMP2B 5. VCP 6. TBK1
35
What is the abnormal protein deposited in the brain for FTLD?
1. Ubiquitin (TDP-43) | 2. Tau
36
What is brain atrophy for FTLD?
Fronto-temporal atrophy
37
What is altered neuro-transmitter function of FTLD?
1. Serotonergic | 2. Dopaminergic
38
What are the symptoms and signs of FTLD?
Behavioural or language deficits
39
What is the macroscopic pathology of FTLD?
* Classically ‘’knife-blade’’ frontotemporal atrophy but severity variable * Variable involvement of parietal lobes * Subcortical structures (amygdala, hippocampus, basal ganglia, thalamus) frequently involved * They have loss of insight
40
What is progressive prosopagnosia?
a clinical syndrome characterized by a progressive and selective inability to recognize and identify faces of familiar people
41
What is the clinical feature of progressive prosopagnosia?
R temporal love atrophy overlap extensively with 'frontal' dementia
42
What is sementic dementia syndrome closely associated with?
Focal L temporal atrophy and this constellation predicts ubiquitin pathology
43
What is FTLD summary?
* Paradigmatic ‘focal’ dementias * Genetically mediated in a significant proportion and this is likely to continue to expand * Behavioural and/or language-based presentations * Variable prominence of other neurological features (esp. parkinsonism) * Asymmetric atrophy with antero-posterior gradient on MRI * Some evidence for molecular imaging signatures
44
What is progressive supranuclear palsy (PSP) + corticobasal degeneration (CBD)?
Overlapping clinical syndrome with parkinsonism plus other neurological and cognitive features, and various histopathological substrates
45
what are features of PSP?
1. Prominent executive deficits 2. Gaze palsy (supranuclear) 3. Early falls and parkinsonism esp affecting axial muscles 4. May have midbrain atrophy on MRI
46
What are features of CBD?
1. Prominent parietal deficits 2. Asymmetric limb apraxia, ridgity +- 'alien limb' 3. May have asymmetric fronto-parietal atrophy on MRI
47
What signs does PSP: midbrain atrophy have on structural MRI?
1. Mickey Mouse sign | 2. Hummingbird sign
48
What are the causes of vascular dementia?
1. Large artery disease (including inflammatory) 2. Cardiac embolic events 3. Small vessel disease 4. Thrombophilias 5. Haematologic factors 6. Haemodynamic mechanims 7. Specific arteriopathies 8. Haemorrhages (primary and secondary) 9. Hereditary e.g. CADASIL, cerebral amyloid angiopathies
49
What are the lesion types for vascular dementia?
1. Arterial territorial infarct 2. Distal field/watershed infarct 3. Lacunar infarct 4. Ischaemic white matter change 5. Incomplete ischaemic injury
50
What is the summary of vascular dementia?
1. A range of causes including both sporadic and genetic vasculopathies 2. Atherosclerosis is the most common but vasculitis is esp important as treatable 3. Many cases lack conventional risk factors or clinical history of stroke 4. Several MRI patterns possible 5. Vessel disease often coexist with primary degeneration
51
What are examples of the classification of Limbic encephalitis?
1. Autoimmune 2. Paraneoplastic 3. Infections 4. Connective tissue diseases/ vasculitides 5. Mimic syndromes
52
What are examples of autoimmune limbic encephalitis?
1. LGI1 most common 2. GABABR, 3. AMPAR 4. CASPR2 - steroid responsive encephalopathies
53
What are examples of Paraneoplastic limbic encephalitis?
1. Small cell lung 2. Testis 3. Lyphoma 4. Ovary 5. Breast
54
What is an example of infections in Limbic encephalitis?
1. Herpesviruses (esp HSV2, HHV6/7)
55
What are examples of connective tissue diseases/vasculitides?
1. Behcets 2. Sjogren's 3. Sarcoid 4. Relapsing polychondritis
56
What is an example of mimic syndrome?
Temporal lobe tumour
57
What are the Limbic encephalitis summary?
1. Causes include paraneoplastic and inflammatory (autoimmune). Expanding range of associations 2. Syndrome of subacute memory impairment with confusion, psychiatric features/ behavioural change (limbic dysfunction) 3. Fluctuation with more protracted episodes of altered conscioussness, abnormal movements (e.g. LGI1) 4. Seizures common 5. Temporal lobe abnormalities on MRI 6. May respond to immunosuppressive treatments

C3 CLINICAL (27 decks)

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