L1 Dementia Flashcards

1
Q

What is dementia a clinical syndrome of?

A

progressive cognitive decline with impaired social or occupational functioning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what % is prevalence in 80+ compared to 40-65?

A

20% to 0.1%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

name 4 different types of dementa

A

alzheimers
dementia with Lewy bodies
frontotemporal lobar degeneration
vascular Dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is the most common type in >65 for the 4 types in order

A

ad
VaD
DLB=other

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what are common patterns of cognitive impairment in Alzheimers

A

episodic, topographical memory

early parietal deficits visuo-perceptual, visuo spatial, calculation, praxis, later global cognitive impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what type of dementia are these symptoms in: executive dysfunction, visual hallucinations, parietal lobe deficits/episodic memory

A

dementia with lewy bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what type of dementia commonly shows behavioural/personality changes, executive dysfunction, language specific subtypes?

A

fronto temporal lobar degeneration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what symptoms are common in vascular dementia

A

vascular dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what does neuroimaging contribute

A
  • exclusion of surgically remediable lesion
  • diagnostic positive predictive value
  • detection and quantification of change
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is the general framework of understanding dementias

A

genetic/other risk factors –> abnormal protein deposited in brain –> loss of brain cells (brain atrophy) –> altered neurotransmitter function –> symptoms and signs of the disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is the pathogenesis of Alzheimer’s disease following the general framework

A
  • genetics: APP, PS1, PS2, ApoE4
  • protein: amyloid plaques, tau positive neurofibrillary tangles
  • atrophy - hippocampal atrophy initially then whole brain
  • altered neurotransmitter: cholinergic
    symptoms: episode memory problems, later global deficits
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what happens in quantifying regional atrophy

A

put scans together, measure if there’s been any change in brain volume then put colour on it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is a symptom of posterior cortical atrophy

A

visual perception/spatial
can’t orient themselves in space or put objects down and forget or can’t place things properly
involvement of parietal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the framework of Dementia with Lewy bodies

A

protein: alpha synuclein
brain atrophy: posterior or generalised
altered neurotransmitter: dopaminergic, cholinergic
symptoms: visual hallucinations, cognitive decline, fluctuations, parkinsonism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are key features of dementia with lewy bodies

A
visual hallucinations
fluctuations in cognition
REM sleep disorder
parkinsonism 
relative absence of disproportionate hippocampal atrophy on MRI
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is the pathogenesis of FTLD

A

genetic: MAPT, progranulin, C9ORF72, CHMP2B, VCP, TBK1

17
Q

what is the macroscopic pathology of FTLD

A
  • front can be shrivelled
  • classically knife-blade frontotemporal atrophy but severity variable
  • variable involvement of parietal lobes
  • subcortical structures (amygdala, hippocampus, basal ganglia, thalamus frequently involved)
18
Q

what symptoms are seen in behavioural variant FTLD

A
loss of texture
pain awareness
utilisation behaviour
grasp reflex
rooting reflex
social emotional
inappropriate remarks
can develop unusual interests
19
Q

what is semantic dementia related to

A

associated with focal left temporal atrophy

20
Q

what are symptoms of progressive supranuclear palsy?

A

prominent executive deficits
gaze palsy (supranuclear)
early falls and parkinsonism esp affecting axial muscles
maybe midbrain atrophy on MRI

21
Q

what are symptoms in corticobasal degeneration

A

prominent parietal deficits
asymmetric limb apraxia, rigidity
maybe aymmetric fronto-parietal atrophy

22
Q

what are signs of PSP on MRI

A

mickey mouse

hummingbird

23
Q

What are causes of vascular dementia

A
large artery disease
cardiac embolic events
small vessel disease
thrombophilias
haematologic factors
haemodynamic mechanisms
specific arteriopathies
haemorrhages
hereditary, cerebral amyloid angiopathies
24
Q

what are lesion types on vascular dementia

A
arterial territorial infarct
distal field/watershed infarct
lacunar infarct
ischaemic white matter change
incomplete ischaemic injury
25
Q

what does vascular dementia show on MRI with multi infarct

A

multiple strokes
stepwise course
plateaus

26
Q

what does vascular dementia show on mri affected in strategic infarct

A

thalamus, basal ganglia

27
Q

what does vascular dementia show in subcortical on MRI

A
dysexecutive syndrome 
memory deficit
impaired recall
relatively intact recognition
subcortical slowing
bradyphrenia
depression
mood changes
episodes with UMN signs, brisk facial jerks
urinary frequency and incontinence
emotional incontinence
extrapyramidal signs
gait disorder
28
Q

what are the MRI features from CADASIL, what is affected

A

involvement of areas not usually involved in atherosclerotic diseases
external capsule
anterior temporal lobe

29
Q

what is common in vascular dementia

A

atherosclerosis

vasculitis

30
Q

what young onset dementias are with peripheral neuropathy

A
CTX
HIV
mitochondrial
Lyme 
neuroacanthocytosis
31
Q

what young onset dementias are with dystonia

A
Wilson's
Kuf's
MLD
Hallervorden-Spatz
neuronal inclusion disorder
HIV
mitochondrial
nvCJD
lymphoma
HD
DRPLA
neuroacanthosytosis
neuroferritinopathy
32
Q

what young onset dementias are present with ataxia

A
Wilson's
Kuf's
Lafora body
NPC
SCAs
MLD
nvCJD
HIV
HD
DRPLA
British
33
Q

what are symptoms of cerebrotendinous xanthomatosis

A

tendon xanthomas
cataracts
dentate nucleus has a high signal on MRI

34
Q

what are MRI features of limbic encephalitis shown on coronal FLAIR

A

coronal FLAIR shows medial temporal signal change reduced after treatment with development of medial temporal lobe atrophy

35
Q

what is limbic encephalitis caused by and what are symtoms?

A

cause - autoimmunity: LGI1, GABA.b.R, AMPAR, CASPR2

symptoms - subacute memory impairment, confusion, behavioural change, seizures

36
Q

what is paraneoplastic limbic encephalitis caused by

A
cancer or tumour in 
small cell lung(anti-Hu, CRMP5/CV2, ANNA-3)
testis (anti-Ma2)
lymphoma (CRMP5/CV2),
ovary (anti-NMDAR)
breast (anti-amphiphysin)
37
Q

what infections are linked to limbic encephalitis

A

herpesviruses e.g. HSV2 HHV6/7

38
Q

what connective tissue diseases are linked to limbic encephalitis

A

behcets
sjogren
sarcoid
relapsing polychondritis

39
Q

what is a mimic syndrome of limbic encephalitis

A

temporal lobe tumour