Cognitive Neurology Flashcards

1
Q

What is the clinical definition of dementia

A

Evidence of significant cognitive decline in at least 2 cognitive domains
Cognitive defects interfere with independence in everyday activities
Not better explained by another process/ do not occur exclusively with delirium)

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

List the main cognitive domains

A
Attention
Executive
Learning and memory
Language
Perceptuo-motor (praxis)
Social cognition
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the two main types of acute cognitive decline

A

Transient global amnesia

Transient epileptic amnesia

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

List the main clinical features of transient global amnesia

A

Abrupt onset antegrade > retrograde amnesia (repetitive)

Preserved knowledge of self

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

How long does transient global amnesia typically last?

A

4-6 hours (<24) and usually a once-off event

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

List the main clinical features of transient epileptic amnesia

A

Forgetful/ repetitive questioning
Can carry out complex activities with no recollection of events
Temporal lobe seizures

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

How long does transient epileptic amnesia typically last?

A

Short-lived (20-30 minutes)

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

What medication does transient epileptic amnesia respond to?

A

Anti-epileptic drugs

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

List the main subacute cognitive disorders

A
Neurodegenerative (CJD)
Toxin, alcohol, CO
Infection (HIV, syphillus)
Functional
Metabolic (B12, Ca, thyroid)
Mood disorders
Inflammatory (limbic encephalitis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

List the main clinical features of functional/ subjective cognitive impairment

A

Everyday forgetfulness impact on functioning
Fluctuation of symptoms which don’t match known disease
Part of generalised functional disorder (reduced concentration, attention, reaction time, memory)

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

What types of people typically get functional disorders?

A

People with previously good memories

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Outline the management of functional disorders

A

Exclude mood disorder

Neurpsychology - CBT

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

Name the most common prion disease

A

Creutzfeldt-jakob disease

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

What is the pathogenesis of CJD?

A

Naturally occuring prion is misfolded causing neurodegeneration

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

List the four subtypes of CJD

A

SPORADIC: rapid onset dementia + neuro signs + myoclonus
VARIANT: painful sensory disturbance + neuropsychological decline
IATROGENIC: cerebellar/ visual onset, multifocal neurological decline
GENETIC: can mimic sporadic, due to mutation in PRNP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the two forms of genetic CJD? What is their main clinical feature?

A

GSS (prolonged ataxia)

FFI (insomnia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What two types of CJD are common in children?

A

Iatrogenic

Genetic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Outline the investigations used to diagnosed CJD

A

EEG
MRI
Spongiform change

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What characteristic feature is present on imaging of variant CJD?

A

Hockey-sticks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the most common type of dementia?

A

Alzheimer’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What type of protein is characteristic of neurodegeneration in Alzheimer’s extracellular and intracellular?

A

Amyloid plaques

Neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Outline the pathogenesis of Alzheimer’s disease?

A

Disruption of cholinergic pathways and synaptic loss

23
Q

List the main clinical features of Alzheimer’s disease

A

Forgetfullness at the start

Progresses to apraxia and visuo-spatial difficulties

24
Q

Outline the aetiology of Alzheimer’s disease depending on age

A
<65 = early onset, most likely genetic 
>65 = sporadic, most likely environmental
25
Q

List the types of atypical aphasia that can present in Alzheimer’s disease

A

Semantic (naming)
Logopenic (repeating)
Non-fluent (effortful)

26
Q

Outline the investigations used to diagnosed AD, and the expected results

A

MMSE initially
MRI - atrophy of temporal/ parietal lobes
SPECT - temperoparietal reduction in metabolism
CSF - reduced amyloid, increased tau ratio

27
Q

Outline the management used in AD

A

Assess vascular risk factors
Acetylcholine boosting drugs (cholinesterase inhibitors e.g. rivastagmine, galantamine, NMDA receptor blokcer e.g. memantine)

28
Q

List the two main types of early onset dementia

A

Fronto-temporal dementia

Huntingdon’s disease

29
Q

What type of protein is characteristic of neurodegeneration in fronto-temporal dementia?

A

Tau protein aggregates

30
Q

Outline the main clinical features of fronto-temporal dementia

A

Behavioural > primary progressive aphasia

Early frontal features (disinhibiton, apathy, loss of empathy, compulsive behaviours, loss of insight)

31
Q

Outline the investigations used to diagnose fronto-temporal dementia, and the expected results

A

MRI - atrophy of frontal-temporal lobes
SPECT - reduced fronto-temporal metabolism
CSF: increased tau/ normal amyloid

32
Q

Outline the management used in fronto-temporal dementia

A

Trazadone/ antipsychotics to help behavioural features
Safety (restricted access to food/ money)
Power of attorney
Nurse specialist

33
Q

List the three main types of late onset dementia

A

Vascular dementia
Dementia with lewy bodies
Parkinson’s disease dementia

34
Q

What is the core criteria for diagnosis of vascular dementia?

A

Cerebrovascular disease

Clear temporal relationship between onset of dementia and CVD

35
Q

Outline the main clinical features of vascular dementia

A

Small vessel disease - reduced attention, executive dysfunction and slowed processing

36
Q

What type of dementia typically presents < 3 months following a stroke?

A

Vascular dementia

37
Q

What type of protein is characteristic of neurodegeneration in vascular dementia, similar to that of Alzheimer’s?

A

Amyloid

38
Q

Outline the investigations and management used in vascular dementia

A
SPECT scan (patchy reduction in uptake)
CT/MRI: mod-severe small vessel disease/ lacunar infarcts

Vascular risk factors
Cholinesterase inhibitor
CPN

39
Q

What type of protein is characteristic of neurodegeneration in dementia with Lewy bodies?

A

Alpha-synuclein

40
Q

Outline the pathogenesis of Lewy body dementia?

A

Insoluble protein leads to disruption of cholinergic and dopaminergic pathways

41
Q

What is the core criteria for diagnosis of Lewy body dementia?

A

Fluctuation of cognition (attention, executive function, visuo-spatial skills)
Recurrent well-formed visual hallucinations
Presence of extra-pyramidal features (parkinsonism) e.g. falls

42
Q

Outline the investigations used to diagnose Lewy body dementia

A

Dopaminergic transport imaging (DaT scan)

Alpha synuclein in CSF

43
Q

Outline the management used in Lewy body dementia

A

Small dose levodopa/ cholinesterase inhibitors

PD nurse specialist/ CPN

44
Q

Parkinson’s disease dementia develops after how many years of the disease?

A

20 years

45
Q

How is DLB distinguished from PD dementia?

A

DLB < 1 year onset of parkinsonism following dementia

PDD >1 year after presentation of parkinsonism, dementia develops

46
Q

Outline the clinical features of Parkinson’s disease dementia

A

Parkinson’s triad + dementia (reduced attention and slowness of processing, impaired visuospatial functions, memory, hallucinations)

47
Q

Outline the management used for Parkinson’s disease dementia

A

Small dose levodopa/ cholinesterase inhibitors
PD nurse specialist/ CPN
(Same as DLB)

48
Q

Outline the pathogenesis of Huntingdon’s disease

A

Expansion of CAG trinucleotide repeat on huntingdon gene produces a neurodegenerative protein

49
Q

List the clinical features of Huntingdon’s disease

A

Dementia (dysexecutive syndrome, slowed speed of processing and memoty)
Change in mood/ personality
Chorea
Late psychosis

50
Q

How is Huntingdon’s disease diagnosed? What are the expected results?

A

Genetic testing

MRI (loss of caudate nucleus)

51
Q

How is Huntingdon’s disease managed?

A

Mood stabilisers
Treatment of chorea
HD nurse specialist

52
Q

List the points of assessment used in dementia

A

Cognitive assessment (Addenbrooke’s)
Imaging (MRI - pattern of atrophy, SPECT)
Bloods (B12, TFT, syphillus, HIV, Ca, genetic panal)
CSF (amyloid, tau, prion)
Neuropsychology (in depth assessment)

53
Q

List aetiology of dementia

A

GENETIC: Huntingdons’s, wilson’s, porphyria
INFECTIOUS: Syphillus, prions, encephalitis
CVD
METABOLIC: Hypothyroidism, thiamine (alcohol)
CEREBRAL TUMOURS
DEGENERATIVE: Alzheimer’s, parkinson’s
DEMYELINATION
TRAUMA: Major head injury
NORMAL PRESSURE HYDROCEPHALUS