Cognitive Neurology Flashcards by Kerry Hendrie

What is the clinical definition of dementia

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)

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List the main cognitive domains

Attention
Executive
Learning and memory
Language
Perceptuo-motor (praxis)
Social cognition

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What are the two main types of acute cognitive decline

Transient global amnesia

Transient epileptic amnesia

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List the main clinical features of transient global amnesia

Abrupt onset antegrade > retrograde amnesia (repetitive)

Preserved knowledge of self

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How long does transient global amnesia typically last?

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

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List the main clinical features of transient epileptic amnesia

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

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How long does transient epileptic amnesia typically last?

Short-lived (20-30 minutes)

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What medication does transient epileptic amnesia respond to?

Anti-epileptic drugs

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List the main subacute cognitive disorders

Neurodegenerative (CJD)
Toxin, alcohol, CO
Infection (HIV, syphillus)
Functional
Metabolic (B12, Ca, thyroid)
Mood disorders
Inflammatory (limbic encephalitis)

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List the main clinical features of functional/ subjective cognitive impairment

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)

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What types of people typically get functional disorders?

People with previously good memories

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Outline the management of functional disorders

Exclude mood disorder

Neurpsychology - CBT

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Name the most common prion disease

Creutzfeldt-jakob disease

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What is the pathogenesis of CJD?

Naturally occuring prion is misfolded causing neurodegeneration

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List the four subtypes of CJD

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

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

GSS (prolonged ataxia)

FFI (insomnia)

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What two types of CJD are common in children?

Iatrogenic

Genetic

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Outline the investigations used to diagnosed CJD

EEG
MRI
Spongiform change

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What characteristic feature is present on imaging of variant CJD?

Hockey-sticks

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What is the most common type of dementia?

Alzheimer’s disease

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

Amyloid plaques

Neurofibrillary tangles

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Outline the pathogenesis of Alzheimer’s disease?

Disruption of cholinergic pathways and synaptic loss

List the main clinical features of Alzheimer’s disease

Forgetfullness at the start

Progresses to apraxia and visuo-spatial difficulties

Outline the aetiology of Alzheimer’s disease depending on age

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

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

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

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

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

Outline the management used in AD

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

List the two main types of early onset dementia

Fronto-temporal dementia | Huntingdon's disease

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

Tau protein aggregates

Outline the main clinical features of fronto-temporal dementia

Behavioural > primary progressive aphasia | Early frontal features (disinhibiton, apathy, loss of empathy, compulsive behaviours, loss of insight)

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

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

Outline the management used in fronto-temporal dementia

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

List the three main types of late onset dementia

Vascular dementia Dementia with lewy bodies Parkinson's disease dementia

What is the core criteria for diagnosis of vascular dementia?

Cerebrovascular disease | Clear temporal relationship between onset of dementia and CVD

Outline the main clinical features of vascular dementia

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

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

Vascular dementia

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

Amyloid

Outline the investigations and management used in vascular dementia

``` SPECT scan (patchy reduction in uptake) CT/MRI: mod-severe small vessel disease/ lacunar infarcts ``` Vascular risk factors Cholinesterase inhibitor CPN

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

Alpha-synuclein

Outline the pathogenesis of Lewy body dementia?

Insoluble protein leads to disruption of cholinergic and dopaminergic pathways

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

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

Outline the investigations used to diagnose Lewy body dementia

Dopaminergic transport imaging (DaT scan) | Alpha synuclein in CSF

Outline the management used in Lewy body dementia

Small dose levodopa/ cholinesterase inhibitors | PD nurse specialist/ CPN

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

20 years

How is DLB distinguished from PD dementia?

DLB < 1 year onset of parkinsonism following dementia | PDD >1 year after presentation of parkinsonism, dementia develops

Outline the clinical features of Parkinson's disease dementia

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

Outline the management used for Parkinson's disease dementia

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

Outline the pathogenesis of Huntingdon's disease

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

List the clinical features of Huntingdon's disease

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

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

Genetic testing | MRI (loss of caudate nucleus)

How is Huntingdon's disease managed?

Mood stabilisers Treatment of chorea HD nurse specialist

List the points of assessment used in dementia

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)

List aetiology of dementia

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