Cognitive Neurology Flashcards
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)
List the main cognitive domains
Attention Executive Learning and memory Language Perceptuo-motor (praxis) Social cognition
What are the two main types of acute cognitive decline
Transient global amnesia
Transient epileptic amnesia
List the main clinical features of transient global amnesia
Abrupt onset antegrade > retrograde amnesia (repetitive)
Preserved knowledge of self
How long does transient global amnesia typically last?
4-6 hours (<24) and usually a once-off event
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
How long does transient epileptic amnesia typically last?
Short-lived (20-30 minutes)
What medication does transient epileptic amnesia respond to?
Anti-epileptic drugs
List the main subacute cognitive disorders
Neurodegenerative (CJD) Toxin, alcohol, CO Infection (HIV, syphillus) Functional Metabolic (B12, Ca, thyroid) Mood disorders Inflammatory (limbic encephalitis)
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)
What types of people typically get functional disorders?
People with previously good memories
Outline the management of functional disorders
Exclude mood disorder
Neurpsychology - CBT
Name the most common prion disease
Creutzfeldt-jakob disease
What is the pathogenesis of CJD?
Naturally occuring prion is misfolded causing neurodegeneration
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
What are the two forms of genetic CJD? What is their main clinical feature?
GSS (prolonged ataxia)
FFI (insomnia)
What two types of CJD are common in children?
Iatrogenic
Genetic
Outline the investigations used to diagnosed CJD
EEG
MRI
Spongiform change
What characteristic feature is present on imaging of variant CJD?
Hockey-sticks
What is the most common type of dementia?
Alzheimer’s disease
What type of protein is characteristic of neurodegeneration in Alzheimer’s extracellular and intracellular?
Amyloid plaques
Neurofibrillary tangles
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
