Dementia Flashcards
Define Cognition
the mental action of acquiring knowledge and understanding thought, experience and the senses
Rapidly progressive cognitive decline should always lead to suspicion of _______________
CJD (prion disease)
Dementia is early onset if under age ?
65
Who is referred to neurology with dementia vs old age psychiatry?
More than 65 years old with gradual onset dementia and no additional neurology go to old age psychiatry
Less than 65 years old or anyone with unusual features (including rapid speed of onset)/ additional neurology should be referred to neurology
Dementia is a clinical syndrome with multiple causes defined by:
- an acquired loss of higher mental function affecting 2 or more cognitive domains
- being of sufficient severity to cause significant social or occupational impairment
- being chronic and stable (different from delirium which is acute and fluctuating)
List some acute cognitive disorders?
Brain injury/ head injury Stroke Viral encephalitis Transient global amnesia Transient epileptic amnesia
Briefly describe transient global amnesia?
lasts for 4-6hrs (no more than 24hrs!), usually a one off episode, person a bit confused and asking repetitive questions, still aware of themselves and who they are, make a full recovery, not sure of the pathophysiology
Briefly describe transient epileptic amnesia?
associated with temporal lobe seizures that may not be witnessed, asking receptive questions, can carry out complex tasks but doesn’t recall events, responds to anti-epileptic medications
List two subacute cognitive disorders?
functional/ subjective cognitive impairment
prion disease
Describe patient complains in functional/ subjective cognitive impairment?
everyday forgetfulness but patient perceives this as having a great impact on functioning, not unusual forgetfulness e.g. the classic go into a room and forget why there, claims that before they had an excellent memory, mismatch between symptoms reported and actual level of functioning, symptoms don’t match neurodegenerative disorders as they say that some day better than others etc as opposed to a gradual decline
What is the most common human prion disease?
Creutzfeldt-Jakob disease
Describe how prion disease causes cognitive impairment/ dementia?
Misfolded native prion protein (PrPc) can’t be broken down by normal mechanisms so it builds up causing subsequent neurodegeneration. There is a domino effect where once a prion protein misfolds every protein it touches also misfolds
Describe presentation of prion disease?
Causes a rapidly progressive dementia leading to death within six months of onset
How is diagnosis of prion disease made?
marked aggressive presentation, also MRI, EEG and CSF analysis
Most common form of CJD is?
sporadic
List three forms of CJD?
sporadic, iatrogenic, variant
Describe sporadic CJD?
occurs in over 50 yo and thought to be due to spontaneous mutations causing misfolding of the prion proteins
Describe iatrogenic CJD?
can occur as prions are resistant to sterilisation so implants from cadavers for growth hormone or for corneal grafts from those infected could cause the disease, there is an incubation period of several years
Describe variant CJD?
affects young people in 20s and linked to exposure to BSE (mad cow disease causing prion) from contaminated meat
What will all patients with CJD ultimately develop?
spongiform change in the brain
Spongiform change in the brain =
CJD (prion disease)
Most common dementia?
Alzheimers disease
Pathological Hallmarks of Alzheimers disease?
Amyloid Beta protein plaques, intracellular neurofibrillary tangles, amyloid angiopathy, loss of neurons and synapses
What is the initial presentation of Alzheimers?
Forgetfulness
Do those with Alzheimers have insight into their disease?
no
At what stage in the alzheimers is memory lost?
early stages
Describe overview of alzheimers symptoms and when they present?
memory loss is first presentation, as the disease progresses language is impaired, develop apraxia, visuospatial difficulties and loss of executive function, personality remains intact until late on
What lobes are primarily involved in alzheimers?
parietal and temporal
Describe investigations for alzheimers?
MRI shows atrophy of temporal and parietal lobes
SPECT shows temporoparietal decreased metabolism
Describe management of alzheimers disease?
address vascular risk factors as AD and VD are interlinked, can give cholinesterase inhibitors to make nerve cells more active e.g. rivastigimine, galantamine which causes a small but significant improvement
Describe course of alzheimers disease?
insidious onset, gradual progression over a decade of longer
Describe age of onset of frontotemporal dementia?
early onset before age 65
Describe genetics in frontotemporal dementia?
in some people it is related to the C9ORF gene and part of the FTD-MND spectrum (as some with MND develop FTD)
What are the two presentations of FTD and what is most common?
Frontal behavioural presentation is most common but also get a progressive aphasia form
Describe presentation of the frontal behavioural form of FTD?
personality change, apathy, disinhibition, carelessness, stereotyped or compulsive behaviours, early loss of insight
Do those with FTD have insight?
no, there is an early loss of insight
How does presentation of FTD differ from presentation of Alzheimers disease?
FTD has behavioural changes as initial presentation, AD is memory as initial presentation (personality and behaviour changes come late in AD)
Describe investigations for FTD?
MRI show atrophy of frontotemporal lobes
SPECT shows decreased FT metabolism
Management of frontotemporal dementia?
Mainly safety management, may give antipsychotics to help with behaviour changes
Describe age of onset of vascular dementia?
late onset over age 65
Describe diagnosis criteria for vascular dementia?
presence of cerebrovascular disease plus a clear temporal relationship between the onset of dementia and cerebrovascular disease
Describe progression of vascular dementia?
step wise progression
Symptoms of vascular dementia?
depend on areas affected
small vessel disease causes decreased attention, executive dysfunction and slow processing
Management of vascular dementia?
Manage vascular risk factors +/- cholinesterase inhibitors (as may have overlap with alzheimers disease)
Dementia with lewy bodies is caused by aggregates of what protein?
alpha synuclein
Dementia with lewy bodies is associated with what motor condition? How is it related?
parkinsons
not everyone with parkinsons gets dementia with lewy bodies but almost everyone with dementia with lewy bodies has parkinsons
Diagnostic criteria for dementia with lewy bodies?
fluctuating cognition plus recurrent well formed visual hallucinations +/- extrapyramidal features of parkinsons
Treatment of dementia with lewy bodies?
L dopa
Describe difference between dementia with Lewy bodies and parkinsons disease dementia?
parkinsons disease dementia refers to those who develop dementia > 1yr after parkinsons diagnosis, DLB is dementia as a presenting complaint or within a year of PD diagnosis
Huntingtons disease is an ____
early onset dementia age 30-50
Huntingtons disease dementia is treated with?
mood stabilisers
