Dementia Flashcards
What is Dementia?
acquired, chronic, and progressive cognitive impairment, sufficient to impair ADLs
What is BPSD?
Behaviour and Psychological Symptoms of Dementia
What is the epidemiology of dementia?
o <65yo = early-onset dementia
o 5-10% population over 65yo; 20% population over 80yo
o Alzheimer’s disease (70% dementia) > Vascular Dementia (VD) > Dementia with Lewy Bodies (DLB)
What are the symptoms of BPSD?
1) Mood changes
2) Abnormal behaviour
3) Hallucinations/delusions
What are the general signs of dementia?
Forgetfulness
Disorientation to time, place and person (wandering, delusions, calling out, sleep disturbance, hallucination and inappropriate behaviour)
What are the MMSE score boundaries?
24-30 – no impairment
18-23 – mild cognitive impairment
<18 – severe cognitive impairment
What are the screening tools for cognitive assessment?
AMTS (score <7 suggests cognitive impairment)
GPCOG (GP Assessment of Cognition)
Which detailed screening tests can you use for dementia?
Addenbrooke’s (ACE-R), MMSE, MoCA
MMSE = 30 questions [old, not widely used]
ACE-R = 100 questions
Which bloods might you do?
TFTs LFTs U/Es + Dipstick HbA1c Vitamin B12 and folate
What are the 4 more common types of dementia?
Alzheimer’s
Vascular
Lewy Body
Frontotemporal
What tests would you do for the 4 types of dementia?
Alzheimer’s: FDG-PET, CSF, MRI (grey matter atrophy, wide ventricles & sulci, temporal lobe atrophy)
Vascular: ECG (AF with emboli), MRI/CT
Lewy Body: 123I-FP-CIP SPECT (DaTScan; a tracer 123I-FP-CIP used in Single Photon Emission CT), I-MIBG
Frontotemporal: FDG-PET, perfusion SPECT, MRI (frontal lobe shrinkage)
What happens at the memory assessment clinic?
Take a collateral history and check bloods
Risk assess the patient
Cognitive assessment – MMSE
Brain scan (check organic pathology)
Which assessments may you use to differentiate delirium from dementia?
Confusion Assessment Method (CAM)
Observational Scale of Level of Arousal (OSLA)
What questions do you ask on the AMTS?
What's the time What year are we in How old are you (42 west street) Where are you now Name two people here What is your DoB When did WW2 end Who is our current prime minister Count backwards from 20 to 1 Recall (42 west street)
What is Alzheimer’s disease?
The most common type (70%) of dementia; steady progressive cognitive decline
What is the aetiology of Alzheimer’s disease?
Amyloid plaques
Tau tangles
Inflammation
What is the normal physiology of Amyloid protein?
- APP cleaved by a-secretase
- sAPPa released and the C83 fragment remains
- C83 is then digested by a-secretase
- Products are then removed
What is the normal physiology of Tau?
Tau protein is a soluble protein present in axons
Tau important for assembly and stability of microtubules
What is the normal physiology of Inflammation in the brain?
Microglial cells are specialist CNS macrophages
What is the pathophysiology of amyloid?
- APP cleaved by b-secretase
- sAPPb released and the C99 fragment remains
- C99 is digested by b-secretase releasing b-amyloid (Ab) protein
- Ab protein forms the toxic aggregates
What is the pathophysiology of Tau?
- Hyperphosphorylated tau is insoluble -> self-aggregates
- The self-aggregates form neurofibrillary tangles (neurotoxic)
- The tangles result ultimately in microtubule instability and neurotoxic damage to neurones
What is the pathophysiology of Inflammation in the brain?
- Increased inflammatory mediators & cytotoxic proteins
- Increased phagocytosis
- Decreased levels of neuroprotective proteins
Which part of the brain is affected first in AD?
Hippocampus
What are the risk factors for AD?
Age (1% at 60y then doubles every 5 years) Genetics Head Injury Vascular Low IQ Poor education
How does genetics affect AD progression?
(8% of risk; 92% sporadic) – genes: APEN, APP, ApoE, etc.
• Presenilin 1 gene (Chr14)
• Presenilin 2 gene (Chr1)
• Beta-amyloid precursor protein (APP) gene (Chr21)
o Co-existent Down’s syndrome increases risk
What are the signs and symptoms of AD?
Atrophy from neuronal loss
Plaque formation
Neurofibrillary tangle formation
Cholinergic loss
How does AD progress?
Early failing memory, wandering, irritability
Middle the “4 A’s” become apparent
Late fully dependent, incontinence, primitive reflexes, EPSE, etc.
What are the 4 A’s of AD?
Amnesia Recent memories lost first; disorientation occurs early
Aphasia Aphasia in finding correct words (Broca’s), speech muddled/disjointed
Agnosia Typically “Visual” (i.e. prosopagnosia – recognising faces)
Apraxia Typically “Dressing” (skilled tasks, despite normal motor functioning)
How does BPSD present?
Psychiatric presentations delusions (15%), depression (20%), GAD
• Do not use antipsychotics to manage long-term (Risperidone has a short-term licence)
Behavioural disturbances aggression, wandering, sexual disinhibition, explosive temper
What is depressive pseudodementia?
S/S: deficits in memory, deficits in executive function, deficits in speech and language
Recent and long-past events (Alzheimer’s = just early), patchy and specific memory loss (all early)
Why is dementia not Wernicke’s aphasia?
Not a true Wernicke’s aphasia as these aphasias are defined as aphasias without disturbance of intellect
What are the prognostic markers for AD?
Bad prognostic indicators = male, depression, behavioural problems, severe focal cognitive deficit
Good prognostic indicator = female
What is the Biological management of AD?
1st line: Anticholinesterases (mild to moderate AD):
• Donepezil – reversible acetylcholinesterase inhibitor
• Galantamine – reversible acetylcholinesterase inhibitor (a7 nAChR agonist)
• Rivastigmine – pseudo-reversible acetylcholinesterase (AChE) inhibitor & butylcholinesterase (BChE) inhibitor
2nd line (moderate or 1st line in severe AD): NMDA (Glutamate) partial receptor agonist: • Memantine – use-dependant non-competitive NMDA receptor blocker with low channel affinity
What is the psychological management of AD?
- 1st line: Structural group cognitive stimulation sessions (mild to moderate AD)
- Exclude depression or GAD
- Other: group reminiscence therapy, validation (reassure) therapy, multisensory therapy (improve other senses)
What is the social management of AD?
- Explain diagnosis (and signpost support) “AD causes dementia which describes a set of symptoms including memory loss and difficulties with thinking, problem-solving or language. AD is a physical disease that affects the brain”
- Optimise health in other areas (i.e. hearing hearing aids, visual glasses)
- Identify future wishes (i.e. advanced directives, lasting power of attorney)
What other things should you do for a patient with AD?
• FOLLOW-UP (every 6 months): with yourself and a single named care manager (with a clearly defined care plan)
• GENERAL: always wear ID, Dossett boxes, change gas to electricity, assistive technology in the house
• CARERS: identify and support any carers involved (signpost information and support; carer’s assessment)
• SOCIAL SUPPORT: personal care support, meal support, day centre availability
• Legally required to inform DVLA and insurers (if diagnosed with any form of dementia; MCI does not need to inform DVLA)
o Outcome renew licence each year, revoked licence, maintain licence
What do you do before giving an anticholinesterase?
o 1st ECG
o Side effects – GI (N&V, diarrhoea, anorexia), other (fatigue, dizziness, headache)
o Absolute contraindications – anticholinergics (block ACh from binding), beta-blockers, NSAIDs, muscle relaxants
o Relative contraindications – asthma, COPD, GI disease, bradycardia, sick sinus syndrome, AV block
What is the aetiology of vascular dementia?
Infarcts caused by thromboemboli or narrowing of arteries due to HTN
What are the risk factors of Vascular Dementia?
age, male, obesity, lack of exercise, smoking, AF, DM, HTN, CVA history (stroke, TIA)
What are the signs and symptoms of VD?
o Sudden onset (may follow CVA), stepwise deterioration
1st: emotional and minor personality changes (labile emotion – tearful elation)
2nd: cognitive deficit
o Focal neurological signs (S/S reflect site of infarct) – i.e. upgoing plantars, some reserved cognition
o Co-morbid depression
o Relatively preserved personality
What is the management of VD?
o Biological:
Daily aspirin (if indicated due to CVA/AF risk)
Reduce risk factors (exercise, less alcohol, treat HTN, stop smoking, treat AF, control DM)
o Psychosocial:
Same as per AD
What is the aetiology of Lewy Body Dementia?
o Lewy Bodies (LB) = a-synuclein with ubiquitin
o Spectrum of diseases including Lewy Bodies = DLB …all the way to… Parkinson’s disease (PD)
In PD, LB are found in the brainstem;
In DLB, LB are found in the brainstem, cingulate gyrus and neocortex
What is the RFs of DLB?
Age
What are the signs and symptoms of DLB?
o Fluctuating confusion with marked variations in alertness levels may resemble delirium I.E. has some lucid intervals (unlike other dementias) o Vivid visual hallucinations (Lilliputian hallucinations) – animals or humans o Parkinsonism (shuffling gait, bradykinesia, rigidity, amimia – n.b. anosmia is an early sign of PD)
o Frequent falls PD = parkinsonism -> dementia
DLB = dementia -> parkinsonism
o (Co-morbid depression)
“Hallucinations and slow movements”
What is the management of DLB?
o Biological:
1st line: acetylcholinesterase inhibitors (Donepezil or Rivastigmine)
Do not offer antipsychotics (increased risk of cerebrovascular disease)
o Psychosocial:
Same as per AD
What is the aetiology of Fronto-temporal Dementia?
atrophy of fronto-temporal regions
o Early onset (20% pre-senile cases) – 40 to 60yo
o 60% sporadic; 40% autosomal inheritance
CHILDLIKE AND EARLY ONSET
What are the S/S of FTLD?
o (1) Frontotemporal dementia -> frontal lobe syndrome (disinhibition, social/personality changes)
o (2) Semantic dementia -> progressive loss of understanding of verbal and visual meaning
o (3) Progressive non-fluent aphasia -> 1st: naming difficulties; 2nd: mutism
o Memory tends to be affected much later (unlike in AD where it may be the first thing to be affected)
What is the pathology of FTLD?
Pathology (two pathologies with no correlation to clinical presentations):
- Tau positive – “Pick’s” bodies (hyperphosphorylated tau) = Pick’s disease (3R), CBD (4R), PSP (4R)
- Tau negative – no Tau = FTLD-U (Frontotemporal Lobar Dementia with Ubiquinated inclusions)
What is the management of FTLD?
o Biological: Antidepressants (treat frontal lobe syndrome) o Psychosocial: Same as per AD OT, SALT, physiotherapy
What is the prognosis of FTLD?
o Death in 5-10 years
What is the inheritance pattern of Huntingtons?
AD (children has 50% chance)
When is the onset of HD?
30-50yo
Why does onset get younger in successive generations?
Trinucleotide expansion disorder -> genetic anticipation = onset and severity may be younger and greater in successive generations (a feature of all trinucleotide expansion disorders, including Fragile X syndrome)
in HUNTINGTIN gene
What are the S/S of HD?
o Movement – chorea, speech/swallowing, stumbling/clumsiness
o Cognitive – organising tasks, flexibility, impulse control, learning new information, difficulty concentrating
o Psychiatric – depression, irritability/mood swings, suicide in 9% of cases, personality change
o Chorea – involuntary jerking or fidgety movements that tend to flow from one area to another
o Lack of insight (they don’t care that anything is wrong – tends to indicate an organic pathology)
What investigations would you do for HD?
o Genetic analysis (HTT gene)
o MMSE may be normal
