S4: Dementia

Question 1

Define Dementia

Answer 1

Dementia is defined as a syndrome of global impairment and deterioration in cognition leading to a decline in functioning in clear consciousness (i.e. consciousness is not affected).
- Alzheimer’s is the most common type of dementia, multi-infarct (vascular) dementia is another type, Lewy-body dementia is another and frontotemporal dementia.

Question 2

What is a syndrome?

Answer 2

A syndrome is a collection of signs and symptoms.

Question 3

Describe clinical syndrome of dementia

Answer 3

• Neuropsychological symptoms -> amnesia, aphasia, apraxia, agnosia (4As), we can measure these by using screening tools such as the MMSE (mini mental state examination), Addenbrooke’s Cognitive Examination or the MOCA (Montreal Cognitive Assessment).
• Neuropsychiatric symptoms -> Referred to as BPSD, behavioural and psychiatric disorders in dementia, examples include depression, paranoia, hallucinations, anxiety, aggression, wandering, personality change. These symptoms appear as the dementia progresses.
• However in order to be diagnosed as dementia, these symptoms must impact on daily living!

Question 4

What are the two categories that activities of daily living fall into?

Answer 4

1. Instrumental (looking after finances, going shopping, cooking, driving).
2. Basic (dressing, washing, going to toilet).

Question 5

How does dementia affect the types of activities differently?

Answer 5

Instrumental activities tend to be affected earlier on in dementia, then as it progresses the basic activities start to get affected.

Question 6

Describe the ICD-10 diagnosis of dementia

Answer 6

G1i) A decline in memory (both verbal and non verbal). Most evident in the learning of new information.
G1ii) Decline in other cognitive abilities. ‘planning and organizing and in general processing of information.
- Decline objectively verified by informant and quantified cognitive assessments.
- Can be quantified as mild, moderate or severe.
G2) Awareness of environment is preserved (i.e. not delirium).
G3) Decline in emotional control or a change in social behaviour (lability, irritability, motivation, apathy).
G4) Symptoms present for > 6 months.

Question 7

What are the 4 main dementia types?

Answer 7

1. Alzheimer’s disease (50-60% of dementia).
2. Vascular dementia (15-20% of dementias).
3. Dementia with Lewy bodies (10-15%).
4. Frontotemporal (4-6%, however tends to affect younger people below 65, in this age group makes up 20%).

Question 8

Describe the epidemiology of dementia

Answer 8

The overall prevalence of dementia in those aged over 65+ is 7-10%, the prevalence actually doubles every 5yrs from 60-90. E.g. 60-65 (1%), 65-70 (2%), 70-75 (3-5%) etc. The incidence of dementia in those over 75+ is 45 per 1000.

Question 9

Diagnosis pathway of dementia

Answer 9

1. GP.
2. Referral to memory clinic.
3. Diagnosis: history, cognitive testing (e.g. Addenbrooks –> different domains of memory tested), collateral history (as early on patient usually realises they have memory problems while later on they may not realise) , any reversible causes (e.g. Tests for folate, thyroid function).
4. Outcome of assessment:
   - No problem or problem.
   - Mild cognitive impairment is where memory is worse than expected for age (abnormal ageing) but no significant impact on daily life. This makes the patient more likely to develop dementia but it is not treated.
   - Dementia –> scan is not always needed for diagnosis.
   - Imaging (clarifying diagnosis e.g. CT scan for dementia, Alzheimer’s).

Question 10

Describe clinical presentation of Alzheimer’s (AD)

Answer 10

HISTORY:
- Usually some awareness of failing memory e.g. forgetting what you ate for breakfast.
- Concentration and general knowledge ok.
FAMILY:
- 2-3 decline, repetitive.
- Preserved language and practical skills, some problems managing accounts.
PHYSICAL:
- Nil of note.
ACE-III (Addenbrooke’s cognitive examination):
- Some impairment on attention.
- Impaired memory (normal registration but very poor delayed recall).
- Mild impairment on naming.
- Language and visuospatial reasonably intact.
MRI/CT head:
- Hippocampal atrophy mainly seen on scans.

Question 11

7 A’s of Alzheimer’s (symptoms)

Answer 11

• Anosognosia: This is a late sign and means that you no longer realise that there is something wrong. You might not understand why you have a memory problem or that you have a memory problem at all.
• Aphasia is the loss of ability to use language. This includes the ability to speak, understand, read and write.
• Altered perception which is when you misinterpret the information your senses are giving you. For some people, this is a bigger problem in the later afternoon or early evening when light changes.
• Apathy is not having drive or initiative. The part of brain that helps you start to do something either to carry out an activity or to communicate is damaged.
• Agnosia means recognising things through senses is damaged e.g. sight, sound, taste, touch and smell.
• Apraxia means you have lost the ability to tell your body how to carry out purposeful movement.
• Amnesia means loss of memory. This is important because everything we do depends on our ability to remember. At the beginning short term memory will be lost and eventually long term memory will go as well.

Question 12

List non cognitive features of AD

Answer 12

They are psychiatric disturbances such as:

• Depression and anxiety.
• Psychosis (can be persecutory delusions e.g. thinks people stealing from them or partition delusions i.e. objects/people coming through walls) and hallucinations.
• Aggression, wandering (walking aimlessly around), shouting-out.
• Personality change.
• Apathy (lack of interest), or the opposite, disinhibition (lack of restraint).

Question 13

List risk factors for AD

Answer 13

• Being female (could be that women live longer).
• Increasing age.
• Vascular risk factors (e.g. having diabetes, hypertension or atrial fibrillation).
• A family history of AD.
• Apo E4 allele status.
• Having hypothyroidism RR2.3.
• Head trauma RR1.8.
• Being less educated (people who are more educated have more IQ/cognitive reserve so AD symptoms come on much slower). More education –> more neuronal connections –> bigger IQ reserve. So dementia is shrinking brain at same rate but dementia doesn’t look as advanced as in uneducated person.
• So a person who has any of these things is at increased risk of AD.

Question 14

Natural history/timeline of AD

Answer 14

• Symptoms of AD are generally present for 1-3 years prior to referral to secondary care.
• The total illness duration is 10-12 years.
• There is a decrease of about 3 points per year on the MMSE test from moderate to severe AD.

Question 15

Pathophysiology of Alzheimer’s

Answer 15

• AD is caused by a number of changes in the brain, we see death of neurones particularly cholinergic neurones.
• Also seen and to blame is the presence of amyloid plaques these are aggregates of misfolded beta amyloid protein, they are derived from improper cleavage of amyloid precursor protein (APP). The amyloid plaques are toxic to nerve cells.
• We also see neurofibrillary tangles, these are hyperphosphorylated tau protein that disrupt neurones cytoskeleton leading to neuronal dysfunction and death.
• Both amyloid plaques and the tangles are needed to lead to neuronal damage.
• ApO E.
• Also we see vascular pathology that leads to inflammation.

Question 16

Drug management of AD

Answer 16

EARLY DEMENTIA:
- Cholinesterase Inhibitors (increase Ach in brain) AND important to monitor pulse on this.
E.g. Donepezil is OD tablet. It is the most commonly used in AD treatment and well tolerated by patients.
E.g. Rivastigmine in patch or BD tablets. If you have GI effects from donepezil this is given or if you struggle taking tablets.
E.g. Galantamine.
LATER DEMENTIA:
- NMDA receptor antagonist. E.g. Memantine to calm down hyperalertness.

Question 17

Describe vascular dementia

Answer 17

• Post Stroke.
• Often mixed with AD: mixed Alzheimer’s and vascular dementia.
• Extensive small vessel cerebrovascular disease.
  Vascular dementia is caused by reduced blood supply to the brain due to diseased blood vessels. This leads to neuronal cell death causing symptoms of dementia.
• Stepwise decline :
  Patchy decline e.g. good normal memory but lose their language.

Question 18

Types of vascular dementia

Answer 18

1. Large vessel disease caused by:
   - Strategic single infarct e.g. in thalamus.
   - Multiple cortical grey matter infarcts (MID).
   - 20-30% post-stroke develop dementia (not immediately after the stroke).
2. Small vessel disease is most common type caused by:
   - Multiple lacunar infarcts (white matter) due to occlusion of single deep perforating artery.

Question 19

Clinical presentation of vascular dementia

Answer 19

• A 1-2yr history of a deterioration in memory, apathy and limited insight, also a stepwise deterioration (being fine for a while, then declining, then remaining stable then declining).
• Family would report that individual has fluctuating cognition and is more confused at night with some emotional lability (pseudobulbar signs, uncontrolled laughing or crying or other emotional displays).
• A PMH of hypertension, high cholesterol, previous MI and recent TIA.
• ACE III (addenbrookes) would find an episodic memory deficit less severe than in AD, also executive defects.
• MRI would find diffuse white matter pathology as a consequence of occlusion of deep blood vessels.

Question 20

Difference Parkinson’s Disease Dementia/ Dementia with Lewy Bodies

Answer 20

PDD and DLB are similar and essentially two conditions on a spectrum:

• PDD is diagnosed if the Parkinsonian symptoms have existed for at least 12 months prior to the dementia.
• DLB is diagnosed if both motor and cognitive symptoms develop within 12 months, or cognitive symptoms prior to motor.

Question 21

Describe Dementia with Lewy Bodies (DLB)

Answer 21

• Someone with Lewy body dementia has neuronal inclusions (protein aggregates) and abnormally phosphorylated neurofilaments, also ubiquination of α synuclein.
• These are found in the paralimbic, neocortical areas and brainstem and this is responsible for the cognitive symptoms, there is a marked decline in Ach.
• We also see Lewy neurites (small cytoplasmic projections of neurones that are degenerated) and some amyloid plaques.

Question 22

Clinical features of DLB

Answer 22

The clinical features of DLB are quite distinct and different from AD, we see:

• Dementia (generally relating to attention and visuospatial).
• Fluctuating cognitive performance and level of consciousness (the most characteristic feature e.g. in middle of convo start staring into space).
• Visual hallucinations (very common, 60% of DLB, seeing people in house (not little hallucinations)).
• Parkinsonism (70% of DLB).
• Falls.
• Very sensitive to antipsychotics (psychosis caused by high DA, giving antipsychotics decreases DA and this worsens.
• Parkinsonian symptoms. We should avoid giving antipsychotics.
• REM sleep disorder (they have vivid dreams generally nightmares, may be acted out).

Question 23

Describe Parkinson’s Disease Dementia:

Answer 23

PDD is dementia that is a direct pathophysiological consequence of Parkinson’s disease, as mentioned earlier it is where someone has Parkinson’s for at least 12 months and then starts to develop dementia.

Question 24

Clinical features of Parkinson’s Disease Dementia

Answer 24

Parkinson’s disease is characterised by motor symptoms, however there is increasing understanding of non-motor symptoms of Parkinson’s including:
- Dementia.
- Depression.
- Psychosis.
- REM sleep disorder.
In terms of epidemiology, 60-75% of PD patients will eventually develop dementia.

Question 25

Drug management of LBD

Answer 25

- Cholinesterase Inhibitors e.g. Rivastigmine in patch or BD tablets, - NMDA receptor antagonist e.g. Memantine. - Avoid antipsychotics. Antipsychotics block dopamine receptors and in parkinsons/parkinsonism there is too low dopamine so you cannot give this.

Question 26

Describe Frontotemporal Dementia

Answer 26

This used to be called Pick's disease and is due to atrophy of the frontal and anterior temporal lobes. It is a rarer cause of dementia but it is a significant cause (20%) of pre-senile dementia, that is dementia in younger people (below 65). The onest of frontotemporal dementia is typically between 45-65 and males are equally affected as females. - 40% of people with FTD show tau inclusions or "Pick bodies" - Atrophy of the frontal lobe and anterior temporal lobe lead to the symptoms seen, we can class people with FTD into different types of FTD based on their symptoms. - Diagnosis: Poor function on verbal fluency, trail making tests, cog estimates, proverbs. Frontal Lobe Battery is a specific frontal lobe tests which can differentiate the types of frontotemporal dementia.

Question 27

Variations of Frontotemporal Dementia

Answer 27

There are two language sub-types of FTD: - Semantic -> speech fluent but vocabulary diminished (e.g. using word thingy). - Progressive non-fluent aphasia -> slow, hesitant speech. There is also a behavioural variant of FTD diagnosed in patients, in these patients we see: - Alteration in personality and social conduct. - Disinhibition or apathy. - Persevation, utilisation behaviours.

Question 28

Describe relationship between alcohol and dementia

Answer 28

It is unclear if alcohol directly causes dementia, but it is prevalent in those who are alcoholics due to: - Frontal changes to frontal lobe. - Vascular causes, head injury, poor diet. - Wernicke's encephalopathy is an acute syndrome caused by dietary thiamine deficiency, often the result of alcoholism. It is characterised by a triad of symptoms; impaired consciousness, ataxia and opthalmoplegia. - Korsakov's is a chronic syndrome caused by thiamine deficiency often due to alcoholism, patients have profound amnesia for new learning (anterograde amnesia). They have good attention/working memory, but due to amnesia they confabulate a lot. - Wernicke's can progress to Korsakov's.

Question 29

Why is it important to diagnose dementia?

Answer 29

It is really important we diagnose dementia and make the referral to the memory clinic, for a number of reasons: - So the individual can receive treatment to help patient function normally for longer. - Services can be provided to the individual that they require. - Allows patient to plan for future e.g. appointing a LPA, sorting out finances, their care wishes etc, nominate someone to make decisions on your behalf. - So patient can access support services.

Question 30

Describe team involved in memory clinic and long term care

Answer 30

- Psychiatrist -> in the assessment, diagnosis and deciding overall management strategy of the condition, also medication management and making complex decisions. - Occupational therapist -> Can assess the home environment, advise on living skills (e.g. safety in the kitchen) and provide aids/adaptations to the patient home allowing them to be independent for longer. - Psychologist -> to do psychometry and analyse any challenging behaviour. - Social worker -> provide day care, organise home carers or even permanent care if required. - In-patient care.

Question 31

List Behavioural and psychological symptoms (BPSP) in dementia

Answer 31

``` Behavioural and psychological symptoms of dementia are common, 80% of dementia patients have BPSD these include symptoms such as: - Apathy - Anxiety - Delusions - Hallucinations - Depression - Agitation - Wandering - Disinhibition   ```

Question 32

Describe challenging behaviour

Answer 32

There are a number of definitions of challenging behaviour, one being that it is behaviour of such an intensity, duration and frequency that the physical safety of the person or other is placed in serious jeopardy or behaviour that is likely to seriously limit or deny access to ordinary community facilities. - It is a manifestation of distress or suffering - Much challenging behaviour can be understood within the framework of a poorly communicated need. - Challenging behaviour in more advanced dementias. - We can work out if a patient has challenging behaviour by asking for experiences of staff that have worked with or cared for the patient.

Question 33

Why might challenging behaviour arise?

Answer 33

Challenging behaviour can arise due to a combination of factors that come together to affect behaviour, mood and thought. - These include the social environment (how are people treating the patient?) - The physical environment (is it dark, is the layout of room good?) - Biological factors (does patient have dementia? A handicap? Are they in pain or on medication? These can affect mood). - Psychological factors (What is the person expecting? What do they want?). - An example would be a care home patient starting to fight with staff as they don't want to be taken for a shower.

Question 34

How do we address challenging behaviour?

Answer 34

We can address challenging behaviour by monitoring and recording behaviour. One way of doing this is by doing ABC charts. These tell us what happened: 1. Before the defined behaviour (antecedent). 2. During the defined behaviour (behaviour). 3. After the behaviour had taken place (consequence). By understanding these we can help combat and treat the challenging behaviour. This may lead to development of a particular care plan. This involves the team thinking about what the unmet need could be and what is the patient communicating by doing the particular behaviour? Once identified the plan is to address that unmet need and meet the communication needs.

Question 35

Describe impact of dementia in the general hospital

Answer 35

- In normal hospitals about 20% of beds are occupied by someone with dementia! This of course has financial implications among others. In hospitals and care there are concerns regarding the care that some elderly people receive, this appeared in the light of recent scandals. - Using the word acopia when referring old individuals is not helpful and is pejorative as it can deny access to further tests. - Older patients are highly sensitive to change of environment when in hospital, they can also acquire infections more easily. This can result in prolonged delirium and cognitive decline.