Dementia

Question 1

Pathophysiology of Lewy body dementia?

Answer 1

alpha-synuclein cytoplasmic inclusions (lewy bodies) in the substantia nigra, paralimbic and neocortical areas

Question 2

Relationship between parkinson’s and lewy body dementia?

Answer 2

Complicated, as dementia is often seen in Parkinson’s disease. also up to 40% of patients with Alzheimer’s disiease have lewy bodies

Question 3

Features of Lewy body dementia?

Answer 3

Progressive cognitive impairment:
- typically occurs before parkinsonism, but usually both features occur within a year of each other (contrast to PD where motor symptoms usually present at least one year before cognitive symptoms)

• cognition may be fluctuating, in contrast to other forms of dementia
• in contrast to AD, early impairments in attention and executive function rather than just memory loss

Parkinsonism

Visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen

Question 4

Diagnosis of Lewy body demenia

Answer 4

Usually clinical

single-photon emission computed tomography (SPECT) is increasingly used. commercially known as a DaTscan.

Question 5

Management of Lewy body dementia?

Answer 5

both acetylcholinestera inhibitors (e.g. donepezil, rivastigmine) and memantine can be used as they are in AD.

Neuroleptics (antipsychotics that block dopamine receptors) should be avoided as patients are extremely sensitive and may develop irreversible parkinsonism.

Question 6

Name the stages of dementia?

Answer 6

Pre-dementia
Early
Middle
Late

Question 7

Features of pre-dementia stage?

Answer 7

subtle symptoms such as anosmia (loss of sense of smell)

up to 10 years before clinical signs of dementia

does not impair daily function, and no functional impairment!!

some forgetfulness, difficultly finding some words from time to time

Question 8

Features of early stage dementia?

Answer 8

symptoms are apparent to other people such as:

• forgetting medication
• misplacing objects
• difficulty with finances
• difficulty around the house, organising
• trouble planning

Question 9

Features of middle stage dementia?

Answer 9

Loose the ability to acquire new information

This stage they tend to require assistance with personal care needs (e.g. hygiene)

more behavioural disturbances (e.g. restlessness, aggression

• lack of insight into their condition, problems with disorientation

Question 10

Features of late stage dementia?

Answer 10

patients often believe they are in a different time in their life (typically earlier in their life) called TIME SHIFT

ability to speak may be lost (few words)

depression and anxiety more common

hallucination may be more present

aggression and restlessness

dangers may not be recognised (e.g. hot stoves)

reduced mobility (e.g. apiration pneumonia due to dysphagia)

poor nutrition

Question 11

most common type of dementia

Answer 11

Alzheimers : 70% of dementia

Question 12

Pathophysiology of Alzheimers disease

Answer 12

Macroscopic:
- widespread cerebral atrophy, particularly in cortex and hippocampus

Microscopic:
- cortical plaques due to deposition of type A-Beta-amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregation of tau protein

• hyperphosphorylation of tau protein

Biochemical:
- deficit of acetylcholine from damage to an ascending forebrain projection

Question 13

Risk factors of AD

Answer 13

Increasing age
Family history of AD

5% inherited as autosomal dominant trait mutations in amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes are thought to cause the inherited form

apoprotein E allele E4 - encodes a cholesterol transport protein

Caucasian ethnicity

Down’s syndrome

Question 14

Pharmacological management of AD?

Answer 14

the 3 acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing milt to moderate AD

memantine (NMDA receptor antagonist) in simple terms second line treatment used for patients:
- patients intolerant or have contraindications to acetylchoninestase inhibitors
- add on drug for patients with moderate to severe disease
- monotherapy in severe AD

Question 15

Non-pharmacological management of AD?

Answer 15

NICE recommends offering ‘a range of activities to promote wellbeing that are tailored to person’s preference’
- offering group cognitive stimulation therapy for patients with mild and moderate dementia
- group reminiscence therapy and cognitive rehabilitation

Question 16

Donepezil contraindications?

Answer 16

relatively CI in patients with bradycardia
adverse effects include insomnia

(anticholinesterase inhibitor)

Question 17

Second most common form of dementia?

Answer 17

Vascular dementia (VD)

Question 18

Pathophysiology of vascular dementia

Answer 18

Not a single disease but a group of syndromes of cognitive impairment caused by different mechanisms causing ischaemia or haemorrhage secondary to cerebrovascular disease

Question 19

Epidemiology of vascular dementia

Answer 19

accounts for 17% of dementia in the UK

Prevalence of dementia following a first stroke varies depending on location and size of the infarct, definition of dementia, interval after stroke and age among other variables. Overall, stroke doubles the risk of developing dementia.

increases with age

Question 20

Main subtypes of VD?

Answer 20

Stroke-related V: multi-infarct or single-infarct dementia

Subcortical VD: caused by small vessel disease

Mixed dementia: presence of both VD and AD

Question 21

Risk factors of VD?

Answer 21

History of stroke or transient ischaemic attack (TIA)
Atrial fibrillation
Hypertension
Diabetes mellitus
Hyperlipidaemia
Smoking
Obesity
Coronary heart disease
A family history of stroke or cardiovascular

rarely, VD can be inherited as in the case of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencepahlpathy)

Question 22

Symptoms of VD?

Answer 22

Symptoms and the speed of progression vary but may include:

Focal neurological abnormalities e.g. visual disturbance, sensory or motor symptoms

The difficulty with attention and concentration

Seizures

Memory disturbance

Gait disturbance

Speech disturbance

Emotional disturbance

Question 23

Diagnosis of VD is based on?

Answer 23

A comprehensive history and physical examination

Formal screen for cognitive impairment

Medical review to exclude medication cause of cognitive decline

MRI scan – may show infarcts and extensive white matter changes

(NICE recommends using the NINDS-AIREN criteria )

Question 24

What criteria is used for diagnosis of VD and what is included in it?

Answer 24

NINDS-AIREN criteria

Presence of cognitive decline that interferes with activities of daily living, not due to secondary effects of cerebrovascular event:
- established using clinical exams and neuropsychological testing

Cerebrovascular disease
- defined by neurological signs and/or brain imaging

A relationship between the above 2 disorders inferred by:
- onset of dementia within 3 months following a recognised stroke
- an abrupt deterioration in cognitive functions
- fluctuating, stepwise progression of cognitive deficits

Question 25

Non-pharmacological management of VD?

Answer 25

Tailored to the individual

Include: cognitive stimulation programmes, multisensory stimulation, music and art therapy, animal-assisted therapy

Managing challenging behaviours e.g. address pain, avoid overcrowding, clear communication

Question 26

Pharmacological management of VD?

Answer 26

There is no specific pharmacological treatment approved for cognitive symptoms

Only consider AChE inhibitors or memantine for people with vascular dementia if they have suspected comorbid Alzheimer’s disease, Parkinson’s disease dementia or dementia with Lewy bodies.

There is no evidence that aspirin is effective in treating patients with a diagnosis of vascular dementia.

No randomized trials found evaluating statins for vascular dementia

Question 27

Order of most common causes of dementia?

Answer 27

1. AD
2. VD
3. Dementia with lewy bodies
4. Frontotemporal dementia (so 3rd most common cause of cortical dementia after AD and lewy body)

Question 28

What are the 3 types of Frontemporal lobar degenerations (FTLD)?

Answer 28

• Frontotemporal dementia (pick’s disease)
• Progressive non fluent aphasia (chronic progressive aphasia, CPA)
• Semantic dementia

Question 29

Common features of frontotemporal lobar dementias?

Answer 29

• onset before 65
• indidious onset
• relatively preserved memory and visuospatial skills
• personality change and social conduct problems

Question 30

what is Picks disease?

Answer 30

known as frontotemporal dementia

characterised by personality change and impaired social conduct. other common features include hyperorality, disinhibition, increased apetite, and perserveration behaviours

Question 31

Pathology of picks disease?

Answer 31

focal gyral atrophy with a knife-blade appearance is characteristic of Pick’s disease

Macroscopic changes:
- atrophy of the frontal and temporal lobes

Microscopic changes include:
- pick bodies (spherical aggregations of tau protein, silver-staining
- Gliosis
- Neurofibrillary tanges
Neurofibrillary tangles
- Senile plaques

Question 32

Management of Frontotemporal dementia?

Answer 32

NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia

Question 33

What is chronic progressive aphasia (CPA) ?

Answer 33

(type of frontotemporal lobe degeneration)

chief factor is non fluent speech. they make short utterances that are agrammatic. comprehension is relatively preserved