Dementia Flashcards

1
Q

What is dementia?

A

Dementia is a syndrome of generalised decline of memory, intellect and personality, without impairment of consciousness and leading to functional impairment.

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2
Q

What are the different types of dementia?

A
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3
Q

Briefly describe the pathophysiology and aetiology of dementia

A

In Alzheimer’s disease there is degeneration of cholinergic neurons in the nucleus basalis of Meynert leading to a deficiency of medulla oblongata pons acetylcholine. Other pathophysiological changes can be divided into microscopic and macroscopic:

Microscopic → Neurofibrillary tangles (intracellularly) and β-amyloid plaque formation (extracellularly).

Macroscopic → Cortical atrophy (commonly hippocampal), widened sulci and enlarged ventricles.

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4
Q

Give examples of irreversible causes of dementia

A

Neurodegenerative: Alzheimer’s disease, fronto-temporal dementia, Pick’s disease, dementia with Lewy bodies (DLB), Parkinson’s disease with dementia and Huntington’s disease.

Infections: HIV, encephalitis, syphilis and CJD.

Toxins: alcohol, barbiturates and benzodiazepines.

Vascular: vascular dementia, multi-infarct dementia and CVD.

Traumatic head injury.

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5
Q

Give examples of reversible causes of dementia

A

Neurological: normal pressure hydrocephalus, intracranial tumours and chronic subdural haematoma.

Vitamin deficiencies: B12 , folic acid, thiamine and nicotinic acid (pellagra).

Endocrine: Cushing’s syndrome and hypothyroidism.

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6
Q

What are the reversible and preventable causes of dementia?

Note: ‘DEMENTIA’

A
  • Drugs (e.g. barbiturates)
  • Eyes and ears (visual/hearing impairment may be confused with dementia)
  • Metabolic (Cushing’s, hypothyroidism)
  • Emotional (depression can present as a pseudodementia),
  • Nutritional deficiencies / normal pressure hydrocephalus
  • Tumours / trauma
  • Infections (e.g. encephalitis)
  • Alcoholism / atherosclerosis (vascular)
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7
Q

Briefly describe the pathophysiology and aetiology of vascular dementia (VaD)

A

Vascular dementia (VaD) occurs as a result of cerebrovascular disease , either due to stroke, multi-infarcts (multiple smaller unrecognized strokes) or chronic changes (arteriosclerosis) in the small vessels.

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8
Q

Briefly describe the pathophysiology and aetiology of Lewy body dementia (DLB)

A

In Lewy body dementia (DLB), there is abnormal deposition of a protein (Lewy body) within the neurons of the brainstem, substantia nigra and neocortex. Outside the brainstem LBs are associated with more profound cholinergic loss than in AD. Within the brainstem, they are associated with dopaminergic loss and parkinsonian-like symptoms.

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9
Q

Briefly describe the pathophysiology and artiology of frontotemporal dementia

A

In fronto-temporal dementia there is specific degeneration (atrophy) of the frontal and temporal lobes of the brain. One type of fronto-temporal dementia is Pick’s disease, where protein tangles (Pick’s bodies) are seen histologically.

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10
Q

Dementias can be divided on the basis of predominance of cortical, subcortical or mixed dysfunction.

Differentiate the location of:

  • Alzheimer’s disease
  • Fronto-temporal
  • Lewy body dementia
  • Vascular dementia
A

Cortical dementias include AD and fronto-temporal dementia.

Subcortical dementias include DLB.

Vascular dementia is mixed.

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11
Q

Briefly differentiate between cortical and subcortical dementia

Note: memory loss, mood, speech and language, personality, coordination, praxis and motor speed

A
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12
Q

How common is dementia?

A

There are currently 800 000 people with dementia in the UK and it is estimated that there will be over one million by 2021.

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13
Q

How does increasing age affect the likelihood of dementia?

A

Dementia increases with age (rare if <55 years; 5– 10% if >65 years; and 20% if >80 years ).

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14
Q

Is dementia more common in men or women?

A

Overall prevalence is similar in ♂ and ♀, but AD is more common in ♀, whereas vascular and mixed dementias are more common in ♂.

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15
Q

What are the risk factors for AD?

A
  • Advancing age
    • The incidence of AD increases with advancing age
  • Family history
    • The lifetime risk of AD in first degree relatives of those affected is 25– 50%
  • Genetics
  • Down’s syndrome
    • The mutations in trisomy 21 are associated with the Down’s syndrome development of pre-senile AD
  • Low IQ
    • Lower educational attainment and lower IQ scores are associated with higher risks of developing dementia
  • Cerebrovascular disease
    • Strong risk factor for vascular dementia which can co-exist with AD
  • Vascular risk factors
    • E.g. Past stroke/MI, smoking, hypertension, diabetes and high cholesterol are risk factors for both AD and vascular dementia
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16
Q

What genes are asscociated with dementia?

A

There are several genes which play a role in Alzheimer’s disease:

Presenilin 1 (chromosome 14), Presenilin 2 (chromosome 2) and amyloid precursor protein (chromosome 21) are genes associated with early onset AD.

ApoE-4 (chromosome 19) is a susceptibility gene that contributes to late onset AD. The ApoE-2 variant is thought to be protective.

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17
Q

Briefly describe the ICD-10 Classification of dementia

A

A. Evidence of the following:

    1. A decline in memory, which is most evident in the learning of new information, although in more severe cases, the recall of previously learned information may also be affected.
  1. A decline in other cognitive abilities, characterised by deterioration in judgement and thinking, such as planning and organising, and in the general processing of information.

B. Preserved awareness of the environment for a period of time long enough to demonstrate (A).

C. A decline in emotional control or motivation, or a change in social behaviour, manifested by one of the following: (1) Emotional lability; (2) Irritability; (3) Apathy; (4) Coarsening of social behaviour.

D. For a confident diagnosis (A) must have been present for at least 6 months .

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18
Q

Briefly describe the symptomatic progression of AD

Note: early stages, disease progression and later stages

A

Early stages: memory lapses, difficulty finding words and forgetting names of people/places.

Disease progression: apraxia, confusion, language problems and difficulty with executive thinking.

Later stages: disorientation to time and place, wandering, apathy, incontinence, eating problems, depression and agitation.

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19
Q

Briefly differentiate between early and late onset AD

A

AD can be classified into early onset or pre-senile (<65 yrs, familial) and late onset or senile (>65 yrs, sporadic), but it usually occurs after the age of 65. It has an insidious onset over years.

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20
Q

What are the clinical features of AD?

A

Loss of memory is the commonest presenting symptom. Initially there is inability to recall new information, and remote memory (long-term memory) declines with disease progress.

Disorientation to time and place is closely related to memory impairment.

Impairment of cognitive and executive functions:

  • Executive functions
  • Visuospatial abilities
  • Language disturbances (dysphasia)
  • Apraxia
  • Agnosia

Non-cognitive symptoms:

  • Perception (hallucinations)
  • Thought content (delusions)
  • Emotion (depression, apathy)
  • Behaviour (wandering, aggression, restlessness)
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21
Q

Briefly describe how executive functions are affected in AD

A

Problem solving, abstract thinking, reasoning, decision making, judgement, planning, organization and processing.

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22
Q

Briefly describe how visuospatial abilities are affected in AD

A

Getting lost, impaired driving and copying figures.

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23
Q

Briefly describe how language is affected in AD

Note: known as dysphagia

A

Word finding difficulties, decreased vocabulary, perseveration (uncontrollable repetition of a particular response, such as a word, phrase, or gesture) and global aphasia (impairment of language, affecting the production or comprehension of speech and the ability to read or write).

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24
Q

What is apraxia?

Note: a feature of AD

A

Inability to carry out previously learned purposeful movements despite normal coordination and strength e.g. dressing, unbuttoning shirt.

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25
Q

What is agnosia?

Note: a feature of AD

A

Impaired recognition of sensory stimuli not attributed to sensory loss or language disturbance e.g. object agnosia, auditory agnosia.

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26
Q

Briefly describe the ICD-10 Criteria of early onset AD

A

A. The general criteria for dementia A– D must be met.

B. No evidence for any other possible cause of dementia or systemic disorder.

Early onset Alzheimer’s disease

A. General criteria for Alzheimer’s met and age of onset is <65.

B. At least one of the following must be met:

  1. Relatively rapid onset and progression;
  2. In addition to memory impairment there is aphasia, agraphia ( ↓ ability to communicate through writing), alexia ( ↓ ability to read), acalculia ( ↓ ability to perform mathematical tasks) or apraxia .
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27
Q

Briefly describe the ICD-10 Criteria for late onset AD

A

A. The general criteria for dementia A– D must be met.

B. No evidence for any other possible cause of dementia or systemic disorder.

Late onset Alzheimer’s disease

A. General criteria for Alzheimer’s met and age of onset is >65.

B. At least one of the following must be met:

  1. Slow, gradual onset and progression;
  2. Predominance of memory impairment over intellectual impairment.
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28
Q

Briefly describe how vascular dementia presents

A

Usually presents in the late sixties or early seventies.

Stepwise rather than continuous deterioration i.e. stepwise increases in severity of symptoms.

Memory loss.

Emotional (depression, apathy) and personality changes (earlier than memory loss).

Confusion is common.

Neurological symptoms or signs (e.g. unilateral spastic weakness of the limbs or increased tendon reflexes, an extensor plantar response or pseudobulbar palsy).

On examination there may be focal neurology (often upper motor neurone signs) and signs of cardiovascular disease elsewhere.

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29
Q

Briefly describe how mixed dementia presents

A

Features of both Alzheimer’s disease and vascular dementia.

30
Q

Briefly describe how dementia with Lewy bodies (DLB) presents

A

Day to day fluctuations in cognitive performance.

Recurrent visual hallucinations.

Motor signs of parkinsonism (tremor, rigidity, bradykinesia).

Recurrent falls, syncope, depression.

Severe sensitivity to neuroleptic drugs.

People with Parkinson’s disease who develop dementia after 12 months are diagnosed as having Parkinson’s disease with dementia as opposed to DLB where dementia and parkinsonian features within 12 months of one another.

31
Q

Briefly describe how fronto-temporal dementia presents

Note: also includes Pick’s disease

A

Usually occurs between the ages of 50 and 60 and develops insidiously.

Family history is positive in 50% of cases.

Early personality changes: e.g. disinhibition (reduced control over one’s behaviour), apathy/restlessness.

Worsening of social behaviour.

Repetitive behaviour.

Language problems: e.g. difficult to find word, problems naming/ understanding words.

Memory is preserved in early stages whereas insight is lost early.

32
Q

Briefly describe how Huntington’s disease presents

A

Autosomal dominant, therefore strong family history.

Abnormal choreiform movements of face, hands and shoulders and gait abnormalities.

Dementia presents later.

33
Q

Briefly describe how normal pressure hydrocephalus presents

A

Average age of onset after 70.

Triad of dementia with prominent frontal lobe dysfunction, urinary incontinence and gait disturbance (wide gait).

34
Q

Briefly describe how Creutzfeldt– Jakob disease (CJD) presents

A

Onset usually before 65.

Rapid progression with death within 2 years.

Disintegration of virtually all higher cerebral functions.

Dementia associated with neurological signs (pyramidal, extrapyramidal, cerebellar).

35
Q

Briefly describe the MSE for dementia

A

Appearance and behaviour: May appear unkempt with poor self-care. Behaviour may be inappropriate e.g. in fronto-temporal dementia due to disinhibition. Uncoordinated or restless.

Speech: slow, confused. Difficulty finding right word. Repetitive.

Mood: low or normal. Disturbance of affect more common in VaD.

Thought: may have delusions.

Perception: hallucinations are a core feature in DLB. May have illusions.

Cognition: affected in all dementias but to varying degrees depending on the type and severity of dementia. Memory impairment is most severe in cortical dementias. There is usually impaired attention and disorientation.

Insight: may be preserved initially but is invariably lost in the latter stages of the disease.

36
Q

What routine investigations should ordered for dementia?

A

Routine investigations

Blood tests:

  • FBC (infection, anaemia)
  • CRP (infection, inflammation)
  • U&Es (renal disease)
  • Calcium (hypercalcaemia)
  • LFTs (alcoholic liver disease)
  • Glucose (hypoglycaemia)
  • Vitamin B12 and folate (nutritional deficiencies)
  • TFTs (hypothyroidism)
37
Q

What non-routine investigations should be ordered for dementia?

Note: guided by clinical assessment

A

Non-routine investigation (guided by clinical assessment)

  1. Urine dipstick
  2. Chest X-ray
  3. Syphilis serology and HIV testing
  4. Brain imaging
  5. ECG
  6. EEG
  7. Lumbar puncture
  8. Genetic tests
  9. Cognitive assessment
38
Q

Why investigate dementia via urine dipstick?

A

Rule out UTI.

39
Q

Why investigate dementia via chest X-ray?

A

Assess for pneumonia and lung tumour.

40
Q

Why investigate dementia via syphilis serology and HIV testing?

A

Only if there are atypical features or special risks.

41
Q

Why investigate dementia via brain imaging?

A

Imaging is only indicated for dementia if there is early onset (<60 years), sudden decline, high risk of structural pathology, focal CNS signs or symptoms (to rule out space-occupying lesions, e.g. subdural haematoma, abscess and tumour), or to monitor disease progression.

42
Q

What methods of brain imaging are used for dementia?

A

CT scan: usual imaging modality. Can identify hippocampal atrophy.

MRI: identifies posterior circulation vascular pathology with much greater sensitivity.

SPECT: rarely used in specialist centres to reliably differentiate between Alzheimer’s disease, vascular dementia and fronto-temporal dementia.

43
Q

Why investigate dementia via ECG?

A

If cardiovascular disease suspected.

44
Q

Why investigate dementia via EEG?

A

If fronto-temporal lobe dementia or CJD is suspected, or where seizure activity is a possibility.

45
Q

Why investigate dementia via lumbar puncture?

A

If meningitis or CJD is suspected.

46
Q

Why investigate dementia via genetic testing?

A

For Huntington’s disease and familial dementia.

47
Q

What cognitive assessments are used for dementia?

A
  • Folstein Mini-Mental State Examination
  • The Abbreviated Mental Test (AMT)
  • The Addenbrooke’s Cognitive Examination (ACE)
  • General Practitioner Assessment of Cognition (GPCOG)
  • The Montreal Cognitive Assessment (MOCA)
48
Q

What functional capacity needs to be assessed in dementia patients?

A

Enquire about functional capacity in dementia patients.

The following areas of functional capacity should be explored:

  • Dressing
  • Continence
  • Self-care
  • Shopping/ housework
  • Ability to manage financial affairs
  • Social contacts
  • Safety in the home
  • Ability to cook
  • Nutrition
  • Orientation
49
Q

Briefly describe The Folstein Mini-Mental State Examination (MMSE)

A

Generally speaking, a quick and informal cognitive assessment can be carried out by recording the following:

  • Orientation in time, place, and person
  • Attention and concentration, e.g. serial sevens test. Record the time taken and the number of errors
  • Memory:
    • Short-term memory
    • Recent memory
    • Remote memory
  • Grasp e.g. name the prime minister and reigning monarch

If cognitive impairment is suspected, you can carry out the Folstein Mini-Mental State Examination (MMSE).

The MMSE is scored out of 30. Scores of less than 22 are indicative of significant cognitive impairment, while scores of 22 to 25 are indicative of moderate cognitive impairment.

50
Q

When is the MMSE invalid?

A

The result is invalid if the patient is delirious or has an affective disorder.

51
Q

Briefly interpret the results of an MMSE

Note: 25-30, 21-24, 10-20, 10-14 and <10

A

Normal: MMSE 25– 30.

Mild: MMSE 21– 24.

Moderate: MMSE 10– 20.

Moderate– severe: MMSE 10– 14.

Severe: MMSE <10.

52
Q

Briefly describe tests to assess the frontal lobe

A

There are a number of frontal lobe tests that are useful adjuncts when considering a diagnosis of fronto-temporal dementia:

  • Verbal fluency and initiation
  • Cognitive estimates
  • Clock drawing test
  • Similarities (conceptualization)
  • Motor sequencing (Luria’s 3 step test)
53
Q

How is verbal fluency and initiation tested?

Note: assesses frontal lobe

A

Ask the patient to recall as many words as possible in one minute starting with the letter ‘S’. Fewer than 10 words is abnormal. Should aim for >15.

54
Q

How is cognitive estimates tested?

Note: assesses frontal lobe

A

Ask the patient to make educated guesses to questions which they are unlikely to know the specific answer to e.g. ‘what is the age of the oldest person in the country?’

55
Q

Briefly describe the clock drawing test

Note: assesses frontal lobe

A

Tests executive function. Ask the patient to draw a large clock face, put the numbers in and then make the clock show ten past five.

56
Q

How is similarities (conceptualisation) tested?

Note: assesses frontal lobe

A

Ask in what way two objects are alike e.g. banana and orange (both fruits), table and chair (both items of furniture), tulip and rose (both types of flower).

57
Q

How is motor sequencing (Luria’s 3 step test) tested?

Note: assesses frontal lobe

A

Tell the patient you are going to show them a series of hand movements. Demonstrate fist, edge, palm 5 times without verbal prompts and ask them to repeat.

58
Q

Briefly describe the first-line and adjunct management strategies from AD

A

First-line

  • Supportive treatment (e.g. OT input for home safety evaluation)
  • Environmental control measures (e.g. motion sensors for patients at risk of wandering)
  • Acetylcholinesterase inhibitors

Adjuncts

  • Antidepressants
  • Antipsychotics
  • Management of insomnia (e.g. trazadone)
  • Management of behavioural and psychological symptoms
  • Adding in or switching to memantine (initially 5 mg OD)
59
Q

Do the DVLA need to be informed following a diagnosis of dementia?

A

After a diagnosis of dementia is made, patients are legally obliged to contact the Driver and Vehicle Licensing Agency (DVLA). They may be able to continue driving subject to medical reports and annual review.

60
Q

What discussions need to take place in case of further deterioration in patients with dementia?

A

Early discussions should take place to allow advance planning prior to cognition deteriorating. Topics include advance statements or decisions , lasting power of attorney and preferred place of care plans.

In the later stages of the disease, if patients are not competent to make a decision, the requirements of the Mental Capacity Act 2005 should be adhered to.

61
Q

Briefly describe the non-pharmacoogical management of dementia

A

The aims of treatment are to promote independence, maintain function and treat symptoms including cognitive, non-cognitive (hallucinations, delusions, anxiety, marked agitation and associated aggressive behaviour), behavioural and psychological:

  • Social support including support groups such as Alzheimer’s Society
  • Increasing assistance with day-to-day activities
  • Information and education Community dementia teams
  • Home nursing and personal care
  • Community services such as meals-on-wheels, befriending services, day centres, respite care and care homes
  • For non-cognitive symptoms or behaviour that challenges, aromatherapy, massage and therapeutic use of music or animal-assisted therapy may be considered
62
Q

What are the principles of managing dementia?

A
  1. Cognitive enhancement (AChE inhibtors)
  2. Treat agitation
  3. Treat low mood and insomnia
  4. Functional support
  5. Social support
  6. Support for carers
63
Q

What is the role of acetylcholinesterase (AChE) inhibitors in managing dementia?

A

The three acetylcholinesterase (AChE) inhibitors (donepezil, galantamine and rivastigmine) are recommended as options for managing mild to moderate Alzheimer’s disease. They can also be used in dementia with Lewy bodies, in support cases where non-cognitive symptoms cause significant distress.

64
Q

What is the role of NMDA (N-methyl-D-aspartate) receptor antagonists in managing dementia?

A

Memantine is an NMDA (N-methyl-D-aspartate) receptor antagonist and is an option for Alzheimer’s disease in the following circumstances:

  • Moderate Alzheimer’s disease in those who are intolerant carers of or have a contraindication to AChE inhibitors
  • Severe Alzheimer’s disease
65
Q

How is challenging behaviour in dementia patients treated?

A

For behaviour that challenges, if non-pharmacological strategies have proved ineffective, a short course of an antipsychotic (e.g. risperidone) can be used. For low mood , antidepressants (e.g. sertraline) can be initiated.

66
Q

What can use of antipsychotic medication in dementia lead to?

A

Use of antipsychotics in dementia with Lewy bodies can cause severe adverse effects including neuroleptic sensitivity reactions or worsening of extrapyramidal features.

67
Q

Briefly describe the mechanism of action of acetylcholinesterase inhibitors

A

Acetylcholinesterase inhibitors are centrally acting agents that work by compensating for the depletion of acetylcholine in the cerebral cortex and hippocampus in AD.

68
Q

When are acetylcholinesterase inhibitors contraindicated?

A

They are cautioned in arrhythmias (sick sinus syndrome and other supraventricular conduction abnormalities), peptic ulcer disease and asthma/COPD. Galantamine is contraindicated in severe renal or hepatic impairment.

69
Q

What are the side effects of acetylcholinesterase inhibitors?

A

Side effects include gastrointestinal disturbances, bradycardia and muscle spasms.

Rivastigmine may cause extrapyramidal side effects.

70
Q

What are the doses of donepezil, rivastigmine and galantamine?

Note: acetylcholinesterase inhibitors

A

Doses:

  • Donepezil (5– 10 mg OD)R
  • ivastigmine (1.5– 6 mg BD)
  • Galantamine (4– 12 mg BD)
71
Q

What differentials should be considered for dementia?

A
  • Normal ageing and mild cognitive impairment
  • Delirium
  • Trauma
    • Stroke, hypoxic or traumatic brain injury
  • Depression (‘pseudodementia’)
    • Poor concentration and impaired memory are common in depression in the elderly
    • Identify whether the low mood or poor memory came first
  • Late onset schizophrenia
  • Amnesic syndrome
    • Severe disruption in memory with minimal deterioration in cognitive functioning
  • Learning disability
  • Substance misuse
  • Drug side effects
    • Opiate and benzodiazepine