Dementia

• Associated deterioration in language (aphasia), perception and comprehension (apraxia)
• Impaired ability to recognise objects (agnosia)
• And/or a disturbance in executive functioning (inability to think abstractly and plan, initiate, sequence, monitor and stop complex behaviour)

Deterioration must represent a progressive decline from a previous higher level of functioning, and consciousness should not be clouded (compare with acute confusional state or delirium).

Memory loss is typically for recent events and long-term memory can be remarkably intact.

Incidence

There are an estimated 163,000 new cases of dementia identified each year in England and Wales.¹
It increases with age:

• 6.7 per 1,000 person years at age 65-69.
• 68.5 per 1,000 person years at age 85 and above.¹

Prevalence

Very rare below 55 years. 3% prevalence by 70 years and doubles every 5.1 years thereafter.
According to UK figures provided by the Alzheimer's Society, prevalence is:²

• 1:1,000 in patients aged 40-65
• 1:50 in 65-70 year-olds
• 1:20 in 70-80 year-olds
• 1:5 in the 80+ age group

Multi-infarct dementia is much less common nowadays. This is almost certainly because practices are diagnosing hypertension and treating it energetically at an early stage.

There are subtle differences in the presentation of different types of dementia. These are covered in detail in their specific records.

In contrast to acute confusional state, which is usually of recent onset and may have a reversible cause, the history should go back at least several months and usually several years.

Consider dementia with Lewy bodies (DLB) in elderly patients presenting with hallucinations, lucid periods, movement disorders, falls or syncope. Making this diagnosis will have important implications for treatment (use of neuroleptics in these patients causes 2 to 3-fold excess mortality).⁵

For objective evidence, carry out a test of cognitive functioning (see below).

The diagnosis of dementia should only be made after:

• Comprehensive history and physical examination. The key to diagnosis is a good history of progressive impairment of memory and other cognitive functioning (usually requiring the help of a spouse, relative or friend).
  
  Make specific notes on the following:⁶
  • Attention and concentration ability
  • Orientation - time, place, person
  • Memory - both short- and long-term
  • Praxis - can they get dressed, lay a table, etc.
  • Language function (usually evident during questioning)
  • Executive function - problem-solving, etc.
• Conduct a formal screen for cognitive impairment - see separate article Screening for Cognitive Impairment.
• Other reversible organic causes have been excluded.

• Ensure no treatable cause has been missed by arranging FBC, ESR or CRP, MSU, U&E, LFT, glucose, Ca²⁺, TFT, B12 and folate (red cell folate). Don't always believe low-normal B12s: cellular deficiency may coexist - if in doubt ,one should treat (homocysteine metabolites may be a better test).⁸
• Consider blood cultures, CXR and CT/MRI scan, psychometric testing as appropriate to confirm diagnosis.
• VDRL/TPHA should not be performed routinely - only if risk factors are present.
• CSF examination should be used if Creutzfeldt-Jakob disease or other forms of rapidly progressive dementia are suspected.⁹
• Patients with mild cognitive impairment (MCI) should be referred to memory assessment clinics. MCI is defined as a decline in cognitive function greater than expected, taking account of the subject's age and education, which is not interfering with activities of daily living (ADL).
• Genetic clinical genotype analysis should only be requested where an inherited cause is suspected.⁶

Priorities for implementation:

• Patients with dementia should not be discriminated against when considering treatment options for other conditions.
• Their valid consent should be sought for treatment wherever possible. This may mean making information available to them in an appropriate form. The use by patients and carers of advocacy services and voluntary organisations should be encouraged. If patients are not competent to make a decision, the requirements of the Mental Capacity Act 2005 (which received Royal Assent on 7 April 2005 and was fully implemented on 1 October 2007) should be followed.
• Carers should receive an assessment of needs as required by the Carers and Disabled Children Act 2004 and the Carers (Equal Opportunities) Act 2004. Carers should be offered individual or group psychoeducation and psychological therapy, peer-support groups, information in a variety of media, and training courses. Issues such as transport, night-sitting, and respite care should also be considered.
• Health and social care managers should take a joint approach to management, and this should include joint written policies and procedures, and joint planning of services which take on board the views of local service users and carers.
• Care managers and coordinators should ensure that a combined care plan takes account of the changing needs of the patient and the carers, is reviewed regularly, and receives the endorsement of the patient and carers.
• Named health and/or social care staff should be assigned to operate the plan.
• A memory assessment service should act as the single point of referral for all patients with a suspected diagnosis of dementia.

This means tailoring services to the individual needs of the person as far as possible. If the patient does not have capacity to make decisions, the Department of Health guidance should all be followed.¹⁰Whilst the patient is in a relatively early stage of the condition, opportunity should be taken to discuss such issues as advanced statements (which outline how they would like decisions taken on their behalf, should they lose capacity), advance decisions about refusing treatment, Power of Attorney, and preferred place of care.
The Mental Capacity Act 2005 has the following principles:

• Adults must be assumed to have capacity to make decisions about their care unless proved otherwise.
• Individuals should be given all available support to help them come to a decision.
• Individuals should retain the right to make what others might consider eccentric or unwise decisions.
• Anything done on behalf of an individual without capacity must be in that person's best interests.
• The rights and basic freedoms of an individual without capacity should be restricted as little as possible.
• With the patient's consent, relatives and carers should be involved in management decisions. Relatives and carers should also have their own needs assessed.

Diversity and equality

This section in the National Institute for Clinical Excellent (NICE) guidance is a reminder about the importance of respecting individuality:
'Patients and carers should be given support appropriate to their needs and should not be discriminated against in terms of race, language, religion or sexuality. Focused support should be available for younger people with dementia, and for those with learning disabilities.'

Social, sexual and financial support

The effects of the condition on existing personal and sexual relationships should be discussed and patients and carers given information about local support services if appropriate.
Information should be provided about financial issues, including direct payments, individual budgets, and possible eligibility for NHS continuing care.

Promoting independence

A care plan should be instituted that addresses activities of daily living (ADL), and considers such issues as the person's environment, toileting skills, and physical exercise.
For people who also have learning disability, consider using the Assessment of Motor and Process Skills (AMPS) at the time of diagnosis.¹¹ The Dementia Questionnaire for Mentally Retarded Persons (DMR) and Dalton's Brief Praxis Test (BPT) may be used for monitoring change in function.

Improving cognitive skills

People with mild-to-moderate dementia may improve if they participate in a structured group stimulation programme.

Community and hospital care

Patients should be cared for in the community as much as possible. However, if they become severely disturbed and need to be contained for their own safety or the safety of others, inpatient care should be considered (this might include those liable to be detained under the Mental Health Act 1983). Inpatient admission would also be justified for patients with complex physical and psychiatric problems who could not be properly assessed in the community.

Palliative and end of life care

Physical, psychological, social and spiritual support should be offered, and dementia patients should have the same access to palliative care services as any other patient. Oral nutrition should be encouraged for as long as possible. Tube feeding may be appropriate in patients with transient dysphagia but should be used as a last resort in patients with severe dementia. Decisions to withhold nutritional support should be taken within a legal and ethical framework.
Fever may be managed with antipyretics and mechanical cooling. Palliative antibiotics should be given after an individual assessment of the patient.
Resuscitation is unlikely to succeed in patients with severe dementia. If no advanced decision has been taken by the patient, the decision to resuscitate should take into account the views of the carers and the multidisciplinary team, the Resuscitation Council UK 's guidance, and the Mental Capacity Act 2005.¹² The decisions should be recorded in the notes and care plan.

Acetylcholinesterase inhibitors

There is currently considerable controversy about the treatment of dementia. The Scottish Intercollegiate Guidelines Network (SIGN) has released guidance, which differs significantly from that of NICE. To summarise, NICE recommends that patients with moderate Alzheimer's disease (whose Mini Mental State Examination (MMSE) score is between 10 and 20 points) should be initiated on an acetylcholinesterase inhibitors. Whereas SIGN recommends that acetylcholinesterase inhibitors should be considered for all Alzheimer's patients, irrespective of whether the dementia is mild, moderate or severe. See separate Alzheimer's disease article for more detail.

• Treatment is started by a doctor who specialises in the care of people with dementia (psychiatrists, neurologists and geriatricians), after appropriate discussion with family and carers.
• These drugs have cholinergic side-effects and should be started at a low dose, and then be titrated according to response.
  • The patient should be reviewed every six months by MMSE score, global, functional and behavioural assessment by the specialist team or in an agreed shared-care arrangement.
  • The views of carers on the patient's condition are discussed at the start of drug treatment and at check-ups.
  • The drug is stopped if the patient's MMSE score falls below 10 points, or if the drug isn't working.
• For people with vascular dementia, acetylcholinesterase inhibitors and memantine should not be prescribed for the treatment of cognitive decline, except as part of properly constructed clinical studies.¹³

The three drugs in this class (donepezil, galantamine or rivastigmine) are all licensed for mild-to-moderate dementia (MMSE score of between 10 and 20). The least expensive of these three drugs is prescribed first. However, if this is not suitable for the patient, another drug could be chosen.

• Donepezil is initiated at 5 mg at night and, if necessary, increased to 10 mg.¹⁴
• Galantamine has nicotinic receptor agonist properties as well as being a reversible inhibitor of acetylcholinesterase. The usual dose is initially 4 mg twice daily for four weeks increased to 8 mg twice-daily for four weeks. The maintenance dose is 8-12 mg twice-daily.¹⁵
• Rivastigmine is a reversible non-competitive inhibitor of acetylcholinesterases.¹⁶ It is initially given at a dose of 1.5 mg twice-daily, increasing in steps of 1.5 mg twice-daily at intervals of at least two weeks according to tolerance and response. The usual range is 3-6 mg twice-daily, with a maximum of 6 mg twice-daily. The notable gastrointestinal side-effects are more common in women than in men.¹⁷

N-methyl-D-aspartate (NMDA) antagonists

Memantine is a N-methyl-D-aspartate (NMDA) antagonist. It is not recommended by NICE except as part of a clinical trial (for moderately severe to severe dementia).^18,19

• Several short-term trials show efficacy of risperidone and olanzapine in reducing the rate of aggression, agitation, and psychosis.
• Alternative treatments include anticonvulsants, such as divalproate and carbamazepine, and short-acting benzodiazepines, such as lorazepam and oxazepam.²⁰
• The cholinergic deficits can contribute to the development of behavioural symptoms, and treatment with acetylcholinesterase inhibitors also shows improvements in behavioural symptoms.

Patients and relatives with a suspected genetic cause for dementia should be offered genetic counselling by the regional genetic services.

The focus for prevention should be the modification of behaviour in middle-aged and older people (reducing smoking, alcohol consumption, obesity and treating other cerebrovascular disease risk factors such as hypertension and hypercholesterolaemia).⁶

The outlook for most types of dementia is poor. Irreversible or untreated dementia usually continues to worsen over time. The condition usually progresses over years until the person's death.

1. Matthews F, Brayne C; The incidence of dementia in England and Wales: findings from the five identical sites of the MRC CFA Study. PLoS Med. 2005 Aug;2(8):e193. Epub 2005 Aug 23. [abstract]
2. Alzheimer's Society; Dementia care and research
3. Dementia in: Brain's diseases of the Nervous system; 10e OMP (1993) p755-62.
4. Holmes C, Cairns N, Lantos P, et al; Validity of current clinical criteria for Alzheimer's disease, vascular dementia and dementia with Lewy bodies. Br J Psychiatry. 1999 Jan;174:45-50. [abstract]
5. McKeith IG, Galasko D, Kosaka K, et al; Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology. 1996 Nov;47(5):1113-24. [abstract]
6. Dementia: Supporting people with dementia and their carers in health and social care, NICE Clinical Guideline (2006)
7. Copeland JR; Assessment of dementia. Lancet. 1998 Mar 14;351(9105):769-70.
8. Savage DG, Lindenbaum J, Stabler SP, et al; Sensitivity of serum methylmalonic acid and total homocysteine determinations for diagnosing cobalamin and folate deficiencies. Am J Med. 1994 Mar;96(3):239-46. [abstract]
9. Management of patients with dementia, SIGN (Feb 2006)
10. Department of Health, Reference guide to consent for examination or treatment (second edition 2009)
11. Assessment of Motor and Process Skills; ampsintl.com 2007
12. Resuscitation Council UK; Decisions Relating to Cardiopulmonary Resuscitation: A Joint Statement from the British Medical Association, the Resuscitation Council (UK) and the Royal College of Nursing (updated 2007)
13. Alzheimer's - donepezil, galantamine, rivastigmine (review) and memantine, NICE Technology Appraisal (2007)
14. Summary of Product Characteristics - Aricept® tablets (donepezil hydrochloride) Eisai Ltd. Electronic Medicines Compendium. Text revised April 2009. Accessed May 2009.
15. Summary of Product Characteristics - Reminyl® tablets (Galantamine) Shire Pharmaceuticals Limited. Electronic Medicines Compendium. Text updated May 2008. Accessed May 2009.
16. Summary of Product Characteristics - Exelon® (rivastigmine hydrogen tartrate) Novartis Pharmaceuticals UK Ltd. Electronic Medicines Compendium. Text revised March 2009. Accessed May 2009.
17. Treatment List - Internet Drug Reference - Exelon
18. Summary of Product Characteristics - Ebixa® 10 mg/g oral drops, solution and 10 mg Film-Coated Tablets. Lundbeck Limited. Electronic Medicines Compendium. Text updated January 2009. Accessed May 2009.
19. NMDA Receptors - Bristol University Centre for Synaptic Plasticity
20. Blennow K, de Leon MJ, Zetterberg H; Alzheimer's disease. Lancet. 2006 Jul 29;368(9533):387-403. [abstract]

• Living well with dementia: A National Dementia Strategy, Department of Health (February 2009)
• Mental wellbeing and older people, NICE Public Health Intervention Guidance (October 2008); Guidance for Occupational therapy and physical activity interventions to promote the mental wellbeing of older people in primary care and residential care
• Experiences of caring for someone with dementia. Audio and video interviews of a number of people who care for someone with dementia. From the Healthtalkonline website.