Dementia

Notes

Introduction

Dementia is a clinical syndrome characterised by a significant deterioration in mental function that leads to impairment of normal function.

Dementia describes a clinical syndrome that is characterised by a significant deterioration in mental function. This subsequently impairs an individual to carry out normal functions.

In healthcare, we measure ‘normal function’ by activities of daily living (ADLs). These are a series of routine activities that people should be able to do without assistance. They can be broadly divided into personal tasks and domestic tasks.

  • Personal: washing, dressing, toileting, continence, transferring (e.g. bed to chair)
  • Domestic: cooking, cleaning, shopping, managing finances, taking medication

There are various underlying causes of dementia of which Alzheimer’s disease is the most common. In this note, we provide a brief overview of dementia. For more specific information about individual causes of dementia please see our other topics.

Epidemiology

Dementia is primarily a disease of older adults.

The World Health Organisation (WHO) estimates that almost 50 million people have a diagnosis of dementia worldwide. The prevalence of dementia increases with age. The estimated prevalence at 60-64 years is 0.9% compared to 41.1% in those aged 95 years and over. A significant proportion of patients with dementia remain undiagnosed and up to 54% of patients with dementia require care home placement.

Aetiology & pathophysiology

Dementia is caused by several different conditions of which Alzheimer's disease is the most common.

Dementia can be caused by several conditions, which all manifest with poor mental performance and impaired normal functioning. The main causes of dementia are listed below:

  • Alzheimer’s disease (AD): 50-75%
  • Vascular dementia (VD): 20%
  • Dementia with Lewy-body (DLB): 15-20%
  • Frontotemporal dementia (FTD): 2%
  • Rare causes: Parkinson’s disease dementia (PDD), Huntington’s disease (HD), Prion disease, and others.

The pathophysiology underlying dementia is complex and depends on the aetiology. For example, in Alzheimer’s disease, the two key pathological changes are senile plaques and neurofibrillary tangles. In vascular dementia, it is due to abnormal cerebral vasculature.

Clinical features

Dementia can be difficult to identify due to the insidious and non-specific symptoms.

Many clinical features are attributable to dementia. Some are characteristic of all dementias whereas others are typical of a particular type, like AD. There is usually a slow onset of symptoms and lack of insight with accommodation to cognitive or functional changes.

It is best to consider clinical features in the following domains: cognitive impairment, behavioural and psychological symptoms of dementia (BPSD), disease-specific features and activities of daily living.

Cognitive impairment

  • Poor memory
  • Language problems: receptive and expressive dysphasia
  • Problems with executive functioning: planning and problem solving
  • Disorientation

BPSD

  • Agitation and emotional lability
  • Depression and anxiety
  • Sleep cycle disturbance
  • Disinhibition: social or sexually inappropriate behaviour
  • Withdrawal/apathy
  • Motor disturbance: wandering is a typical feature of dementia
  • Psychosis

Disease-specific features

  • AD: early impairment of memory. Manifests as short-term memory loss and difficulty learning new information.
  • VD: typically a ‘stepwise’ decline in function. Predominant gait, attention and personality changes. May have focal neurological signs (e.g. previous stroke)
  • DLB: parkinsonism (tremor, rigidity, bradykinesia, postural instability). Fall, syncope and hallucinations predominant feature
  • FTD: marked personality change and behavioural disturbances. Memory and perception relatively preserved.

Activities of daily living

  • Loss of independence: increasing reliance on others for assistance with personal and domestic activities
  • Early stages: problems with higher level function (e.g. managing finances, difficulties at work)
  • Later stages: problems with basic personal care (e.g. washing, eating, toileting) and motor function (e.g. walking, transferring)

Cognitive assessment

A formal mental status examination should be completed using a recognised cognitive assessment tool.

There are multiple cognitive assessment tools, which are designed to test different areas of higher cortical functioning. Cognitive domains assessed include:

  • Attention and concentration
  • Recent and remote memory
  • Language
  • Praxis: planned motor movement (e.g. perform a task)
  • Executive function
  • Visuospatial function

There are a variety of different cognitive assessment tools that range from basic screening tools to in-depth assessments of each cognitive domain. Here we summarise some of the main tools.

Mini-cog

  • Overview: a three item word memory and clock drawing. Screening tool in general practice.
  • Time: 2-4 minutes
  • Setting: General practice
  • Cut-off for dementia: 5/8

Abbreviated mental test score (AMTS)

  • Overview: a ten item scoring tool predominantly used in hospital settings (e.g. hospital ward).
  • Time: < 5 minutes
  • Setting: hospital ward and General practice
  • Cut-off for dementia: 6-8/10

Mini-mental state examination (MMSE)

  • Overview: an eleven item tool. Measures cognitive function. Extensively studied and well-validated. Copyrighted.
  • Time: ≤ 10 minutes
  • Setting: Memory clinic, hospital-setting
  • Cut-off for dementia: 24/30

Montreal cognitive assessment scale (MoCA)

  • Overview: test several domains including executive function, attention, some language, memory and visuospatial skills.
  • Time: 10 minutes
  • Setting: memory clinic, hospital-setting
  • Cut-off for dementia: 26/30

Addenbrookes cognitive examination - III (ACE-III)

  • Overview: longer cognitive assessment tool that assess five domains: attention, memory, verbal fluency, language and visuospatial abilities. Based on the ACE-R, which was originally designed to classify different kinds of dementia
  • Time: 15-20 minutes
  • Setting: memory clinic
  • Cut-off for dementia: 82-88/100

NOTE: particularly in the hospital setting, dementia needs to be differentiated from delirium, which refers to an acute confusional state. Patients with dementia can develop delirium (i.e. acute on chronic confusion). This can be difficult to distinguish and usually requires further assessment after the acute episode.

Diagnosis

It is essential to exclude all alternative causes before making a diagnosis of dementia.

Patients with suspected dementia are usually referred to a memory clinic.

At memory clinic, patients undergo a formal history and examination (including medication review), full complement of baseline investigations including bloods and neuroimaging to exclude an underlying cause, and formal cognitive assessment.

During these investigations, the specific type of dementia may become apparent. In AD, this may be reflected by the lack of other neurological symptoms, absence of major cardiovascular risk factors and predominant impairment in memory, thinking and behaviour.

Diagnostic criteria

There is a diagnostic criteria for dementia based on the Diagnostic and Statistical Manual of Mental Disorders (DSM-V).

We have simplified this into three key components:

  • Functional ability: inability to carry out normal functions. Represents a decline from previous functional level
  • Cognitive domains: impairment involving ≥2 cognitive domains (see chapter on cognitive assessment)
  • Differentials excluded: clinical features cannot be explained by another cause (esp. psychiatric disorders and delirium)

Mild cognitive impairment

This describes cognitive deficits in one or more of the major cognitive domains, but the deficit is insufficient to interfere with independence in daily activities.

Mild cognitive impairment is an increasingly important term because it helps identify patients at risk of progression to dementia. Patients should have regular follow-up and be advised to undertake healthy brain activities (e.g. exercise, socialising).

Differential diagnosis

Dementia is a clinical syndrome that reflects deterioration from an underlying cause, the most common being AD. The main differentials to exclude in a patient with features of dementia are the three ‘D’s’:

  • Depression (and other psychiatric disorders): psychosis can be a feature of dementia.
  • Drugs: consider drugs with anti-cholinergic effects (e.g. anti-histamines, anti-psychotics, anti-epileptics)
  • Delirium: acute confusional state. May be prolonged recovery following episode.

Severity

The severity of dementia can be determined based on the level of functional inability.

Severity of dementia is determined using cognitive assessment tools (e.g. MMSE/MoCA) or rating/assessment tools (e.g. clinical dementia rating - CDR). They are based on the functional limitation of the patient and can be adapted for different types of dementia.

In general, dementia can be divided into mild, moderate or severe.

  • Mild: MMSE 21-26, MoCA 18-25, CDR 1
  • Moderate: MMSE 10-20, MoCA 10-17, CDR 2
  • Severe: MMSE <10, MoCA <10, CDR 3

Investigations

Baseline investigations are essential to exclude an alternative diagnosis.

Typical baseline investigations involve a routine set of blood tests and neuroimaging.

Bloods

  • Full blood count
  • Erythrocyte sedimentation rate (ESR)
  • Urea and electrolytes
  • Bone profile
  • HbA1c
  • Liver function tests
  • Thyroid function tests
  • Serum B12 and folate levels

Other

  • ECG
  • Virology (e.g. HIV)
  • Syphilis testing
  • CXR

Neuroimaging

Typically magnetic resonance imaging (MRI) but CT may be used if MRI not available or unsuitable. Important to exclude an alternative diagnosis (e.g. brain tumour) and can be used to help characterise the type of dementia (e.g. small vessel disease in VD).

Future areas

The use of biomarkers and more advanced imaging (e.g. functional MRI) are being increasingly used in a research capacity to predict the likelihood of developing dementia.

Management

The management of dementia is complex and should involve a full biopsychosocial assessment.

The management of dementia should involve a full assessment of the biological, psychological, and social needs of the patient. With significant deterioration in normal activities of daily living, patients will become dependent on others. This means help from families, organisation of carers, and with more advancing symptoms, need for care home placement.

There are multiple facets to management, which we summarise.

  • Assess capacity and advanced care planning: ideally completed when patients still retain capacity. Consideration of advance statements/decisions and appointment of lasting power of attorney.
  • Physical and mental health: consider co-existing anxiety and depression. Manage physical health needs as normal. Consider delirium if any acute deterioration.
  • Driving: must inform the DVLA. Check website for guidance.
  • Pharmacological: (see below)
  • Non-pharmacological: programmes to improve/maintain cognitive function (e.g. structured group cognitive stimulation programmes). Also exercise, aromatherapy, therapeutic use of music/dancing, massage.
  • Managing BPSD: non-pharmacological interventions. Consider referral to old-age psychiatry if difficult to control. Pharmacological therapy should be used on specialist advice.
  • Care plans: people with dementia require a care manager and care plan. This includes details on diagnosis, treatment, environmental modifications and review plans.
  • End-of-life care: focus on physical, psychological, social, and spiritual needs. Oral nutrition is encouraged as long as possible. Long-term feeding (i.e. NG feeding, gastrostomy tube) is inappropriate in severe dementia. No evidence for increased survival or reduced complications. Resuscitation discussions.

Pharmacological therapy

Medical therapy for the treatment of dementia should be initiated by a specialist treating patients with dementia. The two main drugs are acetylcholinesterase inhibitors and N-methyl-D-aspartic acid receptor antagonists. Pharmacological agents are primarily indicated in patients with AD. They should not be used in patients with mild cognitive impairment. Other factors to consider are medications (e.g. antipsychotics) for difficult to control behaviour and cardiovascular drugs (e.g. aspirin, clopidogrel) for patients with significant cerebrovascular disease.

For more information on specific treatments check out our individual topics on dementia types.

Prognosis

There is no cure for dementia and it is considered a life-limiting condition.

It is estimated that one in three people over the age of 65 will die from dementia and the estimated median survival after diagnosis is 3-9 years (variable).

Progression of dementia has been estimated by WHO, which is based on each stage of severity. Development of delirium on a background of dementia is associated with more rapid progression.

  • Mild: first 2 years
  • Moderate: next 2-4 years
  • Severe: 4-5 years onwards

Last updated: May 2022

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