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Types of Dementia, Symptoms and Treatments Home Instead Senior Care Dementia Course Supplement Dementia is not a natural part of ageing. Not everyone who grows old will develop dementia. Not everyone who develops dementia is old. Young people in their 20’s are sometimes diagnosed with dementia. The word ‘dementia’ describes a set of symptoms that may include memory loss and difficulties with thinking, problem-solving; language; motor skills; sequencing; vision and inhibitions. These changes are often small to start with, but for someone with dementia they have become severe enough to affect daily life. A person with dementia may also experience changes in their mood or behaviour. Dementia is caused when the brain is damaged by diseases, such as Alzheimer’s disease or a series of strokes. Dementia is an umbrella term. There are over 100 individual types of dementia. Alzheimer’s disease is the most common and most well-known cause of dementia. The specific symptoms that someone with dementia experiences will depend on the parts of the brain that are damaged and the disease that is causing the dementia. All kinds of dementia are progressive which means the symptoms will gradually get worse. This factsheet explains what some common types of dementia are, including the causes and symptoms, and how they are sometimes treated. However, just because someone has started to show some signs of cognitive decline, that doesn’t necessarily mean they are developing dementia. Mild Cognitive Impairment (MCI) Mild cognitive impairment (MCI) is a condition in which someone has minor problems with cognition - their mental abilities such as memory or thinking. In MCI these difficulties are worse than would normally be expected for a healthy person of their age. However, the symptoms are not severe enough to interfere significantly with daily life, and so are not defined as dementia. It is estimated that between 5 and 20 per cent of people aged over 65 have MCI. It is not a type of dementia, but a person with MCI is more likely to go on to develop dementia. This page explains what MCI is, the link between MCI and dementia, and the benefits of diagnosing MCI. It then looks at treatments for MCI, ways to cope with the symptoms, and how you can reduce your risk of developing MCI and dementia. Many people who are diagnosed with MCI use this as an opportunity to change their lifestyle for the better. There is a lot that someone can do to help reduce their chances of MCI progressing to dementia. Symptoms The term MCI describes a set of symptoms, rather than a specific disease. A person with MCI has mild problems with one or more of the following: memory - for example, forgetting recent events or repeating the same question reasoning, planning or problem-solving - for example, struggling with thinking things through attention - for example, being very easily distracted language - for example, taking much longer than usual to find the right word for something visual depth perception - for example, struggling to interpret an object in three dimensions, judge distances or navigate stairs.

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Types of Dementia, Symptoms and Treatments

Home Instead Senior Care Dementia Course

Supplement

Dementia is not a natural part of ageing. Not everyone who grows old will develop dementia. Not everyone who develops dementia is old. Young people in their 20’s are sometimes diagnosed with dementia.

The word ‘dementia’ describes a set of symptoms that may include memory loss and difficulties with thinking, problem-solving; language; motor skills; sequencing; vision and inhibitions. These changes are often small to start with, but for someone with dementia they have become severe enough to affect daily life. A person with dementia may also experience changes in their mood or behaviour.

Dementia is caused when the brain is damaged by diseases, such as Alzheimer’s disease or a series of strokes. Dementia is an umbrella term. There are over 100 individual types of dementia. Alzheimer’s disease is the most common and most well-known cause of dementia. The specific symptoms that someone with dementia experiences will depend on the parts of the brain that are damaged and the disease that is causing the dementia. All kinds of dementia are progressive which means the symptoms will gradually get worse. This factsheet explains what some common types of dementia are, including the causes and symptoms, and how they are sometimes treated.

However, just because someone has started to show some signs of cognitive decline, that doesn’t necessarily mean they are developing dementia.

Mild Cognitive Impairment (MCI)

Mild cognitive impairment (MCI) is a condition in which someone has minor problems with cognition - their mental abilities such as memory or thinking. In MCI these difficulties are worse than would normally be expected for a healthy person of their age. However, the symptoms are not severe enough to interfere significantly with daily life, and so are not defined as dementia.

It is estimated that between 5 and 20 per cent of people aged over 65 have MCI. It is not a type of dementia, but a person with MCI is more likely to go on to develop dementia. This page explains what MCI is, the link between MCI and dementia, and the benefits of diagnosing MCI. It then looks at treatments for MCI, ways to cope with the symptoms, and how you can reduce your risk of developing MCI and dementia. Many people who are diagnosed with MCI use this as an opportunity to change their lifestyle for the better. There is a lot that someone can do to help reduce their chances of MCI progressing to dementia.

Symptoms

The term MCI describes a set of symptoms, rather than a specific disease. A person with MCI has mild problems with one or more of the following:

memory - for example, forgetting recent events or repeating the same question reasoning, planning or problem-solving - for example, struggling with thinking things through attention - for example, being very easily distracted language - for example, taking much longer than usual to find the right word for something visual depth perception - for example, struggling to interpret an object in three dimensions, judge distances or

navigate stairs.

These symptoms will have been noticed by the individual, or by those who know them. For a person with MCI, these changes may cause them to experience minor problems or need a little help with more demanding daily tasks (eg paying bills, managing medication, driving). However, MCI does not cause major problems with everyday living. If there is a significant impact on everyday activities, this may suggest dementia.

Most healthy people experience a gradual decline in mental abilities as part of ageing. In someone with MCI, however, the decline in mental abilities is greater than in normal ageing. For example, it's common in normal ageing to have to pause to remember directions or to forget words occasionally, but it's not normal to become lost in familiar places or to forget the names of close family members.

If the person with MCI has seen a doctor and taken tests of mental abilities, their problems will also be shown by a low test score or by falling test scores over time. This decline in mental abilities is often caused by an underlying illness.

Causes

MCI can have a number of different possible causes. Some of these are treatable and some are not.

In some people, MCI is a 'pre-dementia' condition. This means that the brain diseases that cause dementia are already established. These diseases are not generally reversible and so, in time, these people's symptoms will worsen and their condition will progress from MCI to dementia.

For example, some people with MCI have mild memory loss that started gradually. These people are likely to develop Alzheimer's disease as their memory worsens.

Some people with MCI will turn out to have a different, often treatable, cause following assessment by a doctor. This could include depression, anxiety or stress. The same symptoms could also be caused by a physical illness (eg constipation, infection), poor eyesight or hearing, vitamin or thyroid deficiencies, or the side effects of medication. Where this is the case, the person will be diagnosed with this condition - a thyroid deficiency or depression, for example - rather than MCI.

A doctor will not always be able to say what is causing MCI, even after a thorough assessment. It may be necessary to wait a few months or more, to see how the person's symptoms develop.

Many people with MCI develop dementia

People who have MCI are at an increased risk of going on to develop dementia. In research studies carried out in memory clinics, 10-15 per cent of people who had MCI with gradual memory loss went on to develop dementia - usually Alzheimer's disease - each year.

In studies carried out in other settings, the rates of 'conversion' from MCI to dementia are about half this level (about 5 per cent each year), but people with MCI still show a significantly increased risk of dementia.

Although MCI increases someone's risk of developing dementia, not everyone with MCI will get worse and develop dementia. Some people with MCI remain stable over time and some improve and no longer have any problems.

The outcome will generally depend on the cause of the MCI.

These different outcomes are one reason why some doctors find some aspects of MCI to be controversial.

What are the benefits of diagnosing MCI?

The main benefit of diagnosing MCI is that it helps to identify people who are at increased risk of developing dementia. They can then be offered information, advice and support. They can also be kept under review and if they do develop dementia, they should have this diagnosed sooner.

Anyone who is worried about their memory, or has any of the symptoms listed above, should see their GP. A thorough assessment might clear up any anxiety about the cause of symptoms. It may also lead to diagnosis of conditions (eg depression, high blood pressure, poor vision) that can be treated.

The assessment is the same as for suspected dementia. The GP will talk to the person about their symptoms, when they started and how they are affecting their daily life now. The person will have their physical health and any medication they are taking reviewed, and take tests to assess depression and mental abilities.

The GP may be able to make a clear diagnosis at this stage, or they might need to refer the person for more specialist assessment, often at a memory service. Diagnosis may require more than one appointment and may include more detailed assessment by a psychologist. A brain scan may be done, particularly if other potential causes (eg brain bleed, tumour) are suspected.

A doctor will use their own judgement to make the diagnosis. Research guidelines state that MCI should be diagnosed if the person shows all of the following:

symptoms (as listed above) are getting worse and cannot be otherwise explained scores poorly for their age on a test of mental abilities has minor or no problems with more complex daily activities.

If a person is diagnosed with MCI by a specialist, they will usually be discharged back to their GP. What happens next will vary. In some parts of the country, the person will be invited back to the GP or memory service after a set time (often 6 or 12 months) to see whether things have changed. In other parts of the country, they are asked to arrange an appointment themselves, at any time, if they feel their symptoms have got worse.

This is a period of uncertainty for the person and those around them, and they should be given information and advice, and counselling if needed. They may also be offered further support (see 'How is MCI treated?' below).

Many people who are diagnosed with MCI or early-stage dementia take the opportunity to plan ahead, for example by making a power of attorney.

A person diagnosed with MCI who drives - in contrast to someone with dementia - will not always need to tell DVLA (or DVA Northern Ireland). The doctor should be able to advise on this.

How is MCI treated?

There are currently no drugs that have been approved for treating MCI, as opposed to dementia. It was initially hoped that the Alzheimer's drugs donepezil (eg Aricept), rivastigmine (eg Exelon) and galantamine (eg Reminyl) would help with symptoms of MCI, or slow its progression to dementia. However, a lot of trials of these drugs have been done and they have shown no clear benefit to patients.

Nevertheless, there is a lot someone with MCI can do to help lower their chances of developing dementia - see 'Tips for someone diagnosed with MCI' below. MCI is more likely to progress to dementia if the person has a poorly controlled heart condition or diabetes, or has strokes. Therefore, treatment for MCI will often include medication for any heart condition a person may have, or tablets to reduce high blood pressure, prevent clots or lower cholesterol. If depression is diagnosed this will also be treated, with medication, talking therapies or both.

A person with MCI will also be encouraged to lower their risk of developing dementia by adopting a healthy lifestyle and sleeping well. People who smoke should try to stop and people who drink alcohol should keep to recommended

levels. Regular physical exercise also seems to reduce the risk of dementia. Eating a healthy diet and keeping to a healthy weight may also help. Ask the GP or primary care team for advice on all of these.

It is strongly recommended that someone with MCI keeps active, both mentally (eg doing puzzles, reading) and socially (eg seeing friends).

Recent evidence shows that a combined programme of approaches, rather than any one approach alone, can help to improve or maintain mental abilities in people with MCI. These approaches include medical treatment for vascular risk factors (a heart condition, diabetes or high blood pressure), physical activity, learning strategies to improve memory and thinking, and receiving and following advice on memory, health and diet.

In some areas, people with MCI are now routinely referred for regular sessions - sometimes called 'memory protection groups' - to support them with these changes.

Dementia & its Symptoms

When dementia strikes, each person as always, remains unique and will experience dementia in their own way. The different types of dementia also tend to affect people differently, especially in the early stages. Other factors that will affect how well someone can live with dementia include how other people respond to them and the environment around them.

A person with dementia will have cognitive symptoms (to do with thinking or memory). They will often have problems with some of the following:

day-to-day memory – for example, difficulty recalling events that happened recently concentrating, planning or organising – for example, difficulties making decisions, solving problems or carrying out

a sequence of tasks (such as cooking a meal) language – for example, difficulties following a conversation or finding the right word for something visuospatial skills – for example, problems judging distances (such as on stairs) and seeing objects in three

dimensions orientation – for example, losing track of the day or date, or becoming confused about where they are.

A person with dementia will also often have changes in their mood. For example, they may become frustrated or irritable, apathetic or withdrawn, anxious, easily upset or unusually sad. With some types of dementia, the person may see things that are not really there (visual hallucinations) or strongly believe things that are not true (delusions).

Dementia is progressive, which means the symptoms gradually get worse over time. How quickly this happens varies greatly from person to person. As dementia progresses, the person may develop behaviours that seem unusual or out of character. These behaviours may include asking the same question over and over, pacing, restlessness or agitation. They can be distressing or challenging for the person and those close to them.

A person with dementia, especially in the later stages, may have physical symptoms such as muscle weakness or weight loss. Changes in sleep pattern and appetite are also common.

There are many diseases that result in dementia. The most common types of dementia are outlined below:

Alzheimer’s Disease

Alzheimer's disease is the most common cause of dementia. This page describes the symptoms of Alzheimer's disease, how it is diagnosed, and the factors that can put someone at risk of developing it. It also describes the treatments and support that are currently available.

Alzheimer's disease, named after the doctor who first described it (Alois Alzheimer), is a physical disease that affects the brain. There are more than 520,000 people in the UK with Alzheimer's disease. During the course of the disease,

proteins build up in the brain to form structures called 'plaques' and 'tangles'. This leads to the loss of connections between nerve cells, and eventually to the death of nerve cells and loss of brain tissue. People with Alzheimer's also have a shortage of some important chemicals in their brain. These chemical messengers help to transmit signals around the brain. When there is a shortage of them, the signals are not transmitted as effectively. As discussed below, current treatments for Alzheimer's disease can help boost the levels of chemical messengers in the brain, which can help with some of the symptoms.

Alzheimer's is a progressive disease. This means that gradually, over time, more parts of the brain are damaged. As this happens, more symptoms develop. They also become more severe.

The symptoms of Alzheimer's disease are generally mild to start with, but they get worse over time and start to interfere with daily life.

There are some common symptoms of Alzheimer's disease, but it is important to remember that everyone is unique. Two people with Alzheimer's are unlikely to experience the condition in exactly the same way.

For most people with Alzheimer's, the earliest symptoms are memory lapses. In particular, they may have difficulty recalling recent events and learning new information. These symptoms occur because the early damage in Alzheimer's is usually to a part of the brain called the hippocampus, which has a central role in day-to-day memory. Memory for life events that happened a long time ago is often unaffected in the early stages of the disease.

Memory loss due to Alzheimer's disease increasingly interferes with daily life as the condition progresses. The person may:

lose items (eg keys, glasses) around the house struggle to find the right word in a conversation or forget someone's name forget about recent conversations or events get lost in a familiar place or on a familiar journey forget appointments or anniversaries.

Although memory difficulties are usually the earliest symptoms of Alzheimer's, someone with the disease will also have – or go on to develop – problems with other aspects of thinking, reasoning, perception or communication. They might have difficulties with:

language – struggling to follow a conversation or repeating themselves visuospatial problems judging distance or seeing objects in three dimensions; navigating stairs or parking the car

become much harder concentrating, planning or organising – difficulties making decisions, solving problems or carrying out a sequence

of tasks (eg cooking a meal) orientation – becoming confused or losing track of the day or date.

A person in the earlier stages of Alzheimer's will often have changes in their mood. They may become anxious, irritable or depressed. Many people become withdrawn and lose interest in activities and hobbies.

Later stages

As Alzheimer's progresses, problems with memory loss, communication, reasoning and orientation become more severe. The person will need more day-to-day support from those who care for them.

Some people start to believe things that are untrue (delusions) or – less often – see or hear things which are not really there (hallucinations).

Many people with Alzheimer's also develop behaviours that seem unusual or out of character. These include agitation (eg restlessness or pacing), calling out, repeating the same question, disturbed sleep patterns or reacting aggressively.

Such behaviours can be distressing or challenging for the person and their carer. They may require separate treatment and management to memory problems.

In the later stages of Alzheimer's disease someone may become much less aware of what is happening around them. They may have difficulties eating or walking without help, and become increasingly frail. Eventually, the person will need help with all their daily activities.

How quickly Alzheimer's disease progresses, and the life expectancy of someone with it, vary greatly. On average, people with Alzheimer's disease live for eight to ten years after the first symptoms. However, this varies a lot, depending particularly on how old the person was when they first developed Alzheimer's.

Who gets Alzheimer's disease?

Most people who develop Alzheimer's disease do so after the age of 65, but people under this age can also develop it. This is called early-onset Alzheimer's disease, a type of young-onset dementia. In the UK there are over 40,000 people under the age of 65 with dementia.

Developing Alzheimer's disease is linked to a combination of factors, explained in more detail below. Some of these risk factors (eg lifestyle) can be controlled, but others (eg age and genes) cannot.

Age

Age is the greatest risk factor for Alzheimer's. The disease mainly affects people over 65. Above this age, a person's risk of developing Alzheimer's disease doubles approximately every five years. One in six people over 80 have dementia.

Gender

For reasons that are not clear, there are about twice as many women as men over 65 with Alzheimer's disease. This difference is not fully explained by the fact that women on average live longer than men. It may be that Alzheimer's in women is linked to a lack of the hormone oestrogen after the menopause.

Genetic inheritance

Many people fear that the disease may be passed down to them from a parent or grandparent. Scientists are investigating the genetic background to Alzheimer's. There are a few families with a very clear inheritance of Alzheimer's from one generation to the next. In such families the dementia tends to develop well before age 65. However, Alzheimer's disease that is so strongly inherited is extremely rare.

In the vast majority of people, the influence of genetics on risk of Alzheimer's disease is much more subtle. A number of genes are known to increase or reduce a person's chances of developing Alzheimer's. For someone with a close relative (parent or sibling) who was diagnosed with Alzheimer's when over 65, their own risk of developing the disease is increased. However, this does not mean that Alzheimer's is inevitable, and everyone can reduce their risk by living a healthy lifestyle.

People with Down's syndrome are at particular risk of developing Alzheimer's disease, because of a difference in their genetic makeup.

Health and lifestyle

Medical conditions such as diabetes, stroke and heart problems, as well as high blood pressure, high cholesterol and obesity in mid-life, are all known to increase the risk of both Alzheimer's disease and vascular dementia. Anyone can reduce their risk by keeping these under control. Depression is a probable risk factor for dementia; getting it treated early is important.

People who adopt a healthy lifestyle, especially from mid-life onwards, are less likely to develop Alzheimer's disease. This means taking regular physical exercise and keeping to a healthy weight, not smoking, eating a healthy balanced diet and drinking only in moderation.

Leading an active lifestyle that combines regular physical, social and mental activity will help to lower risk.

Treatment and support

There is currently no cure for Alzheimer's disease, but there is a lot that can be done to enable someone to live well with the condition. This will involve drug and non-drug care, support and activities.

The person should have a chance to talk to a professional about their diagnosis. This could be a psychiatrist or mental health nurse, a clinical psychologist, occupational therapist or GP. Information on the support that is available and where to go for further advice is vital in helping someone to stay physically and mentally well. Professionals such as the GP and staff at the memory service or local Alzheimer's Society can advise on what might best meet the needs of the individual and of those caring for them. Additionally, Home Instead Senior Care through its trained Caregivers are specialists in supporting individuals and their families and enabling those diagnosed with dementia remain able to live at home where they are most familiar, happily and safely surrounded by their memories with their one-to-one, dedicated home care support.

There are drug treatments for Alzheimer's disease that can temporarily alleviate some symptoms or slow down their progression in some people. (The names in brackets are common brands of these drugs.)

A person in the mild or moderate stages of Alzheimer's disease or mixed dementia will often be prescribed a drug such as donepezil (eg Aricept), rivastigmine (eg Exelon) or galantamine (eg Reminyl). The drug may help with memory problems, improve concentration and motivation, and help with aspects of daily living such as cooking, shopping or hobbies. A person in the moderate or severe stages of Alzheimer's disease or mixed dementia may be offered a different kind of drug: memantine (eg Ebixa). This may help with mental abilities and daily living, and ease distressing or challenging behaviours such as agitation and delusions.

If someone is depressed or anxious, talking therapies (such as cognitive behavioural therapy) or drug treatments (such as antidepressants) may also be tried. Counselling may help the person adjust to the diagnosis.

There are many ways to help someone remain independent and cope with memory loss. These include practical things like developing a routine or using a weekly pill blister-pack. There are other assistive technology products available such as electronic reminders and calendar clocks.

It is beneficial for a person with Alzheimer's to keep up with activities that they enjoy. Many people benefit from exercising their mind with reading or puzzles. There is evidence that attending sessions to keep mentally active helps (cognitive stimulation). Life story work, in which someone shares their life experiences and makes a personal record, may help with memory, mood and wellbeing. As the dementia worsens, many people enjoy more general reminiscence activities.

Over time, changes in the person's behaviour such as agitation or aggression become more likely. These behaviours are often a sign that the person is in distress. This could be from a medical condition such as pain; because they misunderstood something or someone; or perhaps because they are frustrated or under-stimulated. Individualised approaches should look for, and try to address, the underlying cause. General non-drug approaches often also help. These include social interaction, music, reminiscence, exercise or other activities that are meaningful for the person. They are generally tried before additional drugs are considered, particularly antipsychotics.

Anyone caring for the person is likely to find these behaviours distressing. Support for carers is particularly important at such times.

Mixed dementia

An estimated 10 per cent of people with dementia have more than one type at the same time. This is called mixed dementia. The most common combination is Alzheimer's disease with vascular dementia (caused by problems with the blood supply to the brain). The symptoms of this kind of mixed dementia are a mixture of the symptoms of Alzheimer's disease and vascular dementia.

Vascular dementia

Vascular dementia is the second most common type of dementia (after Alzheimer's disease), affecting around 150,000 people in the UK.

The word dementia describes a set of symptoms that can include memory loss and difficulties with thinking, problem-solving or language. In vascular dementia, these symptoms occur when the brain is damaged because of problems with the supply of blood to the brain. This page outlines the causes, types and symptoms of vascular dementia. It looks at how it is diagnosed and the factors that can put someone at risk of developing it. It also describes the treatment and support that are available.

Causes

Vascular dementia is caused by reduced blood supply to the brain due to diseased blood vessels.

To be healthy and function properly, brain cells need a constant supply of blood to bring oxygen and nutrients. Blood is delivered to the brain through a network of vessels called the vascular system. If the vascular system within the brain becomes damaged - so that the blood vessels leak or become blocked - then blood cannot reach the brain cells and they will eventually die.

This death of brain cells can cause problems with memory, thinking or reasoning. Together these three elements are known as cognition. When these cognitive problems are bad enough to have a significant impact on daily life, this is known as vascular dementia.

Types of vascular dementia

There are several different types of vascular dementia. They differ in the cause of the damage and the part of the brain that is affected. The different types of vascular dementia have some symptoms in common and some symptoms that differ. Their symptoms tend to progress in different ways.

Stroke-related dementia

A stroke happens when the blood supply to a part of the brain is suddenly cut off. In most strokes, a blood vessel in the brain becomes narrowed and is blocked by a clot. The clot may have formed in the brain, or it may have formed in the heart (if someone has heart disease) and been carried to the brain. Strokes vary in how severe they are, depending on where the blocked vessel is and whether the interruption to the blood supply is permanent or temporary.

Post-stroke dementia

A major stroke occurs when the blood flow in a large vessel in the brain is suddenly and permanently cut off. Most often this happens when the vessel is blocked by a clot. Much less often it is because the vessel bursts and bleeds into the brain. This sudden interruption in the blood supply starves the brain of oxygen and leads to the death of a large volume of brain tissue.

Not everyone who has a stroke will develop vascular dementia, but about 20 per cent of people who have a stroke do develop this post-stroke dementia within the following six months. A person who has a stroke is then at increased risk of having further strokes. If this happens, the risk of developing dementia is higher.

Single-infarct and multi-infarct dementia

These types of vascular dementia are caused by one or more smaller strokes. These happen when a large or medium-sized blood vessel is blocked by a clot. The stroke may be so small that the person doesn't notice any symptoms. Alternatively, the symptoms may only be temporary - lasting perhaps a few minutes - because the blockage clears itself. (If symptoms last for less than 24 hours this is known as a 'mini-stroke' or transient ischaemic attack (TIA). A TIA may mistakenly be dismissed as a 'funny turn'.)

If the blood supply is interrupted for more than a few minutes, the stroke will lead to the death of a small area of tissue in the brain. This area is known as an infarct. Sometimes just one infarct forms in an important part of the brain and this causes dementia (known as single-infarct dementia). Much more often, a series of small strokes over a period of weeks or months lead to a number of infarcts spread around the brain. Dementia in this case (known as multi-infarct dementia) is caused by the total damage from all the infarcts together.

Subcortical dementia

Subcortical vascular dementia is caused by diseases of the very small blood vessels that lie deep in the brain. These small vessels develop thick walls and become stiff and twisted, meaning that blood flow through them is reduced.

Small vessel disease often damages the bundles of nerve fibres that carry signals around the brain, known as white matter. It can also cause small infarcts near the base of the brain.

Small vessel disease develops much deeper in the brain than the damage caused by many strokes. This means many of the symptoms of subcortical vascular dementia are different from those of stroke-related dementia.

Subcortical dementia is thought to be the most common type of vascular dementia.

Symptoms

How vascular dementia affects people varies depending on the different underlying causes and more generally from person to person. Symptoms may develop suddenly, for example after a stroke, or more gradually, such as with small vessel disease.

Some symptoms may be similar to those of other types of dementia. Memory loss is common in the early stages of Alzheimer's, but is not usually the main early symptom of vascular dementia.

The most common cognitive symptoms in the early stages of vascular dementia are:

problems with planning or organising, making decisions or solving problems difficulties following a series of steps (eg cooking a meal) slower speed of thought problems concentrating, including short periods of sudden confusion.

A person in the early stages of vascular dementia may also have difficulties with:

memory - problems recalling recent events (often mild) language - eg speech may become less fluent visuospatial skills - problems perceiving objects in three dimensions.

As well as these cognitive symptoms, it is common for someone with early vascular dementia to experience mood changes, such as apathy, depression or anxiety. Depression is common, partly because people with vascular dementia may be aware of the difficulties the condition is causing. A person with vascular dementia may also become generally more emotional. They may be prone to rapid mood swings and being unusually tearful or happy.

Other symptoms that someone with vascular dementia may experience vary between the different types. Post-stroke dementia will often be accompanied by the obvious physical symptoms of the stroke. Depending on which part of the brain is affected, someone might have paralysis or weakness of a limb. Or if a different part of the brain is damaged they may have problems with vision or speech. With rehabilitation, symptoms may get a little better or stabilise for a time, especially in the first six months after the stroke.

Symptoms of subcortical vascular dementia vary less. Early loss of bladder control is common. The person may also have mild weakness on one side of their body, or become less steady walking and more prone to falls. Other symptoms of subcortical vascular dementia may include clumsiness, lack of facial expression and problems pronouncing words.

Progression and later stages

Vascular dementia will generally get worse, although the speed and pattern of this decline vary. Stroke-related dementia often progresses in a 'stepped' way, with long periods when symptoms are stable and periods when symptoms rapidly get worse. This is because each additional stroke causes further damage to the brain. Subcortical vascular dementia may occasionally follow this stepped progression, but more often symptoms get worse gradually, as the area of affected white matter slowly expands.

Over time a person with vascular dementia is likely to develop more severe confusion or disorientation, and further problems with reasoning and communication. Memory loss, for example for recent events or names, will also become worse. The person is likely to need more support with day-to-day activities such as cooking or cleaning.

As vascular dementia progresses, many people also develop behaviours that seem unusual or out of character. The most common include irritability, agitation, aggressive behaviour and a disturbed sleep pattern. Someone may also act in socially inappropriate ways.

Occasionally a person with vascular dementia will strongly believe things that are not true (delusions) or - less often - see things that are not really there (hallucinations). These behaviours can be distressing and a challenge for all involved.

In the later stages of vascular dementia someone may become much less aware of what is happening around them. They may have difficulties walking or eating without help, and become increasingly frail. Eventually, the person will need help with all their daily activities.

How long someone will live with vascular dementia varies greatly from person to person. On average it will be about five years after the symptoms started. The person is most likely to die from a stroke or heart attack.

Who gets vascular dementia?

There are a number of things that can put someone at risk of developing vascular dementia. These are called risk factors. Most of these are things that contribute to underlying cardiovascular diseases. Some of these risk factors (eg lifestyle) can be controlled, but others (eg age and genes) cannot.

Age is the strongest risk factor for vascular dementia. A person's risk of developing the condition doubles approximately every five years over the age of 65. Vascular dementia under the age of 65 is uncommon and affects fewer than 8,000 people in the UK. Men are at slightly higher risk of developing vascular dementia than women.

A person who has had a stroke, or who has diabetes or heart disease, is approximately twice as likely to develop vascular dementia. Sleep apnoea, a condition where breathing stops for a few seconds or minutes during sleep, is also a possible risk factor. Someone can reduce their risk of dementia by keeping these conditions under control, through taking prescribed medicines (even if they feel well) and following professional advice about their lifestyle.

There is some evidence that a history of depression also increases the risk of vascular dementia. Anyone who thinks they may be depressed should seek their doctor's advice early.

Cardiovascular disease - and therefore vascular dementia - is linked to high blood pressure, high cholesterol and being overweight in mid-life. Someone can reduce their risk of developing these by having regular check-ups (over the age of 40), by not smoking, and by keeping physically active. It will also help to eat a healthy balanced diet and drink alcohol only in moderation.

Aside from these cardiovascular risk factors, there is good evidence that keeping mentally active throughout life reduces dementia risk. There is some evidence for the benefits of being socially active too.

Researchers think there are some genetic factors behind the common types of vascular dementia, and that these are linked to the underlying cardiovascular diseases. Someone with a family history of stroke, heart disease or diabetes has an increased risk of developing these conditions. Overall, however, the role of genes in the common types of vascular dementia is small.

People from certain ethnic groups are more likely to develop cardiovascular disease and vascular dementia than others. Those from an Indian, Bangladeshi, Pakistani or Sri Lankan background living in the UK have significantly higher rates of stroke, diabetes and heart disease than white Europeans. Among people of African-Caribbean descent, the risk of diabetes and stroke - but not heart disease - is also higher. These differences are thought to be partly inherited but mainly due to lifestyle factors such as diet, smoking and exercise.

Treatment and support

There is currently no cure for vascular dementia: the brain damage that causes it cannot be reversed. However, there is a lot that can be done to enable someone to live well with the condition. This will involve drug and non-drug treatment, support and activities.

The person should have a chance to talk to a health or social care professional about their dementia diagnosis. This could be a psychiatrist or mental health nurse, a clinical psychologist, occupational therapist or GP. Information on what support is available and where to go for further advice is vital in helping someone to stay physically and mentally well.

Control of cardiovascular disease

If the underlying cardiovascular diseases that have caused vascular dementia can be controlled, it may be possible to slow down the progression of the dementia. For example, after someone has had a stroke or TIA, treatment of high blood pressure can reduce the risk of further stroke and dementia. For stroke-related dementia in particular, with treatment there may be long periods when the symptoms don't get significantly worse.

In most cases, a person with vascular dementia will already be on medications to treat the underlying diseases. These include tablets to reduce blood pressure, prevent blood clots and lower cholesterol. If the person has a diagnosed heart condition or diabetes they will also be taking medicines for these. It is important that the person continues to take any medications and attends regular check-ups as recommended by a doctor.

Someone with vascular dementia will also be advised to adopt a healthy lifestyle, particularly to take regular physical exercise and, if they are a smoker, to quit. They should try to eat a diet with plenty of fruit, vegetables and oily fish but not too much fat or salt. Maintaining a healthy weight and keeping to recommended levels of alcohol will also help. The GP should be able to offer advice in all these areas.

Other treatment and support

Supporting a person with vascular dementia to live well includes treatment for symptoms, support to cope with lost abilities, and help to keep up enjoyable activities. For someone who has had a stroke or has physical difficulties, treatment will also include rehabilitation.

The drugs that are routinely prescribed for Alzheimer's disease do not have benefits for vascular dementia, and are not recommended for it. These drugs may, however, be prescribed to treat mixed dementia (Alzheimer's disease and vascular dementia).

If someone is depressed or anxious, talking therapies (such as cognitive behavioural therapy) or drug treatments may also be tried. Counselling may also help the person adjust to the diagnosis.

There are many ways to help someone remain independent and cope with the cognitive symptoms of vascular dementia. For example, breaking complex tasks down into smaller steps will make them easier. An environment which is not too busy or noisy will make it easier to concentrate. For someone with memory loss, a regular routine and appropriate technology, such as pill boxes or electronic devices, can help.

It is important that a person with any type of dementia stays active and continues to do things they enjoy. Keeping mentally active (cognitive stimulation) is known to help with memory and communication. Life story work, in which someone shares their life experiences and makes a personal record, may help with memory, mood and wellbeing. As the dementia worsens, many people enjoy more general reminiscence activities.

If the person has physical difficulties, for example after a stroke, they will usually benefit from rehabilitation. This could mean working with a physiotherapist (especially for help with weakness, coordination, movement and balance), occupational therapist (for everyday activities) or speech and language therapist (for all aspects of communication).

Over time, changes in the person's behaviour - such as agitation or aggression - become more likely. These are often a sign that the person is in distress. There are many potential causes. For example, the person might be in pain, they may have misunderstood something or someone, or they may be frustrated or under-stimulated. Individualised approaches should try to address the cause. General non-drug approaches (eg social interaction) often help. They should generally be tried before additional drugs are considered, particularly in the case of antipsychotics.

Dementia with Lewy bodies

Dementia with Lewy bodies (DLB) is a type of dementia that shares symptoms with both Alzheimer's disease and Parkinson's disease. It may account for 10-15 per cent of all cases of dementia. DLB can be diagnosed wrongly and is often mistaken for Alzheimer's disease. This section describes the symptoms of DLB and how it is diagnosed, as well as the treatment and support available.

DLB is sometimes known by other names. These include Lewy body dementia, Lewy body variant of Alzheimer's disease, diffuse Lewy body disease and cortical Lewy body disease. All these terms refer to the same condition.

Lewy bodies

Lewy bodies are named after the German doctor who first identified them. They are tiny deposits of a protein (alpha-synuclein) that appear in nerve cells in the brain. Researchers don't have a full understanding of why Lewy bodies appear, or exactly how they contribute to dementia. However, this is linked to two factors:

low levels of important chemicals (mainly acetylcholine and dopamine) that carry messages between nerve cells a loss of connections between nerve cells, which then die.

Lewy bodies are the cause of DLB and Parkinson's disease. They are two of several diseases caused by Lewy bodies that affect the brain and nervous system and get worse over time. These are sometimes called Lewy body disorders.

The way someone is affected by DLB will depend partly on where the Lewy bodies are in the brain:

Lewy bodies at the base of the brain are closely linked to problems with movement (motor symptoms). These are the main feature of Parkinson's disease.

Lewy bodies in the outer layers of the brain are linked to problems with mental abilities (cognitive symptoms), which is a feature of DLB.

People with a Lewy body disorder can have problems with movement and changes in mental abilities at the same time.

A person with Parkinson's disease is at high risk of going on to develop dementia (Parkinson's disease dementia) as their condition progresses. Dementia may be more likely in a person who has developed Parkinson's later in life or who has been living with it for several years.

Who is affected?

DLB accounts for around 4 per cent of all recorded dementia, but there is good evidence that the condition is not always diagnosed correctly. Based on studies of brain tissue after death, scientists think DLB may account for as much as 10-15 per cent of all dementia.

DLB appears to affect men and women equally. As with Alzheimer's disease and vascular dementia, DLB becomes more common over the age of 65. However, it can also affect people under 65.

Other than age, there are few risk factors (such as medical conditions or lifestyle choices) that are known to increase a person's chances of developing DLB. Most people who develop DLB have no clear family history of the condition. A few families seem to have genetic mutations that are linked to inherited Lewy body disease, but these are very rare.

Symptoms

As with most types of dementia, the first symptoms of DLB may affect someone only slightly, but gradually they get worse and cause problems with daily living. Everyone is different, but a person with DLB will usually have some of the symptoms of Alzheimer's disease and some of the symptoms of Parkinson's disease. They will also have some symptoms which are unique to DLB. These are covered in the sections below.

Problems with mental abilities

Problems with attention and alertness are very common in DLB. An important feature of the condition is that these problems may switch between being bad and then better - or the other way round - over the course of the day, by the hour or even a few minutes. Someone with DLB may stare into space for a long time or have periods when their speech is disorganised.

People may also have difficulties judging distances and seeing objects in three dimensions. It is common to struggle with planning, organising and decision-making. Some people also experience depression. Day-to-day memory is often affected in people with DLB, but usually less in the early stages than in early Alzheimer's disease.

Hallucinations and delusions

Visual hallucinations (seeing things that are not there) occur frequently in people with DLB, often in the early stages of the condition. They can happen daily. Visual hallucinations are often of people or animals, and are detailed and convincing to the person with dementia. They can last several minutes and may be distressing. (Someone may also have visual misperceptions, such as mistaking a shadow or a coat on a hanger for a person.) Auditory hallucinations - hearing sounds that are not real, such as knocking or footsteps - can happen but are less common.

Hallucinations and visual misperceptions partly explain why most people with DLB have delusions (persistently thinking things that are not true) at some stage. Some common delusions held by people with DLB are believing that someone is out to get them, that there are strangers living in the house, or that a spouse is having an affair or has been replaced by an identical imposter. The person's relatives and carers may find these delusions very distressing.

Visual hallucinations are also a common reason for a person with DLB to stop driving, because it is no longer safe for them to be on the road. For more on this sensitive issue see our 'Driving and dementia factsheet'.

Movement problems

Up to two-thirds of people with DLB have movement difficulties when the condition is diagnosed, and this number increases as DLB progresses. These symptoms are similar to those of Parkinson's disease, and include slow and stiff (rigid) movement with a blank facial expression.

The person's posture may be stooped and their walk may be shuffling. They may also have difficulty with their balance, and their limbs may sometimes tremble.

Movement problems are one reason why a person with DLB is particularly prone to falls.

Sleep disturbance

Sleep disturbance is another common symptom of DLB and may start years before the diagnosis. Someone with DLB may fall asleep very easily during the day, but have restless, disturbed nights. In the most common night-time sleep problem, the person has violent movements (eg yelling, hitting out) as they try to act out nightmares. This is called rapid eye movement sleep behaviour disorder and is found in different Lewy body disorders such as DLB and Parkinson's disease. For bed partners it can be very distressing or even physically harmful.

Other symptoms

Some people with DLB lose their sense of smell, become constipated or have urinary incontinence (passing urine when they don't intend to). They may also faint or have unexplained episodes when they lose consciousness for a few minutes.

Later stages

DLB is a progressive condition. Over time, symptoms increase and get worse. This is generally over a period of several years.

As the disease progresses, problems with day-to-day memory and other mental abilities become more similar to those of middle- or later-stage Alzheimer's disease. People can also develop behaviours that challenge (eg agitation, restlessness, shouting out).

Worsening movement problems mean that walking gets slower and less steady. As a result, falls become more common. In the later stages of DLB, many people have problems with speech and swallowing, leading to chest infections or risk of choking.

Eventually, someone with DLB is likely to need a large amount of nursing care. How quickly the condition progresses and the life expectancy of a person with DLB vary a lot. On average someone might live for about six to 12 years after the first symptoms, similar to a person with Alzheimer's disease.

Treatment and support

At present, there is no cure for DLB. If the person is able to manage their symptoms, it will help them to live well. Given the range of different types of symptoms, they are likely to need support from a variety of professionals at different times. With correct diagnosis and support from a team of professionals, it is possible to live well with DLB.

When caring for someone with DLB, it is important to be as flexible as possible, bearing in mind that many of the symptoms will vary over time.

Treatment of DLB with drugs is often problematic. This is because everyone responds differently, there is only a small amount of evidence about what works, and there are few approved treatments. Also, medication given to improve hallucinations can make movement problems worse. Similarly, drugs for movement problems may have a negative

effect on mental abilities or make hallucinations worse. There are also serious issues for a person with DLB with use of a specific group of medications known as antipsychotic drugs (see 'Hallucinations and delusions' below).

Managing the symptoms of DLB starts by focusing on those that affect the person the most. Where possible, they should be helped to manage these symptoms in ways that don't use drugs (non-drug approaches), before trying drug treatments.

Hallucinations and delusions

If someone is having hallucinations or delusions, in most cases it is unhelpful to try to convince them that what they are seeing is not there, or that what they believe is untrue. What the person is experiencing is real to them at the time. Instead, carers can offer reassurance that they are there to support the person, and perhaps try distracting them.

It is important to get any glasses or hearing aids checked. Any problems with these can make difficulties with perceiving things worse. Misperceptions (different from hallucinations) can also be triggered by things in the environment such as excess noise and reflective or patterned surfaces.

As in other types of dementia, a person with DLB should be offered non-drug approaches to help them to maintain their mental abilities. Examples of these include social interaction, staying mentally active (cognitive stimulation), developing a structured routine and using practical strategies to live well with memory loss.

There is some evidence that the drugs donepezil and rivastigmine (which are routinely prescribed for Alzheimer's disease) can help with DLB. They improve a person's mental abilities, such as attention and alertness, as well as their ability to do day-to-day tasks. There is less evidence to support use of the related drug galantamine. None of these drugs are currently licensed for use in DLB in Europe, although rivastigmine is licensed for use in Parkinson's disease dementia. For this reason, families may find that doctors are reluctant to prescribe one of these drugs for a person with DLB, though they may do so 'off label' (outside the terms of the licence).

Memantine is a drug often given to people in the later stages of Alzheimer's disease. Some evidence shows that it can also help people with DLB, but not all of the evidence shows this.

However, some drugs may not help and may even harm the person. In particular, the prescription of antipsychotic drugs for hallucinations or delusions in DLB is very problematic. These drugs do not work in everyone and are known to increase the risk of stroke and death in people with dementia.

There is an additional and much greater risk to the use of antipsychotic drugs in people with DLB (in comparison with other types of dementia). Up to half of people taking these drugs have severe reactions, with difficulty moving (including stiffness), becoming more confused, and being unable to perform tasks or communicate. These drugs may even cause sudden death. If someone goes into hospital or sees a new doctor, the person or family should check that medical staff know that the person has DLB and that this diagnosis is clearly recorded in the person's notes.

If a person with DLB must be prescribed an antipsychotic for distressing visual hallucinations or delusions, this should be done only by a specialist after all other approaches have failed. The drug should be given with the utmost care, under constant supervision and with regular monitoring.

Behaviours that challenge

As in other types of dementia, behaviours that are challenging to cope with - for the person and the carer (eg agitation, aggression) - should be seen as a form of communication or a sign of an unmet need.

Carers or professionals should try to identify and meet this need in a way that is tailored to the individual. The cause behind a behaviour may be a medical condition, such as pain, or the side effects of drugs. It may also indicate that the person is frustrated, scared or bored. To manage these, carers should look for specific triggers and try to make appropriate changes in the person's care or environment, such as reducing unnecessary noise and clutter.

This type of behaviour can often be prevented or made better by interaction with other people, or through activities matched to the person's abilities and interests. There is also evidence of benefits from music therapy, physical exercise and hand massage. Life story work or reminiscence can help with low mood. For more information see our 'Changes in behaviour' and Dementia and aggressive behaviour factsheets.

If behaviours that challenge continue and are severe or distressing, drug treatments may be offered. One of the two Alzheimer's drugs - donepezil or rivastigmine - should be tried first. If this fails and an antipsychotic is finally offered to someone with DLB, it is important to be aware of all the risks of a severe reaction (and need for extreme caution) mentioned above.

Sleep disturbance

Steps can be taken to help a person with DLB have more restful nights. Increasing physical exercise, having a regular bedtime and getting-up time, and reducing daytime napping can all help. It can also help to avoid alcohol, caffeine and nicotine close to bedtime, and to keep the bedroom quiet and at a comfortable temperature.

Practical steps can be taken to make the area around the bed safe - remove sharp objects and put a mattress alongside the bed in case the person falls or jumps out.

Carers should also talk to the GP about sleep problems. Drugs such as clonazepam or melatonin can be effective treatments.

Movement problems

A person with movement problems will often benefit from working with an occupational therapist or physiotherapist. These professionals can help the person to move and stay independent, as well as advise on aids and adaptations in the home. If the person has had falls or is worried about falling, the GP may refer them to a falls prevention service. Their risk of falls may be reduced by strength and balance exercises, a sight test, a medication review and making the home safer (eg removing trip hazards).

Support from a speech and language therapist is often helpful if the person develops problems with swallowing or speaking.

The drug levodopa, routinely used to treat Parkinson's disease, is sometimes given to people with DLB and movement problems. Levodopa is, however, less effective in DLB than in Parkinson's disease. It improves movement problems and falls in only around one third of people with DLB. Levodopa can also further increase confusion, reduce mental abilities (eg alertness) or make hallucinations worse.

With correct diagnosis and support from a team of professionals including Home Instead Senior Care, it is possible to live well with DLB.

Frontotemporal Dementia

Frontotemporal dementia (FTD) is one of the less common types of dementia. The term covers a wide range of different conditions. It is sometimes called Pick’s disease or frontal lobe dementia. This page explains what FTD is, its symptoms, and who gets it. It also describes how it is diagnosed and the treatment and support that is available.

The word ‘frontotemporal’ refers to the lobes of the brain that are damaged in this type of dementia. The frontal lobes of the brain, found behind the forehead, deal with behaviour, problem-solving, planning and the control of emotions. An area of usually the left frontal lobe also controls speech.

The temporal lobes – on either side of the brain – have several roles. The left temporal lobe usually deals with the meaning of words and the names of objects. The right temporal lobe is usually involved in recognising faces and familiar objects. For more see the brain and dementia.

Frontotemporal dementia occurs when nerve cells in the frontal and/or temporal lobes of the brain die, and the pathways that connect the lobes change. Some of the chemical messengers that transmit signals between nerve cells are also lost. Over time, as more and more nerve cells die, the brain tissue in the frontal and temporal lobes shrinks.

When the frontal and/or temporal lobes are damaged in this way, this causes the symptoms of FTD. These include changes in personality and behaviour, and difficulties with language. These symptoms are different from the memory loss often associated with more common types of dementia, such as Alzheimer’s disease. As FTD is a less common form of dementia, many people (including some health professionals) may not have heard of it.

Symptoms

The symptoms of FTD dementia vary depending on which areas of the frontal and temporal lobes are damaged. A person may have one of three main types of FTD:

behavioural variant FTD progressive non-fluent aphasia semantic dementia.

As with most forms of dementia, the symptoms can be very subtle at first, but they slowly get worse as the disease progresses.

Behavioural Variant FTD

This is the most common type of FTD. Two thirds of people with FTD are diagnosed with this type. During the early stages, changes are seen in the person’s personality and behaviour.

A person with behavioural variant FTD may:

lose their inhibitions – behave in socially inappropriate ways and act in an impulsive or rash manner. This could include making tactless or inappropriate comments about someone’s appearance

lose interest in people and things (apathy) – lose motivation, but (unlike someone with depression) they are not sad

lose sympathy or empathy – become less responsive to the needs of others and show less social interest or personal warmth. They may also show reduced humour or laugh at other people’s misfortunes. This can make the person appear selfish and unfeeling

show repetitive, compulsive or ritualised behaviours – this can include repeated use of phrases or gestures, hoarding and obsessions with timekeeping. It may also include new interests, such as music or spirituality

crave sweet, fatty foods or carbohydrates and forget table etiquette. They may also no longer know when to stop eating, drinking alcohol or smoking.

It is common for a person with behavioural variant FTD to struggle with planning, organising and making decisions. These difficulties may first appear at work or with managing finances.

In contrast to those with Alzheimer’s disease, people in the early stages of behavioural variant FTD tend not to have problems with day-to-day memory or with visuospatial skills (judging relationships and distances between objects). Someone with FTD may go walking without obvious purpose but, unlike a person with Alzheimer’s, will often find their way home without getting lost.

Recent research shows that FTD can also affect the sensitivity of people with dementia to physical or environmental stimulation such as temperature, sounds and even pain.

It is unusual for a person with behavioural variant FTD to be aware of the extent of their problems. Even early on, people generally lack control over their behaviour or insight into what is happening to them. Their symptoms are more often noticed by the people close to them.

Progressive Non-Fluent Aphasia

In progressive non-fluent aphasia, problems are with speech – ‘aphasia’ means loss of language. Common early symptoms may include:

slow, hesitant speech which may seem difficult to produce – a person may stutter before they can get the right word out, or may mispronounce it when they do

errors in grammar – a person may have ‘telegraphic speech’, leaving out small link words such as ‘to’, ‘from’ or ‘the’

impaired understanding of complex sentences, but not single words.

Semantic Dementia

In semantic dementia, speech is fluent but people begin to lose their vocabulary and understanding of what objects are. Common early symptoms may include:

asking the meaning of familiar words (eg, ‘What is “bread”?’) trouble finding the right word, leading to descriptions instead (eg ‘the thing for opening tins’), or use of less precise

words (eg ‘animal’ instead of ‘cat’) difficulty recognising familiar people or common objects.

In the early stages of both language forms of FTD, other mental abilities (such as memory, visuospatial skills, planning and organising) tend to be unaffected.

Later stages

The rate at which FTD progresses varies greatly, with life expectancy ranging from less than two years to 10 years or more. Research shows that on average, people live for about six to eight years after the start of symptoms but this varies widely

As FTD progresses, the differences between the three types become much less obvious. People with the behavioural variant tend to develop language problems as their condition progresses. They may eventually lose all speech, like a person with one of the language variants.

Similarly, over several years a person with a language variant of FTD (especially semantic dementia) will tend to develop the behavioural problems typical of behavioural variant FTD.

In the later stages of all types of FTD, more of the brain becomes damaged. As a result, the symptoms are often similar to those of the later stages of Alzheimer’s disease. The person may become less interested in people and things and have limited communication. They may become restless or agitated, or behave aggressively. At this late stage, they may no longer recognise friends and family, and are likely to need regular care to meet their needs.

Overlapping motor disorders

About 10–20 per cent of people with FTD also develop a motor disorder, either before or after the start of dementia. A motor disorder is one that causes difficulties with movement. These motor disorders, which are generally uncommon but more likely in people with this form of dementia, are:

Motor Neurone disease Progressive Supranuclear Palsy Corticobasal Degeneration.

The symptoms of these three conditions are similar and can include twitching, stiffness, slow movements and loss of balance or co-ordination. In the later stages, they can often cause difficulties with swallowing. Progressive Supranuclear

Palsy and Corticobasal Degeneration share some symptoms with Parkinson’s disease and are sometimes called ‘atypical parkinsonism’. For more information see rarer causes of dementia.

These motor disorders are all degenerative diseases of the nervous system, meaning that they will get worse over time. If a person has both FTD and motor neurone disease, they can deteriorate more quickly than someone with FTD alone. On average, a person with both conditions will live for two or three years after diagnosis.

Causes

We don’t know exactly what causes FTD. Experts think that the disease is due to a mixture of genetic, medical and lifestyle factors. Even allowing for its under-recognition in older people, FTD does not show the very strong link with ageing seen for more common dementias such as Alzheimer’s disease or vascular dementia.

Autopsy studies show that the death of nerve cells in the frontal and temporal lobes is linked to clumps of abnormal proteins inside the cells, including proteins called tau and TDP-43. The tau protein may take the form of Pick bodies, which gave FTD its original name of Pick’s disease – after Dr Arnold Pick who first studied the dementia.

Frontotemporal dementia is much more likely to run in families than the more common forms of dementia are. About one third of people with the condition have some family history of dementia. About 10–15 per cent of people with FTD have a very strong family history of the condition, with several close relatives in different generations affected. This pattern is most common in the behavioural type of FTD and least common in semantic dementia.

Typically in these cases, FTD is inherited from a parent as a defect (mutation) in one of three genes: MAPT, GRN or C9ORF72.

Each of the children or siblings of someone with a mutation that is known to cause FTD has a 50 per cent chance of carrying the same mutation. Families with a known mutation should be offered a referral to a specialist genetics service for counselling.

Treatment and support

Researchers are working to find effective new treatments for FTD, but there is currently no cure and the progression of the disease cannot be slowed. Treatment tends to focus on helping the person live well by easing their symptoms and supporting them and those around them.

Supporting a person with FTD usually requires input from a team of professionals. These can include a GP, community nurse, psychiatrist and speech and language therapist. When someone has problems with movement or co-ordination, support from a neurologist, physiotherapist or occupational therapist is often needed as well.

Caring for someone with FTD can be particularly challenging, because of the person’s age and the changes in behaviour and communication.

Specialist support groups for younger people with dementia or those with FTD, as well as their carers, can provide invaluable practical and emotional support. Social interaction can also help if the person seems to lose motivation in things or appears bored or lonely.

If a person is found to have a gene mutation that causes FTD, birth relatives will also have to decide whether to have genetic counselling and testing themselves. Testing unaffected family members can be a cause of great anxiety. The result can have emotional, psychological, social, practical and occupational implications. This is why testing would only be done after extensive counselling with a geneticist.

Behavioural changes

Many people with FTD continue to lead an active social life for some years following diagnosis, but changes in their behaviour can begin to make social situations more challenging. It can be less stressful for carers if they try to accept

awkward and potentially embarrassing behavioural symptoms as part of the disease, rather than confront or correct the person, unless the behaviour poses a risk of harm.

The person with dementia will generally lack insight into their condition or the impact of their behaviour on others. They will also generally not have much control over their actions. When a person with FTD behaves inappropriately in public, it can be useful for the Caregiver to try to remove any triggers for this behaviour, or distract the person with something else.

Some carers of people with dementia carry a small card that explains to members of the public that the person has dementia. Alzheimer’s Society / Home Instead Senior Care produces helpcards than you can use for this purpose.

Problems with lack of insight and impulsive behaviour make safe driving very difficult for someone with FTD. Driving is often a very sensitive issue that needs careful handling. For more on this see driving.

It may be easier for a Caregiver to allow the person to carry on with other behaviours, as long as they are harmless. The person may prefer to follow a fixed routine or pursue an obsession (eg with jigsaws or music), and it may be best to let them. However, some of these behaviours, such as compulsive eating and drinking, won’t be harmless. Many carers try to help minimise a person’s opportunity for compulsive eating – for example, by offering food only at mealtimes and in suitable portions or healthier (eg low-fat) options. The person’s use of alcohol may also need to be closely monitored.

It is important to try to manage restlessness, agitation or aggressive behaviour without drugs initially, where possible. This behaviour might result from a person trying to communicate an unmet need, such as feeling frustrated or in pain. Physical exercise and enjoyable, tailored activities, carried out as part of a routine, can help to reduce these types of behaviour.

There is evidence that certain antidepressant drugs can help some people with FTD feel less apathetic (having little interest in people and things) and relieve some behavioural symptoms.

If antipsychotic drugs are being considered for a person with FTD, it is recommended that a specialist advises on the risks and benefits.

There have been a few small trials of drugs used to treat Alzheimer’s disease (donepezil, rivastigmine, galantamine and memantine) in people with FTD. These have had mixed results. In some cases, these drugs made people’s symptoms worse. They are also not licensed for use in FTD and are not widely prescribed.

Language problems

A speech and language therapist with the right experience will be able to support someone with FTD who is gradually losing their language skills. They will try to maximise a person’s existing skills and find new ways for them to communicate.

A therapist can advise a person’s carer on new ways of listening and talking – for example, talking in simpler sentences. In time, a person who is losing their language skills may be taught non-verbal ways of communicating. These can include the use of gestures, drawing or electronic devices.

A speech and language therapist can also help if a person has problems swallowing and can offer practical advice on eating and drinking.

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is caused by an abnormally shaped protein called a prion infecting the brain. Sporadic CJD, which normally affects people over 40, is the most common form of the disease. It is estimated that the disease affects about one out of every 1 million people each year. It is not known what triggers sporadic CJD, but it is not known to be inherited or otherwise transmitted from person to person.

A more recently identified form of CJD, called new variant CJD, was caused by eating meat from cattle infected with bovine spongiform encephalopathy (BSE). This typically affected younger adults. In new variant CJD, there may be many years between a person being infected and the development of symptoms.

In sporadic CJD, the disease usually progresses within a few months. Early symptoms include minor lapses of memory, mood changes and loss of interest. Within weeks the person may complain of clumsiness and feeling muddled, become unsteady walking, and have slow or slurred speech. Symptoms progress to jerky movements, shakiness, stiffness of limbs, incontinence and loss of the ability to move or speak. By this stage the person is unlikely to be aware of their surroundings or disabilities.

People affected by CJD usually die within six months of their early symptoms developing. In a small number of patients the disease may take longer to run its course.

Diagnosis can often be difficult as a range of causes including Alzheimer's disease and vascular dementia need to be ruled out. If no immediate diagnosis is obvious, the neurologist may conclude that the person has a rare neurodegenerative disease - a type of disease that affects the nervous system and gets worse over time - and may refer them to the National Prion Clinic in London for further assessment.

There is no known cure for CJD, however it is an area which is being actively researched.

Young-Onset Dementia

People with dementia whose symptoms started before they were 65 are often described as 'younger people with dementia' or as having young-onset dementia. The age of 65 is used because it is the age at which people traditionally retired. However, this is an artificial cut-off point as opposed to having any biological significance.

Early-onset dementia is caused by broadly similar diseases to dementia in older people ('late-onset dementia'), but there are some important differences. There is a wider range of diseases that cause young-onset dementia and a younger person is much more likely to have a rarer form of dementia.

Young-onset dementia is also more likely to cause problems with movement, walking, co-ordination or balance. This is one reason why younger people with dementia may see a neurologist (a specialist in diseases of the brain and nervous system) rather than - or as well as - a psychiatrist (a specialist in mental health).

However, people under 65 do not generally have the co-existing long-term medical conditions of older people - especially diseases of the heart and circulation. They are usually physically fitter and dementia may be the only serious condition that a younger person is living with.

Early-onset dementia is more likely than late-onset dementia to be hereditary. In perhaps 10 per cent of all people with young-onset dementia the condition seems to have been inherited from a parent. If dementia has been inherited, the diagnosis may have implications for birth relatives of the person such as their siblings (brothers and sisters) or children.

Early onset of common dementias

Alzheimer's disease

Alzheimer's disease develops when proteins build up in the brain to form structures called 'plaques' and 'tangles'. Alzheimer's is the most common type of dementia in younger people and may affect 30 to 35 per cent of younger people with dementia. However, this is a much smaller proportion than in older people with the condition, up to two thirds of whom have Alzheimer's disease.

Another difference is that younger people are much more likely to have an 'atypical' (ie unusual) form of Alzheimer's disease than older people. Atypical Alzheimer's disease is when the first symptoms are not memory loss, which is the most common symptom of late-onset Alzheimer's disease. Instead, the first symptoms are usually problems with vision

(in posterior cortical atrophy), speech (in logopenic aphasia) or planning, decision-making and behaviour (in frontal variant Alzheimer's disease). These atypical forms of Alzheimer's disease account for up to one third of all Alzheimer's disease in younger people but only 5 per cent of all Alzheimer's disease in older people.

In some people with early-onset Alzheimer's disease there is a very clear inheritance of the disease from one generation to the next. This genetic form of the dementia - familial Alzheimer's disease - is caused by rare mutations (defects) in three genes. These mutations are found in between 7 and 12 per cent of all people with early-onset Alzheimer's.

Symptoms of familial Alzheimer's disease usually start in someone's 30s, 40s or 50s; the earlier the symptoms start, the more likely the disease is to be genetic. However, familial Alzheimer's disease is extremely rare. It affects only about 500 known families worldwide and probably accounts for much less than 1 per cent of Alzheimer's disease when all ages, young or old, are considered.

People with Down's syndrome and other learning disabilities can also develop dementia at an early age. Alzheimer's disease is the most common type of dementia in people with Down's syndrome. This increased risk is thought to be associated with the extra copy of chromosome 21 which most people with Down's syndrome have. Chromosome 21 carries the gene for amyloid which forms the plaques in Alzheimer's disease.

Vascular dementia

Vascular dementia occurs when there are problems in the blood supply to the brain. It is closely linked to diabetes and to cardiovascular diseases such as stroke and heart disease. It is probably the second most common type of dementia in younger people - around 15 per cent of dementia in this age group may be vascular dementia.

Symptoms of vascular dementia vary. Early memory loss is less common than in Alzheimer's disease, whereas problems with thinking things through and slower speed of thought are more common. When vascular dementia follows a stroke, physical symptoms (eg limb weakness) are common.

There is a genetic form of vascular dementia known as CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy). CADASIL is rare and is most common in people aged 30 to 50. Symptoms include migraines, repeated strokes, fits, low mood and progressive loss of mental abilities. CADASIL is caused by defects in a gene called NOTCH3 and is inherited in a simple pattern similar to familial Alzheimer's disease.

Frontotemporal dementia

Frontotemporal dementia is caused by damage to the lobes at the front and/or sides of the brain. Around 10 to 15 per cent of younger people with dementia may have this form - much higher than in older people. Frontotemporal dementia is most often diagnosed between the ages of 45 and 65. This is in contrast to Alzheimer's disease, vascular dementia and dementia with Lewy bodies, all of which become more likely with age.

There are three different types of frontotemporal dementia - the behavioural variant, which causes changes in personality and behaviour first, and two types in which language is affected first.

In around 30 per cent of people with this type of dementia, there is good evidence that the disease is genetic. In this instance, genetic counselling and testing may be offered (see 'Genetic testing').

Dementia with Lewy bodies

This type of dementia is caused by the build-up of tiny protein deposits (Lewy bodies) in the brain. About 5 per cent of younger people with dementia may have dementia with Lewy bodies. Lewy bodies also cause Parkinson's disease and about one third of people with Parkinson's eventually develop dementia. Symptoms of dementia with Lewy bodies include varying levels of alertness, and people can also develop hallucinations and the features of Parkinson's disease (eg slower movement, stiffness, trembling of the limbs).

Alcohol-related brain damage

Alcohol-related brain damage includes Korsakoff's syndrome and alcoholic (or alcohol-related) dementia. It occurs in people, most often in their 50s, who regularly consumed excessive amounts of alcohol. Alcohol-related brain damage is caused by a lack of thiamine (vitamin B1), direct damage to nerve cells from alcohol, head injuries (eg falls, fights) and a poor diet.

At least 10 per cent of younger people with dementia may have alcohol-related brain damage. Symptoms overlap with those of Alzheimer's disease and vascular dementia. Alcohol-related brain damage differs from common dementias because it can be halted or even reversed in some people with treatment, abstinence and a good diet.

Rarer forms of dementia

Around 20 to 25 per cent of younger people with dementia are thought to have a rarer cause of the condition - a much higher proportion than in older people. These causes include degenerative neurological conditions (where there is progressive damage to the nervous system) such as Huntington's Disease, Progressive Supranuclear Palsy, Corticobasal Degeneration and Creutzfeldt-Jakob disease (CJD).

In most cases these diseases cause problems with movement as well as dementia. Some of these rarer causes of dementia, such as CJD, can progress very rapidly over just a few months.

Progression

Everyone's experience of dementia is different, but for all the dementias mentioned above most people will become more frail as the condition progresses. This means that they will gradually become dependent on others for their care.

This may be very difficult for all those affected to think about. However, knowing this can enable the person with dementia to think ahead about the kind of treatment and care they might want. Many people decide to set up a Lasting Power of Attorney or make an advance decision before they reach this stage. This gives the person input into how they will be cared for. It also allows carers and family to think about these aspects too.

Other causes

A wide range of other diseases can cause symptoms of dementia, often along with other symptoms (eg spleen or liver problems) which can be very different.

These diseases include inherited metabolic disorders such as Gaucher's disease, Tay Sach's disease and Niemann-Pick's disease. These develop more often in childhood or adolescence and so their development in someone's 30s or 40s is much later than usual.

Other causes of dementia symptoms in younger people include hormone disorders (eg thyroid problems, Addison's Disease), vitamin (eg B12) deficiencies, inflammatory conditions (eg multiple sclerosis) and infections (eg HIV). Memory problems can also be caused by sleep apnoea, where breathing stops for a few seconds or minutes during sleep.

These conditions are not discussed further here but it is important that they are diagnosed because some (eg vitamin deficiency, thyroid problems, sleep apnoea) can be treated.

Treatment

Dementia cannot be cured but there are treatments and support that can help someone live well with the condition. This involves drug and non-drug treatment, support, activities, information and advice.

Common drug treatments help with symptoms of dementia, such as donepezil for Alzheimer's disease or certain antidepressants for frontotemporal dementia. For vascular dementia, drugs will be offered to help to treat the underlying conditions.

Non-drug treatments are also available, usually through the GP. Counselling may help the person adjust to the diagnosis or with relationship issues. Talking therapies may help if the person (or anyone supporting them) becomes depressed or anxious.

Ask a professional about sessions of cognitive stimulation or life story work because these can also help. Non-drug approaches should also be tried first for behavioural changes.

Dementia progresses more quickly if someone is physically unwell, so it is important that the person looks after themselves. This includes regular physical exercise, not smoking, drinking alcohol only in moderation, eating a healthy diet and keeping to a healthy weight.

Age-appropriate services

Services for all people with dementia should try to help them maintain their day-to-day skills, friendships and hobbies, as well as to develop new interests if they wish to. Where possible, they should also support people to continue to live an active life as a member of their local community. Local authorities can allocate a personal budget (only applicable in England) that can help in managing costs for such services. This is dependent, however, on whether the person qualifies for funding.

Services for younger people

Younger people with dementia require dedicated age-appropriate services that are able to meet their specific needs. This could be in the form of a dementia café, activity group, adventure holiday, help at home, day care or residential care. If the person has a very rare dementia then their needs may be very specific.

In practice, such age-appropriate services may not often be available, or not available within a reasonable distance. This is particularly true of supported housing or residential care for people with young-onset dementia. However, some larger care homes now have age-appropriate provision within a dedicated part of the building.

This general lack of age-appropriate services is partly caused by low levels of awareness of the distinct needs of younger people with dementia. Another factor is that young-onset dementia is relatively uncommon, so services tend to be thinly spread.

The result is that younger people with dementia may find that they are referred to a service designed around the needs of older people. Such a service set up for people of a different generation is unlikely to meet the needs of a younger person. For example, activities planned for older people may be less physically demanding and therefore unsuitable for a more active younger person. Or they may draw on experiences or music from a period which has no meaning to a younger person.

Alternatively, a younger person with dementia may find that they are excluded from an older peoples' service because of their age. This can mean that younger people find themselves in the 'gaps' between services, none of which will accept responsibility for their care. People with young-onset dementia and their families understandably say that they find this very frustrating.

Finding age-appropriate services and support

A good starting point to finding out about appropriate support is where the dementia was diagnosed. There may be a dementia adviser or nurse - such as an Admiral Nurse - present who has specialised in young-onset dementia. Such a person can offer individualised advice and support as well as information about age-appropriate services that are available.

It is also a good idea to ask the GP or social services about a needs assessment, also called a community care assessment. This aims to find ways to help maintain independence and quality of life. If the local authority is paying for some of the person's care and support, they should offer them a personal budget. This could be in the form of a direct payment, which can be used flexibly to help meet the person's needs. For example, this could go towards sports or leisure activities or - as the dementia progresses - to pay a Home Instead Senior Care Caregiver to give personal care at home.

Local Alzheimer's Society staff can also advise on what is available in the area. They should be able to offer information and advice, and will run (or know about) dementia cafés and support groups, which are often led by such voluntary sector organisations. In addition, they can advise on befriending, advocacy, or information and support sessions for people with dementia or carers. They may additionally be able to offer a dementia adviser who can provide tailored information and advice.

In some parts of the country there are regional support groups for younger people or those living with specific dementias (eg frontotemporal dementia, familial Alzheimer's disease). These can put younger people with dementia, their families or carers in contact with others in similar circumstances. They offer the opportunity for people to share experiences and strategies for living well.

Alcohol-Related Brain Damage (including Korsakoff's syndrome)

Alcohol-related brain damage (ARBD) is a brain disorder caused by regularly drinking too much alcohol over several years. The term ARBD covers several different conditions including Wernicke-Korsakoff syndrome and alcoholic dementia. None of these is actually a dementia, but they may share similar symptoms. However, in contrast to common causes of dementia such as Alzheimer's disease, most people with ARBD who receive good support and remain alcohol-free make a full or partial recovery. In addition, there is a good possibility that their condition will not worsen.

Patterns of drinking in Great Britain have changed over the past 10 years. Middle-aged people are now the age group which consumes the most alcohol and they are drinking more than in the past, especially middle-aged women. In contrast, younger people (aged 16-24) are now drinking less, particularly when it comes to binge drinking. Against this background, ARBD is an under-recognised and growing problem.

This page outlines the causes, symptoms, diagnosis and treatment of ARBD. It also gives practical tips for carers on supporting someone with the condition.

Alcohol as a risk factor for dementia

Drinking more than the recommended limit for alcohol increases a person's risk of developing common types of dementia such as Alzheimer's disease and vascular dementia. The NHS recommended limits are now a maximum of 14 units each week for men and women, spread over 3 or more days - although lower limits have been suggested for older people because their bodies handle alcohol differently. A small 125ml glass of wine is typically about 1.5 units and a pint of beer, lager or cider is usually 2-2.5 units.

It seems that repeated binge drinking - heavy drinking in one session, often leading to drunkenness - is particularly harmful. Binge drinking is dangerous because it raises the amount of alcohol in the blood to a high level very quickly.

This increased risk of dementia is greatest at higher levels of alcohol consumption - the more you drink, the higher your risk. But you do not need to be an alcoholic or get drunk often to be at increased risk of developing dementia. Regularly drinking even a little above recommended levels probably increases your risk. It also increases your risk of other conditions such as stroke, heart and liver disease, and cancer.

Regularly drinking above recommended limits is seen as one risk factor that contributes towards dementia, rather than being a direct cause. Other lifestyle risk factors that raise a person's chances of developing dementia include smoking, lack of exercise and unhealthy diet. As explained below, regularly drinking at much higher levels than recommended can directly cause problems similar to dementia and so is different.

Alcohol-related brain damage

Some people regularly drink much higher levels than the recommended limits of alcohol. For men, such excessive drinking could mean more than 50 units per week, and for women, more than 35 units/week. Drinking at these high levels not only poses a particularly high risk to someone’s health but it also increases the risk of the person becoming addicted. (Alcohol addiction is where someone has become dependent on alcohol. They have an excessive desire to drink and their drinking is causing problems in their daily life.) Alcohol intake at such high levels over several years directly damages the brain, causing alcohol-related brain damage (ARBD) in some people.

Alcohol-related brain damage leads to slightly different symptoms in different people and causes a range of conditions. The most common form of ARBD is alcoholic dementia which may also be called alcohol-related dementia. ARBD also includes Korsakoff's syndrome, which is also called Korsakoff's psychosis.

Causes

ARBD is defined as long-term decline in memory or thinking caused by excessive alcohol use and a lack of vitamin B1 (thiamine). Thiamine is needed to provide energy to the body. It is especially important for brain and nerve cells because they use so much energy.

Alcohol misuse causes ARBD in a range of ways. Regular heavy drinking over time damages nerve cells because alcohol is a toxin. It also causes chemical changes in the brain and the shrinkage of brain tissue.

The second way that alcohol misuse leads to ARBD is by causing thiamine deficiency. This is partly because heavy drinkers tend to not look after themselves and have poor diet. Alcohol also irritates the stomach lining, leading to vomiting and poor absorption of nutrients. Thiamine deficiency also happens because alcohol interferes with the way the body stores and handles the vitamin.

Alcohol can also cause ARBD through repeated head injuries. People who misuse alcohol are more prone to falls and getting into fights.

Finally, heavy drinking damages blood vessels and is linked to high blood pressure, raised cholesterol levels and an increased risk of heart attacks and strokes. All of these conditions can damage the brain.

It is important to note that not all of these factors are equally important in all forms of ARBD or in everyone with the condition.

Who develops ARBD?

Reliable figures of the number of people with ARBD are not available and the condition is likely to be under-diagnosed. This is partly because having problems with alcohol still carries a stigma within society, so people may not seek help. Awareness of ARBD even among professionals also varies widely. Alcohol-related brain damage may therefore go undiagnosed or unrecorded in a patient's notes.

Evidence from post mortem studies in the UK shows that ARBD affects about 1 in 200 of the general adult population. Among those with alcoholic addiction, this figure rises to as high as one in three. It is not clear why some people develop ARBD while others do not.

People with ARBD tend to be middle-aged, typically in their 40s or 50s, although they can be younger or older. This is younger than the age at which people most often develop common dementias such as Alzheimer's disease. Alcohol-related brain damage is thought to cause more than 10 per cent of 'dementia' in younger people - defined as those aged under 65.

Alcohol-related brain damage is more common among people in poorer communities. It also affects men much more often than women. However, where women are affected, they tend to develop ARBD at a younger age than men and after fewer years of alcohol misuse. Women are more vulnerable to the effects of alcohol.

Korsakoff's Syndrome

Korsakoff's syndrome is a form of ARBD in which the main cause is a clear lack of thiamine. Korsakoff's syndrome is the most well-known form of ARBD and many people think that it is the most common or even only form. However, Korsakoff's syndrome is much less common than other forms of ARBD such as alcoholic dementia.

Korsakoff's syndrome often develops as part of a condition known as Wernicke-Korsakoff syndrome. This consists of two separate but related stages: Wernicke's encephalopathy followed by Korsakoff's syndrome. Wernicke-Korsakoff syndrome is diagnosed in about one in eight people with alcoholism. However, not everyone has a clear case of Wernicke's encephalopathy before Korsakoff's syndrome develops.

How does Wernicke's encephalopathy develop?

An encephalopathy is a disorder that affects the function of the brain. Wernicke's encephalopathy usually develops suddenly, often after abrupt and untreated withdrawal from alcohol. It has a range of different symptoms, but they may not be obvious and it can be difficult to make a diagnosis.

Symptoms of Wernicke's encephalopathy can include:

disorientation, confusion or mild memory loss undernutrition - for example, the person is very underweight involuntary, jerky eye movements or paralysis of the muscles that move the eyes poor balance or unsteadiness, or other signs of damage to a part of the brain called the cerebellum (a region

involved in co-ordinating movement).

If Wernicke's encephalopathy is suspected, immediate medical treatment is essential. The person will need high doses of thiamine (and other B vitamins) injected slowly into a vein. If treatment is done in time, most symptoms will be reversed in a few days. However, permanent brain damage may result if Wernicke's encephalopathy is left untreated or is not treated properly or in time. In some severe cases the person may die.

How does Korsakoff's syndrome develop?

Where Wernicke's encephalopathy is untreated, or is not treated soon enough, Korsakoff's syndrome usually develops, though often gradually. Damage occurs in several regions of the brain - particularly in important small areas deep within the brain, resulting in severe loss of short-term day-to-day memory. Many other abilities may remain intact, such as working memory (information held in our head for a short time before using it, eg working out how much something costs).

Symptoms

As with Korsakoff's syndrome, the symptoms of alcoholic dementia largely reflect the areas in the person's brain that are damaged. Overall, the symptoms of alcoholic dementia are more varied than those of Korsakoff's.

A person with alcoholic dementia often has:

poor planning and organisational skills, and problems with decision-making, judgement and risk assessment problems with impulsivity (eg rash financial decisions) and difficulty controlling emotions (eg irritability or

outbursts) problems with attention and slower reasoning lack of sensitivity to the feelings of other people behaviour which is socially inappropriate.

Unlike Korsakoff's syndrome, however, not everyone with alcoholic dementia has loss of day-to-day memory.

Treatment and support

Unlike Alzheimer's disease or vascular dementia, ARBD is not certain to worsen over time. There is a good chance of stabilisation or improvement if the person is given high doses of thiamine, remains free from alcohol and adopts a healthy diet with vitamin supplements. Brain scans show that, with abstinence, some of the damage caused by excessive drinking can be reversed. However, if the person continues to drink and eats poorly, ARBD is likely to continue to progress.

Treatment and support of a person with ARBD is given in two phases: the initial stabilisation phase and longer term rehabilitative care.

Initial stabilisation

During the initial phase of treatment, which usually lasts up to several weeks, the aim is to withdraw alcohol and stabilise the person's medical condition. Most patients with ARBD will need to stay in hospital for this phase.

Withdrawal of alcohol usually causes the person to have delirium (disorientation which varies over time) as well as intense sweating. They will also often have behavioural problems such as agitation and hallucinations. Treatment will include sedation, replacement of lost fluids and salts, and high doses of thiamine by injection.

Unfortunately, many people with ARBD have a lengthy wait in hospital before gaining access to specialist care. Depending on how serious the ARBD, a person could be discharged to residential care, sheltered accommodation or their usual home with support in the community.

After initial stabilisation, a person with ARBD will need ongoing support from two different kinds of services. Professionals in these services should work closely together to ensure the best care for the person.

Alcohol treatment services

To stay alcohol-free the person will need support from alcohol treatment services which also help people with alcohol problems who do not have ARBD. Special drugs will sometimes be prescribed which either reduce the craving for alcohol or make the person feel unwell (or vomit) if they do drink. They will also need to take thiamine tablets and eat well generally. As well as medication, the person will need non-drug support to stay alcohol-free. This often includes counselling or a 'talking therapy' such as cognitive behavioural therapy focused on their drinking. Support here will also look at a person's social networks and encourage them to attend self-help groups. Some of the common symptoms of ARBD make it harder for a person with the condition to engage with an alcohol treatment programme. This can include denial, lack of insight and impulsivity. Staying alcohol-free is also more challenging if the person has a complex social setting (eg homelessness, isolated from family due to alcohol misuse). Other medical problems such as drug misuse, poor physical health or depression can also add to the challenge. Addressing these broader aspects is important in helping the person stay alcohol-free.

Rarer types of dementia

Alzheimer's disease is the most common cause of dementia, but there are many rarer diseases and conditions that can lead to dementia, dementia-like symptoms or mild cognitive impairment. Rarer forms of dementia account for only around 5 per cent of all dementia cases in the UK. This page outlines some of these rarer causes and gives some suggestions for where to go for more specialist advice and information.

Corticobasal Degeneration (CBD) Huntington's Disease Multiple Sclerosis (MS) Niemann-Pick disease type C Normal Pressure Hydrocephalus (NPH) Parkinson's Disease Dementia (PDD) Posterior Cortical Atrophy (PCA) Progressive Supranuclear Palsy (PSP)