Alzheimer's Society

Alzheimer's disease is the most common cause of dementia, but there are many rarer diseases and syndromes that can lead to dementia. This information sheet outlines some of these rarer causes and gives some ideas about where to go for more specialist advice and information.

Binswanger's disease

Binswanger's disease is a rare form of vascular dementia in which damage occurs to the blood vessels in the deep white matter of the brain.

Symptoms of Binswanger's most often occur in people over the age of 60. Binswanger's mainly affects memory and mental abilities such as thinking and learning. The person may also experience mood swings, tremors, problems in walking and seizures.

The cause of Binswanger's disease is unknown and there is no cure, although the person may be given medication - to control blood pressure or to treat depression, for example.

Dementia with Lewy bodies (DLB)

In dementia with Lewy bodies, microscopic deposits in the brain (Lewy bodies) cause damage to nerve cells. Many of the symptoms of DLB, such as loss of memory and reasoning skills, are similar to those in Alzheimer's disease. In addition, many people with DLB develop Parkinson-type symptoms, such as slowness, stiffness and tremor. Visual hallucinations and falls are common.

Particular care should be taken with certain tranquillising medicines (neuroleptics) as these can be dangerous for people with DLB.

For more information, see the Alzheimer's Society's information sheet What is dementia with Lewy bodies (DLB)? or contact the Pick's Disease Support Group (see under Fronto-temporal dementia).

Down's syndrome

People with Down's syndrome are at particular risk of developing Alzheimer's disease in adult life and this risk increases with age. Research suggests that over half of people with Down's syndrome in their 60s have Alzheimer's. People with other forms of learning disability also have an increased risk of dementia, though not as high as that of people with Down's syndrome.

Problems with communication may mean that early signs of dementia are missed so it is important for carers and relatives to be alert to any changes in behaviour or capabilities. Special tests are being developed to help in diagnosis.

For more information see the information sheet Learning disabilities and dementia or contact:

Down's Syndrome Association

Langdon Down Centre
2a Langdon Park
Teddington TW11 9PS

Telephone 0845 230 0372 (10am-4pm Monday to Friday)
Email info@downs-syndrome.org.uk
Website www.downs-syndrome.org.uk

Fronto-temporal dementia

The term 'fronto-temporal dementia' is used for a range of conditions including Pick's disease, frontal lobe degeneration and the dementia associated with motor neurone disease.

Damage occurs in the frontal or temporal lobe areas of the brain, or both. Younger people - those under the age of 65 - are more likely to be affected. Symptoms vary but often include personality and behaviour changes, problems with judgment and planning and loss of language skills.

For more information see the information sheet What is fronto-temporal dementia (including Pick's disease)? or contact:

The Pick's Disease Support Group

8 Brooksby Close Oadby
Leicester LE2 5AB

Telephone 0116 271 1414
Email info@pdsg.org.uk
Website www.pdsg.org.uk

The Pick's Disease Support Group also provides information and support for people with dementia with Lewy bodies, cortico-basal degeneration, alcohol-related dementia and Korsakoff's syndrome.

HIV and dementia

There are a number of different terms used to describe the dementia caused by the human immunodeficiency virus (HIV). These terms include 'Aids dementia complex', 'HIV dementia' and 'HIV related brain impairment'.

Although HIV can have a direct effect on the brain, the majority of people with HIV do not develop dementia or show any marked decline in their mental abilities. In addition, far fewer cases of HIV-related dementia or cognitive (mental) impairment are being seen in the UK at present owing to the combination of drug therapies that prevent the virus from multiplying.

Symptoms, where they occur, will differ from person to person and even change at different times of the day. They may include forgetfulness, difficulties with concentration and complex thought, apathy, mood swings and hallucinations. Some people may only experience a few very mild symptoms, such as a decline in the ability to think quickly or clearly. These mild impairments do not amount to dementia.

For further information and support contact:

Terrence Higgins Trust

314-320 Grays Inn Road
London WC1X 8DP

Helpline 0845 12 21 200 (weekdays 10am-10pm and weekends 12pm-6pm; calls are charged at local rate)
Email info@tht.org.uk
Website www.tht.org.uk

Huntington's disease

Huntington's disease is a progressive hereditary disease. It most often becomes apparent in adults in their 30s, although it can occur earlier or later. There is also a juvenile type of Huntington's, which affects children.

The course of the disease varies for each person and dementia can occur at any stage of the illness.

Symptoms of dementia associated with Huntington's disease include loss of short term memory and loss of planning and organisational skills. People affected usually lack insight into their condition and other people's needs and may be reluctant to accept help. There may be obsessive behaviour. However, this form of dementia differs from Alzheimer's disease in that those affected continue to recognise people and places.

At present, the dementia associated with Huntington's is untreatable, but a great deal of research is being carried out.

For more information contact:

Huntington's Disease Association

Down Stream Building
1 London Bridge
London SE1 9BG
Telephone 020 7022 1950
Email info@hda.org.uk
Website www.hda.org.uk

Korsakoff's syndrome

Korsakoff's syndrome may result from continual heavy drinking over a long period. It is caused by lack of thiamine (vitamin B1). This may be due to poor nutrition or to poor absorption of vitamins
resulting from the effects of alcohol on the stomach lining, or both. Its main symptom is loss of short term memory. It is not strictly a dementia as damage is confined to small areas in the mid-part of the brain. It can be halted if the person stops drinking and eats healthily.

Alcohol may also have a harmful effect on the nerve cells in the outer layer of the brain, affecting a wide range of skills and abilities. This is sometimes known as alcoholic dementia. However, there may be some recovery if people abstain from alcohol.

There is thought to be considerable overlap between Korsakoff's syndrome and alcoholic dementia. See the information sheet What is Korsakoff's syndrome?, or contact the Pick's Disease Support Group (see under Fronto-temporal dementia), or contact:

Alcohol Concern

Waterbridge House
32-36 Loman Street
London SE1 OEE
Telephone 020 7928 7377
Email contact@alcoholconcern.org.uk
Website www.alcoholconcern.org.uk

Multiple sclerosis (MS)

Some people with multiple sclerosis may experience a loss of some of their mental abilities as their disease progresses. This will occur if damage owing to MS occurs in certain parts of the brain. People
may be affected to different degrees and in different ways over a period of time. The mental abilitiesmost likely to be affected are memory, concentration and problem solving. There may also
be emotional problems, such as mood swings.

The term dementia is not generally used in association with multiple sclerosis because the decline is not usually as severe as it is in Alzheimer's disease, for example. It is more usual to describe the person as experiencing cognitive difficulties.

For further information and support contact:

MS Society

MS National Centre
372 Edgware Road
Staples Corner
London NW2 6ND
Telephone 020 8438 0700
Helpline 0808 800 8000 (weekdays 9am-9pm; calls
are free)
Email info@mssociety.org.uk
Website www.mssociety.org.uk

Niemann-Pick disease type C

Niemann-Pick disease type C is one of a group of rare inherited disorders. It mainly affects school-age children but it can occur at any time from early infancy to adulthood. It is caused by the inability of the body to deal with cholesterol and leads to
progressive loss of movement and difficulties with walking and swallowing.

Dementia is often a particular problem when the disease becomes apparent in late adolescence or early adulthood. The symptoms of dementia include confusion, memory problems, and difficulties in
concentrating and learning. There is no treatment so far and life expectancy varies. However, research has identified the affected gene.

For further information and support contact:

The Niemann-Pick Disease Group (UK)

Kingslaw House
East Brae
East Wemyss
Fife KY1 4RS
Helpline 01592 580672
Email niemann-pick@zetnet.co.uk
Website www.nnpdf.org/npdg-uk

Normal pressure hydrocephalus (NPH)

Normal pressure hydrocephalus (NPH) occurs when an obstruction in the normal flow of spinal fluid causes pressure to build up in the tissues of the brain. Symptoms include difficulties with walking, dementia and urinary incontinence.

People who have had a history of meningitis, encephalitis or head injury are more likely to develop NPH. The condition is sometimes
treatable.

Parkinson's disease

While people with Parkinson's disease have a higher risk of developing dementia than people without Parkinson's disease, the majority remain unaffected.

How dementia occurs in Parkinson's disease is not yet understood. It may be that the microscopic
deposits known as Lewy bodies, which occur in nerve cells in the brain stem in people with Parkinson's, have a role to play, as they do in dementia with Lewy bodies (see above). In addition, the side-effects of certain drugs for Parkinson's may exacerbate symptoms of dementia. Adjusting medication for Parkinson's is sometimes helpful.

Symptoms of dementia associated with Parkinson's disease vary from person to person. The most common are memory loss and loss of the ability to reason and to carry out normal everyday tasks. The person may become obsessional and there may be a loss of emotional control, with sudden outbursts of anger or distress. Visual hallucinations may occur. Symptoms often fluctuate
so that the person seems better or worse at different times.

For further information contact:

Parkinson's Disease Society

215 Vauxhall Bridge Road
London SW1V 1EJ
Telephone 020 7931 8080
Helpline 0808 800 0303 (weekdays 9.30am-5.30pm;
calls are free)
Email enquiries@parkinsons.org.uk
Website www.parkinsons.org.uk

Prion diseases

These are a group of rare diseases in which a transmissible agent, known as prion protein, accumulates in the brain. This causes dementia and neurological symptoms including unsteadiness and
jerky movements.

Different prion diseases occur in humans and
animals. One of these, Creutzfeldt-Jakob disease (CJD), has been identified for some time in a small number of humans. More recently, a new form of CJD, known as variant CJD, has been identified. See the information sheet What is Creutzfeldt-
Jakob disease (CJD)? or contact:

The Creutzfeldt-Jakob Disease Support Network

P.O. Box 346
Market Drayton
Salop TF9 4WN
Helpline 01630 673973
Email info@cjdsupport.net
Website www.cjdsupport.net

Progressive supranuclear palsy (PSP)

Progressive supranuclear palsy (PSP) is a comparatively rare progressive condition, sometimes known as Steele-Richardson-Osliewski syndrome. It affects the brain stem and adjacent areas and some early features resemble those
experienced in Parkinson's disease.

PSP mainly occurs in people over the age of 50, although it occasionally occurs in younger people. A striking feature is paralysis affecting eye movements and problems with vision. Although there are likely to be problems with more complex and abstract thought, the person will remain aware of what is
going on around them. In most cases, the person is more likely to be described as experiencing cognitive difficulties than dementia.

For further information and support contact:

The PSP Association

The Old Rectory
Wappenham
Towcester
Northants NN12 8SQ
Telephone 01327 860 299
Email psp@pspeur.org
Website www.pspeur.org

Thyroid deficiency

An underactive thyroid gland (hypothyroidism) can lead to the symptoms of dementia. Simple tests can detect this condition. The dementia symptoms include loss of interest, apathy, slowing down of mental abilities and poor short term memory.

Treatment involves replacing the naturally occurring thyroid hormones with synthetic hormone preparations. This is more likely to be effective in reversing the dementia if the problem is identified and treated within two years of its onset.

Vitamin deficiency

Vitamin deficiency, particularly a lack of the B vitamins, including thiamine (vitamin B1), riboflavin (vitamin B2), niacin (vitamin B3),
pyrodoxine (vitamin B6), folic acid and vitamin B12, can cause dementia-like symptoms.

Vitamin deficiencies may be due to a poor diet or to problems with the body's absorption of vitamins.
Vitamin deficiencies can be detected through blood tests. Treatment may include vitamin supplements and an improved diet, or in some cases, dealing with conditions that lead to poor
absorption of vitamins.

Information Sheet 442
Last updated: July 2006
Last reviewed: July 2006