Rarer Causes of Dementia - Rarer Causes of Dementia

Rarer Causes of Dementia

Rarer causes of dementia

Alzheimer's disease is the most common cause of dementia, but there are many rarer diseases and syndromes that can lead to dementia. This factsheet 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 and it is usually associated with long-term hypertension. The disease mainly affects memory and mental abilities such as thinking and learning. The person may also experience mood swings, tremors, seizures and problems with walking.

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 and learning disabilities

People with some forms of learning disability have an increased risk of developing dementia in adult life. Those with Down's syndrome are at particular risk of developing Alzheimer's disease, and this risk increases with age. Research suggests that over half of people with Down's syndrome in their 60s also have Alzheimer's disease.

Problems with communication in people with learning disabilities 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 Factsheet 430, Learning disabilities and dementia, or contact the British Institute of Learning Disabilities (see 'Useful organisations' for details).

Dementia with Lewy bodies

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

Particular care should be taken with certain tranquillising medicines (neuroleptics), as these can be dangerous for people with dementia with Lewy bodies.

For more information, see Factsheet 403, What is dementia with Lewy bodies?.

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 judgement and planning, and loss of language skills.

For more information see Factsheet 404, What is fronto-temporal dementia (including Pick's disease)?, or contact the Pick's Disease Support Group (see 'Useful organisations').

HIV-related cognitive impairment

Around one in five people living with the human immunodeficiency virus (HIV) may develop HIV-related cognitive impairment, but the majority of people with HIV do not develop dementia or show any marked decline in their mental abilities.

There can be problems diagnosing HIV-related cognitive impairment because there has been some confusion about the precise definition of the condition, and people can be misdiagnosed as having depression or other conditions instead. Symptoms differ from person to person and may even vary depending on the time of day. They may include forgetfulness, difficulties with concentration and complex thought, apathy, mood swings and hallucinations. Some people may experience only 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.

Many doctors believe that the severity of HIV-related cognitive impairment is diminishing as more people with HIV are taking medications.

For more information, see Factsheet 446, What is HIV-related cognitive impairment?, or contact Mildmay Hospital (see 'Useful organisations').

Huntington's disease

Huntington's disease is a progressive hereditary disease. It usually 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 deterioration of planning and organisational skills. People with the disease usually lack insight into their condition and other people's needs, and may be reluctant to accept help. They may also develop obsessive behaviour. 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 the Huntington's Disease Association (see 'Useful organisations').

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, 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 starts eating 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. There is thought to be considerable overlap between Korsakoff's syndrome and alcoholic dementia.

For more information, see Factsheet 438, What is Korsakoff's syndrome?, or contact the Pick's Disease Support Group (see 'Useful organisations', below).

Multiple sclerosis

Some people with multiple sclerosis (MS) may experience a loss of some of their mental abilities as their disease progresses, 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 abilities most 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 other forms of dementia, such as Alzheimer's disease. It is more usual to describe the person as 'experiencing cognitive difficulties'.

For more information contact the MS Society (see 'Useful organisations').

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 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. Its symptoms include confusion, memory problems, and difficulties in concentrating and learning. There is currently no treatment, and life expectancy varies. However, researchers have identified the affected gene and there is continuing research into this area.

For further information and support contact the Niemann-Pick Disease Group (see 'Useful organisations').

Normal pressure hydrocephalus

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

People with Parkinson's disease have a higher-than-average risk of developing dementia, although the majority remain unaffected. 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 everyday tasks. The person may become obsessive, 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.

It is not yet understood how dementia occurs in Parkinson's disease. 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).

The side-effects of certain drugs for Parkinson's may exacerbate symptoms of dementia, so adjusting medication for Parkinson's is sometimes helpful.

For further information contact the Parkinson's Disease Society (see 'Useful organisations').

Prion diseases

This is 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.

For more information see Factsheet 427, What is Creutzfeldt-Jakob disease (CJD)?, or contact the Creutzfeldt-Jakob Disease Support Network (see 'Useful organisations').

Progressive supranuclear palsy

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 of its early symptoms resemble those of Parkinson's disease.

PSP mainly occurs in people over the age of 50, although it occasionally affects younger people. One striking symptom 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 as 'having dementia'.

For further information and support contact the PSP Association (see 'Useful organisations').

Thyroid deficiency

A grossly underactive thyroid gland (hypothyroidism) can lead to the symptoms of dementia. Simple tests can detect this condition. The 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.

For more information contact Thyroid UK (see 'Useful organisations').

Vitamin deficiency

Severe vitamin B12 deficiency can cause a dementia along with weakness, unsteadiness and visual problems. It may be caused by pernicious anaemia or conditions causing very severe problems of absorption of vitamins from the bowel.

It can be detected by blood testing but nowadays in the developed world the underlying cause is usually recognised long before dementia begins.

Your local Alzheimer's Society branch will always be willing to talk to you and offer advice and information to support your needs.

For more information, Dementia Catalogue, our specialist dementia information resource, is available on the website at alzheimers.org.uk/dementiacatalogue

Useful organisations

Alzheimer's Society

Devon House
58 St Katharine's Way
London E1W 1JX
T 020 7423 3500
0845 300 0336 (helpline open 8.30am-6.30pm weekdays)
E info@alzheimers.org.uk (general information)
helpline@alzheimers.org.uk (helpline)
W alzheimers.org.uk

The UK's leading care and research charity for people with dementia and those who care for them. The helpline provides information, support, guidance and referrals to other appropriate organisations.

British Institute of Learning Disabilities

Campion House
Green Street
Kidderminster DY10 1JL
T 01562 723 010
E enquiries@bild.org.uk
W http://www.bild.org.uk/

Body that works to improve the lives of people with disabilities. Provides a range of published and online information.

CJD Support Network

PO Box 346
Market Drayton TF9 4WN
T 01630 673 993 (helpline)
E info@cjdsupport.net
W http://www.cjdsupport.net/

Supports people with prion diseases, including forms of Creutzfeldt-Jakob disease (CJD). Provides a range of information on the various forms of prion disease, and works with professionals to improve the level of care provided for people with these conditions.

Huntington's Disease Association

Neurosupport Centre
Liverpool L3 8LR
T 0151 298 3298
E info@hda.org.uk
W http://www.hda.org.uk/

Association that provides information, advice, support and useful publications for families affected by Huntington's disease in England and Wales. It can put you in touch with a regional adviser and your nearest branch or support group.

Mildmay Hospital

Hackney Road
London E2 7NA
T 020 7613 6347
W http://www.mildmay.org/

Provides holistic inpatient and day care services for people affected by HIV cognitive impairment. Referrals must be made by other hospital or community services.

MS Society

MS National Centre
372 Edgware Road
London NW2 6ND
T 0808 800 8000 (free helpline, weekdays 9am-9pm)
E helpline@mssociety.org.uk
W http://www.mssociety.org.uk/

Charity providing information and support to anyone affected by MS. Has a national helpline and a network of over 350 local branches.

Niemann-Pick Disease Group (UK)

11 Greenwood Close
The Pastures
Fatfield
Washington NE38 8LR
T 0191 415 0693.
E niemann-pick@zetnet.co.uk
W http://www.niemannpick.org.uk/

Not-for-profit organisation that supports and promotes research to find a cure or treatments for all types of Niemann-Pick Disease and provides support services to individuals and families affected by the disease.

Parkinson's Disease Society of the UK

215 Vauxhall Bridge Road
London SW1V 1EJ
T 0808 800 0303 (helpline 9.30am-5.30pmn)
E via the website (details below)
W http://www.parkinsons.org.uk/

Charity that provides information, advice and support and helpful publications for people with Parkinson's disease. It can put you in touch with your nearest branch, offering information, support and social contact for people with Parkinson's and their families.

Pick's Disease Support Group

8 Brooksby Close
Oadby
Leicester LE2 5AB
T 0845 458 3208
W http://www.pdsg.org.uk/

Supports people with fronto-temporal dementia and other forms of dementia, including alcohol-related brain impairment. Provides information and advice, publishes a range of booklets, and has a network of local contacts. Also supports professionals involved in the care of people with these forms of dementia.

PSP Association

PSP House
167 Watling Street West
Towcester NN12 6BX
T 01327 322 410
E psp@pspeur.org
W http://www.pspeur.org/

Charity that provides information and support for families afflicted by progressive supranuclear palsy.

Thyroid UK

32 Darcy Road
St Osyth
Clacton On Sea
Essex CO16 8QF
T 01255 820 407
E via website (below)
W http://www.thyroiduk.org/

Non-profit organisation campaigning for, and providing information and support to those with Thyroid or related disease.

Factsheet 442

Last updated: October 2008
Last reviewed: October 2008

Reviewed by: Professor Robert Baldwin, Consultant Psychiatrist and Professor, University of Manchester.