IS 18 March 2002 Page 1 Dementia with Lewy bodies This information sheet is for carers of It is only in the last 10 to 15 years that people with dementia with Lewy bodies dementia with Lewy bodies (DLB) has (DLB). Some people with early DLB been recognised, as the result of researchInformation Sheet may also find it useful. If you require by psychiatrists and pathologists. This further information about other issues does not mean that it did not exist regarding dementia call the freephone before. People with the condition would Dementia Helpline on 0808 808 3000, or have been said to have a vascular see our website at www.alzscot.org. dementia or Alzheimer’s disease. This DLB may also be known as Lewy body misdiagnosis still occurs, partly because dementia, diffuse Lewy body disease, not all doctors are yet aware of the dementia of Lewy body type or Lewy condition, but also because it is not body variant of Alzheimer’s disease. always easy to distinguish between these three common types of dementia. This information sheet includes both medical information about the diagnosis Dementia with Lewy bodies is thought and treatment of DLB, and practical to be the second or third most common information for carers, including some cause of dementia, accounting for 15% of the experiences of individual carers. to 20% of cases of dementia which start after the age of 65.1 This means that What is dementia with Lewy there could be about 11,000 people with bodies? DLB in Scotland. Dementia is the general term for a gradual progressive decline in a person’s People who have DLB have microscopic memory and other mental abilities. changes called Lewy bodies2 in the There are many different illnesses which nerve cells of the brain. These were first can cause dementia, but only a few described by Frederich Lewy in 1912. common ones. The best known are He looked at the brains of people with Alzheimer’s disease and vascular Parkinson’s disease, where Lewy bodies dementia. are mostly in one tiny area of the brain called the substantia nigra. In people What is dementia with Lewy with DLB they are much more widely bodies? 1 spread throughout the brain. Symptoms 2 It is not yet clear what the Lewy bodies Diagnosis and tests 3 signify, but it is likely that they are a Course of the illness 3 1 Caring for someone with dlb 4 Campbell S. Stephens S and Ballard C (2001) Dementia with Lewy Bodies: clinical features Treatment and drugs 5 and treatment. Drugs Ageing; 18(6): 397-407 What is the cause? 6 2 Lewy bodies are round deposits within Useful websites 7 damaged nerve cells, which are probably formed as the cells try to protect themselves and they are Acknowledgements 7 distributed in varying degrees throughout all areas of the brain.
IS 18 Page 2 sign of damage to the nerve cells. They people have no insight and may develop contain various proteins, some of which delusional beliefs about the have been identified. When it is clear hallucinations. whether these substances are damaging to the nerve cells or are the result of the Neurological symptoms damage, and how these processes People with DLB quite often have vague happen, we are likely to know a lot neurological problems which can about how Parkinson’s disease and DLB include symptoms that are like those of develop, and therefore what treatments Parkinson’s disease, but which may not could prevent or treat these conditions respond well to the usual treatment effectively. given to people with Parkinson’s disease, the drug L-Dopa. These Symptoms symptoms are often in just one part of Dementia with Lewy bodies shares the the body, and may include: common features of all types of • rigidity and stiffness dementia. There is a gradual loss of • difficulty starting movements mental abilities, including orientation (known as ‘bradykinesia’) and memory, reasoning and intelligence, • slowness of movement with a decline in ability to carry out • tremor. daily tasks. However there are certain specific characteristics of the decline This pattern is different from other kinds which are much more likely in DLB, and of dementia. Neurological problems are less common in Alzheimer’s disease or rare in the early stages of Alzheimer’s vascular dementia. disease, although people in the very late stages develop problems with mobility Patchy mental impairment and other neurological problems. People The person’s memory impairment and with vascular dementia may have some disorientation may be patchy, with some of the neurological problems associated quite normal memory function. He or with having small strokes. she may communicate clearly, but may have difficulties with what are called the Other symptoms which people with ‘executive functions’: problem solving, DLB may get include vague weaknesses planning and the like. However, the of arms or legs, clumsiness or falls. mental decline in some people with DLB These symptoms may come and go. may be just like it is in someone with Alzheimer’s disease. Variability The person’s degree of confusion can Hallucinations vary from moment to moment or day to Seeing or hearing things which are not day. He or she may have episodes of there is not very common in Alzheimer’s quite sudden severe confusion, whichInformation Sheet disease, but 90% of people with DLB then pass. The person’s insight is also have hallucinatory experiences. Usually likely to vary: at times he or she may these will be visual hallucinations such realise that things are not right, that the as people, animals or inanimate objects. hallucinations are imaginary, etc, but at Occasionally the hallucinations are other times he or she may have no auditory – of music or voices. Most apparent understanding of his or her people with DLB find their illness. hallucinations are puzzling, rather than upsetting or frightening. Unlike Sleep disturbance hallucinations in many mental illnesses, People with DLB may be wakeful at the person may have quite a lot of night, sometimes not sleeping at all for insight, realising at times that the night after night. The person may not visitors are imaginary. However some realise this; but it can of course be very
IS 18 Page 3 distressing for carers. Some people with of DLB and will therefore not pick up on DLB seem to experience an unusual the diagnosis quickly. Ask to be referred condition in which they lose the normal to a specialist in order to obtain an paralysis that we normally have when accurate diagnosis. we sleep. This means that they may be physically active, or act out dreams in To diagnose DLB, a specialist (usually the night. There has been a suggestion an old age psychiatrist) will take a that particular drugs (clonzepam and precise history of the pattern of the desipramine) can help this. person’s symptoms. This is so that he or she can be sure first of all that the person Neuroleptic sensitivity has dementia, as there are other reasons At least 50% of people with DLB are why a person may experience symptoms over-sensitive to the side-effects of such as visual hallucinations (for neuroleptic drugs (a type of drug used example, people who are in delirium, the for illnesses such as schizophrenia and confusion that is caused by a physical also used to treat hallucinations and illness). The specialist will then exclude delusions or as tranquillizers)3. Even a other possible diagnoses, including tiny dose may cause the person to vascular dementia and Alzheimer’s become sedated, unsteady or develop disease. symptoms like those of Parkinson’s disease mentioned above. For this reason A CT or CAT scan4, or magnetic it is most important that there is resonance imaging (MRI)5 may reveal someone else there if any of these drugs degeneration, or shrinkage, of the brain. are given to anyone with early dementia. A SPECT scan 6, may be able to show If signs of neuroleptic sensitivity up some of the changes that are develop, the drug should not be given happening, but this is still being again. investigated. However the Lewy bodies themselves can only be seen by looking Diagnosis and tests at brain tissue after death. One problem with DLB is getting a correct early diagnosis, as this dementia Early accurate diagnosis is particularly can often be mistaken for Parkinson’s important with DLB because of the risks disease, depression or another dementia. caused by over-sensitivity to neuroleptic Often relatives and the person with DLB drugs and because other drug treatments do not expect a connection between may help. hallucinations and dementia and will therefore not mention this to the GP. Course of the illness Many GPs will not recognise early signs Unlike Alzheimer’s disease, which usually begins very gradually, DLB often starts quite rapidly or acutely, withInformation Sheet 3 Neuroleptics were originally developed to treat schizophrenia and other major mental illnesses. quite a fast decline in the first few They have been found to be sometimes useful months, although later there may be for other conditions where hallucinations or delusions occur and have also been used as 4 CAT or CT scans are like x-rays but more general tranquillisers, to calm disturbed detailed and allow the specialist to see inside the behaviour in dementia and other conditions. brain. Examples are chlorpromazine (Largactil), 5 risperidone (Risperdal), olanzapine (Zyprexa), MRI uses radio waves and a strong magnetic haloperidol, trifluoperazine (Stelazine) and field to give a clear and detailed picture of the pericyazine (Neulactil). Thioridazine (Melleril) brain. used to be given very commonly to people with 6 dementia, but has now largely been withdrawn SPECT scans look at the pattern of blood flow from use. in the brain.
IS 18 Page 4 some levelling off. DLB tends to Insight progress faster than Alzheimer’s disease Some of the most distressing times for and can last from 5 – 7 years, although carers can be when the person they care this will vary from person to person. for understands what is happening to them. One carer found it particularly Caring for someone with DLB distressing, when her husband was in a care home, on days when he had insight Information and support into his condition. He would ask to come If you need help with a particular home with her, as he did not want to stay problem, specific information or access in the home anymore. For this carer this to a service it is vital that you ask for it. seemed worse than when he was Speak to your GP or social worker or unaware of the changes taking place in Alzheimer Scotland – Action on their lives. Seek support from family, Dementia for information and advice. other carers and professionals to help Contact a local carers group to find out you cope with the distress the fluctuating about other people who are caring or insight of LBD can cause. have cared for someone with DLB, as they will often be a vital source of Sleeping information and support. Some people with DLB are sleepless or physically active in their sleep. (See Very few people know what DLB is, and Sleep disturbance on page 2.) One carer carers often find that there is little reported problems when her husband support from other people, as they do entered very deep sleeping phases and not understand what they are going she could not wake him. This type of through. As a result carers can feel very deep sleep can seem like a stroke. If the isolated. Being able to talk to someone person has any problems related to who understands is very important. sleep, always contact the GP to ensure Alzheimer Scotland’s Dementia there are no other problems and for Helpline, on freephone 0808 808 3000 treatment and advice. is open 24 hours a day for information and emotional support, and can provide Vision a free information pack and help you Some people with LBD experience find services and support near you; or problems with vision, with some people see the website at www.alzscot.org. suffering from double vision. This can cause problems with many activities Dealing with hallucinations and delusions including eating, as the person can no Hallucinations and delusions are often longer see the food clearly to pick it up. the most difficult symptoms to deal Make sure the food is on a plain plate with. They can cause a great deal of and that the table or tablecloth is a emotional strain for carers. Some people different colour from the plate, as thisInformation Sheet with DLB will know they are helps the person to distinguish between experiencing hallucinations, while others the food, plate and table. will not be able to distinguish between reality and what is happening in their Dealing with dribbling hallucinations and delusions. Being able Dribbling can cause embarrassment and to deal with these symptoms is important may prevent the person wanting to go if the carer is to cope effectively. Ask out. Some carers find it hard to deal with for advice and support from the person’s this problem and may feel they cannot specialist or GP, and seek support for take the person out. This is a symptom yourself, for example through a local which people with Parkinson’s disease dementia carer support group. often have. There are drugs which can be prescribed by the person’s GP which help dry up the saliva. However, some
IS 18 Page 5 carers have reported that these drugs can Acetylcholinesterase inhibitor or cause additional constipation problems cognitive enhancer drugs because they are dehydrating. Acetylcholinesterase (AChE) inhibitor drugs are also known as cognitive Other strategies to help enhancers. They include donepezil There are strategies that can help, (Aricept), rivastigmine (Exelon) and especially in the early stages of DLB. galantamine (Reminyl). These drugs These include keeping a set routine, are considered by many psychiatrists as providing written or ‘alarm call’ the first line of therapy in DLB. reminders and providing reassurance. People with DLB will often have times Donepezil and rivastigmine studies have when they have a lot of insight into their shown that AChE inhibitors may be a condition and will need a great deal of safe and effective alternative to support. Speech and language therapists neuroleptic/anti-psychotic treatment. can help people who are having One study showed that people with DLB difficulty in finding the right words or in given donepezil (Aricept) showed an following conversations. Closely improvement in understanding, monitoring the person’s condition and behaviour and psychiatric symptoms, the progression of the dementia will help although it is important to recognise that provide vital information for his or her they are not suitable for everybody and doctors. will not help everyone who tries them.7 However, there is a problem with Services prescribing these drugs for DLB, as they There are many services that may help are only licensed in the UK for the the person with DLB and his or her carer treatment of mild to moderate to cope more easily. The person with Alzheimer’s disease. Speak to the LBD is entitled to a community care specialist treating the person with DLB assessment from the social work to see if one of these drugs might be department to work out what services suitable and if he or she is able to might help. The carer is also entitled to a prescribe it. 8 carer’s assessment. These drugs prevent the breakdown of Contact your local social work acetylcholine, a chemical which carries department to ask for assessments. The messages between brain cells. This Dementia Helpline (0808 808 3000) can means that there is more acetylcholine explain about assessments and services available to transmit messages between and can put you in touch with your local nerve cells. This may temporarily Alzheimer Scotland – Action on improve or stabilise the symptoms of the Dementia project for support. disease.Information Sheet Treatment and drugs There is no cure yet for DLB. Research continues to search for a specific course 7 Samuel W et al (2000) Better cognitive and of therapy. The present form of psychopathologic response to donepezil in treatment usually focuses on treating the patients respectively diagnosed as dementia with symptoms. This means using drugs to Lewy bodies: A preliminary study. International control symptoms such as Parkinson Journal of Geriatric Psychiatry 15, 794-802 type symptoms and hallucinations and 8 delusions. A group of drugs developed Gurling K, Kelly, J, Ramsay C, Rodriguez C. for treating Alzheimer’s disease (2001) Dementia with Lewy Bodies and (‘acetylcholinesterase inhibitors’ or Dementia in Parkinson’s Disease: Special ‘cognitive enhancers’) may also help considerations. In Medication and Dementia. Dementia Services Development Centre people with DLB.
IS 18 Page 6 The effect of these drugs varies from give specific help to the symptoms of person to person. Some will not notice DLB. any effect, while others may find their condition improves slightly or they stay If the person has severe sleep the same when they would have disturbance the doctor will wish to try a expected to deteriorate. People with hypnotic drug, but these are sometimes DLB may see an improvement in ineffective. You may need to suggest behavioural symptoms such as apathy, trying clonazapam and desipramine to hallucinations and delusions. Trials the doctor. indicate that these drugs may delay the progression of the symptoms for up to There has been no research into any nine months on average. Drugs should ‘alternative’ therapies for DLB. not be stopped after nine months as some people may benefit for longer than Problems with drug treatments this.9 Anti-parkinson drugs such as L-dopa (levodopa) and Sinemet (a combination Atypical anti-psychotic drugs of levodopa and carbidopa) which may If treatment with AChE inhibitor drugs help reduce the tremor and loss of fails, you can discuss with the doctor the muscle movement may make the use of atypical neuroleptic/anti- person’s hallucinations and delusions psychotic drugs. Despite what was said worse. above about neuroleptic drug sensitivity (see Neuroleptic sensitivity on page 3) Similarly, neuroleptic/anti-psychotic some people with DLB are able to drugs prescribed for hallucinations may tolerate treatment so that their in fact make the muscle movement hallucinations are reduced. The utmost symptoms worse. Sometimes there is caution is needed. The drugs need to be poor recovery of the muscle movement started in the very lowest doses and the even after stopping the medication. In patient should be monitored regularly in the worst cases, a patient treated with the first days of treatment or after any these drugs could become catatonic, lose change in dose. cognitive function and/or develop more muscle rigidity, which could threaten Early reports on some of the more their life. Some commonly used drugs recently developed ‘atypical’ anti- which should be used with great caution, psychotic drugs have suggested that if at all, for people with DLB are olanzapine (Zyprexa), quetiapine chlorpromazine, haloperidol, or (Seroquel) or risperidone (Risperdal), thioridazine. may be safe to use. What is the cause? Other treatments The cause of DLB is uncertain. ThereInformation Sheet If the person cannot tolerate neuroleptic are certain similarities with Alzheimer’s drugs, and the AChE inhibitor drugs are disease and Parkinson’s disease. Genetic ineffective, but hallucinations or other studies are trying to discover which symptoms are distressing, the doctor will genes may contribute to the usually try other tranquilliser drugs, but development of DLB, but these are still there is no particular evidence that these at an early stage. Just as the amyloid protein may play a central role in Alzheimer’s disease there may be a 9 Australian Alzheimer Association. Feb. 2001 protein or proteins which are central to Website information on drugs. the nerve cell damage in DLB. One www.alzvic.asn.au candidate is alpha-synuclein, which is found within the Lewy bodies themselves.
IS 18 Page 7 Useful websites Alzheimer Scotland – Action on Dementia: www.alzscot.org - over 500 pages about a wide range of issues relating to dementia, including caring, support, treatment, research, campaigns, training, volunteering and local services. Lewynet: www.nottingham.ac.uk/pathology/lewy/l ewyhome.html - mainly medical information on DLB, plus links to other organisations’ websites. Acknowledgements Grateful thanks to carers Kathleen Balchin and Ann Little and to Jenny Henderson and Alan Jacques, who all generously gave their time and experience to help with the development of this information sheet. This information sheet was produced as part of the Dementia Carers Project, funded by the Scottish Executive under the Carers’ Strategy for Scotland.Information Sheet Alzheimer Scotland - Action on Dementia National Office 22 Drumsheugh Gardens Edinburgh EH3 7RN Tel: 0131 243 1453 Fax: 0131 243 1450 E-mail: email@example.com Alzheimer Scotland - Action on Dementia is a company limited by guarantee and is recognised as a charity by the Inland Revenue. Registered in Scotland No. 149069. Scottish Charity No. SC022315. Find us on the internet at www.alzscot.org