Lewy Body Dementia: Causes, Symptoms & Treatment

Lewy body dementia is a form of dementia that can occur as a separate or secondary disease. In this neurodegenerative disease, Lewy bodies appear in the brain, reducing the production of dopamine.

What is Lewy body dementia?

Lewy body dementia is named after neurologist Frederick H. Lewy, who first described the condition in a chapter of his book. The so-called Lewy bodies were first discovered in Parkinson’s disease. These are inclusions found in certain nerve cells as of the brain stem. Since 1989, it has been known that Lewy bodies can also occur in patients who do not show symptoms of Parkinson’s disease. Lewy body dementia is the second most common dementia after Alzheimer’s disease and has no cure. The disease usually begins in older age, most often Lewy body dementia occurs between the ages of 50 and 83.

Causes

As people age, some develop protein clumps in the brain that lead to deficits over time. Lewy bodies are made up of the protein alpha-synuclein; however, it is not yet clear exactly what their role is in the human body. In Lewy body dementia, clumps of this protein form in the brain cells, with inclusions mainly at the nerve endings. Since this is where signal transmission occurs, failure symptoms occur for this reason.

Symptoms, complaints, and signs

Affected individuals suffer from progressive memory impairment, with alertness and mental ability fluctuating very rapidly over the course of a day. Furthermore, patients suffer from visual hallucinations, seeing animals or people, for example. More rarely, auditory hallucinations occur, such as hearing sounds or voices. These psychotic symptoms can only be treated with great difficulty with antipsychotics, as these are extremely poorly tolerated by many patients. Often, the so-called Pisa syndrome or a very pronounced Parkinson’s syndrome then occurs. However, some patients suffer from Parkinson’s symptoms even without antipsychotic treatment. These include hand tremors at rest, muscle stiffness, small-stepped and forward-bent gait, and a reduction in facial expression movements. In addition, behavioral disturbances occur during REM sleep (dream sleep). The affected persons then live out their dreams very strongly because they lack motor inhibition. They scream and talk in their sleep, lash out and may also fall out of bed. Many patients also suffer from depression, hypotonic circulatory disorders as well as urinary incontinence. They usually fall frequently, and sufferers may also lose consciousness. The ability to cope with everyday life is also increasingly lost. Patients have great difficulty planning or carrying out actions and making decisions. The pace of work slows down and there is a reduction in the ability to concentrate and control attention. In the later stages, speech is also impaired, the patients become bedridden, and in the final stage, swallowing disorders also occur. In most cases, the patients then die of pneumonia. Mixed forms are also possible, i.e. symptoms of Alzheimer’s disease also occur. To which symptoms it comes in the course of the disease process, is individually different and depends on which brain areas are affected.

Diagnosis and course of the disease

The physician bases the diagnosis primarily on the typical symptoms that occur in the course of this disease. It is important to remember this very specific form of dementia, as many cases are often misdiagnosed as Alzheimer’s disease. Without the knowledge that psychotic symptoms have already been present for a longer period of time, it is also possible to confuse the disease with delirium. Technical examination procedures are not particularly helpful in the diagnosis of Lewy body dementia. Electroencephalograms show only nonspecific changes, and magnetic resonance imaging (MRI) and computed tomography (CT) do not reveal any characteristic findings. With the help of a dopamine transporter examination, however, Lewy body dementia can be better differentiated from other forms.In the meantime, additional focus is being placed on alternative therapies, such as cognitive or mental training. If you strengthen your body and mind, you can significantly reduce the risk of developing dementia. After diagnosis, the average duration of the disease is between six and eight years, but there are also very rapid or very slow courses.

Complications

In Lewy body dementia, affected individuals suffer from the usual symptoms of dementia. These can thereby significantly limit and reduce the quality of life of the affected person. Not infrequently, patients are then dependent on the help of other people in their daily lives, often posing a danger to themselves. In particular, memory disorders and hallucinations occur. The affected persons are unable to distinguish which events occur in reality. Similarly, patients may hear voices of other people that are not present. It is not uncommon for Lewy body dementia to also lead to circulatory disorders and incontinence. Patients also continue to suffer from depression and various behavioral disorders. It is also not uncommon for the affected person’s sleep to be disturbed and for there to be a significant reduction in the patient’s ability to concentrate. Furthermore, Lewy body dementia can impair communication and also lead to pneumonia. The treatment of Lewy body dementia can be carried out with the help of medication. However, not all symptoms can be limited, so there is not a completely positive course of the disease.

When should you see a doctor?

Disorders of memory performance should be examined more closely by a doctor even if there are minor abnormalities. If there are problems with memory recall, memory impairment or memory gaps, there is cause for concern. If no new knowledge can be acquired or if the affected person objectively reproduces false memories, a doctor is needed. A loss of the usual level of performance and problems in coping with everyday obligations should be investigated and treated. If there are trembling hands, an inner restlessness or problems with the muscles, a doctor should be consulted. Gait unsteadiness, dizziness, or increased risk of accidents should be discussed with a physician. A forward bent gait is considered a particular characteristic of Lewy body dementia. As soon as relatives notice this in the affected person, they should encourage the affected person to see a doctor. Reduced movement, restrictions in mobility or social withdrawal behavior are further warning signs that should be followed up. If incontinence, behavioral problems or circulatory problems occur, a physician is needed. If there is a depressive appearance, mood swings or changes in personality, a visit to the doctor is advisable. If the above abnormalities increase gradually over a period of several months, a doctor should be consulted immediately. Disturbances in concentration, a decrease in the usual pace of work, and problems maintaining attention should also be sought medical attention.

Treatment and therapy

As with most forms of dementia, nerve cell loss cannot be stopped. However, because the psychotic symptoms that are particularly distressing for patients occur as a result of acetylcholine deficiency, they are given cholinesterase inhibitors. These include rivastigmine or donepezil Aricept, for example. If the symptoms do not improve, the antipsychotics clozapine and quetiapine are also used. Clozapine requires special precautions, such as constant blood count monitoring. The motor symptoms of Parkinson’s disease are very difficult to treat, as patients suffering from Lewy body dementia respond extremely poorly to Parkinson’s medications, exacerbating the psychotic symptoms. Relatively well tolerated are low doses of L-dopa. Depression is treated with so-called selective serotonin reuptake inhibitors (SSRIs).

Outlook and prognosis

As the disease progresses, however, not all symptoms become apparent. As it progresses, Lewy body dementia is characterized primarily by tremendous fluctuations in the patient’s mental performance and attention. Increased visual hallucinations occur, which become more and more detailed.At the beginning of Lewy body dementia, patients can still distinguish reality from hallucinations. However, they are no longer able to do this in the advanced stage. In addition, mild symptoms of Parkinson’s disease become visible later on, which are mainly reflected in trembling of the hands, stiffened movements and unsteady gait. Lewy body dementia often leads to pronounced insomnia and hyposomnia. As a result, the sleep-wake rhythm is significantly disturbed. As the patient’s disease progresses, urinary and fecal incontinence then usually occur. The patient’s risk of falling increases decisively due to the increasing restriction of movement. Falls lead to increased disturbances of consciousness and loss of consciousness. Furthermore, fractures and other serious injuries occur more frequently, which in turn lead to further restrictions for the patient. In the later course, the weakening of the patient often increases due to such concomitant diseases. This leads to further deterioration of the immune system.

Prevention

To date, it is not possible to protect against Lewy body dementia. However, there are some factors that reduce the risk of developing this form of dementia. These include physical, mental and social activity, as well as a balanced diet rich in vitamins E, C and beta-carotene. The focus is on a diet low in cholesterol and fat. Preventive measures also include the treatment of cardiac arrhythmias, hypertension and diabetes mellitus.

Follow-up care

For those affected by dementia, aftercare consists of returning to the home environment after an inpatient stay. The challenge is particularly evident in the reliance on family caregivers, who must first settle into their new role. Aftercare therefore not only affects the patient, but also their relatives, who need to be informed and supported in order to avoid being overwhelmed. To ease the situation, a partial inpatient stay in a clinic can be useful, because here the patients are gradually released into everyday life. Through therapeutic offers, a certain autonomy can be regained, depending on the stage of dementia. It is important that those affected are not overburdened by the therapists, as this can result in a renewed outbreak of the disease. The needs of each individual must be sensitively considered. If the patient then moves fully into the home environment, it is also helpful here to receive regular visits from the doctor or to hire a professional nurse to provide support during the difficult initial period. Good daily planning plays a major role in ensuring that the patient is challenged and that there is no void in which the disease can break out. Participation in social life, taking up old hobbies and regular training of body and mind are just a few recommendations.

Here’s what you can do yourself

Creating a weekly schedule provides structure and therefore security. Here, the work to be done and important dates can be entered. Signs on cabinets indicating the respective contents help with orientation in the apartment. Items such as keys and purses can be found more easily by assigning them fixed places. A note with important numbers right next to the telephone provides more security in emergencies. A home emergency call system also provides relief. Clearly structured shopping lists with the name and the required quantity of the product are suitable for shopping. If cooking is made difficult by deficits in action planning, the use of recipes provides relief. Since dementia affects the ability to react, among other things, driving should be avoided. Instead, carpools can be formed, public transportation used or relatives involved. If the affected person was active in sports, it is recommended to obtain information about group offers. Maintaining familiar interests and routines for as long as possible has a positive effect on both body and mind. In advanced cases, it is advisable to draw up a health care proxy. In the event that the person concerned is no longer able to do so, a trusted person is authorized to make decisions in medical matters, to settle financial matters or to sign contracts.