Lewy body dementia – signs, symptoms and treatment
For years, Des O’Sullivan struggled to pinpoint what felt wrong. When his symptoms started, Des was in his early 50s, a busy publishing consultant whose career rested on meeting budgets and deadlines. “At work, things I found straightforward became difficult. I was still good with words, but my maths had gone to hell. I couldn’t put my finger on precisely what was wrong.”
Des, now 67, had begun to shout and scream while he slept, he was hitting his wife Valli who lay beside him, he was hitting the bed. His GP referred him to a sleep clinic.
More referrals followed, more tests and scans. In November 2016, aged 60, Des finally had an answer – a formal diagnosis of dementia with Lewy bodies (DLB). “Until then, I had the common view of dementia, that it starts with memory loss,” says Des. “Lewy body meant nothing to me.”
This is probably true for most of us. So how much do we know about the disease?
What is dementia with Lewy bodies?
Dementia with Lewy bodies is a disease where a number of things occur in the brain: there’s a loss of brain cells and connections between brain cells – known as synapses – and this is probably what drives the symptoms,” says Dr Daniel Erskine, a senior research fellow with Alzheimer’s Research UK at Newcastle University.
“There’s also an accumulation of a protein called alpha-synuclein which stick together to form large, spherical clumps known as Lewy bodies, named after the German doctor who identified them – and that’s what gives the disease its name.” These deposits affect chemicals in the brain which can lead to problems with thinking, movement and behaviour.
Unlike Alzheimer’s though, where there’s a widespread loss across pretty much everywhere, with DLB, it’s specifically in the regions that regulate the activity of the whole brain.
How common is dementia with Lewy bodies?
Pathological data collected from Brains for Dementia Research suggest it’s the second most common form of dementia – accounting for up to 20 per cent of all dementia cases – but clinical diagnosis rates are much lower at 5 per cent.
This year saw the first ‘World Lewy Body Day’ in an effort to spotlight what’s often dubbed the “most common disease we’ve never heard of”. It is often misdiagnosed as Alzheimer’s, Parkinson’s disease or even mental illnesses like schizophrenia or depression, partly because it’s only a fairly recently recognised condition.
What is the cause?
“It used to be thought that the Lewy bodies were causing the death of the brain cells, but now we think they might be a symptom,” says Dr Erskine. “It’s likely that whatever is doing the damage is too small to be seen. Some people think that the protein alpha-synuclein itself sticks together but in much smaller accumulations than Lewy bodies and they cause the damage.”
Another school of thought is that it’s a dysfunction of mitochondria, the parts of the brain cells that produce energy. The regions of the brain that are more vulnerable to this disease are very active cells which need lots of energy so that sort of makes sense.”
“The key point is that it’s not clear,” Dr Erskine adds. “It’s probably a lot of things combined rather than a simple explanation.
Who is at risk?
The most common risk factors are age and sex. “Most people who develop it do so after the age of 65,” says Dr Erskine.
It’s also more commonly diagnosed in men than women (in contrast to Alzheimer’s disease which is more common in women). “In postmortem studies from our brain bank, it would be as high as 70 per cent men,” says Dr Erskine.
There is a genetic component – as there is for almost every disease – but most cases are not inherited. It’s rare for more than one family member to have it.
What are the symptoms?
The main symptoms, according to Rimona Weil, a professor of neurology at the UCL Dementia Research Centre, may include:
Hallucinations: Seeing or hearing things that aren’t actually there. “They are nearly always visual and silent, often of people or animals, and they often occur at certain times of day or in certain places, like the bedrooms.”
Cognitive fluctuations: The ability to pay attention and be alert fluctuates considerably, with periods of being clear and at other times more confused. People can stare into space for a few minutes, and then revert back.
REM sleep disorder: Where people act out their dreams while asleep. They might shout out, laugh or move their arms or legs.
Making mistakes with spatial awareness: For example, bumping into furniture, having a driving accident or difficulty pouring water in a glass.
A change in movement: Similar to the changes seen in Parkinson’s – a slowness, rigidity and sometimes tremor.
Difficulty in planning and making decisions: For instance, organising the home can become an increasing struggle.
Autonomic symptoms: “Where people can have problems with waterworks, bowel function, blood pressure.”
Psychiatric symptoms: “Depression and anxiety are quite common, but [these patients] can also respond to treatment, so they are worth discussing with your healthcare professional.”
What is the difference between dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD)?
Both DLB and PDD fall under the umbrella term of ‘Lewy body dementia’ and both are characterised by the presences of Lewy bodies in the brain.
In cases of PDD, someone with established Parkinson’s disease has then gone on to develop dementia. With DLB, it’s the other way around – the dementia symptoms present first and might (but might not) be followed by Parkinson’s symptoms.
“There are ideas that the pathologies in the brain may start in different areas,” explains Dr Erskine. “It could be that, for DLB, the damage starts in the higher centres of the brain which are used for thinking and spreads down to the lower parts, reaching the brainstem which controls balance, coordination and reflexes. With PDD, it would start in the brainstem and spread to higher areas.”
How does the disease progress?
This is a progressive disease, but people vary in the symptoms that affect them most, even at the later phases.
“There are no official stages and it is very variable between individuals,” says Prof Weil, who leads a weekly clinic in Lewy body dementia. “Patients in my clinic often ask me what the stages will be. One person even said they wished I had a road map of the condition.”
In some, there can be slowing in thought, a withdrawal, in others, the movement symptoms can be more troublesome leading to frailty and falls.
“But it is quite different to Alzheimer’s,” says Prof Weil. “Loss of memory is not so prominent and language is retained really well. You don’t have the loss of the self or inability to recognise people around you that you see in Alzheimer’s, even when DLB is more advanced.”
The average survival time
After diagnosis, it is five to seven years but this is highly variable. One study from Cambridge found survival times after first presenting with symptoms are markedly shorter in DLB compared with Alzheimer’s Disease. Research in the United States found the most common cause of death is “failure to thrive”, followed by pneumonia and swallowing abilities.
What are the treatments?
“Treating it requires a holistic approach as it impacts all body systems,” says Prof Weil.
Cholinesterase inhibitors – for example, rivastigmine or donepezil – can help improve attention and thinking by boosting levels of the neurotransmitter acetylcholine in the brain, allowing more communication between nerve cells. These were developed for people with Alzheimer’s but actually work better in people with DLB.
“About a third of people do well on it, some don’t notice much difference,” says Prof Weil. They can also be used to manage hallucinations, while Parkinson’s medication such as levodopa is often given to people with DLB to treat movement symptoms – although it can sometimes exacerbate confusion and worsen hallucinations.
Antidepressants and anxiety medication, addressing factors that disturb sleep, and making sure that family members are well-supported also form part of an overall treatment plan. Research has shown that caregivers carry a significantly higher burden when compared with caregivers for Alzheimer’s disease.
Although we don’t have any disease modifying treatments, DLB is an area of active research and benefits from scientific discoveries in the Alzheimer’s and the Parkinson’s worlds.
New treatments are being developed for both of these and might have application for DLB in the future. One example is neflamapimod, a drug first trialled for Alzheimer’s which failed, but is now being trialled in DLB and showing more potential.
Taking control with exercise, brain games and being sociable
Taking control as much as possible can make a difference. “Patients who do physical exercise do much better,” says Prof Weil. “There’s not enough research to understand why this is, but clinicians really notice the difference.
The recommendation for Parkinson’s is half an hour five times a week, and that is a good aim for DLB as well. Staying socially engaged is also really important. People ask me a lot about brain games and I’m not against them, but going out and connecting with friends, attending community events makes more of a difference.”
For Des, staying active and involved has been key. Although he took early retirement in 2017, he has stayed busy. He takes regular exercise, which includes yoga and walking his dog twice a day, and is very involved with the dementia community, attending meetings, talks, developing a checklist of early symptoms and giving keynote speeches. He and his wife Valli are the subject of a new film by Alzheimer’s Research UK, which attempts to raise awareness around DLB and show what it’s like to live with.
“You’ve got two options,” he says. “You can wait to die or live well with what you’ve got. That’s what I’ve chosen to do.”
If you or a relative have DLB or PDD and would like to take part in one of Professor Weil’s observational studies, contact her research assistant, Ivelina Dobreva, at i.dobreva@nhs.net
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