For every 100 people who have dementia, about 10-15 will have dementia with Lewy bodies. This means that around 100,000 people in the UK have this type of dementia.
What are the characteristics of Lewy body disease?
Lewy body disease (LBD), sometimes referred to as "dementia with Lewy bodies (DLB)," is the second most common neurocognitive dementia after Alzheimer's disease.
It is characterized by abnormal deposits of a protein called alpha-synuclein inside brain cells. Lewy bodies were named after Dr. Friederich Heinrich Lewy, who first described these structures found in the brains of Parkinson's disease patients at the time of their death.
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These are neuronal inclusions made up of aggregates of filaments caused by abnormal accumulation of the alpha-synuclein protein. These deposits disrupt brain signals, affecting cognitive functions and leading to progressive intellectual impairment and typically severe dependency.
This phenomenon is also characteristic of Parkinson's disease. However, in Parkinson's, Lewy bodies remain localized in the brainstem cells responsible for motor control. In the case of LBD, Lewy bodies are also found in the outer layer of the brain, the cortex, responsible for mental functions. Cortical Lewy bodies are a major pathological feature of Lewy body disease.
"The term 'related to Alzheimer's' or 'Parkinson's-like' is obsolete and should be replaced by the correct designation once the disease is identified," Mr. de Linares emphasizes.
As a caregiver for many years to a partner with LBD, the Association's president often had to fight against this mistaken assumption. During this painful experience, he refused to accept the doctors' diagnosis of Parkinson's disease, firmly believing that the symptoms he had learned to manage belonged to a distinct disorder.
Currently, apart from specific risk factors common to several neurodegenerative diseases, there is no known cause for the characteristic features of Lewy body disease.
What are the symptoms of Lewy body disease?
First and foremost, the patient must have cognitive impairments: attention deficits, orientation problems, difficulties with verbalization, and performing specific simple tasks. Other symptoms can vary significantly from one patient to another, making diagnosis even more challenging. However, there are four main categories of symptoms known as the cardinal symptoms of Lewy body disease.
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Severe fluctuations in cognitive impairments are a highly indicative sign of Lewy body disease.
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Parkinsonian motor symptoms.
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Hallucinations, typically visual.
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Behavior problems during rapid eye movement (REM) sleep. This is a unique form of sleepwalking, which is very common in Lewy body disease.
When a patient presents cognitive impairments along with two of these symptoms, a diagnosis of Lewy body disease can be made with a high degree of certainty.
The clinical diagnostic criteria known as "McKeith criteria" provide clear guidelines in this regard. The challenge in diagnosis mainly lies in the variability of symptoms from one patient to another and often from one period to another in the same patient. It is indeed a disease that must be approached on a case-by-case basis, with careful attention and deep investigation when establishing a diagnosis, to prevent a premature conclusion of Alzheimer's or Parkinson's disease, despite other signs that should attract the physician's attention.
Currently, we can make a precise diagnosis of Lewy body disease, and it is imperative for the well-being of patients and family caregivers to break away from the outdated misconception that still tends to associate it with Parkinson's and Alzheimer's diseases.
Are there treatments for Lewy body disease?
Currently, there are no treatments that can cure this neurodegenerative disease. Treatments for Lewy body disease are primarily symptomatic. They focus on managing symptoms, especially hallucinations, Parkinsonian syndromes, and cognitive deficits.
Why is it imperative to make a correct diagnosis of Lewy body disease?
Some symptoms resembling Alzheimer's and Parkinson's diseases make its diagnosis more complex. "It is imperative to make the distinction," explains Mr. de Linares. An incorrect diagnosis can have consequences for the patient's care and treatment, including the prescription of medications like certain neuroleptics sometimes recommended for Alzheimer's but extremely dangerous for Lewy body disease patients.
A clear diagnosis also guides scientific and medical research on Lewy body disease and increases awareness in nursing homes, among doctors and healthcare professionals, caregivers, and the general public, for better care and tailored support for these individuals. "Research in this area is still lacking.
There are many avenues to explore:
Why do Lewy bodies suddenly spread in the brain and cause all these problems? Why, unlike Parkinson's patients, where the spread remains localized in the brainstem, do Lewy body disease patients experience a much broader distribution, especially in the cortex?
And many other avenues to explore. There is significant research to be done," emphasizes the Association's president.
Furthermore, it is essential to understand why some treatments for Alzheimer's symptoms can be highly effective for Lewy body disease, such as Rivastigmine and Donepezil, while others may be ineffective, dangerous, or even fatal, like first-generation neuroleptics.
Due to a lack of information and awareness campaigns, emergency room doctors or caregivers in facilities where Lewy body disease is not well-known might tend to administer a neuroleptic to a patient with significant behavioral problems. This can have dramatic consequences. "Research genuinely needs to understand this disease, its causes, and appropriate treatments," continued Mr. de Linares during our interview.
Currently, there is a reference center in England for rare dementias but not for Lewy body disease, which is far from rare. This seems absurd to the Association's president and the professors on its scientific committee. Research is in progress in The United Kingdom.
During an exclusive interview for the "Journal du Parlement," Professor Blanc shares the state of his research: "Our work at the ICube Laboratory and the Memory Resource and Research Center in Strasbourg, France is diverse. It provides a unique, transversal approach to LBD, focusing on several priorities... The first is to precisely describe the prodromal stage, that is, before dementia sets in, in the medical sense of the term – the period preceding the dramatic consequences that take over the patient's daily life when they can no longer use the phone, sort papers, or drive. The disease has been established for 15 or 20 years by then.
Thus, the challenge is to identify it earlier. This is especially critical because it leads to questions about early treatment. If we can treat early, we can halt or even destabilize the disease to prevent it.
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