Mickey Berkowitz's "Impersonating Disease": What is Dementia with Lewy Bodies?
- Oren Rice
- Published in Walla Health
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Israeli basketball legend Mickey Berkowitz is battling dementia with Lewy bodies – the second most common type of degenerative dementia after Alzheimer’s. The disease combines cognitive decline with visual hallucinations, Parkinson’s-like symptoms and sharp swings between lucidity and confusion. Actor Robin Williams also had it – but it was only diagnosed at his autopsy
Exposure Mickey Berkowitz's illness (72), one of the greatest athletes in Israeli history – Dementia with Lewy bodies – brought to public awareness a disease that many in the public did not know its name until now. His wife, Shelly, said frankly to Niv Raskin on Channel 12: "Sometimes I think it's worth wishing for him to stop. As harsh as it may sound, that's my opinion. It's a damned difficult disease that doesn't respect the patient." So what is this disease, and why is it considered one of the most challenging to diagnose and treat?
Dementia with Lewy bodies is the second most common form of progressive neurodegenerative dementia, after Alzheimer's. According to the Mayo Clinic , it is caused by the accumulation of protein deposits called "Lewy bodies": clumps of a protein called alpha-synuclein that build up in nerve cells in areas of the brain responsible for thinking, memory, and movement. They are named after neurologist Friedrich Lewy, who first identified them in 1912 in Parkinson's disease patients—interestingly, in Alois Alzheimer's lab in Munich.
According to Johns Hopkins Hospital and the National Institute on Aging , the disease affects more than a million people in the United States alone. Despite its high prevalence, it is known to be the most misdiagnosed form of dementia of all—a difficulty that stems from its wide range of symptoms, which tend to mimic other illnesses entirely.
Four unique symptoms
Unlike Alzheimer's, where memory loss is often the first and most prominent sign, dementia with Lewy bodies presents a more complex and broad picture. According to the Mayo Clinic and the medical literature, the diagnosis is usually based on ongoing cognitive decline, combined with at least two of four key symptoms:
- Visual hallucinations – One of the earliest and most characteristic symptoms, in which the patient sees shapes, animals, or people that do not exist, sometimes repeatedly.
- Parkinson's disease-like symptoms – Muscle stiffness, slowness of movement, tremors, and difficulty walking and balancing.
- Cognitive volatility – Sharp and unexpected changes in the level of alertness and concentration, in which the patient may go between moments of complete clarity and moments of deep confusion, sometimes on the same day.
- REM sleep behavior disorder – in which the person “acts out” their dreams with physical movements while sleeping – a symptom that may appear years before all the others.
In addition to these four, the disease also damages the autonomic nervous system, which is responsible for regulating blood pressure, pulse, body temperature, and digestion. This damage can cause dizziness, falls, constipation, and unstable blood pressure.
“The terrorist inside my husband’s mind”
Distinguishing between dementia with Lewy bodies and Alzheimer's is not just an academic or scientific matter, but has direct therapeutic significance. According to the Mayo Clinic, about half of patients with the disease also suffer from Alzheimer's pathology, which makes accurate diagnosis even more difficult. But the essential difference lies in the response to medications: dementia with Lewy bodies patients are unusually sensitive to certain antipsychotic medications, and taking them can seriously worsen their condition. For this reason, accurate diagnosis is critical – as a mistake can actually lead to dangerous treatment.
One of the most frustrating aspects of the disease is the difficulty in diagnosing it while the patient is still alive. According to the Alzheimer's Association of America, there is no single test that can definitively diagnose it—it is a "clinical" diagnosis, meaning a professional assessment based on the combination of symptoms and the exclusion of other possible causes. The only way to confirm the diagnosis with absolute certainty is an autopsy, which allows for direct identification of Lewy bodies in brain tissue.
In recent years, there has been significant progress in the ability to diagnose the disease during the patient's lifetime, thanks to a new laboratory test called the Seed Amplification Assay. Its principle of operation is sophisticated: normal alpha-synuclein protein is added to a sample from the body, and if even a tiny bit of the defective protein is present, it "infects" the normal protein and causes it to fold incorrectly as well. Thus, within the reaction chain, the presence of a very small amount of the defective protein - the one that previously would have escaped detection - can be revealed.
Two sources of samples stand out in particular: spinal fluid (obtained by lumbar puncture) and a simple skin sample (biopsy). According to studies published last year, the accuracy of the test in identifying the disease is about 85%-87% for both of these sources – a significant achievement for a diagnosis that previously relied almost entirely on clinical impressions. The skin sample is attracting a lot of interest because of its relative simplicity: it is a small sample, much less invasive than a lumbar puncture.
Alongside the new laboratory tests, doctors are now also using a specialized brain imaging test (DaTscan), which checks the density of dopamine receptors in the brain and helps differentiate between dementia with Lewy bodies and Alzheimer's.
It is important to note: these tests are not yet considered a definitive diagnosis, but rather a valuable tool alongside clinical evaluation.
The world-famous case that illustrated this difficulty for doctors in diagnosing the disease is that of actor Robin Williams. Williams was diagnosed with Parkinson's disease during his lifetime, but it was only at his autopsy, after his death in 2014, that it was discovered that he had advanced dementia with Lewy bodies – one of the most severe cases ever recorded. His widow Susan later described how the symptoms of the disease would "pop in and out like a game of hide-and-seek," appearing and disappearing unexpectedly. She devoted a moving article in the medical journal Neurology to the family's experience, entitled "The terrorist inside my husband's brain."
Is there a treatment?
Currently, there is no cure for the disease or its progression, but some of the symptoms can be alleviated. Drug treatment focuses on several fronts simultaneously.
To treat cognitive symptoms – problems with attention, memory and alertness – drugs originally developed for Alzheimer’s disease, from the cholinesterase inhibitor family (such as rivastigmine and donepezil, known by their trade names Exelon and Aricept), are used. In dementia with Lewy bodies, they may be particularly effective, and sometimes even more so than in Alzheimer’s patients – probably because the lack of acetylcholine, one of the important chemical messengers for brain function, is particularly prominent in this disease. Another advantage of these drugs: they may also help reduce visual hallucinations, one of the core symptoms of the disease. In moderate to severe cases, another drug is sometimes added (memantine, known as Namenda).
For Parkinson's-like movement symptoms – stiffness, tremors and slowness – specific medications (from the levodopa family) can sometimes be given, but with great caution and in low doses, because they may worsen hallucinations and other mental symptoms. And to alleviate the sleep disorder, in which the patient "acts out" their dreams while sleeping, the hormone melatonin is often used.
Fatal diagnosis
Misdiagnosis poses the greatest danger to patients. Patients with dementia with Lewy bodies are unusually and dangerously sensitive to certain antipsychotic medications (neuroleptics), primarily older drugs such as haloperidol. Administration of these medications can cause a severe, sometimes life-threatening, allergic reaction. Anticholinergics, benzodiazepines, and common over-the-counter sleeping pills can also worsen a patient’s condition. For this reason, any drug treatment requires the supervision of a neurologist experienced in the disease, and accurate diagnosis is not just an academic matter – it can make a life-threatening difference.
Regarding the course of the disease: According to the US National Institute on Aging, dementia patients with Lewy bodies live an average of five to eight years from the time of diagnosis, although the range is wide and ranges from two to 20 years. The rate of progression and clinical picture vary greatly from person to person, depending on their age, general health and the severity of their symptoms.
- Families of dementia and Alzheimer's patients can get help fromAmda Association – Receive information, support and guidance