Lewy Body Dementia Preparing For End Stage End Of Life Care

Lewy Body Dementia
Lewy Body Dementia

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Dated May 7, 2020

This webinar was recorded and can be viewed for free on YouTube here.

Source Stanford PD Community Blog

The presentation began with an acknowledgment from Dr. Katz about the presentation: these are difficult topics that may be sad or challenging to think about.

Still, it is important to have this kind of information to help you plan and prepare for the future. Topics included what caregiving is like in advanced LBD, end-of-life care, indicators of the individual’s prognosis, and advance care planning.

Within the palliative care field, there is a concept called “total pain,” which posits that the physical pain experienced by the sick individual is only part of all the suffering caused by the disease.

The illness impacts the entire family, including the caregiver, and all family members may experience suffering in the physical, psychological, social, and spiritual realms. Unfortunately, physicians rarely bring up end-of-life issues, and less than half of caregivers say they felt prepared for what was to come.

Caregiving in advanced LBD is very challenging and often causes grief, guilt, and existential distress. 

Anticipatory grief, a feeling of mourning for an expected future that has been lost, is common. Most caregivers at this stage of illness never feel that they are doing a good enough job of caregiving, even though they are.

This feeling of guilt is common and is a normal part of the situation that should be acknowledged. Finding support from a friend circle, support group, faith community, or family is essential. Spiritual care or counseling can be very helpful for the caregiver, family, and person with LBD during this time, to help find perspective.

Resilience isn’t only about enduring; it’s about how you are able to recharge and bounce back. It is not about becoming stoic and being able to endure an increasingly burdened situation; rather, it is about your ability to take a break and recover from a challenging situation.

Caregiver burnout is real: it affects health outcomes and leads to higher mortality in caregivers. Burnout is a healthy, adaptive response – an alarm to notify us that something needs to change before this gets worse. It’s important to notice the alarm is ringing, often by practicing mindfulness and self-care.

What does self-care look like?

  • Monitor yourself for signs of caregiver burnout: feelings of exhaustion, irritation, or frustration.
  • Promote your own well-being and seek balance.
  • Think of it as putting on your own “oxygen mask” on before you help others.
  • Build resilience in yourself.
  • Strengthen meaning and purpose in life: remember why you have chosen to care for this person.
  • Connect with yourself and with others: “connection is protection.”
  • Broaden your understanding of your options and choices (self-efficacy): remember that you do have a choice in how you handle situations, even if they are quite difficult.

Palliative care focuses on improving the quality of life of patients and their families facing serious illness. It seeks to affirm life and provide relief from pain, while offering a support system to the family and the patient. While palliative care services can be particularly essential later in the disease, increasingly it is recognized as having great value earlier in the course of the illness as well. In some studies, palliative care not only improved the quality of life, but also the length of life.

Motor symptoms

Increased slowness and stiffness can cause discomfort and pain and can lead to contractures. Contractures are stiffness or constriction in the muscles, tendons, or ligaments, which prevent the extremity from moving and can cause severe pain. Falls often increase due to the loss of balance and impulsivity. Supervision around-the-clock becomes necessary. Patients become wheelchair-dependent, and, eventually, many will become bedridden.

There are a number of strategies and techniques to manage these issues:

  • Range of motion exercises (independent or assisted) and massage to help prevent contractures
  • Skin care to ensure skin is clean and dry
  • Change positioning every 2 hours to prevent formation of pressure ulcers
  • Use a gait belt to help walk and transfer safely
  • Mechanical lifts may be needed eventually
  • Use specialized cushions or mattress to prevent pressure ulcers
  • Broda specialized positioning and mobility chairs

Dr. Katz mentioned a 2019 study in the New England Journal of Medicine entitled “Rehabbed to Death,” which found that for a minority of older adults, sometimes a stay in rehab facility after a hospitalization is actually a gateway into a cycle that sends the individual from the rehab facility back to the hospital, then back to rehab, in their final months of life. The rehab facility in this case is not really able to “rehabilitate” or improve the person’s health or quality of life; more accurately, this should be called “post-acute” care. This can be in part due to payment structures within Medicare and Medicaid that perpetuate the cycle, and it does not generally represent a good quality of life for someone in their final months or weeks.

If your loved one with LBD is hospitalized and you are recommended to send them to sub-acute rehab, it is more of a post-acute care convalescent home rather than a rehab facility. It is something to be aware of.

Communication

In advanced LBD, communication often becomes quite difficult. Voice changes, poor attention, confusion, and word-finding problems are common; impaired communication can also lead to anxiety or agitation.

Some basic tips:

  • Say “yes, and…” – do not argue, even if they are hallucinating or confused.
  • Speak clearly in a calm voice. Tone is often more important than content. Be aware of your tone and facial expressions.
  • Ask “yes” or “no” questions rather than open-ended questions.
  • Maintain eye contact.
  • Offer a reassuring response to frequently asked questions.
  • Find a place and time to talk without distractions.
  • Refer to people by their names instead of pronouns like he or she, to avoid confusion.
  • Talk about one thing at a time.
  • Have patience.
  • When giving instructions, use simple sentences – one piece of information at a time.
  • Recognize what you’re up against.
  • Understand that there will be good days and bad days.

Psychiatric symptoms

Those with advanced LBD often experience hallucinations that may be severe and frightening. Make their neurologist and psychiatrist aware if this is happening.

Behavioral treatments include figuring out or anticipating some possible triggers of the hallucinations or agitation. Cover reflective surfaces, as mirror images are often disturbing to those with advanced LBD. Use a calm tone of voice and avoid confrontation.

At the end of life, hallucinations and psychosis are often so severe that they need medication treatments to help control the symptoms.

The “Five R’s” from the Family Caregiver Handbook:

  • Remain calm.
  • Respond to the person’s feelings.
  • Reassure the person.
  • Remove yourself.
  • Return when you are calm.

Eating and taking medications

Most people will need help with feeding once the disease is advanced. Keep the patient as involved and independent as they can be; finger foods can sometimes allow them to feed themselves for longer than a fork and knife. Using a plate in a contrasting color can help your loved one to be able to distinguish the food from the plate. Always position them in the upright position when eating, and continue to sit upright for 20-30 minutes after a meal.

Sometimes, those with LBD may refuse to eat due to confusion or hallucinations. They may also experience swallowing problems, such as choking, aspirating food into the lungs, or forgetting how to swallow so that food stays in the mouth.

Recommendations:

  • Do not use thickeners; evidence shows they can actually worsen quality of life.
  • Try carbonated beverages.
  • Try smoothie-consistency drinks; thin liquids are most difficult.
  • Give medications with applesauce or something of a similar consistency.
  • Alternate between a bite of food and a sip of fluid.
  • Have them do a chin tuck when swallowing.
  • Benevolent trickery is sometimes necessary.

Weight loss

Weight loss is expected for those with LBD and is an indication of end-stage disease. Anorexia (lack of eating) and cachexia (weakness and wasting of the muscles) are common. It may seem counter-intuitive, but high-calorie supplements or appetite stimulants will not help with these issues and can actually worsen quality and quantity of life. It can be very hard to see the number on the scale continuing to drop, but it’s not something we can fix – it’s a hallmark of end-stage LBD.

Toileting and incontinence

Give your loved one as much privacy and independence as is consistent with their safety. Give them extra time, assess if they need help, and acknowledge that it may be uncomfortable or embarrassing for them. Timed voiding – visiting the bathroom at regular times throughout the day – can be very helpful to keep them comfortable and avoid accidents. A pad inside the absorbent briefs can provide extra protection. The Liberty catheter is an external catheter that goes on at bedtime, which can be helpful. Consider keeping a urinal or commode at the bedside so they do not have to get up to the bathroom overnight.

Dental care

It’s important to continue dental care. Brush teeth twice a day; eventually, they will likely need assistance with this task. A little bit of lemon juice can aid in swallowing and help clean the palate.

Practices to avoid in advanced LBD

In the past, patients with swallowing difficulties were often given thickened liquids. However, recent studies have shown this is actually a harmful practice and should not be continued! Thickened liquids are generally so unpalatable that people often refuse to drink them, which can lead to dehydration and kidney damage.

Feeding tubes are another practice in those with dementia that have now been shown to be counter-productive: artificial nutrition does not prolong or improve life for those with advanced dementia. Rather, assisted oral feedings are the best course. Post-operative pain and infection after feeding tube insertion, as well as increased likelihood of delirium and repeat surgeries, are some of the reasons why this practice is no longer recommended. Caregiver burden often increases as well.

Fluctuations in alertness

As LBD progresses, individuals will have more and more fluctuations of alertness. This often presents as seeming “zoned out” for a few minutes, and then they come back to normal. It is not a medical emergency, and there is no known treatment.

End-of-life care: Days to weeks

At this point, most people are bedridden. Focus on creating comfortable surroundings. Bring nature indoors, if possible, and play your loved one’s favorite music. Soothing touch, such as a gentle massage, can be relaxing and pleasurable. Smells such as lavender oil can be pleasant as well.

In terms of medications, if Sinemet (carbidopa-levodopa) has been helpful for them, continue it for as long as possible. Reduce other medications to only what is necessary, or will help them remain comfortable. If liquid formulations of medications are available, switch to those; if the person is unable to swallow, then suppositories may be the best option. Most patients at this stage of LBD will need sedating medications to control the agitation and psychosis that generally increase at the end of life.

Prognostic indicators in LBD

There are certain indicators that provide a prognosis – the likely course or time frame of late-stage LBD. These indicators are used by Medicare to determine eligibility for hospice services, which require an estimate of six months or less of life.

For those with weight loss, these are the same criteria as for “Adult Failure to Thrive,” a different medical diagnosis. The main criteria for hospice eligibility are a body mass index (BMI) of less than 22, and a score on the Palliative Performance Scale (PPS) of 40 or less, indicating someone who is mainly in bed and needs assistance with activities of daily living (ADLs). These two factors are strong indicators that someone is likely within 6 months of the end of their life, and should receive hospice services to ensure the best care.

For those with dementia, hospice eligibility requires at least one of the following in the past 12 months:

  • Aspiration pneumonia
  • Pyelonephritis (kidney infection)
  • Septicemia (infection of the blood)
  • Stage 3 or 4 pressure ulcers
  • Recurrent fever
  • 10% weight loss (or greater) in the past 12 months
  • Albumin less than 2.5 mg/dL (measurement of protein indicating malnutrition) and greater than or equal to 7C on the FAST scale:
  1. Speaks 5-6 words per day
  2. Speaks only 1 word clearly per day
  3. Cannot walk without personal assistance
  4. Cannot sit up without personal assistance
  5. Can no longer hold up head independently

What do people die of in LBD?

There have been very few studies on this question. For most, aspiration pneumonia – a lung infection caused by inhaling food or oral secretions into the lungs – is the cause of death. Sometimes aspiration pneumonia leads to sepsis, an extreme reaction by the body in response to a systemic infection, which can be fatal. Reduced mobility, falls, or fractures can also be life-threatening. Failure to thrive, which is the severe weight loss associated with late-stage dementia, is another cause as well.

Hospice care: Role of the neurologist

Right now, only about 5% of patients with LBD will receive hospice services

It’s important that more LBD patients and families receive the help and care available to them through hospice. Your loved one’s neurologist can still play a helpful role even after hospice is involved, by helping to communicate and coordinate with the hospice team and educating them about the specific needs of LBD patients, such as medications that are commonly used in a hospice setting, but which should NOT be given to those with LBD.

Medications to avoid in hospice patients with LBD:

  • Haloperidol (Haldol)
  • Metoclopramide
  • Phenergan

Alternative medications that are safer to use in LBD are quetiapine (Seroquel) for agitation/psychosis and ondansetron (Zofran) for nausea.

The Advance Healthcare Directive is a legal document that allows an individual to spell out their preferences for life-saving medical interventions and end of life care, ahead of time, and designates an individual who is empowered to make healthcare decisions on their behalf, if necessary. This requires that you speak with the loved one about their wishes while they are still able to consider such decisions.

www.prepareforyourcare.org

Most people who go into cardiac arrest are not able to be resuscitated successfully; only 5% of people with dementia who undergo CPR survive. It is not like the movies; the chances of a good recovery are actually quite small, contrary to the portrayal in the movies. The POLST (Physician’s Order for Life Sustaining Treatment) is a form, usually yellow, that tells emergency professionals what to do in a crisis when the patient cannot speak for themselves.

Dr. Katz cited a quote from the book Being Mortal: Medicine and What Matters in the End by Atul Gawande, MD:

“… Our most cruel failure in how we treat the sick and the aged is the failure to recognize that they have priorities beyond merely being safe and living longer.”

Two questions to ask around advance care planning:

  • If you look ahead, what worries you the most?
  • When you look to the future, what are you hoping for? (in addition to a cure)

“Our ultimate goal, after all, is not a good death, but a good life to the very end.” – Atul Gawande, MD

Lewy Body Dementia: A Doctor’s Story

Lewy Body Dementia
Lewy Body Dementia

Below we post yet another touching and revealing personal testimony from a doctor concerning Lewy Body Dementia.

When I finished my training, I was taught that the vast majority of dementia was Alzheimer’s disease, with occasional cases of multi-infarct dementia as well as odd syndromes such as Kreutzfeld-Jacob disease and genetic, traumatic, toxic and tumor-related syndromes.

Parkinson’s disease, we were taught, caused a tremor and freezing up of a person’s movements and only very rarely was associated with any kind of memory loss.

These teachings helped us modern doctors leave behind terms such as “senility” or “hardening of the arteries” to explain cognitive loss. We still had no useful tools to change the course of dementia, but we were more scientific in our description of it.

In the last several years, however, neurologists have determined that there is a very common dementia that is associated with Parkinson’s disease.

Lewy body disease or Lewy body dementia was a condition that I had been taught was not only uncommon but only accurately diagnosed at autopsy or with a brain biopsy.

It appears, now, that it is quite common, comprising up to 30% of cases of dementia.

It is more common in men than in women, like Parkinson’s disease but not like Alzheimer’s disease and is more common in people who have higher educational attainment.

It is characterized by collections of protein known as Lewy bodies that are found throughout the cerebral cortex, rather than just in the movement centers of the midbrain, as in Parkinson’s disease.

There are some medical tests that will help to diagnose Lewy body disease, but they are not commonly performed. It is most commonly diagnosed by the identifying 2 of 4 common clinical features. These are:

  1. Fluctuating cognition with varying levels of consciousness and alertness. They will fall deeply asleep and be unarousable or be very slow and confused, lasting hours sometimes, then improve to a more normal baseline, laughing and participating in conversations. Family will sometimes think they have had a stroke or a seizure.
  2. Visual hallucinations. These may be quite detailed.
  3. REM sleep disorder. Patients will often talk in their sleep or do complex movements, often getting up and walking or performing complex behaviors. This can be disturbing and even dangerous to a bed partner.
  4. Motor features of Parkinson’s, including pill-rolling tremor, slow movements and rigidity. These features almost always follow the development of memory loss.

Patients have other clinical features such as depression, anxiety, apathy and loss of executive function (unable to clean a closet, sort and pay bills or put together a photo album.) They often have autonomic dysfunction, with fainting spells due to drop in blood pressure or inability to tolerate changes in temperature. They become unstable in their walking or standing and fall frequently. They have urinary incontinence. They have delusions that are detailed and hard to shake.

My father had this and I didn’t recognize it until about 3 months before his death.

He was a very smart man, having graduated in physics from Caltech and then worked with early computers and was an important part of the space program. He worked on developing electric cars and wind power and studied the feasibility of oil shale and tar sands (not feasible and not efficient, he concluded.)

He brought a supercomputer to the island of Maui and helped jump-start their technology industry. He was funny and engaging and had a knack for encouraging others by being a springboard for their ideas. He was the most compelling conversationalist I have ever known and played a mean game of scrabble.

When he lost his wife to cancer 12 years ago he began to notice some disturbing memory failures. He put those down to the stresses of home hospice and the depression that followed her death. He was capable with his laptop computer and enjoyed the early handheld computing devices, but when he got his first smartphone, the technology became more difficult for him to learn.

He could use email, but forgot how it worked and never learned to access it with his iPhone. He took on the position of board president with an academic organization and wasn’t able to keep up with what he needed to do. He resigned and felt terrible about that. He tried to take blood pressure medication but would pass out unexpectedly so stopped it. He kept all of his mail and couldn’t figure out how to file it, feeling ashamed about the state of his desk. He flailed and talked in his sleep so much that his wife had to go to bed elsewhere. He would wake up at 4 in the morning, inconsolably sure that he needed to catch a plane or teach a class.

He fell frequently and when he walked or stood, would lean to one side, unaware that he was doing it. It was nerve-wracking to walk with him because he refused to take an arm and never learned to use a walker or cane. He slept more, often while sitting up, and had periods of unresponsiveness that were alarming. His falls were not minor and he gashed his face and ripped the skin on his arms, broke his hand and hit his head hard enough to spend an agitated night in the hospital. His writing became small, shaky and cramped.

It was clear he had dementia and that it wasn’t a normal kind. He saw a neurologist who thought he might have Parkinson’s disease and a wonderful gerontologist who diagnosed Alzheimer’s disease. His wife, who had been attending a dementia support group, had heard about how common Lewy body dementia was and suggested it might be that. I read the most recent literature and decided she was right. Not only did he have it, so had many of my patients over the years who I had thought had Alzheimer’s disease.

The fluctuations. The falls. The detailed hallucinations and delusions. The executive dysfunction. It isn’t subtle how different it is from Alzheimer’s disease. These are the people who come into the emergency department frequently when they are clearly worse than normal but improve overnight and return home, even though we think that’s a bad idea. They do fine until they fluctuate again and then are back. These are the difficult to handle patients with the mean delusions who drive their families or spouses nuts but are unmanageable in nursing homes. If Alzheimer’s disease were vanilla ice cream, Lewy body dementia would be rocky road, with real rocks.

My father was a sweet guy but this disease made him critical, unkind and selfish. But only sometimes. Only when we were so deliberately stupid that we didn’t understand that his reputation would be ruined if he didn’t get to the airport or to the lecture he was supposed to be giving. Only if we tried to help him walk when he could clearly do it better without our pushing him off balance. Only when he woke up scared and didn’t understand what was going on. At other times he was kind and appreciative and full of sweetness, humor and wisdom that he could no longer put express with words.

It was possible for him to stay at home with his wife for a long time with the help of caregivers. Eventually, however, the combination of impulsiveness, weakness and sleep disturbance made even 24 hour caregiver support inadequate and he had to be moved to a memory care center.

The facility was really wonderful, catering to the dotty, delirious and demented, many of them with what appeared to be Lewy body dementia. He perked up briefly after moving, but then began to sleep more, sitting up in his wheelchair. He still had up times, explaining the chemical properties of tungsten, listening to a talk I needed to practice and offering good questions. He became weaker, unable to hold a cup or a fork, barely able to lift a cookie. Eventually he didn’t wake up at all and two days later died peacefully, in the care of hospice.

Since his diagnosis, I have been much more aware of those demented people who don’t have Alzheimer’s disease. It helps to know, so we don’t compare them unfavorably. They aren’t just difficult people with dementia, they are regular people with difficult dementia.

Robin Williams, the gifted actor and comedian, developed a set of disturbing symptoms in the last years of his life that were unexplained but progressive and horrible. He said goodnight to his wife one evening (she slept in a separate room because of his sleep behaviors), went to his bedroom and hung himself. An autopsy showed severe Lewy body dementia. She wrote a letter to the journal Neurology detailing their medical odyssey. It is heartbreaking to read.

We have no useful treatments for Lewy body dementia. Sometimes benzodiazepines help a bit with the sleep disorder, but my experience was that they did nothing. Anti-psychotic medication is not helpful and often can worsen the symptoms significantly. Parkinson’s medication can help with the rigidity and tremor, but my experience was that, by the time that was prominent it was more helpful for my father not to have the ability to act out his impulsivity. A wheelchair was much safer. Cholinesterase inhibitors, used in Alzheimer’s disease, are recommended but don’t help much and can cause sleep problems, nausea and drooling.

I will miss my father. I will think about his voice, his conversation, his smile, his love of math and his scientific contributions. I will remember his smell, his wispy white hair and the way he loved to have his back scratched hard. I will not miss his last 6 months, though, and I resent those nasty Lewy bodies that infested his wonderful brain.

Dr. Janice M. Boughton is an internist in Moscow, Idaho and is affiliated with multiple hospitals in the area, including Gritman Medical Center and Good Shepherd Health Care System-Hermiston. She received her medical degree from Johns Hopkins University School of Medicine and has been in practice for more than 20 years.

Susan Williams: The Terrorist Inside My Husband’s Brain. Lewy Body Dementia.

Lewy Body Dementia (2)

We post the personal testimony of Susan Schneider Williams, widow of the late Robin Williams.

I am writing to share a story with you, specifically for you. My hope is that it will help you understand your patients along with their spouses and caregivers a little more. And as for the research you do, perhaps this will add a few more faces behind the why you do what you do. I am sure there are already so many.

This is a personal story, sadly tragic and heartbreaking, but by sharing this information with you I know that you can help make a difference in the lives of others.

As you may know, my husband Robin Williams had the little-known but deadly Lewy body disease (LBD). He died from suicide in 2014 at the end of an intense, confusing, and relatively swift persecution at the hand of this disease’s symptoms and pathology. He was not alone in his traumatic experience with this neurologic disease. As you may know, almost 1.5 million nationwide are suffering similarly right now.

Although not alone, his case was extreme. Not until the coroner’s report, 3 months after his death, would I learn that it was diffuse LBD that took him. All 4 of the doctors I met with afterwards and who had reviewed his records indicated his was one of the worst pathologies they had seen. He had about 40% loss of dopamine neurons and almost no neurons were free of Lewy bodies throughout the entire brain and brainstem.

Robin is and will always be a larger-than-life spirit who was inside the body of a normal man with a human brain. He just happened to be that 1 in 6 who is affected by brain disease.

Not only did I lose my husband to LBD, I lost my best friend. Robin and I had in each other a safe harbor of unconditional love that we had both always longed for. For 7 years together, we got to tell each other our greatest hopes and fears without any judgment, just safety. As we said often to one another, we were each other’s anchor and mojo: that magical elixir of feeling grounded and inspired at the same time by each other’s presence.

One of my favorite bedrock things we would do together was review how our days went. Often, this was more than just at the end of the day. It did not matter if we were both working at home, traveling together, or if he was on the road. We would discuss our joys and triumphs, our fears and insecurities, and our concerns. Any obstacles life threw at us individually or as a couple were somehow surmountable because we had each other.

When LBD began sending a firestorm of symptoms our way, this foundation of friendship and love was our armor.

The colors were changing and the air was crisp; it was already late October of 2013 and our second wedding anniversary. Robin had been under his doctors’ care. He had been struggling with symptoms that seemed unrelated: constipation, urinary difficulty, heartburn, sleeplessness and insomnia, and a poor sense of smell—and lots of stress. He also had a slight tremor in his left hand that would come and go. For the time being, that was attributed to a previous shoulder injury.

On this particular weekend, he started having gut discomfort. Having been by my husband’s side for many years already, I knew his normal reactions when it came to fear and anxiety. What would follow was markedly out of character for him. His fear and anxiety skyrocketed to a point that was alarming. I wondered privately, Is my husband a hypochondriac? Not until after Robin left us would I discover that a sudden and prolonged spike in fear and anxiety can be an early indication of LBD.

He was tested for diverticulitis and the results were negative. Like the rest of the symptoms that followed, they seemed to come and go at random times. Some symptoms were more prevalent than others, but these increased in frequency and severity over the next 10 months.

By wintertime, problems with paranoia, delusions and looping, insomnia, memory, and high cortisol levels—just to name a few—were settling in hard. Psychotherapy and other medical help was becoming a constant in trying to manage and solve these seemingly disparate conditions.

I was getting accustomed to the two of us spending more time in reviewing our days. The subjects though were starting to fall predominantly in the category of fear and anxiety. These concerns that used to have a normal range of tenor were beginning to lodge at a high frequency for him. Once the coroner’s report was reviewed, a doctor was able to point out to me that there was a high concentration of Lewy bodies within the amygdala. This likely caused the acute paranoia and out-of-character emotional responses he was having. How I wish he could have known why he was struggling, that it was not a weakness in his heart, spirit, or character.

In early April, Robin had a panic attack. He was in Vancouver, filming Night at the Museum 3. His doctor recommended an antipsychotic medication to help with the anxiety. It seemed to make things better in some ways, but far worse in others. Quickly we searched for something else. Not until after he left us would I discover that antipsychotic medications often make things worse for people with LBD. Also, Robin had a high sensitivity to medications and sometimes his reactions were unpredictable. This is apparently a common theme in people with LBD.

During the filming of the movie, Robin was having trouble remembering even one line for his scenes, while just 3 years prior he had played in a full 5-month season of the Broadway production Bengal Tiger at the Baghdad Zoo, often doing two shows a day with hundreds of lines—and not one mistake. This loss of memory and inability to control his anxiety was devastating to him.

While I was on a photo shoot at Phoenix Lake, capturing scenes to paint, he called several times. He was very concerned with insecurities he was having about himself and interactions with others. We went over every detail. The fears were unfounded and I could not convince him otherwise. I was powerless in helping him see his own brilliance.

For the first time, my own reasoning had no effect in helping my husband find the light through the tunnels of his fear. I felt his disbelief in the truths I was saying. My heart and my hope were shattered temporarily. We had reached a place we had never been before. My husband was trapped in the twisted architecture of his neurons and no matter what I did I could not pull him out.

In early May, the movie wrapped and he came home from Vancouver—like a 747 airplane coming in with no landing gear. I have since learned that people with LBD who are highly intelligent may appear to be okay for longer initially, but then, it is as though the dam suddenly breaks and they cannot hold it back anymore. In Robin’s case, on top of being a genius, he was a Julliard-trained actor. I will never know the true depth of his suffering, nor just how hard he was fighting. But from where I stood, I saw the bravest man in the world playing the hardest role of his life.

Robin was losing his mind and he was aware of it. Can you imagine the pain he felt as he experienced himself disintegrating? And not from something he would ever know the name of, or understand? Neither he, nor anyone could stop it—no amount of intelligence or love could hold it back.

Powerless and frozen, I stood in the darkness of not knowing what was happening to my husband. Was it a single source, a single terrorist, or was this a combo pack of disease raining down on him?

He kept saying, “I just want to reboot my brain.” Doctor appointments, testing, and psychiatry kept us in perpetual motion. Countless blood tests, urine tests, plus rechecks of cortisol levels and lymph nodes. A brain scan was done, looking for a possible tumor on his pituitary gland, and his cardiologist rechecked his heart. Everything came back negative, except for high cortisol levels. We wanted to be happy about all the negative test results, but Robin and I both had a deep sense that something was terribly wrong.

On May 28th, he was diagnosed with Parkinson disease (PD).

We had an answer. My heart swelled with hope. But somehow I knew Robin was not buying it.

When we were in the neurologist’s office learning exactly what this meant, Robin had a chance to ask some burning questions. He asked, “Do I have Alzheimer’s? Dementia? Am I schizophrenic?” The answers were the best we could have gotten: No, no, and no. There were no indications of these other diseases. It is apparent to me now that he was most likely keeping the depth of his symptoms to himself.

Robin continued doing all the right things—therapy, physical therapy, bike riding, and working out with his trainer. He used all the skills he picked up and had fine-tuned from the Dan Anderson retreat in Minnesota, like deeper 12-step work, meditation, and yoga. We went to see a specialist at Stanford University who taught him self-hypnosis techniques to quell the irrational fears and anxiety. Nothing seemed to alleviate his symptoms for long.

Throughout all of this, Robin was clean and sober, and somehow, we sprinkled those summer months with happiness, joy, and the simple things we loved: meals and birthday celebrations with family and friends, meditating together, massages, and movies, but mostly just holding each other’s hand.

Robin was growing weary. The parkinsonian mask was ever present and his voice was weakened. His left hand tremor was continuous now and he had a slow, shuffling gait. He hated that he could not find the words he wanted in conversations. He would thrash at night and still had terrible insomnia. At times, he would find himself stuck in a frozen stance, unable to move, and frustrated when he came out of it. He was beginning to have trouble with visual and spatial abilities in the way of judging distance and depth. His loss of basic reasoning just added to his growing confusion.

It felt like he was drowning in his symptoms, and I was drowning along with him. Typically the plethora of LBD symptoms appear and disappear at random times—even throughout the course of a day. I experienced my brilliant husband being lucid with clear reasoning 1 minute and then, 5 minutes later, blank, lost in confusion.

Prior history can also complicate a diagnosis. In Robin’s case, he had a history of depression that had not been active for 6 years. So when he showed signs of depression just months before he left, it was interpreted as a satellite issue, maybe connected to PD.

Throughout the course of Robin’s battle, he had experienced nearly all of the 40-plus symptoms of LBD, except for one. He never said he had hallucinations.

A year after he left, in speaking with one of the doctors who reviewed his records, it became evident that most likely he did have hallucinations, but was keeping that to himself.

It was nearing the end of July and we were told Robin would need to have inpatient neurocognitive testing done in order to evaluate the mood disorder aspect of his condition. In the meantime, his medication was switched from Mirapex to Sinemet in an effort to reduce symptoms. We were assured Robin would be feeling better soon, and that his PD was early and mild. We felt hopeful again. What we did not know was that when these diseases “start” (are diagnosed) they have actually been going on for a long time.

By now, our combined sleep deficit was becoming a danger to both of us. We were instructed to sleep apart until we could catch up on our sleep. The goal was to have him begin inpatient testing free of the sleep-deprived state he was in.

As the second weekend in August approached, it seemed his delusional looping was calming down. Maybe the switch in medications was working. We did all the things we love on Saturday day and into the evening, it was perfect—like one long date. By the end of Sunday, I was feeling that he was getting better.

When we retired for sleep, in our customary way, my husband said to me, “Goodnight, my love,” and waited for my familiar reply: “Goodnight, my love.”

His words still echo through my heart today.

Monday, August 11, Robin was gone.

After Robin left, time has never functioned the same for me. My search for meaning has replicated like an inescapable spring throughout nearly every aspect of my world, including the most mundane.

Robin and I had begun our unplanned research on the brain through the door of blind experience. During the final months we shared together, our sights were locked fast on identifying and vanquishing the terrorist within his brain. Since then, I have continued our research but on the other side of that experience, in the realm of the science behind it.

Three months after Robin’s death, the autopsy report was finally ready for review. When the forensic pathologist and coroner’s deputy asked if I was surprised by the diffuse LBD pathology, I said, “Absolutely not,” even though I had no idea what it meant at the time. The mere fact that something had invaded nearly every region of my husband’s brain made perfect sense to me.

In the year that followed, I set out to expand my view and understanding of LBD. I met with medical professionals who had reviewed Robin’s last 2 years of medical records, the coroner’s report, and brain scans. Their reactions were all the same: that Robin’s was one of the worst LBD pathologies they had seen and that there was nothing else anyone could have done. Our entire medical team was on the right track and we would have gotten there eventually. In fact, we were probably close.

But would having a diagnosis while he was alive really have made a difference when there is no cure? We will never know the answer to this. I am not convinced that the knowledge would have done much more than prolong Robin’s agony while he would surely become one of the most famous test subjects of new medicines and ongoing medical trials. Even if we experienced some level of comfort in knowing the name, and fleeting hope from temporary comfort with medications, the terrorist was still going to kill him. There is no cure and Robin’s steep and rapid decline was assured.

The massive proliferation of Lewy bodies throughout his brain had done so much damage to neurons and neurotransmitters that in effect, you could say he had chemical warfare in his brain.

One professional stated, “It was as if he had cancer throughout every organ of his body.” The key problem seemed to be that no one could correctly interpret Robin’s symptoms in time.

I was driven to learn everything I could about this disease that I finally had the name of. Some of what I learned surprised me.

One neuropathologist described LBD and PD as being at opposite ends of a disease spectrum. That spectrum is based on something they share in common: the presence of Lewy bodies—the unnatural clumping of the normal protein, α-synuclein, within brain neurons. I was also surprised to learn that a person is diagnosed with LBD vs PD depending on which symptoms present first.

After months and months, I was finally able to be specific about Robin’s disease. Clinically he had PD, but pathologically he had diffuse LBD. The predominant symptoms Robin had were not physical—the pathology more than backed that up. However you look at it—the presence of Lewy bodies took his life.

The journey Robin and I were on together has led me to knowing the American Academy of Neurology and other groups and doctors. It has led me to discover the American Brain Foundation, where I now serve on the Board of Directors.

This is where you come into the story.

Hopefully from this sharing of our experience you will be inspired to turn Robin’s suffering into something meaningful through your work and wisdom. It is my belief that when healing comes out of Robin’s experience, he will not have battled and died in vain. You are uniquely positioned to help with this.

I know you have accomplished much already in the areas of research and discovery toward cures in brain disease. And I am sure at times the progress has felt painfully slow. Do not give up. Trust that a cascade of cures and discovery is imminent in all areas of brain disease and you will be a part of making that happen.

If only Robin could have met you. He would have loved you—not just because he was a genius and enjoyed science and discovery, but because he would have found a lot of material within your work to use in entertaining his audiences, including the troops. In fact, the most repeat character role he played throughout his career was a doctor, albeit different forms of practice.

You and your work have ignited a spark within the region of my brain where curiosity and interest lie and within my heart where hope lives. I want to follow you. Not like a crazed fan, but like someone who knows you just might be the one who discovers the cure for LBD and other brain diseases.

Thank you for what you have done, and for what you are about to do.

DISCLOSURE

Susan Schneider Williams serves on the Board of Directors for the American Brain Foundation (americanbrainfoundation.org) but reports no disclosures relevant to the manuscript.

Go to Neurology.org for full disclosures.

Recognising Diagnosing Misdiagnosing Lewy Body Dementia: The Doctor’s Perspective

Lewy Body Dementia
Lewy Body Dementia UK

Lewy Body Dementia is often mistaken for other forms of dementia.

Below we post a detailed report and perspective from a qualified doctor Charles E. Driscoll, MD.

Frequently misdiagnosed as Alzheimer’s disease, Lewy body dementia is notable for its up-and-down swings that precede the eventual decline.

As a family physician/geriatrician, I sometimes speak to primary care physicians about dementia with Lewy bodies (DLB) because of the understanding my spouse and I have gained while caring for our family members during their personal walk with LB disease.

I once asked an audience of 400 primary care providers who among them was currently caring for a patient with DLB. Fewer than a dozen hands went up.

After my presentation, when I called for another show of hands, most in the room realized they probably had been caring for a patient with DLB under the assumption that it was another type of dementia.

If your first reaction to DLB is “never heard of it,” you’re not alone, as the disorder was not consistently recognized until 1996 and not listed in the International Classification of Diseases until 2005. This article addresses the key points of what we have learned and information that will help in the care of these patients.

What Defines LBD?
Lewy body dementia (LBD) describes two distinct but related types of dementia: DLB and Parkinson’s disease with dementia (PDD). LBD is now recognized as the second most common cause of dementia after Alzheimer’s disease (AD), affecting more than 1.5 million people and their families and accounting for 20% to 25% of all dementias.1

Mental dysfunction is caused by neurodegeneration accompanied by the cerebral deposition of an abnormal protein called alpha-synuclein as compared with AD, which is associated with the protein beta-amyloid with neurofibrillary tangles.

Unlike AD, which mainly affects the cerebral cortex, LBD can affect the cortex and substantia nigra, altering both cognitive and motor functions. If dementia appears first, followed by or concurrent with PD motor symptoms, it is usually referred to as DLB, but if motor dysfunction presents initially with dementia occurring more than one year later, it is diagnosed as PDD.2

The current consensus is that these neurodegenerative conditions, along with other LB conditions (eg, multiple system atrophy, LB variant of Alzheimer’s, Down syndrome, amyotrophic lateral sclerosis, dementia with brain iron accumulation), are all part of a spectrum of disorders. Experts are still struggling to establish agreement on the taxonomy.

Establishing the Diagnosis
LBD is frequently initially misdiagnosed as AD in its earlier stages until the defining characteristics begin to appear. Unlike the gradual, steady decline of AD, LBD is notable for its up-and-down swings before eventual decline. Some family members initially are confused about what they are observing because their family member can function apparently normally one day and exhibit dramatic symptoms the next.

The key to appropriate LBD management is earlier recognition. Several consequences of misdiagnosing this condition include exposing the patient to potentially harmful medications (eg, exaggerated response to benzodiazepines and neuroleptic drugs), missing an opportunity to choose effective therapies (eg, better response to cholinesterase inhibitors), and delaying the opportunity to help families understand and cope with the significant burden of this illness.

Here are clues that the dementia you are observing is LBD. Unlike early AD, LBD often begins with episodic loss of long-term memory and more marked impairments of executive (eg, making decisions; sequential tasking), visuo-spatial, and attentive functions. People with LBD also show more problems with impulse control and behavior. Research has concluded that LBD patients exhibit a higher rate of periodic limb movements and rapid eye movement (REM) sleep behavior disorder that can help to differentiate DLB from AD.3,4

A diagnosis of LBD requires some combination of dementia and the following2:

• three core symptoms: fluctuating cognition (bad days and good “showtime” days), vivid visual hallucinations and/or delusions, and motor dysfunction; or

• three suggestive symptoms: REM sleep behavior disorder with acting out of dreams or excessive daytime sleepiness, abnormal brain CT/MRI, and extreme sensitivity to haloperidol and tranquilizers.

DLB is considered probable with the presence of dementia plus two core symptoms or one core and one or more suggestive symptoms. It is considered possible with dementia plus one core symptom or one or more suggestive symptoms.

A histological diagnosis can be made at autopsy with the findings of alpha-synuclein–positive Lewy bodies extensively distributed within the neocortical and limbic areas (see Figure 1 below). Unfortunately, there is no accurate premortem test that will nail down the LB diagnosis. At this time, there is research in the use of imaging techniques to aid in the diagnosis.

Single photon emission CT testing has identified an association with occipital hypoperfusion. MRIs have shown DLB-specific changes, including preservation of hippocampal and medial temporal lobe volumes, compared with AD. Recently, presynaptic dopaminergic deficits in the striatum have been visualized using a 123I-radiolabeled marker (DaTSCAN). These techniques are still in the realm of research institutions and not widely available.

Typical Disease Progression
LBD generally is thought to progress to death faster than AD. Some authorities quote a prognosis of between two and seven years after onset until death. Men are more likely to die sooner than women. There also is a shorter course to nursing home placement for DLB patients than for those with AD. DLB patients are higher utilizers of emergency department and outpatient care, community services, and drug therapies.2

The disease evokes many emotions and a great deal of misunderstanding for families. Remembering that all forms of dementia are terminal, note that DLB patients qualify for hospice care to assist in the management of their various symptoms. Early referral often is helpful for families providing care at home. Additionally, referral to a family support group can be beneficial. The best information can be accessed from the Lewy Body Dementia Association (www.lbda.org).

Helpful Medications
The mainstay of LBD therapy is cholinesterase inhibitors (eg, donepezil, galantamine), which work better for the cognitive and behavioral symptoms of patients with DLB than those with AD. If the side effects experienced with one inhibitor are troublesome, another may be tried and may work better. If patients experience difficulty taking pills, a patch formulation (rivastigmine) is available. When a patient takes a cholinesterase inhibitor, the reduction in hallucinations and delusions can be remarkable.

Selective serotonin reuptake inhibitors can be useful for some patients with depression and anxiety, but the required dosages are smaller than those used for non-DLB patients. Disruptive behaviors (eg, aggression, wandering, hallucinations, delusions) are common in DLB and are frequently the precipitating event for a visit to a physician. Because behaviors are similar to those seen with delirium, the first step in dealing with them should be the elimination of organic causes.

Examining for a urinary tract infection, constipation with impaction, and occult infections is always justified. When families insist on pharmacological management, the atypical class of antipsychotics (eg, quetiapine) provide better management of disruptive behaviors because at least one-half of LBD patients are particularly sensitive to traditional antipsychotics and benzodiazepines. Fatal neuroleptic malignant syndrome with fever, restlessness, rigidity, and muscle cell breakdown has resulted from the use of haloperidol.

A higher prevalence of cardiovascular problems and abnormal electrical activity evident on an electrocardiogram (ECG) is more common in DLB patients. Because many medications can produce cardiotoxic effects, a black box warning has been placed on the atypical antipsychotics. An ECG should be performed to look for evidence of prolonged Q-T interval or some other conduction defect. This requires a careful explanation to ensure informed consent and agreement about the risks4 in the event of drug use.

The use of benzodiazepines or narcoleptic medications will often produce a paradoxical response in DLB, and this can be an early supportive clue to the diagnosis. DLB patients also tend to have more autonomic dysfunction than AD patients, and polypharmacy may result from attempts to control hypotension, constipation, urinary retention, myotonia, and excessive salivation as separate issues rather than as a part of the whole.

Many DLB patients will manifest the motor symptoms of Parkinsonism, but unfortunately anti-Parkinson drugs often make the cognitive and behavioral problems worse and thereby force a choice between the lesser of two evils. If simultaneous treatment is to be attempted, the dictum of “start very low and go very slow” certainly applies to patients with DLB.

Where Do We Go From Here?
DLB is underrecognized by physicians, and among the general public there is a very limited awareness of the different types of dementia. For years everything was labeled as AD, with little reason to do otherwise, since physicians’ knowledge base precluded any effective management. Now there are good reasons to discriminate these conditions—and the earlier the better.

DLB is far more apt to overstress and burden families.5 In facilitating a support group, my wife and I have found that getting people to share their experiences makes them realize the commonality of the bizarre symptoms they see in their loved ones and normalizes the experience so that they feel better able to try new coping skills at home. Grieving is common in DLB families, even long before the death occurs, and a support group provides recognition of this fact and significant comfort.

Many caregivers are themselves aged spouses or siblings and are themselves experiencing the onset of personal health issues. They need to be reminded to keep their own doctors’ appointments and be spiritually nurtured in order to remain strong in caregiving. Getting adequate and early assistance for family caregivers helps reduce the strain of caring for someone with dementia.

Managing patients with DLB is a difficult and sometimes frustrating task for physicians. Teamwork by office members will help to spread out the busyness of communication with families, medication management, and coordination of community services. The literature about DLB is sometimes hard to find and in a variety of journals. Monitoring the information for physicians on the Lewy Body Disease Association website is a good way to identify new advances in the science and resources that may be useful in patient care.

— Charles E. Driscoll, MD, is a clinical professor of family medicine at the University of Virginia and a clinical associate professor of family medicine at Virginia Commonwealth University in Richmond. He is board certified in family medicine and geriatrics with special interest in dementia and frailty in the elderly.

— Jean Driscoll, MS, along with her husband, established the first Lewy Body support group for families in Virginia. Together they serve as the group’s facilitators.

Resources for Patients
• A Caregiver’s Guide to Lewy Body Dementia by Helen Buell Whitworth and Jim Whitworth: This book is a comprehensive guide to all things Lewy body. The Whitworths also write a blog, The Lewy Body Rollercoaster, which answers questions, references other sources, and announces new developments (http://lbdtools.com). Jim Whitworth is a cofounder of the Lewy Body Dementia Association.

• A Long Dark Night — A Caregiver’s Journey With Dementia by Philip Edward Phlegar Weeks: Weeks is an Episcopal bishop who, in this book, explains his feelings and frustrations with the Lewy body dementia affecting his spouse. He addresses spirituality, sexuality, and the difficulty of getting community services coordinated.

• Creating Moments of Joy for the Person With Alzheimer’s or Dementia: A Journal for Caregivers by Jolene Brackey: The fourth edition of this work by Brackey, an activities director in a memory care unit, includes practical solutions and strategies for caregivers wrapped in humor, warmth, and a message of hope.

• Learning to Speak Alzheimer’s: A Groundbreaking Approach for Everyone Dealing With the Disease by Joanne Koenig Coste: Coste is a nationally recognized expert in communications techniques. After her experience with a spouse who had Alzheimer’s, she offers her five basic tenets for use by caregivers and provides hundreds of practical tips.

• Living With Lewy’s: Empowering Today’s Dementia Caregiver by Amy and Gerald Throop: The authors’ decade of caregiving experience provides the basis for this book. It was written to provide a better understanding of the dementia process and to share practical, safe methods of dealing with specific disabilities and behaviors.

Lewy Body Dementia: Who is vulnerable? Who is at risk of LBD?

Lewy Body Dementia

Many people message this blog for answers to the question: am i at risk?

Below we post a list of risk factors that (could) be relevant.

A risk factor is something that could increase the chance of developing a disease.

Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.

But, risk factors can also be seen as characteristics at the biological, psychological, family, community, or
cultural level that precede and are associated with a higher likelihood of negative outcomes.

Until recently, the only known risk factor for developing Lewy body dementia was considered to be an older age.

Research has made some gains lately in sifting out what might increase the risk of developing Lewy body dementia.

As people age, they generally have a greater risk of developing Lewy body dementia.

The typical age range for the development of Lewy body dementia is between 50 and 85, although it can occur outside those ages.

In one study, researchers found that the peak age range for Lewy body dementia to develop is between 70-79.

Not Smoking

Interestingly, people with a history of smoking cigarettes have a lower risk of developing Lewy body dementia.2 However, the negative health effects of smoking are such that this is never recommended as a way to prevent Lewy body dementia.

Low Education Levels

More years of education are correlated with a reduced risk of Lewy body dementia.2

Depression and Anxiety

A history of depression and anxiety increase the risk of developing Lewy body dementia.2

Less Caffeine Intake

A history of higher caffeine consumption is associated with a lower risk of Lewy body dementia.2 Caffeine intake has also been correlated with a lower risk of Alzheimer’s disease.

Family History

About 10% of Lewy body dementia cases appear to be tied to heredity, where the person inherits the disease from a parent. When someone has had Lewy body dementia or Parkinson’s disease, his or her family members have a higher risk of developing Lewy body dementia.2 These familial cases of Lewy body dementia appear to occur often in younger people.

Mutations in genes known as SNCA and SNCB can cause Lewy body dementia. Some research has found that people with a variant of the GBA gene may have a higher risk of developing Lewy body dementia. Being positive for the APOE 4 gene was also found to be higher in those who developed Lewy body dementia. APOE ε4 has been tied to a significantly higher risk of Alzheimer’s disease.3

ADHD

According to one study published in the European Journal of Neurology, almost half of the participants in the study had adult attention-deficit/hyperactivity disorder (ADHD), compared to only 15% of those with Alzheimer’s disease.4

Gender

Men have a higher chance of developing Lewy body dementia than women do. Approximately twice as many males as females develop Lewy body dementia.1

Stroke

One study found that a prior stroke was correlated with an increased risk of Lewy body dementia.5

Hypertension

High blood pressure has been correlated with a higher risk or both Alzheimer’s disease and Lewy body dementia.5

Diabetes Mellitus

Diabetes mellitus, specifically type 2, has been strongly associated with a higher occurrence of Alzheimer’s disease, so much so that Alzheimer’s is sometimes referred to as “type 3 diabetes.” Other research has also determined that diabetes carries a higher risk of Lewy body dementia.5

Hyperlipidemia

Hyperlipidemia, commonly referred to as high cholesterol levels, also increases the risk of both Alzheimer’s disease and Lewy body dementia.5 High cholesterol levels are connected with cardiovascular diseases, which have been tied to increased dementia risk.

Oophorectomy History

One other factor identified as being connected with a higher risk of developing Lewy body dementia is a history of an oophorectomy, which is the removal of one or both of the ovaries in women.2

Can You Prevent Lewy Body Dementia?

If you have a family history of Lewy body dementia or Parkinson’s disease, it’s understandable to be concerned about developing Lewy body dementia.

Like other types of dementia, there’s not a guaranteed way to completely prevent Lewy body dementia.

However, understanding the factors that increase the risk helps us identify opposing strategies that can decrease this likelihood, and these strategies are generally connected with better physical health, as well.

Fascinating History Insight Observation Lewy Body Dementia

Lewy Body Dementia
Lewy Body Dementia

When dealing with, and trying to understand Lewy Body Dementia, it always helps if we can draw upon many different sources and experiences.

Below we have dug up a fascinating insight from times gone by, but nevertheless a very important piece of history concerning Lewy Body Dementia.

We think you will find this very informative and fascinating, as did we.

With many thanks to Jama Network

Mervyn Peake

Mervyn Peake (1911-1968) was an accomplished British artist, poet, novelist, and playwright. He was a prolific and talented illustrator and wrote hundreds of poems, 4 novels, and several plays.

His exceptional career was prematurely ended by a neurodegenerative illness variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism.

However, a detailed review of biographical accounts produces substantial evidence in support of a probable diagnosis of dementia with Lewy bodies, a clinical entity remaining undiagnosed outside specialty dementia clinics.

Peake developed signs of parkinsonism and insidious cognitive decline during his fifth decade. A breakdown in his writing style has frequently been cited as reflecting his encroaching dementia.

Visual hallucinations are portrayed in sketches, and together with paranoid delusions are apparent in poetry composed during his illness.

His deterioration was progressive and punctuated by well-described episodes of confusion and psychosis.

His occasional preservation of insight is poignantly captured in drawings of figures with dunce caps or pointed heads, often with expressions of fear and apprehension etched with an economy of strokes.

Peake spent his final years in various psychiatric institutions but continued to exhibit lucid intervals even late into his illness.

His tragic deterioration remained undiagnosed at the time, but in retrospect, his progressive dementia with parkinsonism, visual hallucinations, and marked cognitive fluctuations likely represents one of the earliest recognized historical cases of dementia with Lewy bodies.

Dementia with Lewy bodies (DLB) is estimated to account for 15% to 20% of cases of dementia, based on autopsy data1 and epidemiologic studies.2 Wider recognition of this disorder has emerged only recently, coinciding with descriptions published since the 1980s.3 To date, reports of prominent historical cases are lacking, although with increasing awareness, such cases are more likely to be recognized in retrospect.

A probable diagnosis is apparent in the neurobehavioral deterioration of accomplished British artist, poet, novelist, and playwright, Mervyn Peake (1911-1968), whose celebrated Gormenghast novels were recently adapted into a critically acclaimed television miniseries by the British Broadcasting Corporation.4 

Peake’s persistent attempts at artistic expression in the face of a relentless, cognitively devastating illness provide us with a unique and intensely personal account of a historical case of DLB.

Mervyn Peake was born in Kuling (Lushan), China, in 1911. His father, a British missionary physician, brought his family back to England in 1922. As a young man, Peake entered art school and first exhibited his work in 1931. He soon established a reputation as a talented illustrator. He was called up for military service in 1940, and while serving in the army, started work on his Gormenghast novels.

After the war, he was commissioned to document the aftermath of the conflict and completed a series of heartbreaking sketches of concentration camp survivors at Belsen. He continued to write and illustrate, producing many of his finest paintings, drawings, and poems during the late 1940s (Figure 1). He was much admired as an art instructor, and during this period also scripted several plays.5

These examples are taken from some of Mervyn Peake's earlier work, prior to the onset of cognitive decline. A, Self-portrait. Oil on canvas (National Portrait Gallery, London). B, Illustration for a 1949 edition of Robert Louis Stevenson's Treasure Island. Published with permission from the Mervyn Peake estate.

These examples are taken from some of Mervyn Peake’s earlier work, prior to the onset of cognitive decline. A, Self-portrait. Oil on canvas (National Portrait Gallery, London). B, Illustration for a 1949 edition of Robert Louis Stevenson’s Treasure Island. Published with permission from the Mervyn Peake estate.

Peake first showed signs of a mysterious neurodegenerative illness around 1956, developing signs of parkinsonism and symptoms of cognitive decline in his fifth decade.5 Perplexed biographers have alternately speculated diagnoses of Alzheimer disease,6 Parkinson disease,7 or postencephalitic parkinsonism5 after questioning a history of viral encephalitis6 or encephalitis lethargica.5 

The disintegration of Peake’s writing style has frequently been regarded as reflective of his encroaching dementia.7 He also suffered from bouts of depression, which influenced his later work. After a stressful period that culminated with a number of disastrous play reviews, his behavior started to become erratic, and his wife reported that he experienced hallucinations.8 

His deterioration was progressive, punctuated by well-described episodes of confusion and psychosis but relieved by intermittent lucid periods. He was ultimately diagnosed as having parkinsonism and “premature senility.”8

In 1960, neurosurgical approaches for Parkinson disease were being attempted, and Peake underwent one of these procedures in an effort to ameliorate his parkinsonian symptoms. The effects were disappointing.

As a last resort, an appointment was arranged with the preeminent British neurologist of the day, Sir Russel Brain, but he was likewise unable to assist the ailing artist. Peake spent the next several years of his life in various institutions, treated with neuroleptics and electroconvulsive therapy, until his death in 1968 at the age of 57 years.8 

His work has since drawn substantial critical acclaim and is presently the subject of increasing public interest. His influence on subsequent generations of artists and writers has been profound.

Dementia with lewy bodies

In 1912, Friederich Lewy described the presence of intracytoplasmic inclusion bodies in the dopaminergic neurons of the substantia nigra in patients with Parkinson disease.9 Lewy bodies in the midbrain are spherical, eosinophilic inclusions, differing slightly in appearance from those described in the neocortex.

Reports of cortical Lewy bodies were rare until new staining techniques for ubiquitin were introduced in the 1970s. The occurrence of cortical Lewy bodies in cases of dementia was firmly established by the 1980s.3 Antibodies to α-synuclein, which immunostain Lewy bodies, have since replaced the use of the ubiquitin stain, and DLB is now classified among the spectrum of diseases regarded as synucleinopathies.10

Consensus criteria for the diagnosis of DLB were published in 1996 and refined in 1999 (Table 1).11,12 The prospective sensitivity and specificity of the consensus criteria for DLB are reported at 0.83 and 0.95, respectively.13 Previous retrospective studies yielded comparable specificities (0.87-1.00) but lower sensitivities (0.22-0.89).14 

Hence, although the criteria might not identify every individual harboring the condition, there is high certainty of a diagnosis of DLB if the criteria are met.

Age at onset generally ranges from 50 to 83 years,15 although autopsy-verified cases have been documented in patients with initial symptoms at an age as young as 27 and 33 years.16 The mean survival time is usually comparable with that of Alzheimer disease.15

Consensus Criteria for a Diagnosis of Dementia With Lewy Bodies (DLB)11,12

Consensus Criteria for a Diagnosis of Dementia With Lewy Bodies (DLB)

Historical evidence

Peake’s progressive cognitive decline is apparent in his later work. His pronounced difficulties with attention and visuospatial ability are typical of DLB, in contrast with the more prominent decline in memory function observed in Alzheimer disease.11 Commencing with his later illustrations, one biographer observed that, “there is a coarseness of feeling, a lack of sympathy for the persons he portrays.”8

By 1958, his drawings were more caricaturelike and later became “geometric and then almost abstract”8(p219) in keeping with Peake’s inexorable deterioration of visuospatial function. In this respect, his case is comparable with those of Willem de Kooning17 and William Utermohlen,18 in whom similar changes were noted in artistic ability with advancement of probable Alzheimer disease, likely arising from involvement of posterior isocortical association areas, with resultant deterioration of visuospatial function.

Although problems with memory were apparent by 1960, Peake’s “attention span had become so short that he could no longer read a story and retain the idea for an illustration long enough to draw it.”8(p223)

Thus, both cognitive domains predominantly affected in DLB were especially involved. Conversely, a tenuous preservation of insight was captured in drawings of figures in dunce caps or with pointed heads, often with expressions of fear and apprehension etched with an economy of strokes (Figure 2A), distinguishing him from artists diagnosed as having probable Alzheimer disease.18

Sketches by Mervyn Peake made late in the course of his dementia. A, Figure with dunce cap, a repetitive theme. B, Animal-like floating creatures surround a tormented individual, drawn while Peake was suffering from visual hallucinations. Published with permission from the Mervyn Peake estate.

Sketches by Mervyn Peake made late in the course of his dementia. A, Figure with dunce cap, a repetitive theme. B, Animal-like floating creatures surround a tormented individual, drawn while Peake was suffering from visual hallucinations. Published with permission from the Mervyn Peake estate.

Peake’s parkinsonian signs and symptoms have been well documented in biographies.8 In his mid 40s, he developed shaking of the hands and was tentatively diagnosed as having Parkinson disease after evaluation at the National Hospital, Queen’s Square (London, England).5 

The shaking “spread to his legs as well as his hands” and exhibited “remorseless progression” over the years.8(p213) The beneficial effects of L-dopa in the management of Parkinson disease were not discovered until the 1960s, and so treatment in the preceding decade was limited to the use of anticholinergic medications and experimental surgical pallidotomy. Later photographs of Peake reveal the stooped posture and inscrutable expression characteristic of parkinsonism.

Peake was tormented by recurring visual hallucinations, the early development of which—soon after the onset of his other symptoms—is further suggestive of DLB. Many well-formed and detailed examples are evident in his sketches (Figure 2B). These hallucinations, together with the paranoid delusions he experienced while in the hospital, were incorporated into poetry composed during his institutionalization:

Other poems, perhaps indicating auditory as well as visual hallucinations, and letters to his wife reflecting his frightening delusions, are further discussed in a recent biography.5

By the time he entered his sixth decade, Peake “was with us only in flashes, and those flashes were often over before we had grasped what he had said, or could reply. Most of the time he sat in silence, head bowed.”20(p37) A remarkable example of the striking fluctuation in his mental status follows:

After dinner he was sitting humped on the sofa when suddenly he made a motion towards Maeve, and as she leaned over him he indicated that he wanted some paper and something to draw with. My wife produced a sheaf of quarto typing paper and placed a ball-point pen in his swollen hand. We went on talking while he sat with the sheaf of white paper on his knee. . . . Then I noticed that his hand had ceased its normal shaking, and that he was sitting upright with the paper held firmly, concentrating. For more than an hour, he seemed to lose touch with his illness altogether. He covered page after page with wonderful and preposterous beasts, leaping, snarling, laughing, cavorting. As soon as one was finished he turned to a fresh page and drew another. We self-consciously kept up our conversation, although all three of us were watching him in amazement. He appeared unaware of our presence. And then the spell was broken. He dropped the pen, and his hand began to shake again.”20(pp37-38)

This is a compelling description of a dramatic fluctuation in cognition and alertness, which is typical of DLB and not other forms of dementia, such as Alzheimer disease. There are abundant examples of similar observations in accounts of his illness.5,7,8

Several features supportive of a diagnosis of DLB are also apparent in biographical material of Peake’s neurobehavioral decline. Repeated falls were observed, as “his sense of balance would suddenly desert him, and he would lurch and fall against objects and people.”21(p204) Moreover, there is frequent reference to neuroleptic sensitivity, since “although the doctors gave him tranquilizers, the tremors in his hands and legs increased.”8(p218)

Thorazine and chlorpromazine were the most common neuroleptics used in the 1950s and 1960s. His wife often ironically observed that the medications doctors gave him only seemed to make him worse.8 One of his physicians also noted that some of the treatments “aggravated the parkinsonism, rather than improved it.”21 Worsening confusion and exacerbation of parkinsonism are common consequences of neuroleptic use in DLB.12,15

Peake’s symptoms developed at a younger age than most patients with DLB, although the duration of his illness was more in keeping with the diagnosis. Throughout his institutionalization, he was victim to systematized delusions and hallucinations.

His occasional bouts of depression intensified over time. In addition, rapid eye movement sleep behavior disorder may have been present, since Peake was plagued by nightmares and “a restless nervous energy that prevented him from sleeping at night.”8(p212) However, the published material does not allow for verification of this possibility.

Previous speculation has centered on the possibility that Peake suffered from postencephalitic parkinsonism,21 but such an explanation is dubious on several counts. The pandemic of von Economo encephalitis (encephalitis lethargica, or “sleeping sickness”), which was first described in 1916 and dwindled to obscurity by 1929, was associated with a syndrome of parkinsonism observed to manifest up to 4 or 5 years after the acute illness.

There is no reliable record of acute encephalitis in Peake, and by the 1940s, cases of postencephalitic parkinsonism had become exceedingly rare.22 Moreover, no instances of oculogyric crises or convulsive movements, dramatic features typical of postencephalitic parkinsonism, were ever reported to occur in Peake. Finally, progressive dementia was not a prominent part of the clinical picture in the postencephalitic syndrome, which was generally limited to chronic parkinsonian phenomena.22

Mervyn Peake’s deterioration was a mystery at the time, but in retrospect, his progressive neurobehavioral decline associated with parkinsonism, visual hallucinations, and cognitive fluctuations likely represents one of the earliest recognizable, and possibly best-documented, historical cases of DLB. There is abundant biographical evidence that he exhibited all of the core features (only 2 of which are required for a probable diagnosis of DLB), as well as most of the ancillary features supportive of the diagnosis.

The burgeoning popularity of Peake’s work serves as a testament to his brilliant expressions of imagination. That such a gifted artist succumbed to a dementing illness with a predilection for attention and visuospatial function is particularly tragic.

His later work stands as a unique and defiant record of his struggle with the disease. Recognition of the likely cause of his affliction as his popularity continues to grow may ultimately serve to heighten awareness of this distinct and devastating form of dementia.Article Information

Corresponding author and reprints: Demetrios J. Sahlas, MD, MSc, FRCP(C), Department of Medicine, Division of Neurology, University of Toronto, Sunnybrook and Women’s College Health Sciences Centre, 2075 Bayview Ave, A4 21, Toronto, Ontario, M4N 3M5, Canada (e-mail: dj.sahlas@sw.ca).

Dr Sahlas is supported by a joint personnel award from the Canadian Institutes of Health Research, Ottawa, Ontario, and the Heart and Stroke Foundation of Canada, Ottawa.

I would like to thank Sebastian Peake, the artist’s son, and the Peake family for their interest as well as for their kind permission to reproduce the poetry and artwork contained herein.

The helpful staff at the Merril Collection, Toronto Public Library, is gratefully acknowledged. Special thanks are extended to Sandra E. Black, MD, for a critical review of the manuscript.References1.

Weiner MF Dementia associated with Lewy bodies.  Arch Neurol.1999;56:1441-1442.Google Scholar2.Holmes  CCairns  NLantos  P  et al Validity of current clinical criteria for Alzheimer’s disease, vascular dementia and dementia with Lewy bodies.  

Br J Psychiatry.1999;174:45-50.Google Scholar3.Kosaka  KYoshimura  MIkeda  KBudka  H Diffuse type of Lewy body disease.  Clin Neuropathol.1984;3:185-192.Google Scholar4.Not Available Gormenghast [videotape].  London, England: British Broadcasting Corp; 2000.5.Yorke  M Mervyn Peake: My Eyes Mint Gold: A Life.  New York, NY: The Overlook Press; 2002:258-259, 286-289.6.Jones  L A reverie of bone.  New Worlds.1967;151:51-54.Google Scholar7.Batchelor  J Mervyn Peake: A Biographical and Critical Exploration. 

London, England: Gerald Duckworth & Co Ltd; 1974.8.Winnington  GP Vast Alchemies.  London, England: Peter Owen Publishers; 2000.9.Lewy  FH Paralysis agitans, I: pathologische anatomie.  In: Lewyandowsky  M, ed.  Hadbuch der Neurologie. Berlin, Germany: Springer; 1912:920-933.Google Scholar10.Galvin  JELee  VM-YTrojanowski  JQ Synucleinopathies.  Arch Neurol.2001;58:186-190.Google Scholar11.McKeith  IGGalasko  DKosaka  K  et al Consensus guidelines for the clinical and pathological diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop.  

Neurology.1996;47:1113-1124.Google Scholar12.McKeith  IGPerry  EKPerry  RH Report on the second dementia with Lewy body international workshop: consortium on dementia with Lewy bodies.  Neurology.1999;53:902-905.Google Scholar13.McKeith  IGCalard  CGPerry  RH  et al Prospective validation of consensus criteria for the diagnosis of dementia with Lewy bodies.  Neurology.2000;54:1050-1058.Google Scholar14.McKeith  IGO’Brien  JTBallard  C Diagnosing dementia with Lewy bodies.  Lancet.1999;354:1227-1228.Google Scholar15.McKeith  IG Dementia with Lewy bodies.  

Br J Psychiatry.2002;180:144-147.Google Scholar16.Gibb  WRGEsiri  MMLees  AJ Clinical and pathological features of diffuse cortical Lewy body disease (Lewy body dementia).  Brain.1987;110:1131-1153.Google Scholar17.Espinel  CH de Kooning’s late colours and forms: dementia, creativity, and the healing power of art.  Lancet.1996;347:1096-1098.Google Scholar18.Crutch  SJIsaacs  RRossor  MN Some workmen can blame their tools: artistic change in an individual with Alzheimer’s disease.  Lancet.2001;357:2129-2133.Google Scholar19.

Peake  M A Reverie of Bone.  London, England: Bertram Rota; 1967.20.Mullins  E How many miles to Babylon?  Mervyn Peake Review.1985;19:37-81.Google Scholar21.Watney  J Mervyn Peake.  London, England: Michael Joseph; 1976.22.Dickman  MS von Economo encephalitis.  Arch Neurol.2001;58:1696-1698.Google Scholar

Signs Of LBD, Diagnosis Of Lewy Body Dementia, Plus Vaccine.

Lewy Body Dementia

Please see foot of this post below for info on research Lewy Body Dementia vaccine.

The word “dementia” describes a condition affecting a person’s memory and thinking that is a decline from how he or she used to function and that is severe enough to affect day-to-day life.

Alzheimer’s disease dementia and Lewy body dementia are the two most common types.

Lewy body dementia gets its name from the abnormal protein clumps that are seen on autopsies of the brains of people with Lewy body dementia.

The protein alpha-synuclein – a protein found in the brain, not one you eat – clumps into spheres called Lewy bodies which can be seen using a microscope.

These are named after F. H. Lewy, the person who first described them.

The diagnosis Lewy body dementia is an umbrella term that includes two different conditions: dementia with Lewy bodies and Parkinson’s disease dementia.

In dementia with Lewy bodies, a person develops memory and thinking problems before or at the same time as he or she develops movement problems that resemble Parkinson’s disease.

In Parkinson’s disease dementia, a person who has experienced Parkinson’s disease movement problems for years then also develops trouble with memory and thinking.

These two conditions share many of the same features.

In addition to memory and thinking problems and movement problems, people with these conditions can have fluctuations in their alertness and concentration, hallucinations and paranoia, acting out dreams during sleep (something called REM sleep behavior disorder), low blood pressure with standing, daytime sleepiness and depression, among other symptoms.

Diagnosis is important

Getting the correct diagnosis is critical for patients and families.

While no one wants to hear that they have a disease that currently can’t be cured, patients and families often feel relief that they finally have an explanation for what’s happening.

The diagnosis of Lewy body dementia is often missed due to lack of awareness by physicians, patients and families.

Even for people eventually receiving a diagnosis of Lewy body dementia, research shows their first diagnosis is commonly incorrect.

In that study, 26 percent of people later diagnosed with Lewy body dementia were first diagnosed with Alzheimer’s disease and 24 percent were given a psychiatric diagnosis like depression.

Knowing the correct diagnosis lets patients and families connect to resources such as the Lewy Body Dementia Association, an organization dedicated to helping people living with this disease.

The organization provides education on Lewy body dementia, helps patients and families know what to expect, links patients and families to support and resources and connects them to research opportunities.

Once a diagnosis is made, physicians can also suggest potentially helpful treatments.

Medications can include carbidopa/levodopa (Sinemet®), a drug that helps with slow movements, and cholinesterase inhibitors, which are drugs developed for Alzheimer’s disease that may also help people with Lewy body dementia.

Avenues for research

There is a great deal that we still need to learn about the Lewy body dementias.

Increasing research is a priority of the National Institutes of Health.

Earlier this year, experts published new criteria for the diagnosis of dementia with Lewy bodies, aiming to improve accurate diagnosis.

There are also currently multiple research studies trying to find drugs to help people with Lewy body dementias, including studies to investigate drugs hoped to improve thinkinghallucinations and walking.

For Parkinson’s disease dementia, a new drug called pimavanserin was approved by the Food and Drug Administration in 2016 to treat hallucinations. 

Vaccine Lewy Body Dementia

Current research studies are testing drugs hoped to improve memory and thinking.

Scientists also hope to learn more about the alpha-synuclein protein clumps in the Lewy body diseases.

Recent vaccine studies suggested that the body might be able to create antibodies against alpha-synuclein.

This could be the first step toward a vaccine to help people with Parkinson’s disease and dementia with Lewy bodies.

If effective, a vaccine would prompt the immune systems of people with these diseases to create antibodies to attack and clear the protein clumps.

With advances in diagnosis and treatment, there is reason for hope.

How To Manage Lewy Body Dementia Symptoms

Lewy Body Dementia UK

HOW TO MANAGE SYMPTOMS WITHOUT DRUGS

As carers we want to try and do as much as we can for our loved ones naturally, without using drugs.

Lewy body dementia is a complex disease that includes physical, cognitive, and behavioral symptoms.

The behavioral symptoms can be the most upsetting and difficult-to-manage parts of the disease. Hallucinations, delusions, aggression, agitation, apathy, depression, and anxiety are common.

Because antipsychotic drugs are especially dangerous for people with Lewy body and could make difficult symptoms even worse, it’s best to first try non-drug treatments before resorting to medication.

Non-drug treatments are also helpful in improving symptoms to potentially reduce medication dosage.

We share 10 non-drug ways to help manage and reduce the severity of Lewy body dementia symptoms.

These suggestions should only be used if the situation is safe. If anyone is in danger of physical harm, immediately get medical help.

1. Tolerate behavior that doesn’t cause harm, focus on reassurance and distraction
People with Lewy body dementia often experience hallucinations or delusions that cause strange behavior or false accusations.

But if their behavior isn’t aggressive or harmful, they don’t seem to have any physical pain or discomfort, and they’re not upset to an extreme degree, one solution is to tolerate the behavior and not try to convince them of our reality to stop the behavior.

Instead, respond to their emotions and concerns rather than the facts or exact words. Provide comfort as needed and assure them that they’re safe.

In these cases, the side effects of medication could be far worse than the hallucinations or delusions themselves.

2. Check for physical causes
Sometimes, new behavioral symptoms or a worsening of symptoms could be caused by physical pain or discomfort that the person isn’t able to verbally express or describe.

Common physical issues include severe arthritis, injury, fever, urinary tract infection (UTI)bed sores, and constipation. In some cases, simply being tired, needing to pee, or being hungry can also trigger negative behavior.

When physical pain is well treated, negative behavioral symptoms often decrease.

3. Check for medication side effects
Sometimes medications used to treat Lewy Body dementia symptoms or other common health conditions can increase behavioral problems.

For example, over-the-counter sleep aids, bladder control medications, and drugs used to treat the physical symptoms of Lewy Body (tremors, shuffling walk, stiffness in arms or legs) can cause confusion, agitation, hallucinations, and delusions.

And benzodiazepines, common sedative medications to treat anxiety, can cause increased anxiety or worsen cognitive function in people with Lewy Body dementia.

If your older adult is taking any of these medications, speak with their doctor to find out if there are alternatives that are less likely to affect people with Lewy Body, if it can be eliminated, or if the dose can be lowered.

4. Modify their environment
Clutter, noise, and crowds can contribute to the hallucinations and delusions that typically trigger behavioral challenges.

Reducing clutter and minimizing distracting noise, people, or activity can make it easier for someone with dementia to function, reduce their anxiety, and reduce the chance that their eyes will play tricks on them and cause them to become confused or upset about what they think they’re seeing.

5. Use kind, soothing responses to comfort and calm
Someone who has dementia is no longer able to process logic and reason the way we would. Keep them as calm and happy as possible by avoiding conflicts.

Assuming they’re not hurting themselves or others, this means going along with what they say, not correcting or arguing with them, and not quizzing them about what they remember.

If they’re agitated or concerned, validate their feelings and offer comfort through gentle hugs and engaging activities.

6. Create daily routines and keep tasks simple
Routine and simplicity both reduce the chances that they’ll get angry or agitated.

Having clear structure and consistent routine each day reduces uncertainty and confusion and creates a reassuring rhythm to life.

Break down everyday tasks into smaller steps to make them simpler and reduce frustration.

Overall, focusing on successes rather than pointing out failures (and making success possible) boosts self-esteem and positive feelings.

7. Encourage exercise and physical therapy
To help with the physical symptoms of Lewy body, physical therapy options include cardiovascular, strength, and flexibility exercises, as well as gait training.

Working on general physical fitness is also helpful.

Regular exercise also boosts mood, improves physical health, and often reduces aches and pains. All these factors contribute to reducing negative behavior.

8. Consider speech therapy for swallowing problems
If Lewy Body is causing problems with swallowing, that will interfere with nutrition and cause hunger. Neither is good for someone’s mood or health – and it’s natural get “hangry” (hungry + angry) when we’re too hungry.

If that’s happening, consider speech therapy.

Speech therapists teach techniques that make swallowing easier and safer. They also make recommendations on the types and consistency of food and drinks that help with swallowing.

9. Consider alternative therapies
There are a variety of therapies that may help both the person with Lewy Body as well as caregivers.

Occupational therapy may help someone with dementia maintain skills and improve independence and confidence.

Musicart, or can also reduce anxiety, improve mood, and give a sense of accomplishment.

Pet therapy, or using animals to improve moods and behaviors, boosts mood in many people. Caring for a pet, even if the pet is only visiting, can promote a sense of accomplishment and purpose.

Aromatherapy can boost mood and promote relaxation. Massage therapy can loosen stiff muscles, improve circulation, and promote relaxation.

Individual and family therapy or counseling can also be useful for learning strategies to manage emotional and behavioral symptoms. This can also help caregivers learn techniques to keep calm when conflicts come up.

10. Participate in caregiver support groups
Caregiver support groups have many benefits, including hearing from people in similar situations, being able to vent frustrations, and getting tried-and-true advice.

Getting support is essential for helping you stay as calm as possible in tough situations as well as for finding creative solutions to behavior challenges.

What to do if Lewy body dementia symptoms don’t improve

In some cases, non-drug methods aren’t enough to reduce or manage challenging symptoms caused by Lewy body dementia.

When someone’s behavior is aggressive, dangerous, overly disruptive, or significantly impacts their quality of life, additional help is needed.

The best place to start is the doctor who is treating your older adult’s Lewy body dementia. They’re familiar with their health history, current medications, and any past reactions to medications.

If their doctor isn’t able to help and/or the behavior symptoms are severe, a neurologist, geriatric psychiatrist (also called a geropsychiatrist), or geriatrician who specializes in tough dementia cases may be more helpful.

The Facts About Lewy Body Dementia

Lewy Body Dementia

Please help us to help others, spread the word and share our link for Lewy Body Dementia UK.

Without your help and support we can do very little apart from being just another website on the internet, reaching very few people who may need information and help. But with your help, then others who also really need support too can benefit who may not even know we exist.

You can help us to help others, by sharing our link upon your social network platforms or by bookmarking us to share later.

If we can reach just one person or family suffering at this time, then we have succeeded in our aim and purpose, but we would like to reach so many more than just one.

We thank you deeply and sincerely.

Most people mistakenly believe that all types of dementia share similar symptoms, like the hallmark signs of forgetfulness and confusion that are associated with Alzheimer’s disease (AD). However, Lewy body dementia (LBD), the second most common type after AD, is characterized by unique symptoms that make it difficult to diagnose and treat.

It’s important for family caregivers who are looking after aging loved ones to familiarize themselves with some of the basic signs of various age-related diseases like LBD. Noticing strange new behaviors or quirks early on can help ensure a senior gets the medical attention they need in a timely manner.

Facts About Lewy Body Dementia

  1. It affects millions of seniors.
    According to the Lewy Body Dementia Association, LBD affects approximately 1.4 million individuals and their families. Although it is widely unknown, it is the second most common form of dementia after Alzheimer’s disease.
  2. LBD is not Alzheimer’s disease.
    Many people use the terms “dementia” and “Alzheimer’s disease” interchangeably, but dementia is an umbrella term used to describe a progressive neurological disorder that affects cognitive function, of which there are many different kinds. Alzheimer’s is one form of dementia that is characterized by impaired memory, issues with decision making, trouble with problem solving and difficulty learning new skills.
    There are crucial differences between AD and LBD. For one thing, people with LBD experience unpredictable changes in cognition, regardless of the time of day, whereas people with Alzheimer’s tend to have more trouble in the late afternoon and evening—a symptom known as “sundowning.” Those with LBD also tend to have more visual hallucinations and movement issues, while the hallmarks of AD include memory loss and trouble performing familiar tasks. Furthermore, abnormal alpha-synuclein protein deposits in the brain called Lewy bodies are the hallmark biological marker of LBD, unlike Alzheimer’s, which is caused by the accumulation of beta-amyloid plaques and tau tangles in brain tissue.
  3. LBD is difficult to diagnose.
    As with Alzheimer’s, LBD can only be conclusively diagnosed through an examination of brain tissue after a person has died. However, medical advancements have significantly improved the certainty with which doctors can diagnose living patients.
    Doctors consider the person’s symptoms, interview their family members, perform physical and mental evaluations, obtain a family and personal medical history, and conduct blood tests and brain imaging tests like MRI, PET and CT scans. Symptoms of LBD include problems with depth perception, hallucinations (often visual), delusions, paranoia, Parkinsonism (body stiffness, tremors, trouble walking), and physical issues like heart rate and blood pressure fluctuations, constipation, and fainting spells. To be diagnosed with LBD, an individual must have dementia as well as several of these symptoms.
  4. Medications and LBD can have adverse interactions.
    An accurate, timely diagnosis of LBD is essential to avoiding dangerous medication reactions. Many drugs prescribed to people with Alzheimer’s disease and Parkinson’s disease—especially antipsychotic drugs used to control hallucinations—can have a dangerous effect on people with LBD. Neuroleptic malignant syndrome (NMS) is a neurological disorder brought on by a negative reaction to antipsychotic medications that often occurs in people with LBD. Individuals who develop NMS can experience muscular rigidity, high fever, variable blood pressure and severe sweating. Anticholinergic medications, benzodiazepines and over-the-counter sleeping pills can also have negative effects on seniors with Lewy body dementia.
  5. Parkinson’s disease and LBD are very similar.
    People with LBD often exhibit the same symptoms as those with Parkinson’s disease dementia (PDD). While the two conditions start off differently, their biological underpinnings are closely related, and people with Parkinson’s disease can be diagnosed with either PDD or LBD. The factor that physicians use to distinguish between these two conditions is the timing of the onset of cognitive symptoms. People who develop dementia within a year of their Parkinson’s diagnosis are thought to have LBD, while those whose dementia symptoms start beyond the one-year mark are thought to have PDD.
  6. LBD affects sleep quality.
    Sleep issues and dementia often go hand in hand, but there’s a specific sleep condition that appears to disproportionately affect people with LBD. Studies indicate that as many as two-thirds of LBD patients struggle with REM sleep behavior disorder (RBD). RBD is an ailment that causes movement, speaking and gesturing during the REM (rapid eye movement) stage of sleep, which is usually characterized by paralysis in healthy individuals.
  7. Like most dementias, LBD is unpredictable.
    One of the biggest challenges facing seniors with LBD and their families is the fact that symptoms of the disease tend to worsen and improve erratically. Periods of mental fog, aggressive behavior, movement issues and vivid hallucinations can last seconds, minutes, hours or days. Fluctuations in symptoms can be caused by underlying infections, medications or general progression of the disease. Because of this, it can be nearly impossible to determine how far LBD has progressed in a given individual. However, if the exacerbating factor is capable of being fixed (like a urinary tract infection), then the person with LBD can often return to their prior level of functioning after receiving treatment for the secondary issue.
  8. LBD has no cure.
    There is currently no way to cure or halt the progression of LBD. Instead, treatments are aimed at controlling the cognitive, psychiatric and motor symptoms of the disorder. Those who have been diagnosed with the condition may benefit from palliative care, which focuses on using drugs and nonpharmaceutical treatments to manage symptoms and improve a patient’s comfort and quality of life. For instance, cognitive issues may be addressed with cholinesterase inhibitors, a type of medication that promotes brain cell function by regulating the neurotransmitter acetylcholine. To improve sleep quality and minimize the effects of RBD, careful treatment with clonazepam (Klonopin) or melatonin might be prescribed. Levodopa may be used to mitigate the motor effects of severe Parkinsonism. Occupational, speech and physical therapies are the most common nonpharmaceutical approaches to helping people with LBD manage their condition and maintain day-to-day functionality. Average life expectancy after diagnosis is about 8 years, with progressively increasing cognitive and functional disability.

Lewy Body Dementia Advanced Stage End Of Life Care

Lewy Body Dementia

This post was not the easiest post we have ever put up on our site concerning lewy body dementia.

Right at the beginning when we first started this information website we made the decision that what was needed, and what carers and loved ones wanted, was the bear facts.

No matter how bitter and hard these facts are to read, people just want the hard facts, that’s why they search the internet, they want the truth.

We decided to set up this site to try and put lots of different information together in one place from all over the internet, so that you don’t have to do the searching.

We just wanted to help and make things easier, if only in a small way.

But, it’s not always easy when choosing what does, and does not go up on our site.

We have added the link to the essay below at the foot of this post.

Advanced stage and end-of-life care

Within the palliative care field, there is a concept called “total pain,” which posits that the physical pain experienced by the sick individual is only part of all the suffering caused by the disease.

The illness impacts the entire family, including the caregiver, and all family members may experience suffering in the physical, psychological, social, and spiritual realms.

Unfortunately, physicians rarely bring up end-of-life issues, and less than half of caregivers say they felt prepared for what was to come.

Caregiving in advanced LBD is very challenging and often causes grief, guilt, and existential distress. 

Anticipatory grief, a feeling of mourning for an expected future that has been lost, is common. Most caregivers at this stage of illness never feel that they are doing a good enough job of caregiving, even though they are.

This feeling of guilt is common and is a normal part of the situation that should be acknowledged. Finding support from a friend circle, support group, faith community, or family is essential. Spiritual care or counseling can be very helpful for the caregiver, family, and person with LBD during this time, to help find perspective.

Resilience isn’t only about enduring; it’s about how you are able to recharge and bounce back. It is not about becoming stoic and being able to endure an increasingly burdened situation; rather, it is about your ability to take a break and recover from a challenging situation.

Caregiver burnout is real: it affects health outcomes and leads to higher mortality in caregivers. Burnout is a healthy, adaptive response – an alarm to notify us that something needs to change before this gets worse. It’s important to notice the alarm is ringing, often by practicing mindfulness and self-care.

What does self-care look like?

  • Monitor yourself for signs of caregiver burnout: feelings of exhaustion, irritation, or frustration.
  • Promote your own well-being and seek balance.
  • Think of it as putting on your own “oxygen mask” on before you help others.
  • Build resilience in yourself.
  • Strengthen meaning and purpose in life: remember why you have chosen to care for this person.
  • Connect with yourself and with others: “connection is protection.”
  • Broaden your understanding of your options and choices (self-efficacy): remember that you do have a choice in how you handle situations, even if they are quite difficult.

Palliative care focuses on improving the quality of life of patients and their families facing serious illness. It seeks to affirm life and provide relief from pain, while offering a support system to the family and the patient.

While palliative care services can be particularly essential later in the disease, increasingly it is recognized as having great value earlier in the course of the illness as well. In some studies, palliative care not only improved the quality of life, but also the length of life.

Motor symptoms

Increased slowness and stiffness can cause discomfort and pain and can lead to contractures. Contractures are stiffness or constriction in the muscles, tendons, or ligaments, which prevent the extremity from moving and can cause severe pain.

Falls often increase due to the loss of balance and impulsivity. Supervision around-the-clock becomes necessary. Patients become wheelchair-dependent, and, eventually, many will become bedridden.

There are a number of strategies and techniques to manage these issues:

  • Range of motion exercises (independent or assisted) and massage to help prevent contractures
  • Skin care to ensure skin is clean and dry
  • Change positioning every 2 hours to prevent formation of pressure ulcers
  • Use a gait belt to help walk and transfer safely
  • Mechanical lifts may be needed eventually
  • Use specialized cushions or mattress to prevent pressure ulcers
  • Broda specialized positioning and mobility chairs

Dr. Katz mentioned a 2019 study in the New England Journal of Medicine entitled “Rehabbed to Death,” which found that for a minority of older adults, sometimes a stay in rehab facility after a hospitalization is actually a gateway into a cycle that sends the individual from the rehab facility back to the hospital, then back to rehab, in their final months of life.

The rehab facility in this case is not really able to “rehabilitate” or improve the person’s health or quality of life; more accurately, this should be called “post-acute” care. This can be in part due to payment structures within Medicare and Medicaid that perpetuate the cycle, and it does not generally represent a good quality of life for someone in their final months or weeks.

If your loved one with LBD is hospitalized and you are recommended to send them to sub-acute rehab, it is more of a post-acute care convalescent home rather than a rehab facility. It is something to be aware of.

Communication

In advanced LBD, communication often becomes quite difficult. Voice changes, poor attention, confusion, and word-finding problems are common; impaired communication can also lead to anxiety or agitation.

Some basic tips:

  • Say “yes, and…” – do not argue, even if they are hallucinating or confused.
  • Speak clearly in a calm voice. Tone is often more important than content. Be aware of your tone and facial expressions.
  • Ask “yes” or “no” questions rather than open-ended questions.
  • Maintain eye contact.
  • Offer a reassuring response to frequently asked questions.
  • Find a place and time to talk without distractions.
  • Refer to people by their names instead of pronouns like he or she, to avoid confusion.
  • Talk about one thing at a time.
  • Have patience.
  • When giving instructions, use simple sentences – one piece of information at a time.
  • Recognize what you’re up against.
  • Understand that there will be good days and bad days.

Psychiatric symptoms

Those with advanced LBD often experience hallucinations that may be severe and frightening. Make their neurologist and psychiatrist aware if this is happening.

Behavioral treatments include figuring out or anticipating some possible triggers of the hallucinations or agitation. Cover reflective surfaces, as mirror images are often disturbing to those with advanced LBD. Use a calm tone of voice and avoid confrontation.

At the end of life, hallucinations and psychosis are often so severe that they need medication treatments to help control the symptoms.

The “Five R’s” from the Family Caregiver Handbook:

  • Remain calm.
  • Respond to the person’s feelings.
  • Reassure the person.
  • Remove yourself.
  • Return when you are calm.

Eating and taking medications

Most people will need help with feeding once the disease is advanced. Keep the patient as involved and independent as they can be; finger foods can sometimes allow them to feed themselves for longer than a fork and knife.

Using a plate in a contrasting color can help your loved one to be able to distinguish the food from the plate. Always position them in the upright position when eating, and continue to sit upright for 20-30 minutes after a meal.

Sometimes, those with LBD may refuse to eat due to confusion or hallucinations. They may also experience swallowing problems, such as choking, aspirating food into the lungs, or forgetting how to swallow so that food stays in the mouth.

Recommendations:

  • Do not use thickeners; evidence shows they can actually worsen quality of life.
  • Try carbonated beverages.
  • Try smoothie-consistency drinks; thin liquids are most difficult.
  • Give medications with applesauce or something of a similar consistency.
  • Alternate between a bite of food and a sip of fluid.
  • Have them do a chin tuck when swallowing.
  • Benevolent trickery is sometimes necessary.

Weight loss

Weight loss is expected for those with LBD and is an indication of end-stage disease. Anorexia (lack of eating) and cachexia (weakness and wasting of the muscles) are common. It may seem counter-intuitive, but high-calorie supplements or appetite stimulants will not help with these issues and can actually worsen quality and quantity of life. It can be very hard to see the number on the scale continuing to drop, but it’s not something we can fix – it’s a hallmark of end-stage LBD.

Toileting and incontinence

Give your loved one as much privacy and independence as is consistent with their safety. Give them extra time, assess if they need help, and acknowledge that it may be uncomfortable or embarrassing for them.

Timed voiding – visiting the bathroom at regular times throughout the day – can be very helpful to keep them comfortable and avoid accidents. A pad inside the absorbent briefs can provide extra protection.

The Liberty catheter is an external catheter that goes on at bedtime, which can be helpful. Consider keeping a urinal or commode at the bedside so they do not have to get up to the bathroom overnight.

Dental care

It’s important to continue dental care. Brush teeth twice a day; eventually, they will likely need assistance with this task. A little bit of lemon juice can aid in swallowing and help clean the palate.

Practices to avoid in advanced LBD

In the past, patients with swallowing difficulties were often given thickened liquids. However, recent studies have shown this is actually a harmful practice and should not be continued! Thickened liquids are generally so unpalatable that people often refuse to drink them, which can lead to dehydration and kidney damage.

Feeding tubes are another practice in those with dementia that have now been shown to be counter-productive: artificial nutrition does not prolong or improve life for those with advanced dementia. Rather, assisted oral feedings are the best course.

Post-operative pain and infection after feeding tube insertion, as well as increased likelihood of delirium and repeat surgeries, are some of the reasons why this practice is no longer recommended. Caregiver burden often increases as well.

Fluctuations in alertness

As LBD progresses, individuals will have more and more fluctuations of alertness. This often presents as seeming “zoned out” for a few minutes, and then they come back to normal. It is not a medical emergency, and there is no known treatment.

End-of-life care: Days to weeks

At this point, most people are bedridden. Focus on creating comfortable surroundings. Bring nature indoors, if possible, and play your loved one’s favorite music. Soothing touch, such as a gentle massage, can be relaxing and pleasurable. Smells such as lavender oil can be pleasant as well.

In terms of medications, if Sinemet (carbidopa-levodopa) has been helpful for them, continue it for as long as possible. Reduce other medications to only what is necessary, or will help them remain comfortable.

If liquid formulations of medications are available, switch to those; if the person is unable to swallow, then suppositories may be the best option. Most patients at this stage of LBD will need sedating medications to control the agitation and psychosis that generally increase at the end of life.

Prognostic indicators in LBD

There are certain indicators that provide a prognosis – the likely course or time frame of late-stage LBD. These indicators are used by Medicare to determine eligibility for hospice services, which require an estimate of six months or less of life.

For those with weight loss, these are the same criteria as for “Adult Failure to Thrive,” a different medical diagnosis. The main criteria for hospice eligibility are a body mass index (BMI) of less than 22, and a score on the Palliative Performance Scale (PPS) of 40 or less, indicating someone who is mainly in bed and needs assistance with activities of daily living (ADLs).

These two factors are strong indicators that someone is likely within 6 months of the end of their life, and should receive hospice services to ensure the best care.

For those with dementia, hospice eligibility requires at least one of the following in the past 12 months:

  • Aspiration pneumonia
  • Pyelonephritis (kidney infection)
  • Septicemia (infection of the blood)
  • Stage 3 or 4 pressure ulcers
  • Recurrent fever
  • 10% weight loss (or greater) in the past 12 months
  • Albumin less than 2.5 mg/dL (measurement of protein indicating malnutrition) and greater than or equal to 7C on the FAST scale:
  1. Speaks 5-6 words per day
  2. Speaks only 1 word clearly per day
  3. Cannot walk without personal assistance
  4. Cannot sit up without personal assistance
  5. Can no longer hold up head independently

What do people die of in LBD?

There have been very few studies on this question. For most, aspiration pneumonia – a lung infection caused by inhaling food or oral secretions into the lungs – is the cause of death. Sometimes aspiration pneumonia leads to sepsis, an extreme reaction by the body in response to a systemic infection, which can be fatal.

Reduced mobility, falls, or fractures can also be life-threatening. Failure to thrive, which is the severe weight loss associated with late-stage dementia, is another cause as well.

Hospice care: Role of the neurologist

Right now, only about 5% of patients with LBD will receive hospice services

It’s important that more LBD patients and families receive the help and care available to them through hospice. Your loved one’s neurologist can still play a helpful role even after hospice is involved, by helping to communicate and coordinate with the hospice team and educating them about the specific needs of LBD patients, such as medications that are commonly used in a hospice setting, but which should NOT be given to those with LBD.

Medications to avoid in hospice patients with LBD:

  • Haloperidol (Haldol)
  • Metoclopramide
  • Phenergan

Alternative medications that are safer to use in LBD are quetiapine (Seroquel) for agitation/psychosis and ondansetron (Zofran) for nausea.

The Advance Healthcare Directive is a legal document that allows an individual to spell out their preferences for life-saving medical interventions and end of life care, ahead of time, and designates an individual who is empowered to make healthcare decisions on their behalf, if necessary. This requires that you speak with the loved one about their wishes while they are still able to consider such decisions.

Most people who go into cardiac arrest are not able to be resuscitated successfully; only 5% of people with dementia who undergo CPR survive. It is not like the movies; the chances of a good recovery are actually quite small, contrary to the portrayal in the movies. The POLST (Physician’s Order for Life Sustaining Treatment) is a form, usually yellow, that tells emergency professionals what to do in a crisis when the patient cannot speak for themselves.

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