Lewy Body Dementia: What Families Should Know

A family in Troy told us their father had been seeing a cat in the upstairs hallway for the better part of a year. Not every day. He knew the cat was not really there and was not frightened by it, so nobody mentioned it to a doctor. What finally sent them to a neurologist was something else. He had started shouting and swinging his arms in his sleep, hard enough that his wife moved into the spare room.

Those two things turned out to be the same illness.

Lewy body dementia is the second most common form of neurodegenerative dementia after Alzheimer's, and the Lewy Body Dementia Association puts the number affected in the United States at more than a million people. Most families have never heard the name until a doctor says it to them. It is also the dementia we most often see identified late, because the early years look like something else.

Two illnesses that share one name

Lewy body dementia is an umbrella over two diagnoses, and the difference between them is mostly a question of what showed up first.

Dementia with Lewy bodies. Thinking changes come first. Movement changes, if they come at all, come later.

Parkinson's disease dementia. Movement changes come first and lead to a Parkinson's diagnosis. If cognitive changes follow more than a year after the movement symptoms, the diagnosis becomes Parkinson's disease dementia.

Underneath, they are the same biology. Both involve abnormal deposits of a protein called alpha-synuclein, named Lewy bodies after the neurologist who described them in 1912. Over time, people with either diagnosis develop a similar mix of thinking, movement, sleep and behavioral symptoms. The label says more about the order the symptoms arrived in than about what the care will eventually ask for.

Why it does not look like Alzheimer's at the start

Families come in expecting memory to be the headline, because that is the dementia everyone knows. In Lewy body dementia, memory is often not where the trouble starts.

What the National Institute on Aging describes instead is trouble with attention, with visual and spatial judgment, and with the kind of thinking that holds several steps in mind at once. A parent may misjudge a curb or a doorway, or lose the thread of a task that used to be automatic, while recalling last Thanksgiving perfectly well.

Four features tend to travel with it:

Visual hallucinations, often early. These are usually detailed and realistic. Children, animals, a person standing in a doorway. Many people know the images are not real, which is part of why families do not report them.

Cognitive fluctuations. Attention and alertness swing, sometimes day to day and sometimes within a single afternoon.

Parkinson's-like movement changes. Slowness, stiffness, a shuffling walk, a stooped posture, tremor, trouble with balance.

Acting out dreams in sleep. The clinical name is REM sleep behavior disorder, and it is the one that most often precedes everything else.

The good day and the bad day are the same week

Of everything on that list, the fluctuations cause families the most private misery, because they are so easy to read as a character problem.

A mother is sharp on the phone Sunday and cannot follow a conversation Monday. She stares into space all morning and is entirely herself at dinner. A son who visits on the wrong day comes away certain his sister has been exaggerating. And the appointment lands on a good hour, which is how a neurologist can end up with an incomplete picture too.

None of that is willful. Unpredictable swings in concentration, alertness and wakefulness are a defining feature of the illness, and they are hard for a doctor to catch inside a twenty minute visit.

Two things help. Keep a plain log for two or three weeks before an appointment, with the date, the time of day and what the person could and could not do. And when you describe your parent to a clinician, describe the range rather than the average. Not "she gets confused sometimes," but "on Tuesday she could not find the bathroom in her own house, and on Thursday she balanced her checkbook."

The sleep symptom that arrives years ahead

REM sleep behavior disorder deserves its own paragraph because of when it shows up.

Most of us are effectively paralyzed while dreaming. In this disorder that mechanism fails and the person acts the dream out. Yelling, kicking, punching, falling out of bed. The dreams often involve being chased or attacked, which is why the movements are so forceful. Spouses get hurt. So do the people dreaming.

It can appear years before any thinking or movement symptom, and it is worth reporting on its own, long before anyone is thinking about dementia. It is also one of the more treatable pieces of the picture. The LBDA describes it as often quite responsive to treatment, which cannot be said about much else here.

If a parent is thrashing in their sleep, that is a medical finding, not a quirk. Say it at the next appointment.

Why the diagnosis has to travel with the person

This is the part we would ask every family to take away, because it is the one with real safety in it.

People with Lewy body dementia can be severely sensitive to antipsychotic medication as a class. The Lewy Body Dementia Association states that up to half of people with LBD treated with any antipsychotic medication may have a severe reaction, including worsening confusion, heavy sedation, and increased or possibly irreversible parkinsonism.

The same warning carries an instruction we would be wrong to leave out, because a warning with nothing to do about it is just fear. If severe fever or muscle rigidity appears, contact a doctor immediately. The LBDA describes that combination as potentially life-threatening, and as something treated by stopping the medication. Fever and rigidity together, in someone recently started on a new medicine, is a same-day call and not a wait-and-see.

We want to be careful about what that does and does not mean.

It does not mean a family should refuse a category of medicine or overrule a physician. Prescribing is a medical decision, made by a doctor who knows the whole person and weighs the risk of a medication against the risk of leaving a frightening or dangerous symptom untreated. Sometimes that lands on treating. The call belongs to the prescriber, and it should.

It also does not mean stopping anything on your own. The National Institute on Aging says plainly that a person with LBD should talk with their doctor before reducing or stopping a medication, and that applies to a family reading an article like this one and feeling alarmed. Raise it, do not act on it.

And antipsychotics are not the only class worth naming to a prescriber. The LBDA lists several others that may cause sedation, movement problems or confusion in LBD: benzodiazepines, anticholinergics such as antispasmodics, older antidepressants, some medications used for parkinsonian symptoms, and certain common over-the-counter medicines, including the antihistamines found in many sleep aids and allergy products. That last one matters because nobody thinks of it as a drug. It sits in a kitchen drawer and gets handed over without a thought.

What it does mean is that no prescriber should ever be making that call without knowing the diagnosis. And in practice, the diagnosis is exactly what goes missing at the moment it matters most, because the moment it matters most is usually an emergency.

So carry it. Not in someone's memory, in writing.

• A card in the wallet and a note in the phone, naming the diagnosis and the diagnosing physician
• The same information given at every point of contact, including the emergency department, urgent care, a surgeon, an anesthesiologist, a dentist, a new specialist, a walk-in clinic on a holiday weekend
• Before any surgery, an actual conversation with the anesthesiologist, arranged in advance rather than mentioned on the morning. The LBDA is direct about why: people with LBD often respond to certain anesthetics and to surgery with acute confusion or delirium, and can have a sudden significant drop in functional abilities that may be permanent. It also notes that regional or spinal anaesthesia is less likely to produce postoperative confusion than a general, which is a question for the anesthesiologist rather than a decision for the family. And if anyone tells you to stop all medications before surgery, check that instruction with the treating doctor rather than following it flat
• A current list of everything the person takes, prescriptions, over the counter medicines, vitamins and supplements included
• A habit of reporting any sudden change after a new medication is started, rather than waiting for the next scheduled visit

The National Institute on Aging makes the same point plainly: certain medications can worsen LBD symptoms, so responses to medication are worth paying close attention to and telling a doctor about.

That is a job a family can do, and it does not require anyone to practice medicine.

What the daily care actually asks for

Some of what this illness needs is ordinary and some of it is specific.

Falls are the specific one. Stiffness and a shuffling gait already narrow a person's margin, and the illness also disturbs the automatic systems that regulate blood pressure, so standing too quickly can bring on lightheadedness or a faint. Slow transitions, good lighting, clear floors and unhurried help matter more here than in most dementias.

Hallucinations ask for a response rather than a correction. Arguing a person out of what they are plainly seeing does not work and usually frightens them. When the images are not distressing they often need nothing. When they are, calm company and a change of room are the first tools, and a doctor is the next.

Then there is the unglamorous list from the autonomic side of the illness: constipation, trouble regulating body temperature, urinary incontinence, poor sense of smell, daytime sleepiness. Later on, swallowing can become difficult and eating slows down.

Nearly all of that is care that runs on attention and time rather than equipment. It is a large part of why we keep our Troy homes small.

Where Michigan families get help

Michigan is better served on this than most states, and almost nobody knows it.

The University of Michigan runs the Rinne Lewy Body Dementia Initiative through the Michigan Alzheimer's Disease Center in Ann Arbor, and it hosts support groups built for this diagnosis rather than for dementia in general. Two meet on Zoom on the second Tuesday of each month, at 2 p.m. for people living with LBD and 3 p.m. for caregivers, plus an evening caregiver group at 6 p.m. The virtual groups require registration so they can send the link. An in-person group meets at the center in Ann Arbor on the fourth Thursday at 2 p.m., no registration needed. First-time attendance goes through Renee Gadwa at [email protected].

For a family in Troy, the Zoom groups are the realistic ones, and they are why we mention this at all. A caregiver who cannot leave the house can still sit in a room with twenty people who know exactly what a bad Tuesday looks like.

The Lewy Body Dementia Association staffs a helpline, the Lewy Line, at 800-539-9767. The Alzheimer's Association helpline runs 24 hours a day at 800-272-3900 and covers this diagnosis, not only Alzheimer's. For the broader practical picture in this county, we wrote separately about where Oakland County families find dementia help.

What the course looks like

Lewy body dementia is progressive and there is no cure. The National Institute on Aging gives an average of five to seven years from diagnosis to death, with a range from two to twenty, and notes that the pace varies enormously from person to person. Some symptoms do respond to treatment for a period of time.

Families ask early whether this diagnosis means moving twice, once into care and again later into something else. Whether a particular home can meet a particular person's needs comes down to an assessment of that person, not to the name of the illness. Falls risk, swallowing, sleep, how the day actually goes. That is a conversation to have honestly at the front end rather than to discover a year in.

The short version

Lewy body dementia is common, it looks nothing like the dementia most people picture, and it is frequently identified late. If a parent is acting out dreams, seeing things that are not there, or swinging between sharp and absent inside the same week, those belong together in front of a doctor rather than filed separately as odd.

And once the diagnosis exists, make sure it never travels alone. Every prescriber, every time, in writing. It is the single most useful thing a family can carry.