Living With Lewy Body Dementia

In 2010, Judy Kauser, then 68, started having pains in her leg. It progressed so much that her family physician suggested the source was a back problem, or sciatica. When steroid medications didn't help, she was sent for injections for inflammation, but the pain continued and worsened, requiring her to use a walker. Her husband, Dennis, says Judy became confused and disoriented, but he and the doctors blamed it on the pain medication.

It would take another two years before Judy was diagnosed with dementia with Lewy bodies.

The Lewy Body Dementia Association estimates the condition affects more than 1.4 million individuals in the U.S. alone. It's the second most common type of progressive dementia after Alzheimer's disease, yet it's often underdiagnosed.

"It's the most common disease you've never heard of," says Dr. James Galvin, a neurology and psychiatry professor and associate dean for clinical research at the Charles E. Schmidt College of Medicine at Florida Atlantic University.

What Is Dementia With Lewy Bodies?

Lewy Body Dementia is an umbrella term that refers to both Parkinson's disease dementia and dementia with Lewy bodies -- the abnormal protein deposits in the brain that can lead to problems with thinking, movement, behavior and mood. While it often resembles Alzheimer's disease, dementia with Lewy bodies involves differing symptoms, according to Galvin, who's also on the board of directors and a member of the scientific advisory board at the Lewy Body Dementia Association.

In Alzheimer's disease, memory loss is more common -- at any earlier point -- than it is in dementia with Lewy bodies. And although Alzheimer's disease can cause problems with walking and balance, those with dementia with Lewy bodies are more likely to exhibit more disabling physical symptoms such as stiffness and hunched posture. Hallucinations, disruptions to the body's nervous system and REM sleep behavior disorder also occur more frequently in early-stage dementia with Lewy bodies compared with Alzheimer's disease.

Symptoms of dementia with Lewy bodies include: fluctuating cognition; recurring hallucinations; spontaneous symptoms of Parkinson's disease such as stiffness and trouble walking; REM sleep behavior disorder; sensitivity to antipsychotic medications; and low levels of dopamine in brain images. However, not all symptoms always appear at first.

Other signs of the condition include repeated fainting, loss of consciousness, difficulties with simple body functions like digestion, hallucinations, inability to perceive visual information like the surrounding environment and other psychiatric problems.

"The two things that are most easy to test are memory and movement, which leads people to often initially be diagnosed with Alzheimer's disease or Parkinson's disease," Galvin says. "That's why it's very confusing and often not diagnosed until later in the course of the disease. Until recently, there's really been no other good way to measure those symptoms."

Any of the symptoms can appear in any order, he adds. If movement symptoms are first, a doctor might make a Parkinson's disease diagnosis. Or if memory problems are most visible, the patient might be diagnosed with Alzheimer's disease. If psychiatric symptoms are most noticeable, someone may be diagnosed with a bipolar disorder or schizophrenia rather than dementia with Lewy bodies, Galvin says, adding: "Of course, all of those diagnoses are wrong."

Risk Factors

In a study published in the journal Neurology in 2013, Dr. Bradley Boeve, chair of behavioral neurology at the Mayo Clinic in Rochester, Minnesota, and colleagues reported that there are key risk factors associated with dementia with Lewy bodies.

They studied 147 men and women with the disease and compared them with 236 people with Alzheimer's disease. Of the 19 risk factors included in the study, the researchers determined that depression and low caffeine intake were not only risk factors for both Parkinson's disease and Alzheimer's disease, but also increased the risk for dementia with Lewy bodies more so than in either disease. Another recent study helps explain the caffeine phenomena. In July, an analysis published in the Journal of Alzheimer's Disease proposed that moderate and regular coffee consumption may have neuroprotective effects. The benefits may be explained by the coffee's ability to activate adenosine A2A receptors, which are known to improve damage in the brains of people with Alzheimer's disease.

Other risk factors for dementia with Lewy bodies include a personal medical history of anxiety, depression and stroke, a family history of Parkinson's disease and carrying a genetic marker for dementia: APOE4.

The Mayo Clinic suggests that men who are over age 60 and have a family member with the condition have a greater risk for developing the disease. Still, diagnosing the disease remains a problem, Galvin says.

Diagnosis and Treatment

"It's a difficult diagnosis because there's no standardized way to collect biomarkers," Galvin continues, adding that other diseases must first be ruled out.

Tests may include a neurological exam that looks at the body's reflexes, eye movements, balance and sense of touch, as well as a memory test to determine whether it's Alzheimer's disease or dementia with Lewy bodies. Brain scans can rule out other conditions, such as vitamin deficiencies, Alzheimer's disease or strokes.

"If you don't have the right diagnosis, it's very difficult to balance how the medicines are used," Galvin explains. "For example, medicines that improve movement can worsen hallucinations, and medications that treat hallucinations can worsen movement."

If a doctor doesn't take all that into account, someone may be misdiagnosed and treated with psychiatric medications that have been proven to worsen dementia symptoms, Galvin adds. That's why the U.S. Food and Drug Administration warns that the drugs be avoided in those with the disease.

In the case of the Kausers, Judy was initially diagnosed with mild cognitive impairment -- or MCI -- which describes the fine line between a person's normal memory or thinking and dementia. When that happened, Dennis turned to a caregiver support group that helped him understand MCI and how he could help his wife, who once taught grades from prekindergarten to high school special education.

Trips back and forth from the couple's home in Missouri to their winter home in Sarasota, Florida, confused her until they permanently snow-birded south for one-floor living and ease of access to the doctors she needed to address her symptoms. She'd tell Dennis, "This is our house, but it doesn't feel like our house," Dennis, now 73, remembers. "She was talking to her mother and father, who had been dead for a long time."

The repeated hallucinations led the couple to another specialist, who diagnosed Judy with dementia with Lewy bodies. By 2013, Judy was on the max doses for Aricept and Namenda -- two of only four drugs approved by the U.S. Food and Drug Administration to treat Alzheimer's disease and dementia.

"Generally she was functioning, but gradually it got worse," Dennis explains. "We could still do things, but everything got slower."

Life After Diagnosis

"It's a faster progressing disease than Alzheimer's disease," Galvin says. The average duration is about five to eight years after the onset of obvious symptoms, but patients can live with dementia with Lewy bodies for up to 20 years before succumbing to the disease.

In 2014, Dennis enlisted the help of a registered nurse for night care, since Judy's unrest was keeping him awake at night. But the days were trying, too, and he ultimately placed her in an assisted living facility that year -- which he calls a difficult decision. He visited nearly every day and continued seeing a caregiver support group until she passed away in March 2015 at age 72.

Laura Gitlin, a professor and director of the Center for Innovative Care in Aging at The Johns Hopkins School of Nursing in Baltimore, says there are proactive measures families can take to make coping with dementia with Lewy bodies a little easier.

"A family member can be caring for a person for upwards of 20 years. The effects of these diseases are pretty profound," says Gitlin, author of "A Caregiver's Guide to Dementia: Using Activities and Other Strategies to Prevent, Reduce and Manage Behavioral Symptoms."

Families need support, education about the disease and the disease process and the strength to ask for help from other family members when needed. She recommends families work together to ensure finances regarding the patient's long-term care are in place, and to make sure someone takes over power of attorney of health and legal decisions.

"Having the discussion early on with the person who is affected and other family members as to what health care decisions should and can be made ... is very important," Gitlin says, adding that these early discussions can help the family evolve as the disease progresses.

For Dennis, going to a caregiver group was just as important as visiting Judy. To find resources for such support, visit the Lewy Body Dementia Association's website, Eldercare Locator or the Family Caregiver Alliance.

Samantha Costa is a Health + Wellness reporter at U.S. News. You can follow her on Twitter, connect with her on LinkedIn or email her at scosta@usnews.com.