Lewy body dementia, the second-most-common progressive dementia after Alzheimer’s disease, receives relatively little attention and few research dollars, and often goes undiagnosed. Comedian Robin Williams, perhaps the most famous person to have died from this disease, was diagnosed only after his death, when neuropathologists found the protein- and lipid-laden Lewy body deposits throughout his brain and brainstem. “I had never even heard of the disease,” his widow, Susan Schneider Williams, told the audience at the International Lewy Body Dementia conference, held June 24–26 in Las Vegas. For her husband, as for many patients with the disorder, the diagnostic uncertainty led doctors to prescribe medications that worsened his disease. The lack of a clear diagnosis also made it difficult to comprehend the multitude of distressing symptoms, including paranoia, delusions, and the unpredictable fluctuations of his cognitive skills throughout the day. “He thought he was going crazy,” Susan Williams said. “A diagnosis would have helped us cope.”

  • Dementia with Lewy bodies often goes unrecognized.
  • Use of a standardized assessment boosted diagnosis by one-third.
  • Management guidelines improved health outcomes for patients and caregivers.

Could researchers improve the diagnosis and management of DLB by providing more information and clear guidelines to physicians? A U.K. program shows how this might work. In Las Vegas, John O’Brien of the University of Cambridge described the DIAMOND-Lewy research program, a collaboration between Cambridge and Newcastle University that is funded by the National Institute for Health Research. The acronym stands for “improving the DIagnosis And Management Of Neurodegenerative Dementia of Lewy body type.” DIAMOND-Lewy encompasses Parkinson’s disease dementia (PDD) as well as dementia with Lewy bodies (DLB). It provides a checklist to help physicians diagnose and manage DLB and PDD patients. The checklist is based on the current consensus criteria for these diseases (McKeith et al., 2017). In an initial study, use of the checklist improved recognition of Lewy body dementia at hospital clinics in England, and led to some benefits for patients and caregivers over a six-month period.

Researchers in Las Vegas called this an important advance, and one of the most exciting things they heard at the conference. “The DIAMOND-Lewy project is truly impressive in scope and detail. The recommendations are comprehensive, which will surely enable more optimal management of these complex patients,” Bradley Boeve of the Mayo Clinic in Rochester, Minnesota, told Alzforum.

As a first step to improving care, DIAMOND-Lewy researchers assessed what barriers hinder diagnosis. In two U.K. regions, the North East and East Anglia, it took patients more than a year on average and 20 clinical consults to be diagnosed with DLB, as compared with six months and 3.5 clinical consults for other dementias. Half of DLB patients initially received a different diagnosis. Why was diagnosis so difficult? O’Brien and colleagues found that, in part, physicians lacked knowledge about the disease. For example, many doctors did not ask their patients about sleep disturbances, a key early sign of DLB. Another issue, however, was that doctors believed nothing could be done to improve the lives of people with DLB, and were reluctant to make the diagnosis.

To combat the first problem, the DIAMOND Lewy team developed two different tool kits—one to standardize diagnosis of DLB, and the other for PDD (Thomas et al., 2016). They revised the DLB tool kit a year later to reflect newly updated criteria for diagnosing the disorder (Jun 2017 news; Thomas et al., 2018). The DLB and PDD tool kits are freely available, along with a video describing their use. The DLB tool kit provides specific screening questions to help assess whether a patient has any of the four core features of the disease: cognitive fluctuations, REM sleep disorder, visual hallucinations, and parkinsonism. The presence of two or more indicates probable DLB. The tool kit also queries the three indicative DLB biomarkers—abnormal dopaminergic imaging, damage to cardiac nerves, and confirmation of REM sleep problems by polysomnography. One of these plus a core feature likewise indicates probable DLB.

Did use of the DLB tool kit improve diagnosis? In Las Vegas, O’Brien presented unpublished data that suggests it does. In the 18 months prior to adopting the instrument, an average of 4.6 percent of the 4,504 dementia cases seen in the North East and East Anglia regions were labeled DLB. After the tool kit was introduced, DLB diagnoses jumped to 6.2 percent of 2,058 dementia cases seen, a 35 percent increase. By contrast, use of the PDD tool kit did not change diagnoses of this disease, which held steady at about 9 percent of dementia cases. Diagnoses were not confirmed by autopsy.

What about the second need identified by the study—better management of patient health? DLB patients typically fare worse than those with other dementias, such as Alzheimer’s, noted John Paul Taylor of Newcastle University in the U.K. They are admitted to the hospital more often, accumulate higher bills, report lower quality of life, and die sooner. Could standardizing the way physicians manage the disease improve outcomes?

For this, DIAMOND-Lewy researchers developed a separate tool kit aimed at defining the best clinical care. They reviewed the literature on physician care for people with Lewy body dementia, and summarized the treatment measures in 252 statements. These statements dealt with issues such as which medications to prescribe for people with DLB or PDD, which ones to avoid, what dosages to use, and what non-pharmacological interventions might be helpful. Then the researchers convened a panel of 26 experts to evaluate these measures. The panel discussed each statement and voted whether to accept or reject it as a best practice, ending up with 161 consensus care statements in the tool kit. A one-page overview lists the primary management strategies for each symptom, as well as practices to avoid. For example, antipsychotics can hasten disease progression for DLB patients, and dopamine replacement therapy needs to be carefully titrated, as high doses can worsen hallucinations. The evidence underpinning this management tool kit is in press at Lancet Neurology.

To evaluate this management tool kit, the researchers shared it with 11 memory or movement disorder health services in the North East and East Anglia, while 11 other services maintained the usual standard of care. Among the 22 clinics, 127 people newly diagnosed with DLB or PDD were assessed at baseline, three, and six months. Participants were mostly men, with an average age in the late 70s. Eighty-four percent completed the study. Sixty percent were diagnosed with DLB and 40 percent with PDD.

Did the tool kit improve health? Reporting unpublished data, the researchers found no difference on several measures of cognitive and motor function. However, 40 percent of the usual care group were rated as steeply declining on the clinical global impression (CGI) scale over the six months, versus 20 percent of the tool kit group. Caregivers reported a similar impression, with less decline in patients cared for under the new guidelines. In addition, caregivers in the tool kit group self-reported less depression, stress, and perceived emotional burden than those in the control group did. Economically, the tool kit group fared better as well. Medication costs rose, but this was offset by a large drop in healthcare and social care costs, resulting in an overall financial savings.

The findings are encouraging, especially because not every health service that received the tool kit used it effectively, Claire Bamford of Newcastle noted in Las Vegas. Her team interviewed 31 clinicians who took part in the study. Almost uniformly, doctors praised the design of the tool kit, saying they liked how information was highlighted, the different levels of detail available on how to manage each symptom, and guidelines on medication dosage. One doctor called it a “one-stop shop” for Lewy body dementia information. Clinicians expressed high confidence in the quality of the information, and said it made them think more about the whole patient and their health needs, rather than treating each symptom separately.

However, clinics varied widely in how they used the tool kit. Only some integrated it into their practices, while others simply held copies on file. When it was used successfully, it was often because one person at the health service championed the tool kit and took the lead in incorporating it into clinical practice, Bamford said. In ongoing work, the DIAMOND-Lewy team is looking for ways to support use of this tool by clinics and to roll it out more broadly to primary care clinics and inpatient wards. Audience members in Las Vegas suggested that a phone app, which could be easily updated as standards change, might be most effective and user-friendly.—Madolyn Bowman Rogers


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News Citations

  1. DLB Guidelines Get a Makeover

Paper Citations

  1. . Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017 Jul 4;89(1):88-100. Epub 2017 Jun 7 PubMed.
  2. . Development of assessment toolkits for improving the diagnosis of the Lewy body dementias: feasibility study within the DIAMOND Lewy study. Int J Geriatr Psychiatry. 2016 Dec 8; PubMed.
  3. . Revision of assessment toolkits for improving the diagnosis of Lewy body dementia: The DIAMOND Lewy study. Int J Geriatr Psychiatry. 2018 Oct;33(10):1293-1304. Epub 2018 Aug 9 PubMed.

External Citations

  1. DIAMOND-Lewy

Further Reading