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. 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
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.
Use of a standardized assessment boosted diagnosis by one-third
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.
Management guidelines improved health outcomes for patients and caregivers
As a first step to improving care, DIAMOND-Lewy researchers assessed what barriers hinder diagnosis. In two UK 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.
The DIAMOND DLB Tool Kit
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:
DIAMOND-Lewy research programme Toolkits & Videos
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?
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AlzForum.Org: New Tool Kit Helps Physicians Recognize and Manage Lewy Body Dementias