Dementia with Lewy bodies, new international guidelines

ROCHESTER, Minn. -- Susan Williams, wife of the late actor and comedian Robin Williams, called Lewy body disease "the terrorist inside my husband's brain. An estimated 1.4 million Americans have dementia with Lewy bodies, making it the second-leading cause of dementia after Alzheimer's disease. A degenerative and ultimately fatal brain disease, dementia with Lewy bodies frequently is misdiagnosed as Alzheimer's or Parkinson's disease.
According to research reported online Wednesday, June 7, in Neurology, the international Dementia with Lewy Bodies Consortium issued new guidelines about diagnosing and treating the disease and called for more clinical trials into the illness.
"The updated clinical criteria and associated biomarkers hopefully will lead to earlier and more accurate diagnosis, and that is key to helping patients confront this challenging illness and maximize their quality of life," says Bradley Boeve, M.D., a co-author of the paper and a neurologist at Mayo Clinic, which sponsored an international dementia with Lewy bodies conference with roughly 400 clinicians, scientists, patients and care partners in Fort Lauderdale, Florida, in December 2015.
Yesterday's report is the first publication to come out of the conference. The previous consensus guidelines were published in 2005. By weighting clinical signs and biomarkers, the new recommendations give detailed guidance to help diagnose dementia with Lewy bodies. Its' symptoms include:
  • Cognitive problems: Confusion, reduced attention span and memory loss
  • Fluctuating attention: Drowsiness, staring into space, daytime naps and disorganized speech
  • Visual hallucinations: Seeing animals or people that aren't there.
  • Sleep difficulties: Physically acting out dreams while asleep and excessive daytime sleepiness
  • Movement disorders: Slowed movement, rigid muscles, tremors or shuffling walk
  • Poor regulations of body function: Dizziness, falls and bowel issues.
  • Depression: Persistent sadness and loss of interest
"Despite dementia with Lewy bodies being relatively common, some physicians and many in the public still have never heard of this disorder," Dr. Boeve says.
Protein deposits -- named after Frederick Lewy who discovered them -- develop in brain cells and are believed to cause some to die and others to malfunction. The new guidelines give treatment recommendations, such as using medications called cholinesterase inhibitors to try to improve cognition by triggering nerve impulses from one brain cell to the next.
"Previously, there has been little agreement on the optimal medication and non-medication approaches for patients and their families," Dr. Boeve notes.
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The report has more than 60 contributors, worldwide experts in their field, including first author Ian McKeith, M.D., of Newcastle University in England and senior author Kenji Kosaka, M.D., of Yokohama City University Medical Center in Japan.
In addition to Dr. Boeve, Mayo Clinic co-authors are Dennis Dickson, M.D.; Tanis Ferman, Ph.D.; Neill Graff-Radford, M.D.; Kejal Kantarci, M.D.; Angela Lunde; Pamela McLean, Ph.D.; Melissa Murray, Ph.D.; and Owen Ross, Ph.D.
The National Institute on Aging and National Institute of Neurological Disorders and Stroke of the National Institutes of Health supported this work, among others. The authors noted conflict of interest disclosures.
About Mayo Clinic
Mayo Clinic is a nonprofit organization committed to clinical practice, education and research, providing expert, whole-person care to everyone who needs healing. For more information, visit mayoclinic.org/about-mayo-clinic or newsnetwork.mayoclinic.org/.

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