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From the Institute for Ageing and Health (Drs. McKeith, O’Brien, E.K. Perry, Burn, Kalaria, Kenny, Mukaetova-Ladinska, and R.H. Perry), University of Newcastle upon Tyne, UK; Department of Neuropathology (Dr. Dickson), Mayo Clinic Jacksonville, FL; Division of Pathology (Dr. Lowe), School of Molecular Medical Sciences, University of Nottingham, UK; Department of Neurology (Dr. Emre), Istanbul University, Istanbul Faculty of Medicine, Turkey; UBC Division of Neurology (Dr. Feldman), University of British Columbia, Vancouver, Canada; Department of Neurology (Dr. Cummings), UCLA Medical School Alzheimer’s Disease Center, Los Angeles, CA; Parkinson’s Disease Research, Education and Clinical Center (Dr. Duda), Philadelphia VAMC, PA; Memory Disorders Program (Dr. Lippa), Drexel University School of Medicine, Philadelphia, PA; Section of Geriatric Psychiatry (Dr. Aarsland), Stavanger University Hospital, Norway; Department of Geriatric & Respiratory Medicine (Dr. Arai), Tohoku University School of Medicine, Miyagi, Japan; Wolfson Centre for Age-Related Diseases (Dr. Ballard), London, UK; Department of Neurology (Dr. Boeve), Mayo Clinic, Rochester, MN; Institute of Nuclear Medicine (Dr. Costa), HPP Medicina Molecular, SA, Porto, Portugal; Section of Neurology (Dr. del Ser), Hospital Severo Ochoa, Madrid, Spain; Hopital La Salpêtrière (Dr. Dubois), Paris, France; Depts. of Neuroscience (Dr. Galasko) and Pathology (Dr. Hansen), University of California San Diego, La Jolla; MCSA Alzheimer’s Disease Research Unit (Dr. Gauthier), McGill Centre for Studies in Aging, Quebec, Canada; Department of Neurological Sciences (Dr. Goetz), Rush University Medical Center, Chicago, IL; Fundación Jiménez Diaz (Dr. Gómez-Tortosa), Servicio de Neurología, Madrid, Spain; Prince of Wales Medical Research Institute (Dr. Halliday), Sydney, Australia; Laboratory of Neurogenetics (Dr. Hardy), National Institute on Aging, Bethesda, MD; Department of Neuropathology & Neuroscience (Dr. Iwatsubo), University of Tokyo, Japan; Dept. of Neurology CB 7025 (Dr. Kaufer), University of North Carolina School of Medicine, Chapel Hill; Department of Neurology (Dr. Korczyn), Tel-Aviv University, Israel; Department of Psychiatry (Dr. Kosaka), Fukushimera Hospital, Toyohashi, Japan; Center for Neurodegenerative Disease Research (Drs. Lee and Trojanowski), University of Pennsylvania School of Medicine, Philadelphia; Reta Lila Weston Institute of Neurological Studies (Dr. Lees), Royal Free & UCL Medical School, London, UK; Department of Neurology (Dr. Litvan), Movement Disorder Program, University of Louisville School of Medicine, KY; Department of Clinical Sciences (Dr. Londos), University Hospital, Malmoe, Sweden; Neuropsychology Research Program (Dr. Lopez), University of Pittsburgh Medical College, PA; Department of Radiology (Dr. Minoshima), University of Washington, Seattle; Department of Neurology (Dr. Mizuno), Juntendo University School of Medicine, Tokyo, Japan; Servicio de Neurologia (Dr. Molina), Hospital 12 de Octubre, Madrid, Spain; Clinique Neurologique (Dr. Pasquier), Centre Hospitalier et Universitaire, Lille, France; Department of Neurodegeneration and Restorative Research Center of Neurological Medicine (Dr. Schulz), University of Göttingen, Germany; and Department of Neurology & Neurobiology of Ageing (Dr. Yamada), Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
From the Institute for Ageing and Health (Drs. McKeith, O’Brien, E.K. Perry, Burn, Kalaria, Kenny, Mukaetova-Ladinska, and R.H. Perry), University of Newcastle upon Tyne, UK; Department of Neuropathology (Dr. Dickson), Mayo Clinic Jacksonville, FL; Division of Pathology (Dr. Lowe), School of Molecular Medical Sciences, University of Nottingham, UK; Department of Neurology (Dr. Emre), Istanbul University, Istanbul Faculty of Medicine, Turkey; UBC Division of Neurology (Dr. Feldman), University of British Columbia, Vancouver, Canada; Department of Neurology (Dr. Cummings), UCLA Medical School Alzheimer’s Disease Center, Los Angeles, CA; Parkinson’s Disease Research, Education and Clinical Center (Dr. Duda), Philadelphia VAMC, PA; Memory Disorders Program (Dr. Lippa), Drexel University School of Medicine, Philadelphia, PA; Section of Geriatric Psychiatry (Dr. Aarsland), Stavanger University Hospital, Norway; Department of Geriatric & Respiratory Medicine (Dr. Arai), Tohoku University School of Medicine, Miyagi, Japan; Wolfson Centre for Age-Related Diseases (Dr. Ballard), London, UK; Department of Neurology (Dr. Boeve), Mayo Clinic, Rochester, MN; Institute of Nuclear Medicine (Dr. Costa), HPP Medicina Molecular, SA, Porto, Portugal; Section of Neurology (Dr. del Ser), Hospital Severo Ochoa, Madrid, Spain; Hopital La Salpêtrière (Dr. Dubois), Paris, France; Depts. of Neuroscience (Dr. Galasko) and Pathology (Dr. Hansen), University of California San Diego, La Jolla; MCSA Alzheimer’s Disease Research Unit (Dr. Gauthier), McGill Centre for Studies in Aging, Quebec, Canada; Department of Neurological Sciences (Dr. Goetz), Rush University Medical Center, Chicago, IL; Fundación Jiménez Diaz (Dr. Gómez-Tortosa), Servicio de Neurología, Madrid, Spain; Prince of Wales Medical Research Institute (Dr. Halliday), Sydney, Australia; Laboratory of Neurogenetics (Dr. Hardy), National Institute on Aging, Bethesda, MD; Department of Neuropathology & Neuroscience (Dr. Iwatsubo), University of Tokyo, Japan; Dept. of Neurology CB 7025 (Dr. Kaufer), University of North Carolina School of Medicine, Chapel Hill; Department of Neurology (Dr. Korczyn), Tel-Aviv University, Israel; Department of Psychiatry (Dr. Kosaka), Fukushimera Hospital, Toyohashi, Japan; Center for Neurodegenerative Disease Research (Drs. Lee and Trojanowski), University of Pennsylvania School of Medicine, Philadelphia; Reta Lila Weston Institute of Neurological Studies (Dr. Lees), Royal Free & UCL Medical School, London, UK; Department of Neurology (Dr. Litvan), Movement Disorder Program, University of Louisville School of Medicine, KY; Department of Clinical Sciences (Dr. Londos), University Hospital, Malmoe, Sweden; Neuropsychology Research Program (Dr. Lopez), University of Pittsburgh Medical College, PA; Department of Radiology (Dr. Minoshima), University of Washington, Seattle; Department of Neurology (Dr. Mizuno), Juntendo University School of Medicine, Tokyo, Japan; Servicio de Neurologia (Dr. Molina), Hospital 12 de Octubre, Madrid, Spain; Clinique Neurologique (Dr. Pasquier), Centre Hospitalier et Universitaire, Lille, France; Department of Neurodegeneration and Restorative Research Center of Neurological Medicine (Dr. Schulz), University of Göttingen, Germany; and Department of Neurology & Neurobiology of Ageing (Dr. Yamada), Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
* To whom correspondence should be addressed. E-mail: i.g.mckeith{at}ncl.ac.uk.
Abstract-- The dementia with Lewy bodies (DLB) Consortium has revised criteria for the clinical and pathologic diagnosis of DLB incorporating new information about the core clinical features and suggesting improved methods to assess them. REM sleep behavior disorder, severe neuroleptic sensitivity, and reduced striatal dopamine transporter activity on functional neuroimaging are given greater diagnostic weighting as features suggestive of a DLB diagnosis. The 1-year rule distinguishing between DLB and Parkinson disease with dementia may be difficult to apply in clinical settings and in such cases the term most appropriate to each individual patient should be used. Generic terms such as Lewy body (LB) disease are often helpful. The authors propose a new scheme for the pathologic assessment of LBs and Lewy neurites (LN) using alpha-synuclein immunohistochemistry and semiquantitative grading of lesion density, with the pattern of regional involvement being more important than total LB count. The new criteria take into account both Lewy-related and Alzheimer disease (AD)-type pathology to allocate a probability that these are associated with the clinical DLB syndrome. Finally, the authors suggest patient management guidelines including the need for accurate diagnosis, a target symptom approach, and use of appropriate outcome measures. There is limited evidence about specific interventions but available data suggest only a partial response of motor symptoms to levodopa: severe sensitivity to typical and atypical antipsychotics in ~50%, and improvements in attention, visual hallucinations, and sleep disorders with cholinesterase inhibitors.
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