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Author affiliations
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.
Address correspondence and reprint requests to Professor I.G. McKeith, Institute for Ageing and Health, Wolfson Research Centre, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, UK; e-mail: i.g.mckeith{at}ncl.ac.uk
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.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the December 27 issue to find the title link for this article.
This article was previously published in electronic format as an Expedited E-Pub on October 19, 2005, at www.neurology.org.
*Author affiliations are listed in the appendix at the end of this article. Members of the Consortium on DLB are listed in appendix E-1 at www.neurology.org.
The third International Workshop meeting on DLB held in Newcastle upon Tyne in September 2003, was supported by unrestricted project grants in excess of $10,000 from Amersham Health, Novartis Pharma, Pfizer/Eisai, and Shire Pharmaceuticals. Dr. I.G. McKeith has received sponsorship and research grants from Novartis, Pfizer/Eisai, Janssen Cilag, and GE Healthcare, Dr. J.T. O'Brien from GE Healthcare, Dr. H. Feldman from Janssen Pharma, Pfizer/Eisai, and Novartis, and Dr. C. Lippa from Amersham Health, Novartis, Pfizer/Eisai, and Shire.
A follow-up meeting of some of the participants in the workshop in September 2004 in Washington, DC, was supported by the NIH (NIA/National Institute of Neurological Disorders and Stroke).
Received February 24, 2005. Accepted in final form August 2, 2005.
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Z. Walker, E. Jaros, R. W H Walker, L. Lee, D. C Costa, G. Livingston, P. G Ince, R. Perry, I. McKeith, and C. L E Katona Dementia with Lewy bodies: a comparison of clinical diagnosis, FP-CIT single photon emission computed tomography imaging and autopsy J. Neurol. Neurosurg. Psychiatry, November 1, 2007; 78(11): 1176 - 1181. [Abstract] [Full Text] [PDF]
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B. F. Boeve, M. H. Silber, C. B. Saper, T. J. Ferman, D. W. Dickson, J. E. Parisi, E. E. Benarroch, J. E. Ahlskog, G. E. Smith, R. C. Caselli, et al. Pathophysiology of REM sleep behaviour disorder and relevance to neurodegenerative disease Brain, November 1, 2007; 130(11): 2770 - 2788. [Abstract] [Full Text] [PDF]
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H. Oka, M. Yoshioka, M. Morita, K. Onouchi, M. Suzuki, Y. Ito, T. Hirai, S. Mochio, and K. Inoue Reduced cardiac 123I-MIBG uptake reflects cardiac sympathetic dysfunction in Lewy body disease Neurology, October 2, 2007; 69(14): 1460 - 1465. [Abstract] [Full Text] [PDF]
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K. L Brickell, J. B Leverenz, E. J Steinbart, M. Rumbaugh, G. D Schellenberg, D. Nochlin, T. H Lampe, I. E Holm, V. V. Deerlin, W. Yuan, et al. Clinicopathological concordance and discordance in three monozygotic twin pairs with familial Alzheimer's disease J. Neurol. Neurosurg. Psychiatry, October 1, 2007; 78(10): 1050 - 1055. [Abstract] [Full Text] [PDF]
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W. A. Rocca, J. H. Bower, J. E. Ahlskog, A. Elbaz, B. R. Grossardt, S. K. McDonnell, D. J. Schaid, and D. M. Maraganore Risk of Cognitive Impairment or Dementia in Relatives of Patients With Parkinson Disease Arch Neurol, October 1, 2007; 64(10): 1458 - 1464. [Abstract] [Full Text] [PDF]
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C. F. Lippa, B. F. Boeve, J. E. Parisi, and B. M. Keegan A 75-year-old man with cognitive impairment and gait changes Neurology, September 11, 2007; 69(11): 1183 - 1189. [Full Text] [PDF]
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M. Andersson, E. H. Wiig, L. Minthon, and E. Londos A Quick Test for Cognitive Speed: A Measure of Cognitive Speed in Dementia With Lewy Bodies American Journal of Alzheimer's Disease and Other Dementias, September 1, 2007; 22(4): 313 - 318. [Abstract] [PDF]
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V. Bogaerts, S. Engelborghs, S. Kumar-Singh, D. Goossens, B. Pickut, J. van der Zee, K. Sleegers, K. Peeters, J.-J. Martin, J. Del-Favero, et al. A novel locus for dementia with Lewy bodies: a clinically and genetically heterogeneous disorder Brain, September 1, 2007; 130(9): 2277 - 2291. [Abstract] [Full Text] [PDF]
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K. Seppi and O. Rascol Dementia with Lewy bodies and Parkinson disease with dementia: Can MRI make the difference? Neurology, August 21, 2007; 69(8): 717 - 718. [Full Text] [PDF]
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M. K. Beyer, J. P. Larsen, and D. Aarsland Gray matter atrophy in Parkinson disease with dementia and dementia with Lewy bodies Neurology, August 21, 2007; 69(8): 747 - 754. [Abstract] [Full Text] [PDF]
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H. Matsuda Role of Neuroimaging in Alzheimer's Disease, with Emphasis on Brain Perfusion SPECT J. Nucl. Med., August 1, 2007; 48(8): 1289 - 1300. [Abstract] [Full Text] [PDF]
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D. Weisman, M. Cho, C. Taylor, A. Adame, L. J. Thal, and L. A. Hansen In dementia with Lewy bodies, Braak stage determines phenotype, not Lewy body distribution Neurology, July 24, 2007; 69(4): 356 - 359. [Abstract] [Full Text] [PDF]
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K Mondon, A Gochard, A Marque, A Armand, D Beauchamp, C Prunier, D Jacobi, B de Toffol, A Autret, V Camus, et al. Visual recognition memory differentiates dementia with Lewy bodies and Parkinson's disease dementia J. Neurol. Neurosurg. Psychiatry, July 1, 2007; 78(7): 738 - 741. [Abstract] [Full Text] [PDF]
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S. Amici, S. M. Brambati, D. P. Wilkins, J. Ogar, N. L. Dronkers, B. L. Miller, and M. L. Gorno-Tempini Anatomical Correlates of Sentence Comprehension and Verbal Working Memory in Neurodegenerative Disease J. Neurosci., June 6, 2007; 27(23): 6282 - 6290. [Abstract] [Full Text] [PDF]
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A. Minguez-Castellanos, C. E. Chamorro, F. Escamilla-Sevilla, A. Ortega-Moreno, A. C. Rebollo, M. Gomez-Rio, A. Concha, and D. G. Munoz Do {alpha}-synuclein aggregates in autonomic plexuses predate Lewy body disorders?: A cohort study Neurology, June 5, 2007; 68(23): 2012 - 2018. [Abstract] [Full Text] [PDF]
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D. R. Williams, J. L. Holton, C. Strand, A. Pittman, R. de Silva, A. J. Lees, and T. Revesz Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome Brain, June 1, 2007; 130(6): 1566 - 1576. [Abstract] [Full Text] [PDF]
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C. C. Rowe, S. Ng, U. Ackermann, S. J. Gong, K. Pike, G. Savage, T. F. Cowie, K. L. Dickinson, P. Maruff, D. Darby, et al. Imaging {beta}-amyloid burden in aging and dementia Neurology, May 15, 2007; 68(20): 1718 - 1725. [Abstract] [Full Text] [PDF]
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K. Ishii, T. Soma, A. K. Kono, K. Sofue, N. Miyamoto, T. Yoshikawa, E. Mori, and K. Murase Comparison of Regional Brain Volume and Glucose Metabolism Between Patients with Mild Dementia with Lewy Bodies and Those with Mild Alzheimer's Disease J. Nucl. Med., May 1, 2007; 48(5): 704 - 711. [Abstract] [Full Text] [PDF]
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J. E. Galvin, C. M. Roe, and J. C. Morris Evaluation of Cognitive Impairment in Older Adults: Combining Brief Informant and Performance Measures Arch Neurol, May 1, 2007; 64(5): 718 - 724. [Abstract] [Full Text] [PDF]
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J. E. Galvin, C. M. Roe, M. A. Coats, and J. C. Morris Patient's Rating of Cognitive Ability: Using the AD8, a Brief Informant Interview, as a Self-rating Tool to Detect Dementia Arch Neurol, May 1, 2007; 64(5): 725 - 730. [Abstract] [Full Text] [PDF]
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J. B. Leverenz, C. E. Yu, T. J. Montine, E. Steinbart, L. M. Bekris, C. Zabetian, L. K. Kwong, V. M-Y. Lee, G. D. Schellenberg, and T. D. Bird A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology Brain, May 1, 2007; 130(5): 1360 - 1374. [Abstract] [Full Text] [PDF]
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S. Bombois, C.-A. Maurage, M. Gompel, V. Deramecourt, M.-A. Mackowiak-Cordoliani, R. S. Black, R. Lavielle, A. Delacourte, and F. Pasquier Absence of beta-Amyloid Deposits After Immunization in Alzheimer Disease With Lewy Body Dementia Arch Neurol, April 1, 2007; 64(4): 583 - 587. [Abstract] [Full Text] [PDF]
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J. L. Whitwell, C. R. Jack Jr, J. E. Parisi, D. S. Knopman, B. F. Boeve, R. C. Petersen, T. J. Ferman, D. W. Dickson, and K. A. Josephs Rates of cerebral atrophy differ in different degenerative pathologies Brain, April 1, 2007; 130(4): 1148 - 1158. [Abstract] [Full Text] [PDF]
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C. F. Lippa, J. E. Duda, M. Grossman, H. I. Hurtig, D. Aarsland, B. F. Boeve, D. J. Brooks, D. W. Dickson, B. Dubois, M. Emre, et al. DLB and PDD boundary issues: Diagnosis, treatment, molecular pathology, and biomarkers Neurology, March 13, 2007; 68(11): 812 - 819. [Abstract] [Full Text] [PDF]
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J. L. Whitwell, S. D. Weigand, M. M. Shiung, B. F. Boeve, T. J. Ferman, G. E. Smith, D. S. Knopman, R. C. Petersen, E. E. Benarroch, K. A. Josephs, et al. Focal atrophy in dementia with Lewy bodies on MRI: a distinct pattern from Alzheimer's disease Brain, March 1, 2007; 130(3): 708 - 719. [Abstract] [Full Text] [PDF]
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