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NEUROLOGY 1996;47:1113-1124
© 1996 American Academy of Neurology

Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB)

Report of the consortium on DLB international workshop

I.G. McKeith, MD, D. Galasko, MD, K. Kosaka, MD, E.K. Perry, DSc, D.W. Dickson, MD, L.A. Hansen, MD, D.P. Salmon, PhD, J. Lowe, DM, S.S. Mirra, MD, E.J. Byrne, MRC Psych, G. Lennox, MD, N.P. Quinn, MD, J.A. Edwardson, PhD, P.G. Ince, MD, C. Bergeron, MD, A. Burns, MD, B.L. Miller, MD, S. Lovestone, MRC Psych, D. Collerton, MSc, E.N.H. Jansen, MD, C. Ballard, MD, R.A.I. de Vos, MD, G.K. Wilcock, DM, K.A. Jellinger, MD and R.H. Perry, DSc

For the Consortium on Dementia with Lewy Bodies; *See pages 1122 and 1123 for the Consortium of Dementia with Lewy Bodies participants.
Presented at the Proceedings of the First International Workshop of the Consortium on DLB, Newcastle upon Tyne, UK, October 1995.
Supported by Parke Davis and Company, The French Foundation for Alzheimer Research, Janssen-Cilag Ltd, Sandoz Pharma Ltd, Bayer AG, F Hoffmann-La Roche AG, The Wellcome Trust, Eli Lilly and Company, Astra Arcus AB, Lederle Laboratories, Merck Sharp & Dohme Ltd, Institute for the Health of the Elderly, University of Newcastle upon Tyne, Medical Research Council (UK), Newcastle upon Tyne City Health NHS Trust, Royal Victoria Infirmary and Associated Hospitals NHS Trust.
Received April 1, 1996. Accepted in final form May 16, 1996.
Address correspondence and reprint requests to Dr McKeith, MD, BS, FRCPsych, Department of Old Age Psychiatry, Institute for the Health of the Elderly, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, U.K.

Recent neuropathologic autopsy studies found that 15 to 25% of elderly demented patients have Lewy bodies (LB) in their brainstem and cortex, and in hospital series this may constitute the most common pathologic subgroup after pure Alzheimer's disease (AD).The Consortium on Dementia with Lewy bodies met to establish consensus guidelines for the clinical diagnosis of dementia with Lewy bodies (DLB) and to establish a common framework for the assessment and characterization of pathologic lesions at autopsy. The importance of accurate antemortem diagnosis of DLB includes a characteristic and often rapidly progressive clinical syndrome, a need for particular caution with neuroleptic medication, and the possibility that DLB patients may be particularly responsive to cholinesterase inhibitors. We identified progressive disabling mental impairment progressing to dementia as the central feature of DLB. Attentional impairments and disproportionate problem solving and visuospatial difficulties are often early and prominent. Fluctuation in cognitive function, persistent well-formed visual hallucinations, and spontaneous motor features of parkinsonism are core features with diagnostic significance in discriminating DLB from AD and other dementias. Appropriate clinical methods for eliciting these key symptoms are described. Brainstem or cortical LB are the only features considered essential for a pathologic diagnosis of DLB, although Lewy-related neurites, Alzheimer pathology, and spongiform change may also be seen. We identified optimal staining methods for each of these and devised a protocol for the evaluation of cortical LB frequency based on a brain sampling procedure consistent with CERAD. This allows cases to be classified into brainstem predominant, limbic (transitional), and neocortical subtypes, using a simple scoring system based on the relative distribution of semiquantitative LB counts. Alzheimer pathology is also frequently present in DLB, usually as diffuse or neuritic plaques, neocortical neurofibrillary tangles being much less common. The precise nosological relationship between DLB and AD remains uncertain, as does that between DLB and patients with Parkinson's disease who subsequently develop neuropsychiatric features. Finally, we recommend procedures for the selective sampling and storage of frozen tissue for a variety of neurochemical assays, which together with developments in molecular genetics, should assist future refinements of diagnosis and classification.

NEUROLOGY 1996;47: 1113-1124




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AM J ALZHEIMERS DIS OTHER DEMENHome page
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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J. Neurol. Neurosurg. PsychiatryHome page
D de Jong, R W M M Jansen, Y A L Pijnenburg, W J A van Geel, G F Borm, H P H Kremer, and M M Verbeek
CSF neurofilament proteins in the differential diagnosis of dementia
J. Neurol. Neurosurg. Psychiatry, September 1, 2007; 78(9): 936 - 938.
[Abstract] [Full Text] [PDF]


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BrainHome page
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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Am. J. Pathol.Home page
W. Yang, R. L. Woltjer, I. Sokal, C. Pan, Y. Wang, M. Brodey, E. R. Peskind, J. B. Leverenz, J. Zhang, D. P. Perl, et al.
Quantitative Proteomics Identifies Surfactant-Resistant {alpha}-Synuclein in Cerebral Cortex of Parkinsonism-Dementia Complex of Guam but Not Alzheimer's Disease or Progressive Supranuclear Palsy
Am. J. Pathol., September 1, 2007; 171(3): 993 - 1002.
[Abstract] [Full Text] [PDF]


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NeurologyHome page
W. Jagust, B. Reed, D. Mungas, W. Ellis, and C. DeCarli
What does fluorodeoxyglucose PET imaging add to a clinical diagnosis of dementia?
Neurology, August 28, 2007; 69(9): 871 - 877.
[Abstract] [Full Text] [PDF]


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NeurologyHome page
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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Am J EpidemiolHome page
K. J. Anstey, C. von Sanden, A. Salim, and R. O'Kearney
Smoking as a Risk Factor for Dementia and Cognitive Decline: A Meta-Analysis of Prospective Studies
Am. J. Epidemiol., August 15, 2007; 166(4): 367 - 378.
[Abstract] [Full Text] [PDF]


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BrainHome page
P. A. Kempster, D. R. Williams, M. Selikhova, J. Holton, T. Revesz, and A. J. Lees
Patterns of levodopa response in Parkinson's disease: a clinico-pathological study
Brain, August 1, 2007; 130(8): 2123 - 2128.
[Abstract] [Full Text] [PDF]


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Br. J. PsychiatryHome page
A. STRYDOM, G. LIVINGSTON, M. KING, and A. HASSIOTIS
Prevalence of dementia in intellectual disability using different diagnostic criteria
The British Journal of Psychiatry, August 1, 2007; 191(2): 150 - 157.
[Abstract] [Full Text] [PDF]


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J. Neurol. Neurosurg. PsychiatryHome page
L M Allan, C G Ballard, J Allen, A Murray, A W Davidson, I G McKeith, and R A Kenny
Autonomic dysfunction in dementia
J. Neurol. Neurosurg. Psychiatry, July 1, 2007; 78(7): 671 - 677.
[Abstract] [Full Text] [PDF]


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Arch NeurolHome page
H. Boecker, A. O. Ceballos-Baumann, D. Volk, B. Conrad, H. Forstl, and P. Haussermann
Metabolic Alterations in Patients With Parkinson Disease and Visual Hallucinations
Arch Neurol, July 1, 2007; 64(7): 984 - 988.
[Abstract] [Full Text] [PDF]


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J. Neurosci.Home page
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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Psychosom. Med.Home page
A. S. Buchman, P. A. Boyle, R. S. Wilson, Y. Tang, and D. A. Bennett
Frailty is Associated With Incident Alzheimer's Disease and Cognitive Decline in the Elderly
Psychosom Med, June 1, 2007; 69(5): 483 - 489.
[Abstract] [Full Text] [PDF]


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NeurologyHome page
D. Galasko, D. Salmon, A. Gamst, J. Olichney, L. J. Thal, L. Silbert, J. Kaye, P. Brooks, R. Adonay, U.-K Craig, et al.
Prevalence of dementia in Chamorros on Guam: Relationship to age, gender, education, and APOE
Neurology, May 22, 2007; 68(21): 1772 - 1781.
[Abstract] [Full Text] [PDF]


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NeurologyHome page
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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JNMHome page
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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Am. J. Pathol.Home page
B. M. Dufty, L. R. Warner, S. T. Hou, S. X. Jiang, T. Gomez-Isla, K. M. Leenhouts, J. T. Oxford, M. B. Feany, E. Masliah, and T. T. Rohn
Calpain-Cleavage of {alpha}-Synuclein: Connecting Proteolytic Processing to Disease-Linked Aggregation
Am. J. Pathol., May 1, 2007; 170(5): 1725 - 1738.
[Abstract] [Full Text] [PDF]


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NeurologyHome page
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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NeurologyHome page
M. S. Forman, E. J. Mufson, S. Leurgans, D. Pratico, S. Joyce, S. Leight, V. M.-Y. Lee, and J. Q. Trojanowski
Cortical biochemistry in MCI and Alzheimer disease: Lack of correlation with clinical diagnosis
Neurology, March 6, 2007; 68(10): 757 - 763.
[Abstract] [Full Text] [PDF]


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J. Neurol. Neurosurg. PsychiatryHome page
M. K Beyer, C. C Janvin, J. P Larsen, and D. Aarsland
A magnetic resonance imaging study of patients with Parkinson's disease with mild cognitive impairment and dementia using voxel-based morphometry
J. Neurol. Neurosurg. Psychiatry, March 1, 2007; 78(3): 254 - 259.
[Abstract] [Full Text] [PDF]


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BrainHome page
B. R. Reed, D. M. Mungas, J. H. Kramer, W. Ellis, H. V. Vinters, C. Zarow, W. J. Jagust, and H. C. Chui
Profiles of neuropsychological impairment in autopsy-defined Alzheimer's disease and cerebrovascular disease
Brain, March 1, 2007; 130(3): 731 - 739.
[Abstract] [Full Text] [PDF]


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BrainHome page
A. Lannuzel, G. U. Hoglinger, S. Verhaeghe, L. Gire, S. Belson, M. Escobar-Khondiker, P. Poullain, W. H. Oertel, E. C. Hirsch, B. Dubois, et al.
Atypical parkinsonism in Guadeloupe: a common risk factor for two closely related phenotypes?
Brain, March 1, 2007; 130(3): 816 - 827.
[Abstract] [Full Text] [PDF]


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J. Neurosci.Home page
M. L. Kramer and W. J. Schulz-Schaeffer
Presynaptic {alpha}-Synuclein Aggregates, Not Lewy Bodies, Cause Neurodegeneration in Dementia with Lewy Bodies
J. Neurosci., February 7, 2007; 27(6): 1405 - 1410.
[Abstract] [Full Text] [PDF]


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AM J ALZHEIMERS DIS OTHER DEMENHome page
L. Rozzini, B. V. Chilovi, E. Bertoletti, M. Conti, I. Delrio, M. Trabucchi, and A. Padovani
Cognitive and Psychopathologic Response to Rivastigmine in Dementia With Lewy Bodies Compared to Alzheimer's Disease: A Case Control Study
American Journal of Alzheimer's Disease and Other Dementias, February 1, 2007; 22(1): 42 - 47.
[Abstract] [PDF]


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AM J ALZHEIMERS DIS OTHER DEMENHome page
P. K. Panegyres and K. Frencham
Course and Causes of Suspected Dementia in Young Adults: A Longitudinal Study
American Journal of Alzheimer's Disease and Other Dementias, February 1, 2007; 22(1): 48 - 56.
[Abstract] [PDF]


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AM J ALZHEIMERS DIS OTHER DEMENHome page
G. Gliebus and C. F. Lippa
The Influence of {beta}-Blockers on Delayed Memory Function in People With Cognitive Impairment
American Journal of Alzheimer's Disease and Other Dementias, February 1, 2007; 22(1): 57 - 61.
[Abstract] [PDF]


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Arch NeurolHome page
G. K. Tofaris, T. Revesz, T. S. Jacques, S. Papacostas, and J. Chataway
Adult-Onset Neurodegeneration With Brain Iron Accumulation and Cortical {alpha}-Synuclein and Tau Pathology: A Distinct Clinicopathological Entity
Arch Neurol, February 1, 2007; 64(2): 280 - 282.
[Abstract] [Full Text] [PDF]


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J. Neuropsychiatry Clin. Neurosi.Home page
D. E. Clarke, R. v. Reekum, M. Simard, D. L. Streiner, M. Freedman, and D. Conn
Apathy in Dementia: An Examination of the Psychometric Properties of the Apathy Evaluation Scale
J Neuropsychiatry Clin Neurosci, February 1, 2007; 19(1): 57 - 64.
[Abstract] [Full Text] [PDF]




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