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November 01, 1996; 47 (5) View and Review

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, D. Galasko, K. Kosaka, E.K. Perry, D.W. Dickson, L.A. Hansen, D.P. Salmon, J. Lowe, S.S. Mirra, E.J. Byrne, G. Lennox, N.P. Quinn, J.A. Edwardson, P.G. Ince, C. Bergeron, A. Burns, B.L. Miller, S. Lovestone, D. Collerton, E.N.H. Jansen, C. Ballard, R.A.I. de Vos, G.K. Wilcock, K.A. Jellinger, R.H. Perry
First published November 1, 1996, DOI: https://doi.org/10.1212/WNL.47.5.1113
I.G. McKeith
MD
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D. Galasko
MD
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K. Kosaka
MD
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E.K. Perry
DSc
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D.W. Dickson
MD
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L.A. Hansen
MD
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D.P. Salmon
PhD
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J. Lowe
DM
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S.S. Mirra
MD
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E.J. Byrne
MRC Psych
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G. Lennox
MD
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N.P. Quinn
MD
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J.A. Edwardson
PhD
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P.G. Ince
MD
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C. Bergeron
MD
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A. Burns
MD
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B.L. Miller
MD
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S. Lovestone
MRC Psych
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D. Collerton
MSc
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E.N.H. Jansen
MD
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C. Ballard
MD
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R.A.I. de Vos
MD
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G.K. Wilcock
DM
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K.A. Jellinger
MD
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R.H. Perry
DSc
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Citation
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, D. Galasko, K. Kosaka, E.K. Perry, D.W. Dickson, L.A. Hansen, D.P. Salmon, J. Lowe, S.S. Mirra, E.J. Byrne, G. Lennox, N.P. Quinn, J.A. Edwardson, P.G. Ince, C. Bergeron, A. Burns, B.L. Miller, S. Lovestone, D. Collerton, E.N.H. Jansen, C. Ballard, R.A.I. de Vos, G.K. Wilcock, K.A. Jellinger, R.H. Perry
Neurology Nov 1996, 47 (5) 1113-1124; DOI: 10.1212/WNL.47.5.1113

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Abstract

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

  • Copyright 1996 by Advanstar Communications Inc.
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  • Article
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    • Pathologic assessment and diagnostic criteria for DLB
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