Diagnosis and management of dementia with Lewy bodies

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 approximately 50%, and improvements in attention, visual hallucinations, and sleep disorders with cholinesterase inhibitors.

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Affiliated Institutions

• Mayo Clinic US
• University of California, Los Angeles US
• Hospital Universitario Severo Ochoa ES
• Sorbonne Université FR
• King's College London GB
• Newcastle University GB
• Universidad Complutense de Madrid ES
• University College London GB
• University of Göttingen DE
• Mayo Clinic in Florida US
• Drexel University US
• University of Louisville Hospital US
• University of British Columbia CA
• University of Pennsylvania US
• Lund University SE
• University of North Carolina at Chapel Hill US
• University of Nottingham GB
• Universidad Autónoma de Madrid ES
• National Institutes of Health US
• Jacksonville College US
• Tel Aviv University IL
• Fukushimura Hospital JP
• University of Pittsburgh US
• Stavanger University Hospital
• Juntendo University JP
• University of California, San Diego US
• Istanbul University TR
• McGill University CA
• Kanazawa University JP
• University of Washington US
• Tohoku University JP
• The University of Tokyo JP
• Rush University US
• WinnMed US
• Neuroscience Research Australia AU
• Newcastle upon Tyne Hospitals NHS Foundation Trust GB

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