Diagnosis and Management of Dementia With Lewy Bodies: Third Report of the DLB Consortium

Neurology. 2005 Dec 27;65(12):1863-72. doi: 10.1212/01.wnl.0000187889.17253.b1. Epub 2005 Oct 19.

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.

Publication types

  • Practice Guideline
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Brain / metabolism
  • Brain / pathology*
  • Brain / physiopathology*
  • Corpus Striatum / metabolism
  • Corpus Striatum / pathology
  • Corpus Striatum / physiopathology
  • Diagnosis, Differential
  • Dopamine Plasma Membrane Transport Proteins / metabolism
  • Drug Tolerance / physiology
  • Humans
  • Lewy Bodies / metabolism
  • Lewy Bodies / pathology
  • Lewy Body Disease / diagnosis*
  • Lewy Body Disease / drug therapy
  • Lewy Body Disease / physiopathology*
  • REM Sleep Behavior Disorder / diagnosis
  • REM Sleep Behavior Disorder / etiology
  • REM Sleep Behavior Disorder / physiopathology
  • alpha-Synuclein / metabolism

Substances

  • Dopamine Plasma Membrane Transport Proteins
  • alpha-Synuclein