Abstract:
Clinical criteria for dementia with Lewy bodies (DLB) have been
proposed, but their formulation, reliability, and validity require
further study. Pathologic criteria for DLB are also undergoing
evolution. Two studies were conducted with the goal of identifying the
components of these evolving criteria that may benefit from further
refinement; one study evaluated the components of the clinical criteria
and another study operationalized the pathologic criteria for DLB.
Twenty-four patients with a premorbid diagnosis of probable or possible
Alzheimer's disease (AD) (n 3D 18), Parkinson's disease (PD) (n 3D
5), or progressive supranuclear palsy (PSP) (n 3D 1) were studied.
Inter-rater reliability and validity of the clinical criteria were
determined by a retrospective chart review, done by five neurologists,
and a blinded pathologic evaluation. The Consortium on dementia with
Lewy bodies (CDLB) pathologic criteria were operationalized to compare
past criteria and test the validity of the evolving clinical criteria on
the dementia patients. Three or more cortical fields (at 250 x
magnification) with many (four or more) Lewy bodies (LBs) on ubiquitin
immunoreactive sections were required to meet the CDLB neocortical score
of > 6. Fifteen of the AD patients had at least one LB in a cortical
section, four had many LBs, while three had no LBs; all patients with
movement disorder had at least one LB in a cortical section. The
sensitivity/specificity ratio of the CDLB probable DLB clinical criteria
based upon many LBs being present was 75%/79%. Reformulated clinical
criteria that require the presence of extrapyramidal signs significantly
predicted those patients with many LBs versus those with few or no LBs
(chi 2 3D 5.48, p 3D 0.02) and increased clinical specificity to 100%.
This preliminary study identifies components of the evolving clinical
and pathologic criteria for DLB that require further refinement