ABSTRACT
Dementia with Lewy bodies (DLB) is an increasingly recognized diagnostic
entity among neurodegenerative disorders. Clinically, DLB is distinguished
as a global dementia syndrome that is accompanied by cognitive fluctua-
tions, parkinsonism, and visual hallucinations. Neuropathologically, DLB
is characterized by the presence of neuronal inclusions called Lewy bodies
in various brain regions.1 Thus, patients with DLB and patients with
Parkinson’s disease (PD) and dementia (PDD) share common clinical and
neuropathological features: both are parkinsonian dementia syndromes that
involve neurodegeneration and abnormal aggregation of the -synuclein
protein, the major constituent of Lewy bodies. However, it is controversial
whether DLB and PDD represent different diseases or syndromic variants of
the same underlying disease process, one commencing initially with motor
dysfunction and the other with cognitive dysfunction.2,3 The relative impor-
tance of concomitant Alzheimer’s disease (AD) neuropathological changes in
both DLB and PDD is also debated. In the absence of a resolution of these
controversies, the perspective of serving patients should be given priority. To
that end, it is important to understand the basis of the controversy and its
impact on clinical care. This chapter reviews the clinical and neurobiological
features of DLB and PDD and provides practical guidelines on the man-
agement of patients who present with DLB relative to patients with PD who
later develop a global dementia syndrome.
