Objective: To determine whether the dementia associated with REM sleep beha
vior disorder (RBD) differs from Alzheimer's disease (AD) and, if so, wheth
er differences in cognitive performance between RBD/dementia and AD resembl
e reported differences between dementia with Lewy bodies (DLB) and AD. Meth
ods: This retrospective study compares neurocognitive performance between 3
1 patients with degenerative dementia and polysomnography-confirmed RBD and
31 patients without brainstem Lewy body pathology who met Consortium to Es
tablish a Registry for Alzheimer's Disease (CERAD) clinical and neuropathol
ogic criteria for AD. The patient groups did not differ in dementia severit
y (based on Global Deterioration Scale score) or duration. Results: RBD pre
ceded or coincided with the onset of cognitive decline in 94% of the patien
ts. All but one patient with RBD/dementia bad one or more of the following
clinical features of DLB: visual hallucinations, extrapyramidal signs, or f
luctuating cognition/alertness. The data revealed significantly worse perfo
rmance on attention, perceptual organization, visual memory, and letter flu
ency for the RBD/dementia group, whereas the AD group showed significantly
worse performance on confrontation naming and verbal memory. Conclusions: P
atients with RBD and degenerative dementia demonstrate a significantly diff
erent pattern of cognitive performance from patients with AD. Most of the p
atients in the RBD/dementia sample also meet criteria for possible or proba
ble DLB, and the pattern of cognitive differences from AD is similar to rep
orted comparisons between DLB and AD. The cognitive and clinical data provi
de evidence to suggest that the dementia associated with RBD may represent
DLB.