William T Hu, Manuel Hazim, Chadwick M Hales, James L Lah and Allan I Levey
Objective: To identify clinical features of dementia with Lewy bodies (DLB) least likely associated with
Alzheimer's disease pathology, and to determine whether it is associated with a unique neuropsychological profile.
Methods: Clinical records of 98 patients given the diagnosis of DLB at a specialty cognitive and behavioral
neurology clinic in a tertiary referral center were retrospectively reviewed for core, suggestive, and supportive
features of DLB as well as demographic variables, cerebrospinal fluid (CSF) Alzheimer's biomarkers, and
longitudinal neuropsychological analyses.
Results: Core, suggestive, and supportive features were common in this cohort, with 69% and 39% of patients
assigned the diagnosis of probable DLB and possible DLB fulfilling consensus criteria for probable DLB. 26 of 98
clinically diagnosed DLB patients had CSF Alzheimer's biomarker analysis, and visual hallucination was the only
feature not associated with CSF suggestive of Alzheimer's disease. 42 of 98 patients had longitudinal
neuropsychological analyses, and patients with visual hallucinations had worse baseline executive functions but
slower longitudinal decline in executive functions than patients without visual hallucinations.
Conclusion: Visual hallucination in clinically diagnosed DLB is associated with CSF biomarkers consistent with a
non-AD disorder and a unique longitudinal neuropsychological profile. DLB patients with visual hallucinations can be
considered a unique DLB endophenotype for future biomarker discovery and validation.