Core Clinical Features Associated With Survival in Patients With Dementia With Lewy Bodies.

Abstract

This analysis used clinical data from prospectively followed participants meeting criteria for probable dementia with Lewy bodies (DLB) in the Mayo Clinic Alzheimer's Disease Research Center (ADRC) between 1998 and 2024. DLB is characterized by unique core features of visual hallucinations (VHs), parkinsonism, REM sleep behavior disorder, and cognitive fluctuations with a variable disease course. DLB is associated with a poor prognosis, but whether these unique DLB core clinical features influence survival is unknown. We aimed to determine whether core clinical features are associated with survival in patients with probable DLB. Patients followed in the Mayo Clinic ADRC between 1998 and 2024 underwent annual clinical assessments. Those who met clinical criteria for probable DLB were analyzed. Time-dependent Cox proportional hazard models using age as the time scale determined associations between the individual and cumulative number of core clinical DLB features and survival. The prognostic significance of core features present at the time DLB criteria were met was assessed in separate models. Models were adjusted for sex and duration from the onset of cognitive symptoms to DLB diagnosis. Of 488 patients with probable DLB meeting inclusion criteria, 118 (24%) were women with a mean age of 71.9 ± 8.4 years at the time of meeting probable DLB criteria. Shorter survival was associated with the development of VHs (hazard ratio [HR] 3.25, 95% CI 2.46-4.29) and parkinsonism (HR 2.28, 95% CI 1.54-3.39) during the disease course and VHs at the time of DLB diagnosis (HR 1.60, 95% CI 1.18-2.16). All four core features were also associated with shorter survival (4 core features vs 2 core features, HR 3.58 95% CI 2.66-4.80, 4 core features vs 3 core features, HR 2.46, 95% CI 1.86-3.25). In 191 patients (45 women (24%) with a mean age of 71.2 ± 8.6 years at probable DLB diagnosis) with autopsy-confirmed DLB, VHs, parkinsonism, and all four core features were associated with shorter survival. Sex was not associated with survival. VHs, parkinsonism, and the development of all 4 core features were associated with shorter survival in probable and in autopsy-confirmed DLB. These findings have important prognostic and management implications for patients with DLB and their caregivers.