20 Clinical characteristics of pathological confirmed early onset dementia with lewy bodies

Abstract

Background Early-onset dementia (EOD) is characterized by distinct clinical profiles and prognosis when compared to late-onset dementia (LOD). As the second most common neurodegenerative form of dementia, little is known about the clinical profile of early-onset Dementia with Lewy Bodies (DLB). A current challenge for clinicians when managing patients with DLB is the suboptimal diagnosis rate which will affect treatment efficacy and outcome. To address this knowledge gap, by hypothesizing early-onset DLB will have a distinct profile when compared to Alzheimer’s disease (AD), we accessed and reviewed data of patients with pathological confirmed DLB from National Alzheimer’s Coordinating Center (NACC) database. Methods Patients with first visit that fulfill criteria for dementia of AD or DLB were analyzed. Early onset age was defined as less than 65 years old. Variables included in the analyses include baseline demographics, cognitive, behavioral, motor symptoms, neuropsychological battery scores and clinician diagnosis. Comparisons were made between early-onset AD (EOAD) versus early-onset DLB (EODLB), and early versus late-onset DLB. Results This study included 363 patients with EOAD, 32 EODLB and 147 late-onset DLB. Patients with EODLB were more likely to present with psychosis, apathy, REM sleep behavioral disorder, and motor symptoms. While EOAD patients were more likely to present with cognitive symptoms as first recognized and predominant presentation and perform worse in memory assessment. Motor as first recognized presentation, slowness, visual hallucination, caregiver reporting of agitation and apathy were the significant predictors to differentiate the two. Late-onset DLB patients were less depressed and more impaired in memory and executive function related scores than EODLB. Significant number of EODLB patients were misdiagnosed as EOAD (46.9%, p Conclusions EODLB is characterized by motor and neuropsychiatric symptoms while neuropsychological tests appear less reliable to differentiate EODLB from EOAD. Given that misdiagnosis of DLB remain significantly high, we propose a more careful and comprehensive clinical approach may improve the diagnosis rate. Acknowledgement The NACC database is funded by NIA/NIH Grant U01 AG016976. NACC data are contributed by these NIA funded ADCs: P30 AG019610 (PI Eric Reiman, MD), P30 AG013846 (PI Neil Kowall, MD), P50 AG008702 (PI Scott Small, MD), P50 AG025688 (PI Allan Levey, MD, PhD), P50 AG047266 (PI Todd Golde, MD, PhD), P30 AG010133 (PI Andrew Saykin, PsyD), P50 AG005146 (PI Marilyn Albert, PhD), P50 AG005134 (PI Bradley Hyman, MD, PhD), P50 AG016574 (PI Ronald Petersen, MD, PhD), P50 AG005138 (PI Mary Sano, PhD), P30 AG008051 (PI Steven Ferris, PhD), P30 AG013854 (PI M. Marsel Mesulam, MD), P30 AG008017 (PI Jeffrey Kaye, MD), P30 AG010161 (PI David Bennett, MD), P50 AG047366 (PI Victor Henderson, MD, MS), P30 AG010129 (PI Charles DeCarli, MD), P50 AG016573 (PI Frank LaFerla, PhD), P50 AG016570 (PI Marie-Francoise Chesselet, MD, PhD), P50 AG005131 (PI Douglas Galasko, MD), P50 AG023501 (PI Bruce Miller, MD), P30 AG035982 (PI Russell Swerdlow, MD) , P30 AG028383 (PI Linda Van Eldik, PhD), P30 AG010124 (PI John Trojanowski, MD, PhD), P50 AG005133 (PI Oscar Lopez, MD), P50 AG005142 (PI Helena Chui, MD), P30 AG012300 (PI Roger Rosenberg, MD), P50 AG005136 (PI Thomas Montine, MD, PhD), P50 AG033514 (PI Sanjay Asthana, MD, FRCP), P50 AG005681 (PI John Morris, MD), and P50 AG047270 (PI Stephen Strittmatter, MD, PhD). This study is supported by Singhealth Foundation Grant (NRS 15/001), NNI Centre Grant (NCG CS02) and National Medical Research Council, Singapore (NMRC/IRG/015).