Objective: To examine the incidence, clinical features, and survival of early-onset Dementia with Lewy Bodies and Parkinson’s Disease Dementia (DLB/PDD) (≤55 years of age) in a population-based cohort of residents in seven counties in Minnesota.

Background: Due to their rarity, there is a lack of studies investigating early-onset DLB/PDD.

Method: We used an early-onset Parkinsonism population-based incident cohort defined by the expanded Rochester Epidemiology Project (eREP) between 2010-2015. A specialist reviewed all the charts to confirm the clinical diagnosis of DLB and PDD.

Results: In the years 2010-15, we identified 6 incident cases of early-onset DLB/PDD, all men. The incidence was 0.44 cases per 100,000 person-years. Most of the patients used levodopa (83%) and 1 only developed Levodopa-induced dyskinesia (LID) 1.7 years after Levodopa initiation and 2.6 years after DLB/PDD onset. Rest tremor and gait instability were present in all the patients; whereas bradykinesia was reported in 84% and rigidity in 67%; in addition, bradykinesia and rigidity occurred about 1.7 years after the diagnosis.
Whereas among non-motor symptoms, 5 (83%) had REM sleep behavior disorder (RBD), 2 (33%) had cognitive fluctuations, and all of them (100%) had visual hallucinations.
At time of data collection, 1 (17%) patient had died: 2.6 years after DLB/PDD onset due to aspiration pneumonia.

Conclusion: The incidence of early-onset DLB/PDD in seven counties in Minnesota (2010-2015) was 0.44 per 100,000 person-years. Cardinal motor symptoms had prevalence ranging from 67% to 100%; whereas LID was reported in one patient.
The majority of the patients had RBD and/or visual hallucinations; cognitive fluctuations were present in one third of the 6 early-onset DLB/PDD.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/early-onset-dementia-with-lewy-bodies-and-parkinsons-disease-dementia-a-population-based-study-2010-2015/