Background: Dementia with Lewy bodies (DLB) is the second most common degenerative dementia in older people. However, rates of misdiagnosis are high, and little is known of its natural history and outcomes. Very few previous studies have been able to access routine clinical information for large, unbiased DLB cohorts in order to establish initial presentation, neuropsychological profile, and mortality.
Objective: To examine in detail, symptom patterns at presentation and their association with outcomes, including mortality, in a large naturalistic DLB cohort from a secondary care sample.
Methods: A retrospective cohort design was used to identify a DLB cohort (n = 251) from Cambridgeshire and Peterborough NHS Foundation Trust (CPFT). Information relating to first consultation, diagnosis, and DLB diagnostic features were extracted.
Results: A wide range of presenting complaints and differential initial diagnoses were identified for the cohort. Along with memory loss (27.1%) and hallucinations (25.4%), low mood (25.1%) was noted as a key presenting complaint among the DLB cohort. Rates of REM sleep disorder were considerably lower (8.4%) than would be expected. Deficits in non-amnestic cognitive domains were associated with reduced mortality compared with amnestic-only presentations.
Conclusion: Individuals later diagnosed with DLB present initially to secondary care with a wide range of symptoms and complaints, some of which are not immediately suggestive of a DLB diagnosis. More examinations of large cohorts such as this are needed to further elucidate the complex presentation and clinical course of DLB, and to confirm whether amnestic-only presentation confers a worse outcome.
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http://dx.doi.org/10.3233/JAD-180877 | DOI Listing |
Alzheimers Dement
December 2024
Xiangya Hospital, Central South University, Changsha, Hunan, China.
Background: Genetics plays an important role in dementia with Lewy bodies (DLB) and remains poorly understood. Previous research has identified several genes associated with DLB, including APOE, GBA, SNCA, BIN1, TMEM175, PLCG2, and CNTN1. To date, genetic studies on DLB have focused on Caucasian population.
View Article and Find Full Text PDFBackground: Directed by the enzyme pair PINK1 and PRKN, mitophagy is a crucial mitochondrial quality control mechanism that selectively decorates damaged mitochondria with phosphorylated ubiquitin (pS65-Ub), facilitating their lysosomal degradation. The dynamic pS65-Ub signal accumulates upon enhanced activation from increased mitochondrial damage or upon reduced autophagic-lysosomal flux. Previous studies including ours demonstrated altered mitophagy and elevated pS65-Ub levels in Parkinson's and Alzheimer's disease brains that also independently associated with α-synuclein, tau, or amyloid pathology.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Peking Union Medical College Hospital, Beijing, China.
Background: Previous studies on APOE have mostly focused on APOE ε4, while less attention has been paid to APOE ε2. The aim of this study was to clarify the effect of APOE ε2 on different cognitive domains in dementia patients.
Method: All subjects were from the Peking Union Medical College Hospital (PUMCH) dementia cohort and included clinical diagnoses of AD, VaD, FTLD, and LBD.
Alzheimers Dement
December 2024
Alzheimer Center Amsterdam, Neurology, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, Netherlands.
Background: Young onset dementia (YOD) is characterized by an atypical clinical manifestation, and it is unclear to what extent impairments in everyday functioning are part of this manifestation. This study aims to describe the prevalence and differences of difficulties in instrumental activities of daily living (IADL) in YOD.
Methods: In this cross-sectional study, 394 subjects with sporadic YOD (onset<65 years,mean(M)age 58.
Eur Radiol
December 2024
Alzheimer Center Amsterdam, Neurology, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, The Netherlands.
Objectives: Distinguishing dementia with Lewy bodies (DLB) from Alzheimer's disease (AD) dementia, particularly in patients with DLB and concomitant AD pathology (DLB/AD+), can be challenging and there is no specific MRI signature for DLB. The aim of this study is to examine the additional value of MRI-based brain volumetry in separating patients with DLB (AD+/-) from patients with AD and controls.
Methods: We included 1518 participants from four cohorts (ADC, ADNI, PDBP and PredictND); 147 were patients with DLB (n = 76, DLB/AD+; n = 71, DLB/AD-), 668 patients with AD dementia, and 703 controls.
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