Dementia with Lewy bodies: findings from an international multicentre study

Abstract

Objectives. To describe the baseline demographic, neuropsychiatric and neurological data of a large selected clinical sample of patients with dementia with Lewy Bodies (DLB) from an international multicentre trial with rivastigmine. To examine the usefulness of the Consensus Criteria for the diagnosis of DLB in different countries. Methods. Seventeen centres from Spain, the UK and Italy recruited patients diagnosed clinically as probable DLB according to recent Consensus Criteria (McKeith et al., 1996). A standard clinical protocol including inclusion/exclusion criteria, collection of demographic and medical data, cognitive (Mini Mental State Examination: MMSE), motor (Unified Parkinson's Disease Rating Scale: UPDRS) and neuropsychiatric (Neuropsychiatric Inventory: NPI) examinations, was applied after obtaining informed consent. Data were summarised and compared across countries with uni‐ and multivariate analyses. Results. One hundred and twenty patients were recruited: 56.7% males, mean (SD) age 73.9 (6.4) years, range 57 – 87 years. Sixty percent fulfilled all three core diagnostic features of DLB, and 40% only two (‘parkinsonism’ 92.4%, ‘cognitive fluctuations’ 89.1%, ‘visual hallucinations’ 77.3%). ‘Systematised delusions’ (46%) and ‘repeated falls’ (42%) were the most frequent supportive diagnostic features. There were no differences across countries in demographic, diagnostic or clinical features. Patients showed a wide range of psychopathology which was weakly correlated with cognitive impairment. Some mild extrapyramidal signs (EPS) were observed in most patients. Conclusions. The Consensus Criteria for DLB can be consistently applied across many different sites for multicentre studies. ‘Parkinsonism’ and ‘cognitive fluctuations’ as core features and ‘systematised delusions’ and ‘repeated falls’ as supportive features are the most frequent diagnostic clues. Neuropsychiatric disturbances, in particular apathy, delusions, hallucinations and anxiety, and mild symmetric EPS are frequent in DLB and are only related weakly to cognitive impairment. Copyright © 2000 John Wiley & Sons, Ltd.

Visuospatial deficits predict rate of cognitive decline in autopsy-verified dementia with Lewy bodies.

“…However, DLB patients with more severe visuospatial deficits carried less NFT burden, suggesting that a process related to the formation of Lewy bodies rather than AD pathology contributed to their clinical syndrome. These results also bolster the contention that the DLB phenotype is strongest in individuals who have minimal NFT (Cormack et al, 2004; Del Ser et al, 2000; McKeith et al, 2005; Merdes et al, 2003). …”

“…In addition to the hallmark α-synuclein inclusions (i.e., Lewy bodies) in neocortical, limbic, and subcortical brain regions, the majority of patients with DLB also develop concomitant neurofibrillary tangles (NFT) and neuritic plaques (NP) in the same limbic and neocortical distribution as in AD (Armstrong, Cairns, & Lantos, 1998; Hansen et al, 1990), although fewer NFT are typically found in DLB (Gelb, Oliver, & Gilman, 1999; Hansen et al, 1990). The clinical phenotype associated with DLB appears to be weaker in patients who have extensive NFT (Cormack, Aarsland, Ballard, & Tovee, 2004; Del Ser et al, 2000; Merdes et al, 2003). In patients with Lewy bodies, a clinical syndrome that is strongly characteristic of DLB is associated with greater Lewy body burden (Cormack et al, 2004; Harding, Broe, & Halliday, 2002) and neurochemical disruption (Ballard et al, 2000; Ballard et al, 2002; Perry et al, 1991; Teaktong et al, 2005).…”

Gender and Pathology-Specific Effect of Apolipoprotein E Genotype on Psychosis in Alzheimer’s Disease

“…In our study, this association was confirmed to be much more prominent and statistically significant in females. Studies examining the role of Lewy body pathology on psychosis have focused on subjects with dementia with Lewy bodies, where psychosis, particularly visual hallucinations, and to a lesser degree delusions are characteristic (37, 38). A study by Engelborghs and colleagues on 11 subjects with dementia with Lewy bodies showed a relationship between ε4 allele and delusions (39).…”

Extrapyramidal features in Parkinson's disease with and without dementia and dementia with Lewy bodies: A cross‐sectional comparative study

“…Motor features mediated by non‐dopaminergic systems have been associated with incident dementia. Parkinsonism is also common in DLB, occurring at some point during the course of the illness in 75 to 80% cases 17–19. There are no prospective clinicopathological studies available to clarify whether the extrapyramidal features of DLB differ from those of PD.…”