We found that DLB comprised 4.2% of all dementia cases in a representative clinical population in NHS secondary care services. This is a considerably lower figure than that cited by both neuropathological studies and previous meta-analyses [10, 18]. We also found dementia diagnosed in only 9.7% of cases of PD, much lower than the 20–30% seen in the systematic review [13] and subsequent population and clinic-based studies of PDD prevalence [14,15,16].
Our study was deliberately designed to determine the frequency of diagnoses in routine clinical services, and reflects current real-life practice for patients being assessed in specialist services within secondary care. Services were selected by the research team primarily on the basis of their generalisability to psychiatry of old age and neurology/geriatric medicine services, throughout the UK.
The most likely reason that rates found in our cohorts are lower than those reported in meta-analysis of other hospital-referred populations, and indeed nearer to community-based estimates, is probably to be found in the methodology employed. Our study was based upon scrutiny of routine clinical records from services receiving mainly community-based referrals. This cohort therefore represents a broader, more generalisable dementia population than those investigated in prevalence studies conducted within specialist centres that often show larger prevalence rates.
Nevertheless, our observed range in prevalence in a case series likely also reflects a lower rate of disease detection, rather than true disease prevalence in some populations. This is supported by the differences in prevalence of DLB observed between our NE and EA cohorts, and the wide range in rates observed in neighbouring services within the same region. This variation in detection may be related to a number of factors; the effect on medical education, training, and service development of Newcastle University’s long history of LBD research may have contributed to higher rates in NE. Varying sensitivity to core DLB features may play a role in detection; Walker et al. [23] noted that prevalence studies incorporating a neurological examination reported higher prevalence rates of DLB. It is also possible that not all practitioners comprising participating services are fully aware of consensus criteria, but the high level of agreement between diagnoses made within services and those made by the expert panel (98%) would suggest that consensus diagnostic guidelines are in routine use in participating services.
Despite our belief that our findings represent variation in DLB detection, variation in true disease prevalence cannot be entirely ruled out. Environmental factors or a combination of environmental factors in the pathogenesis of DLB have been proposed [24]. It is not possible to discount the possibility that the variation in regional diagnostic rates seen within this study simply reflect the degree of exposure to causative or precipitating biological factors, but the intra-regional variation which was also seen would argue against this.
Contrary to the findings of the meta-analysis, which reported a positive relationship between age and DLB prevalence (although this was not statistically significant), we identified an inverse correlation between these two factors, and found the mean age of DLB patients at diagnosis to be lower than that of non-DLB dementia patients. This may be a reflection of a more aggressive course and increased mortality in DLB, or that DLB becomes less common clinically with advancing age as other pathologies become more prevalent leading to a mixed pathological and clinical picture. Our study design and information systems did not allow us access to accurate mortality data, although increased mortality in DLB has been described [6].
DLB was also more prevalent among men than women in our cohort, a finding which also conflicts with the lack of significant association identified in meta-analysis [10]. A male preponderance has been observed in neuropathological DLB samples [25] but population samples have both supported and refuted this hypothesis [26, 27]. Our very large sample size and multi-servicing sampling make our data the strongest support for a male preponderance of DLB from clinical samples to date.
Dementia prevalence in our PD cohorts was much lower than has been reported previously. A variation in prevalence of dementia was not identified between regions, yet higher age and lower MMSE scores at diagnosis of dementia suggest that dementia is diagnosed later on in the disease in EA. However, as the age at PD diagnosis was also older in EA, once again the possibility that there may be an environmental factor driving earlier onset in NE cannot be discounted. Another reason behind the difference in age may be the differences in life expectancy between the regions – the latest figures show this to be 80.4/83.8 years (male/female) in EA and 78.0/81.7 years in NE [28] – similar to the age differences we observed between the two regions in the study. It is, however, possible that clinicians in the NE region have a lower threshold for making both diagnoses. It should also be noted that the mean age at the mid-point of our screening period across both regions was 77.6 years and was similar to the median of the mean ages in studies analysed in the systematic review by Aarsland et al. (74.9 years) [13].
The strong inverse correlation between age at onset of PD and the time to diagnosis of dementia is consistent with age being a risk factor for PDD [29].
As with DLB, the most likely cause of the lower prevalence rate of PDD in our case series is because we have reported the observed rate of diagnosis of PDD as made by clinicians in routine practice. Previous studies have sought to identify dementia specifically in their PD populations using standardised diagnostic tools. Although clinical diagnoses agreed with those made by our independent clinician panel in 99% of PDD and PD cases, it is likely that our findings reflect lower detection rates of PDD within the PD population.
A lower rate of diagnosis in clinical practice has important implications for the patients and their carers who benefit from a diagnosis being made. The development of dementia has a profound effect on the patient and carer, and allows for the provision of support services to cater for these. Dementia leads to loss of insight, poor judgement, poor financial decision-making, increased carer stress, impaired driving skills, and an increased falls risk, amongst other difficulties [17]. Healthcare providers would also need to adapt their services to cater for a higher population of their patients experiencing the difficulties of having dementia.
Strengths of this study include the very large sample size compared to previous studies, its multi-site nature (when previous estimates have usually involved only single sites), its representativeness, in that access to all cases within a service was allowed, and, since we used clinically made diagnoses, its clinical relevance. Potential limitations include the fact that we could not compare diagnostic rates made by clinicians with “true” prevalence, which would have required full clinical examination of all 12,500 cases and would not have been possible. Another important limitation of the study is that our methodology permitted investigation of DLB and PDD prevalence as determined by primary clinical dementia syndrome alone. We were therefore unable to determine the contribution of co-existing AD neuropathology in such cases, although no mechanism currently exists to accurately determine such cases on the basis of clinical presentation [21].
