For whom and for what the definition of severe dementia is useful: an EDCON consensus.

The European Dementia Consensus Network (EDCON) is a special project of the Madariaga Foundation located in Brussels. The Madariaga Foundation seeks to facilitate collaboration between European countries and between the public and private sector. This paper will review the differences in the definitions of Severe Dementia and summarise the EDCON consensus on their implications for management. EDCON recommends that:--The attributes of the person suffering from dementia should be given as much attention (and are as important for care) as the severity of cognitive decline in dementia;--The dementia syndrome (particularly in it's severe form) is inadequately defined by criteria which only includes the domain of cognition;--Physical, legal, social and cultural factors defining the environment of patients and their families should be carefully examined and that the results of this examination should be used in conjunction with the results of the somatic and psychiatric assessment in planning care and placement of the patient;--patients with severe dementia should have access to palliative care; - family members should be included in the care plans for those with severe dementia who are in institutional care.

Clustering of behavioural and psychological symptoms in dementia (BPSD): a European Alzheimer's disease consortium (EADC) study.

OBJECTIVES: This cross-sectional study investigated the relationships between behavioural and psychological symptoms of dementia (BPSD). METHODS: We recruited 194 patients with dementia from the memory clinic practice of six European Alzheimer's Disease Consortium centres. BPSD were assessed using a pre-designed questionnaire comprising the Neuropsychiatric Inventory (NPI). BPSD scores were analysed by Spearman's correlation analysis and principal components factor analysis. Results were confirmed by performing Varimax rotation of the factors. RESULTS: The NPI symptoms occurred very frequently: 96% of the patients displayed at least one symptom. Among them, apathy (59.6%) and depression (58.5%) were the most common abnormalities, followed by irritability (44.6%), anxiety (44%) and agitation (41.5%). Four NPI-based factors were identified (58% of the common variance): psychosis factor (irritability, agitation, hallucinations and anxiety), psychomotor factor (aberrant motor behaviour and delusions), mood liability factor (disinhibition, elation and depression) and instinctual factor (appetite disturbance, sleep disturbance and apathy). CONCLUSION: Psychosis, psychomotor factor, mood liability factor and instinctual factor form four distinct behavioural syndromes in dementia. We report the novel observation that depression clusters together with disinhibition and elation within a mood liability factor. This finding highlights the nature of mood, and mood oscillations, from depression to disinhibition within the cluster, and has implications for treatment by taking into consideration the poles of the mood scale and any oscillation between them.

Long-Term use of rivastigmine in patients with dementia with Lewy bodies: an open-label trial.

Patients with dementia with Lewy bodies (DLB) have progressive deficits in cognition, parkinsonism, and neuropsychiatric symptoms. Cholinesterase inhibitors have been used to ameliorate cognitive decline and neuropsychiatric symptoms in short-term trials. In this study, patients with DLB were treated with rivastigmine up to 96 weeks. Improvement from baseline was seen in cognitive function as measured by the Mini-Mental State Examination (MMSE), and neuropsychiatric symptoms as measured by the Neuropsychiatric Inventory (NPI) over the first 24 weeks of treatment. By 96 weeks, neither the MMSE scores nor the NPI scores were significantly worse than at baseline.

Dementia with Lewy bodies treated with rivastigmine: effects on cognition, neuropsychiatric symptoms, and sleep.

Dementia with Lewy bodies (DLB) is a common cause of the dementia syndrome. Symptomatic treatment of the fluctuating cognition, visual hallucinations, and sleep disturbance that characterize this condition is challenging; neuroleptics are relatively contraindicated. We describe eight patients fulfilling the consensus diagnostic criteria for probable DLB who were treated with rivastigmine. Clinical features rated were: cognition by the Modified Mini-Mental State Examination (3MS); and behavioral and psychiatric symptoms by the Neuropsychiatric Inventory (NPI). Additional information was obtained from family and nursing reports. Seven patients showed resolution or improvement in cognition and neuropsychiatric symptoms as demonstrated by improvement in their 3MS and NPI scores. They also became more independent in mobility and activities of daily living, and the majority returned to live in their own home. Of the seven patients with sleep disruption, six improved. One case had no improvement in his symptomatology and the rivastigmine was stopped. Outcomes in this case series suggest that rivastigmine is well tolerated in clinical practice.

Rivastigmine in the treatment of dementia with Lewy bodies: preliminary findings from an open trial.

The objective of this study was to assess the tolerability and efficacy of rivastigmine in a group of patients with probable dementia with Lewy bodies (DLB), using an open label study. Open label treatment was with rivastigmine up to maximum tolerated dose (mean 9.6 mg daily, range 3-12 mg). Eleven patients with DLB, mean age 78.5 years, were treated with this cholinesterase inhibitor. After 12 weeks of treatment, mean Neuropsychiatric Inventory scores fell by 73% for delusions, 63% for apathy, 45% for agitation and 27% for hallucinations. Five of the patients (45%) experienced very significant clinical improvements that had not been achieved with other treatments, including low dose neuroleptics. Medication was well tolerated and parkinsonian symptoms tended to improve. Cholinesterase inhibition may be a safe and effective alternative to neuroleptic treatment in DLB. Such effects may also prove to be applicable to the management of neuropsychiatric symptoms in Parkinson's disease and Alzheimer's disease.

Clinical associations of lymphopenia in elderly persons admitted to acute medical and psychiatric wards.

Lymphopenia is a common finding in old people admitted to medical and psychiatric wards. We describe a pilot study of the clinical associations, and prognostic significance of lymphopenia in elderly persons admitted to acute medical and psychiatric wards. Consecutive patients admitted to acute medical and psychiatric wards were prospectively selected according to initial peripheral lymphocyte count (PLC) into lymphopenic (PLC < 1.0 X 10(9)/litre, n = 41), and non-lymphopenic (PLC > 1.5 x 10(9)/litre, n = 23). Results of routine haematological and biochemical investigations were recorded, as well as drug history and medical diagnoses. Anthropometric measurements, assessment of functional ability (Barthel ADL Index), and cognitive function (Mini-Mental State Examination) were then performed by investigators blind to lymphocyte status. Patients were contacted between 3 and 6 months following recruitment into the study. Lymphopenia was associated with functional ability as measured by a lower Barthel score (p = 0.004), and cognitive impairment as measured by the Mini-Mental State Examination (p = 0.02). No association was found with medical diagnostic groupings, drugs known to cause lymphopenia, nutritional status, or survival. Lymphopenia may be a significant marker of vulnerability, and a larger study is required to elucidate the veracity and mechanisms of lymphopenia-associated debility.

Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson's diseases.

OBJECTIVE: Motor and cognitive function were compared in patients with Lewy body dementia, Parkinson's disease, or Alzheimer's disease, to identify features that may be clinically useful in differentiating Lewy body dementia from Alzheimer's disease and Parkinson's disease. METHODS: A range of neuropsychological function and extrapyrimidal signs (EPS) was assessed in 16 patients with Lewy body dementia, 15 with Parkinson's disease, 25 with Alzheimer's disease, and 22 control subjects. RESULTS: The severity of total motor disability scores increased in the following order: controls approximately = Alzheimer's disease << Parkinson's disease < Lewy body dementia. Compared with patients with Parkinson's disease, patients with Lewy body dementia had greater scores for rigidity and deficits in the finger tapping test, but rest tremor and left/right asymmetry in EPS were more evident in Parkinson's disease. Patients with Lewy body dementia were also less likely to present with left/right asymmetry in EPS at the onset of their parkinsonism. "Sensitivity" to neuroleptic drugs was noted in 33% of patients with Lewy body dementia. Alzheimer's disease and Lewy body dementia groups had greater severity of dementia compared with the Parkinson's disease group and controls. Neuropsychological evaluation disclosed severe but similar degrees of impaired performances in tests of attention (digit span), frontal lobe function (verbal fluency, category, and Nelson card sort test) and motor sequencing in both Lewy body dementia and Alzheimer's disease groups, than Parkinson's disease and controls. In the clock face test, improved performance was noted in the "copy" compared to "draw" part of the test in controls, patients with Alzheimer's disease, and those with Parkinson's disease, but not in the patients with Lewy body dementia, who achieved equally poor scores in both parts of the test. CONCLUSIONS: EPS in Lewy body dementia resemble those seen in idiopathic Parkinson's disease, although less rest tremor and left/right asymmetry but more severe rigidity favours a diagnosis of Lewy body dementia. The unique profile of patients with Lewy body dementia seen in the clock face test suggests that this simple and easy to administer test may be useful in the clinical setting to differentiate Lewy body dementia and Alzheimer's disease.

Overview of differential diagnosis.

Alzheimer's disease (AD) is an example of cortical dementia, where most of the pathological changes lie in cortical association areas and the predominant clinical features are symptomatic of cortical dysfunction. The differential diagnosis of AD is complex and is usually made in two stages: diagnosis of the dementia syndrome and diagnosis of the underlying etiology. Each of these stages is complicated by the existence of conditions that mimic dementia in presentation and by the risk of inappropriately diagnosing AD where other conditions are responsible for the dementia state. Despite the fact that the concept of dementia, in terms of a decline in cognitive function and behavioral change, has existed for many years, there is still a general lack of agreement about the components of the syndrome. This article reviews the issues surrounding the differential diagnosis of the dementia syndrome and explores the points of differentiation between AD and some similar etiologies encountered when making a differential diagnosis.

Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop.

Recent neuropathologic autopsy studies found that 15 to 25% of elderly demented patients have Lewy bodies (LB) in their brainstem and cortex, and in hospital series this may constitute the most common pathologic subgroup after pure Alzheimer's disease (AD). The Consortium on Dementia with Lewy bodies met to establish consensus guidelines for the clinical diagnosis of dementia with Lewy bodies (DLB) and to establish a common framework for the assessment and characterization of pathologic lesions at autopsy. The importance of accurate antemortem diagnosis of DLB includes a characteristic and often rapidly progressive clinical syndrome, a need for particular caution with neuroleptic medication, and the possibility that DLB patients may be particularly responsive to cholinesterase inhibitors. We identified progressive disabling mental impairment progressing to dementia as the central feature of DLB. Attentional impairments and disproportionate problem solving and visuospatial difficulties are often early and prominent. Fluctuation in cognitive function, persistent well-formed visual hallucinations, and spontaneous motor features of parkinsonism are core features with diagnostic significance in discriminating DLB from AD and other dementias. Appropriate clinical methods for eliciting these key symptoms are described. Brainstem or cortical LB are the only features considered essential for a pathologic diagnosis of DLB, although Lewy-related neurites, Alzheimer pathology, and spongiform change may also be seen. We identified optimal staining methods for each of these and devised a protocol for the evaluation of cortical LB frequency based on a brain sampling procedure consistent with CERAD. This allows cases to be classified into brainstem predominant, limbic (transitional), and neocortical subtypes, using a simple scoring system based on the relative distribution of semiquantitative LB counts. Alzheimer pathology is also frequently present in DLB, usually as diffuse or neuritic plaques, neocortical neurofibrillary tangles being much less common. The precise nosological relationship between DLB and AD remains uncertain, as does that between DLB and patients with Parkinson's disease who subsequently develop neuropsychiatric features. Finally, we recommend procedures for the selective sampling and storage of frozen tissue for a variety of neurochemical assays, which together with developments in molecular genetics, should assist future refinements of diagnosis and classification.

Incidence of dementia in a representative British sample.

In a four-year follow-up study of 1042 elderly people (aged 65 years or older), randomly sampled from the community, levels of dementia were assessed using a two-phase case-finding procedure (screening followed by clinical interview) among survivors. Clinical information on those not reinterviewed was provided by death certificates, hospital case notes, or postal questionnaires. The weighted four-year cumulative incidence of dementia was 3.7% (95% confidence intervals: 2.4%-5.0%), with age-specific rates of 0.9%, 2.8%, 5.2%, 9.0%, and 8.7% for the age groups 65-69, 70-74, 75-79, 80-84, and 85-89 years respectively. While consistent with data from other British regions, it remains likely that these rates underestimate true incidence.

Neuropsychiatric systemic lupus erythematosus in elderly people: a case series.

Five elderly patients presenting with neuropsychiatric systemic lupus erythematosus were referred to the sectorised psychiatry service of the department of health care of the elderly. They represented 2% of patients admitted over a period of two years. Two patients presented with a subacute confusional state, two with dementia, and one with depression. Three patients responded well to treatment. This suggests that systemic lupus erythematosus (SLE) is more common in elderly people than was originally thought and is a potentially treatable cause of organic brain disorder. The absence of reports of elderly patients with SLE is likely to be due to the continued application of the American Rheumatism Association's revised 1982 classification criteria, which are inappropriate for this population.