Diagnosis and management of Alzheimer's disease and other disorders associated with dementia. The role of neurologists in Europe

In 1998 a task force to develop guidelines for diagnostic evaluation and treatment of dementia was initiated by the European Federation of Neurological Societies (EFNS) scientist panel on dementia. The aims of the task force were to provide evidence-based recommendations and to highlight the role of the neurologist in the management of patients with Alzheimer's disease and other disorders associated with dementia. We based our recommendations on a review of available evidence-based guidelines supplemented with further literature reviews. The recommendations were derived from consensus meetings and relate to individual patient management, as there are inadequate data on the cost-effectiveness of different diagnostic evaluations and treatments for dementia. Their specific applications will depend upon available resources. The particular contributions of the neurologist include: early identification and differential diagnosis of rare and common brain disorders causing cognitive and behavioural symptoms, referral for and interpretation of ancillary investigations, and identification and treatment of vascular and other concurrent diseases. A review of the management of dementia in Europe revealed considerable variation. In some countries neurologists have taken the lead in the management of patients with dementia, while in other countries the neurologist is rarely involved. We recommend that neurologists should have a clear role in the management of dementia in the whole of Europe. They should be involved in the diagnostic evaluation of dementia and facilitate the development of multidisciplinary teams for evaluation and management of patients with cognitive disturbances. The increasing role of neurology in the management of patients with dementia has important implications for training and education

Recommendations for the diagnosis and management of Alzheimer's disease and other disorders associated with dementia: EFNS guideline

The aim of this international guideline on dementia was to present a peer-reviewed evidence-based statement for the guidance of practice for clinical neurologists, geriatricians, psychiatrists, and other specialist physicians responsible for the care of patients with dementia. It covers major aspects of diagnostic evaluation and treatment, with particular emphasis on the type of patient often referred to the specialist physician. The main focus is Alzheimer's disease, but many of the recommendations apply to dementia disorders in general. The task force working group considered and classified evidence from original research reports, meta-analysis, and systematic reviews, published before January 2006. The evidence was classified and consensus recommendations graded according to the EFNS guidance. Where there was a lack of evidence, but clear consensus, good practice points were provided. The recommendations for clinical diagnosis, blood tests, neuroimaging, electroencephalography (EEG), cerebrospinal fluid (CSF) analysis, genetic testing, tissue biopsy, disclosure of diagnosis, treatment of Alzheimer's disease, and counselling and support for caregivers were all revised when compared with the previous EFNS guideline. New recommendations were added for the treatment of vascular dementia, Parkinson's disease dementia, and dementia with Lewy bodies, for monitoring treatment, for treatment of behavioural and psychological symptoms in dementia, and for legal issues. The specialist physician plays an important role together with primary care physicians in the multidisciplinary dementia teams, which have been established throughout Europe. This guideline may contribute to the definition of the role of the specialist physician in providing dementia health care

MAPT S305I mutation : implications for argyrophilic grain disease

Frontotemporal lobar degeneration (FTLD) with mutations in the tau gene (MAPT) causes familial frontotemporal dementia with tau pathology. Many of these mutations result in morphological phenotypes resembling sporadic tauopathies, although, to date, no such cases mimicking argyrophilic grain disease (AgD) have been documented. We now present a case with a novel S305I MAPT mutation and a morphological phenotype showing resemblance to AgD. At the age of 39, the patient developed behavioural and personality changes and lack of verbal fluency with later poor performance on naming tasks and rigidity in the extremities. After a short disease course of 1.5 years, the patient died. A unique neuropathological phenotype with neuronal diffuse cytoplasmic tau immunoreactivity, oligodendroglial-coiled bodies, argyrophilic grains, and non-argyrophilic, but tau-immunopositive and ubiquitin-immunonegative pre-grains were observed, whereas classical neurofibrillary tangles, Pick bodies, and neuritic plaques were absent. The tau-positive abnormal structures were composed only of 4R-tau isoforms and, ultrastructurally, straight filaments. Neuronal loss was greatest in the medial temporal cortex, hippocampus, and amygdala. These pathological features resemble AgD. The novel S305I substitution has a strong effect on MAPT exon 10 splicing, thereby causing a striking increase in 4R-tau isoforms. Our observation not only widens the phenotypic spectrum of FTLD with MAPT mutation but also underpins the notion that the predominance of similar neuropathological findings in sporadic AgD cases may be viewed as features of a distinct disease entity

Costs of dementia in Hungary

Objective The main aim of this paper is to give an overview on the quality of life, health care utilisation and costs of dementia in Hungary. Method A cross-sectional non-population based study of 88 consecutive dementia patients and their caregivers was conducted in three GP practices and one outpatient setting in 2008. Resource Utilization in Dementia (RUD), Mini Mental State Examination (MMSE) and quality of life (EQ-5D) were surveyed and cost calculations were performed. Costs of patients living at home were estimated by the current bottom-up cost-of-illness calculations, while costs of nursing home patients were considered by official reimbursement to determine the disease burden from a societal viewpoint. Results The mean age of the patients was 77.4 years (SD=9.2), 59% of them were female. The mean MMSE score was 16.70 (SD=7.24), and the mean EQ-5D score was 0.40 (SD=0.34). The average annual cost of dementia was 6,432 Euros per patient living at home and 6,086 Euros per patient living in nursing homes. For the whole demented population (based on EuroCoDe data) we estimated total annual costs of 846.8 million Euros; of which 55% are direct costs, 9% indirect costs and 36% informal care cost. Compared to acute myocardial infarction the total disease burden of dementia is 26.3 times greater. Conclusions This is the first study investigating resource utilisation, costs, and quality of life of dementia patients in the Central and Eastern European region. Compared to the general population of Hungary EQ-5D values of the demented patients are lower in all age groups. Dementia related costs are much lower in Hungary compared to Western European countries. There is no remarkable difference between the costs of demented patients living at home and in nursing homes, from the societal point of view