EFNS-ENS Guidelines on the diagnosis and management of disorders associated with dementia

BACKGROUND AND OBJECTIVES: The last version of the EFNS dementia guidelines is from 2007. In 2010, the revised guidelines for Alzheimer's disease (AD) were published. The current guidelines involve the revision of the dementia syndromes outside of AD, notably vascular cognitive impairment, frontotemporal lobar degeneration, dementia with Lewy bodies, corticobasal syndrome, progressive supranuclear palsy, Parkinson's disease dementia, Huntington's disease, prion diseases, normal-pressure hydrocephalus, limbic encephalitis and other toxic and metabolic disorders. The aim is 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 dementing disorders. It represents a statement of minimum desirable standards for practice guidance. METHODS: The task force working group reviewed evidence from original research articles, meta-analyses and systematic reviews, published by June 2011. The evidence was classified (I, II, III, IV) and consensus recommendations graded (A, B, or C) according to the EFNS guidance. Where there was a lack of evidence, but clear consensus, good practice points were provided. RESULTS AND CONCLUSIONS: New recommendations and good practice points are made for clinical diagnosis, blood tests, neuropsychology, neuroimaging, electroencephalography, cerebrospinal fluid (CSF) analysis, genetic testing, disclosure of diagnosis, treatment of behavioural and psychological symptoms in dementia, legal issues, counselling and support for caregivers. All recommendations were revised as compared with the previous EFNS guidelines. The specialist neurologist together with primary care physicians play an important role in the assessment, interpretation and treatment of symptoms, disability and needs of dementia patients.     

EFNS guidelines for the diagnosis and management of Alzheimer’s disease

Background and objectives: In 2008 a task force was set up to develop a revision of the European Federation of the Neurological Societies (EFNS) guideline for the diagnosis and management of Alzheimer’s disease (AD) and other disorders associated with dementia, published in early 2007. The aim of this revised international guideline 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 AD. Mild cognitive impairment and non‐Alzheimer dementias are not included in this guideline. Methods: The task force working group reviewed evidence from original research articles, meta‐analysis, and systematic reviews, published before May 2009. The evidence was classified and consensus recommendations graded (A, B, or C) according to the EFNS guidance. Where there was a lack of evidence, but clear consensus, good practice points were provided. Results: The recommendations for clinical diagnosis, blood tests, neuropsychology, neuroimaging, electroencephalography, cerebrospinal fluid (CSF) analysis, genetic testing, disclosure of diagnosis, treatment of AD, behavioural and psychological symptoms in dementia, legal issues, counselling and support for caregivers were all revised as compared with the previous EFNS guideline. Conclusion: A number of new recommendations and good practice points are made, namely in CSF, neuropsychology, neuroimaging and reviewing non‐evidence based therapies. The assessment, interpretation, and treatment of symptoms, disability, needs, and caregiver stress during the course of AD require the contribution of many different professionals. These professionals should adhere to these guideline to improve the diagnosis and management of AD.     

EFNS‐ENS Guidelines on the diagnosis and management of disorders associated with dementia

Background and objectives

The last version of the EFNS dementia guidelines is from 2007. In 2010, the revised guidelines for Alzheimer's disease (AD) were published. The current guidelines involve the revision of the dementia syndromes outside of AD, notably vascular cognitive impairment, frontotemporal lobar degeneration, dementia with Lewy bodies, corticobasal syndrome, progressive supranuclear palsy, Parkinson's disease dementia, Huntington's disease, prion diseases, normal‐pressure hydrocephalus, limbic encephalitis and other toxic and metabolic disorders. The aim is 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 dementing disorders. It represents a statement of minimum desirable standards for practice guidance.

Methods

The task force working group reviewed evidence from original research articles, meta‐analyses and systematic reviews, published by June 2011. The evidence was classified (I, II, III, IV) and consensus recommendations graded (A, B, or C) according to the EFNS guidance. Where there was a lack of evidence, but clear consensus, good practice points were provided.

Results and conclusions

New recommendations and good practice points are made for clinical diagnosis, blood tests, neuropsychology, neuroimaging, electroencephalography, cerebrospinal fluid (CSF) analysis, genetic testing, disclosure of diagnosis, treatment of behavioural and psychological symptoms in dementia, legal issues, counselling and support for caregivers. All recommendations were revised compared with the previous EFNS guidelines. The specialist neurologist together with primary care physicians play an important role in the assessment, interpretation and treatment of symptoms, disability and needs of dementia patients.     

Guidance for the preparation of neurological management guidelines by EFNS scientific task forces – revised recommendations 2012

This paper is meant to provide guidance to anyone wishing to write a neurological guideline for diagnosis or treatment, and is directed at the Scientist Panels and task forces of the European Federation of Neurological Societies (EFNS). It substitutes the previous guidance paper from 2004. It contains several new aspects: the guidance is now based on a change of the grading system for evidence and for the resulting recommendations, and has adopted The Grading of Recommendations, Assessment, Development and Evaluation system (GRADE). The process of grading the quality of evidence and strength of recommendations can now be improved and made more transparent. The task forces embarking on the development of a guideline must now make clearer and more transparent choices about outcomes considered most relevant when searching the literature and evaluating their findings. Thus, the outcomes chosen will be more critical, more patient‐oriented and easier to translate into simple recommendations. This paper also provides updated practical recommendations for planning a guideline task force within the framework of the EFNS. Finally, this paper hopes to find the approval also by the relevant bodies of our future organization, the European Academy of Neurology.     

The prevalence, diagnosis and treatment of depression in dementia patients in chronic care facilities in the last six months of life

OBJECTIVE: To assess the prevalence, diagnosis and treatment of depression among dementia patients and normal controls in chronic care facilities in the last six months of life. METHOD: We reviewed perimortal data concerning dementia severity, depressive symptoms and diagnoses, and medication use for 279 dementia patients and 24 normal controls brought to autopsy through an Alzheimer's Disease Resource Center. RESULTS: Major depression was highly prevalent among both dementia patients and normal controls in chronic care facilities in the last six months of life. This depression was under-diagnosed by physicians. Documentation of depressive symptoms by medical support staff has improved over time. However, physician diagnosis of depression has not improved. Recognition of depression was significantly lower for patients with severe dementia. Depression was under-treated in both dementia patients and normal controls, although treatment rates may be increasing. Anxiolytics and hypnotics were often used in lieu of, or in addition to, antidepressant therapy. CONCLUSIONS: Major depression was highly prevalent in both dementia patients and normal controls, indicating that depression is an important issue for the elderly in the last six months of life irrespective of cognitive status. Under-diagnosis of depression may be an important clinical issue. As physician diagnosis of depression has not improved with time, further physician training and/or awareness initiatives may be warranted. Depression, a treatable cause of excess morbidity and mortality, was undertreated in all groups studied. However, treatment rates may be improving. The prevalent use of anxiolytics and hypnotics for depressed patients is problematic.     

Factors affecting timely recognition and diagnosis of dementia across Europe: from awareness to stigma

BACKGROUND: Timely recognition and diagnosis of dementia is the pre-condition for improving dementia care, but diagnosis often occurs late in the disease process. OBJECTIVE: To compare facilitators and obstacles to the timely recognition of dementia across eight European Union states, in order to implement established policies for earlier diagnosis. METHODS: A modified focus group technique, including a pre and posterior procedure. RESULTS: Twenty-three participants from different disciplines, purposively sampled for professional expertise in dementia research and innovative practice, attended two focus groups. Stigma in ageing and dementia, accompanied by a sense that there is little to offer until later on in the disease, underpinned the widespread reluctance of GPs to recognise dementia at an early stage and were major obstacles to the timely diagnosis of dementia across all eight countries. Dementia care services varied widely across Europe. Countries with the greatest development of dementia health care services were characterised by national guidelines, GPs fulfilling a gatekeeper function, multi-disciplinary memory clinics and innovative programmes that stimulated practice and new services. Dementia-related stigma was perceived as being less prominent in these countries. CONCLUSIONS: Overcome of delays in the timely diagnosis of dementia needs more than specialist services. They should address the processes associated with stigma, age and dementia, especially where these relate to physician practice and diagnostic disclosure. Stigma is perceived as variable across European States, with a promising finding that its impact is relatively small in countries with the widest range of dementia care services.     

Review of the therapeutic management of Parkinson's disease. Report of a joint task force of the European Federation of Neurological Societies (EFNS) and the Movement Disorder Society-European Section (MDS-ES). Part II: late (complicated) Parkinson's disease

To provide evidence-based recommendations for the management of late (complicated) Parkinson's disease (PD), based on a review of the literature. Complicated PD refers to patients suffering from the classical motor syndrome of PD along with other motor or non-motor complications, either disease-related (e.g. freezing) or treatment-related (e.g. dyskinesias or hallucinations). MEDLINE, Cochrane Library and INAHTA database literature searches were conducted. National guidelines were requested from all EFNS societies. Non-European guidelines were searched for using MEDLINE. Part II of the guidelines deals with treatment of motor and neuropsychiatric complications and autonomic disturbances. For each topic, a list of therapeutic interventions is provided, including classification of evidence. Following this, recommendations for management are given, alongside ratings of efficacy. Classifications of evidence and ratings of efficacy are made according to EFNS guidance. In cases where there is insufficient scientific evidence, a consensus statement ('good practice point') is made.     

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

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.     

Guidelines for legal and financial counseling of Alzheimer's disease patients and their families

As soon as there is reasonable evidence for the diagnosis of Alzheimer's disease (primary progressive degenerative dementia), the physician should urge legal and financial counseling of the patient and the family in planning for the patient's long-term care. The general purposes and the process of such counseling are described as a guide for physicians who care for these patients and their families. The issues of informed consent, competency, powers of attorney, guardianship, inter vivos trusts, wills, and living wills are discussed. Timely planning eases the burden on the family and assists the physician in the patient's care during the later stages of the disorder.     

Review of the therapeutic management of Parkinson's disease. Report of a joint task force of the European Federation of Neurological Societies and the Movement Disorder Society–European Section. Part I: early (uncomplicated) Parkinson's disease

The aim of the study was to provide evidence-based recommendations for the management of early (uncomplicated) Parkinson's disease (PD), based on a review of the literature. Uncomplicated PD refers to patients suffering from the classical motor syndrome of PD only, without treatment-induced motor complications and without neuropsychiatric or autonomic problems. MEDLINE, Cochrane Library and International Network of Agencies for Health Technology Assessment (INAHTA) database literature searches were conducted. National guidelines were requested from all European Federation of Neurological Societies (EFNS) societies. Non-European guidelines were searched for using MEDLINE. Part I of the guidelines deals with prevention of disease progression, symptomatic treatment of motor features (parkinsonism), and prevention of motor and neuropsychiatric complications of therapy. For each topic, a list of therapeutic interventions is provided, including classification of evidence. Following this, recommendations for management are given, alongside ratings of efficacy. Classifications of evidence and ratings of efficacy are made according to EFNS guidance. In cases where there is insufficient scientific evidence, a consensus statement (good practice point) is made.     

Guidance for the preparation of neurological management guidelines by EFNS scientific task forces.

The aim of an EFNS neurological management guideline is to provide guidance for clinical neurologists, other health care professionals and health care providers about important aspects of management of neurological disease. It represents the view of an expert task force appointed by the chairperson of the scientific committee with the agreement of the chairperson of a Scientist Panel. It will be a peer-reviewed statement of minimum desirable standards for the guidance of practice based on the best available evidence. It is not intended to have legally binding implications in individual cases.     

Appropriateness of psychostimulant prescription to children: theoretical and empirical perspectives.

OBJECTIVES: 1) To consider study designs for evaluating how appropriately stimulant medications are prescribed to children. 2) To investigate appropriateness by seeking evidence of consensus around how stimulant management should be undertaken, collecting and reviewing evidence of actual physician practice in relation to use of stimulants, and evaluating the closeness of fit between recommended and actual practice. METHOD: Electronic databases and other published and unpublished material were searched for the decade 1987-1997 to identify 1) authoritative recommendations for medical management of attention-deficit hyperactivity disorder (ADHD) and 2) surveys of physician practice. Available information was analyzed qualitatively for commonalities and trends and for concordance between recommendations and practice. RESULTS: Despite differences in emphasis between experts, common themes were identifiable regarding prerequisites and follow-up for use of stimulants. Overall, it was rare to find physician practice in accordance with expert recommendations more than 70% of the time, suggesting that management may be suboptimal in at least 30% of children treated with medication for ADHD. Across surveys, consistent differences appear in physician practice patterns related to practice type. Management by specialist consultants approximates standards recommended by experts, while management by primary care practitioners diverges from these standards. CONCLUSIONS: There are grounds for concern about the quality and appropriateness of medical management for children with ADHD. The methodology used in this study holds promise for wider application, with planning and refinement. There is a need to find ways to improve care for children with ADHD provided by primary care physicians.     

Diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy in clinical practice: A survey among Dutch neurologists

The diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is often a challenge. The clinical presentation is diverse, accurate biomarkers are lacking, and the best strategy to initiate and maintain treatment is unclear. The aim of this study was to determine how neurologists diagnose and treat CIDP. We conducted a cross‐sectional survey on diagnostic and treatment practices among Dutch neurologists involved in the clinical care of CIDP patients. Forty‐four neurologists completed the survey (44/71; 62%). The respondents indicated to use the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2010 CIDP guideline for the diagnosis in 77% and for treatment in 50%. Only 57% of respondents indicated that the presence of demyelinating electrophysiological findings was mandatory to confirm the diagnosis of CIDP. Most neurologists used intravenous immunoglobulins (IVIg) as first choice treatment, but the indications to start, optimize, or withdraw IVIg, and the use of other immune‐modulatory therapies varied. University‐affiliated respondents used the EFNS/PNS 2010 diagnostic criteria, nerve imaging tools, and immunosuppressive drugs more often. Despite the existence of an international guideline, there is considerable variation among neurologists in the strategies employed to diagnose and treat CIDP. More specific recommendations regarding: (a) the minimal set of electrophysiological requirements to diagnose CIDP, (b) the possible added value of nerve imaging, especially in patients not meeting the electrodiagnostic criteria, (c) the most relevant serological examinations, and (d) the clear treatment advice, in the new EFNS/PNS guideline, would likely support its implementation in clinical practice.     

EFNS guidelines on the molecular diagnosis of channelopathies,epilepsies, migraine,stroke, and dementias

Objectives: These EFNS guidelines on the molecular diagnosis of channelopathies, including epilepsy and migraine, as well as stroke, and dementia are designed to summarize the possibilities and limitations of molecular genetic techniques and to provide diagnostic criteria for deciding when a molecular diagnostic work‐up is indicated. Search strategy: To collect data about planning, conditions, and performance of molecular diagnosis of these disorders, a literature search in various electronic databases was carried out and original papers, meta‐analyses, review papers, and guideline recommendations were reviewed. Results: The best level of evidence for genetic testing recommendation (B) can be found for a small number of syndromes, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, severe myoclonic epilepsy of infancy, familial recurrent hemorrhages, familial Alzheimer’s disease, and frontotemporal lobar degeneration. Good practice points can be formulated for a number of other disorders. Conclusion: These guidelines are provisional, and the future availability of molecular genetic epidemiological data about the neurogenetic disorders under discussion in our article will allow improved recommendation with an increased level of evidence.     

A roadmap to advance dementia research in prevention,diagnosis, intervention,and care by 2025

Objective

National and global dementia plans have focused on the research ambition to develop a cure or disease‐modifying therapy by 2025, with the initial focus on investment in drug discovery approaches. We set out to develop complementary research ambitions in the areas of prevention, diagnosis, intervention, and care and strategies for achieving them.

Methods

Alzheimer's Society facilitated a taskforce of leading UK clinicians and researchers in dementia, UK funders of dementia research, people with dementia, and carer representatives to develop, using iterative consensus methodology, goals and recommendations to advance dementia research.

Results

The taskforce developed 5 goals and 30 recommendations. The goals focused on preventing future cases of dementia through risk reduction, maximising the benefit of a dementia diagnosis, improving quality of life, enabling the dementia workforce to improve practice, and optimising the quality and inclusivity of health and social care systems. Recommendations addressed gaps in knowledge and limitations in research methodology or infrastructure that would facilitate research in prioritised areas. A 10‐point action plan provides strategies for delivering the proposed research agenda.

Conclusions

By creating complementary goals for research that mirror the need to find effective treatments, we provide a framework that enables a focus for new investment and initiatives. This will support a broader and more holistic approach to research on dementia, addressing prevention, surveillance of population changes in risk and expression of dementia, the diagnostic process, diagnosis itself, interventions, social support, and care for people with dementia and their families.     

Longitudinal analysis of dementia diagnosis and specialty care among racially diverse Medicare beneficiaries

IntroductionThere is insufficient understanding of diagnosis of etiologic dementia subtypes and contact with specialized dementia care among older Americans.MethodsWe quantified dementia diagnoses and subsequent health care over five years by etiologic subtype and physician specialty among Medicare beneficiaries with incident dementia diagnosis in 2008/09 (226,604 persons/714,015 person-years).ResultsEighty-five percent of people were diagnosed by a nondementia specialist physician. Use of dementia specialists within one year (22%) and five years (36%) of diagnosis was low. “Unspecified” dementia diagnosis was common, higher among those diagnosed by nondementia specialists (33.2%) than dementia specialists (21.6%). Half of diagnoses were Alzheimer's disease.DiscussionAscertainment of etiologic dementia subtype may inform hereditary risk and facilitate financial and care planning. Use of dementia specialty care was low, particularly for Hispanics and Asians, and associated with more detection of etiological subtype. Dementia-related professional development for nonspecialists is urgent given their central role in dementia diagnosis and care.     

EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives

Despite being one of the most devastating diseases known, there is little evidence for diagnosing and managing patients with amyotrophic lateral sclerosis (ALS). Although specific therapy is lacking, correct early diagnosis and introduction of symptomatic and specific therapy can have a profound influence on the care and quality of life of the patient and may increase survival time. This document addresses the optimal clinical approach to ALS. The final literature search was performed in the spring of 2005. Consensus recommendations are given graded according to the EFNS guidance regulations. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. People affected with possible ALS should be examined as soon as possible by an experienced neurologist. Early diagnosis should be pursued and a number of investigations should be performed with high priority. The patient should be informed of the diagnosis by a consultant with a good knowledge of the patient and the disease. Following diagnosis, the patient and relatives should receive regular support from a multidisciplinary care team. Medication with riluzole should be initiated as early as possible. PEG is associated with improved nutrition and should be inserted early. The operation is hazardous in patients with vital capacity < 50%. Non-invasive positive pressure ventilation improves survival and quality of life but is underused. Maintaining the patients ability to communicate is essential. During the entire course of the disease, every effort should be made to maintain patient autonomy. Advance directives for palliative end of life care are important and should be fully discussed early with the patient and relatives respecting the patients social and cultural background.     

Dementia consults: predictors of compliance by primary care physicians

This study was designed to determine rates of primary care physician compliance with recommendations made by physician-consultants at an Alzheimer's disease center. A mail survey was sent to primary care physicians, who were asked to indicate which recommendations had been implemented and to select reasons for lack of adherence. The response rate was 53 percent (49/92). Some 83 percent of recommendations were implemented. Compliance with medical recommendations was higher (87 percent) than with social or behavioral issues (69 percent). Reasons for noncompliance indicated that patient or family barriers were responsible in 48 percent of cases, while physicians were responsible in 24 percent. These findings validate the development of two educational programs: curricula that support the primary care physician in conveying the value of these interventions and guidelines to assist specialists in communicating treatment recommendations.     

Diagnosis and treatment of amyotrophic lateral sclerosis according to EFNS recommendations (2005)

The article is focused on recent EFNS recommendations for the diagnosis and management of amyotrophic lateral sclerosis (ALS). In the commentary, the need for use of EFNS recommendations for ALS in clinical practice in Poland is emphasized. The importance of 1) complex respiratory and nutritional care, 2) symptomatic treatment, 3) multidisciplinary teamwork and palliative care, as well as 4) an individual rehabilitation programme and 5) communication status improvement, is stressed as a necessary step for improving the quality of life of Polish ALS patients.