EFNS Guidelines on cognitive rehabilitation: report of an EFNS Task Force

In 1999, a Task Force was set up under the auspices of the European Federation of Neurological Societies with the aim to evaluate the existing evidence for the clinical effectiveness of cognitive rehabilitation. This review led to the development of a set of guidelines to be used in the management of adult patients with cognitive disorders due to acquired focal neurological damage.     

EFNS guideline on mild traumatic brain injury: report of an EFNS task force

In 1999, a Task Force on Mild Traumatic Brain Injury (MTBI) was set up under the auspices of the European Federation of Neurological Societies. Its aim was to propose an acceptable uniform nomenclature for MTBI and definition of MTBI, and to develop a set of rules to guide initial management with respect to ancillary investigations, hospital admission, observation and follow-up.     

EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force

Background: The evidence base for the diagnosis and management of amyotrophic lateral sclerosis (ALS) is weak. Objectives: To provide evidence‐based or expert recommendations for the diagnosis and management of ALS based on a literature search and the consensus of an expert panel. Methods: All available medical reference systems were searched, and original papers, meta‐analyses, review papers, book chapters and guidelines recommendations were reviewed. The final literature search was performed in February 2011. Recommendations were reached by consensus. Recommendations: Patients with symptoms suggestive of ALS should be assessed as soon as possible by an experienced neurologist. Early diagnosis should be pursued, and investigations, including neurophysiology, performed with a 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/carers should receive regular support from a multidisciplinary care team. Medication with riluzole should be initiated as early as possible. Control of symptoms such as sialorrhoea, thick mucus, emotional lability, cramps, spasticity and pain should be attempted. Percutaneous endoscopic gastrostomy feeding improves nutrition and quality of life, and gastrostomy tubes should be placed before respiratory insufficiency develops. Non‐invasive positive‐pressure ventilation also improves survival and quality of life. Maintaining the patient’s 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 should be discussed early with the patient and carers, respecting the patient’s social and cultural background.     

EFNS guideline on the diagnosis and management of alcohol-related seizures: report of an EFNS task force

Despite being a considerable problem in neurological practice and responsible for one-third of seizure-related admissions, there is little consensus as to the optimal investigation and management of alcohol-related seizures. The final literature search was undertaken in September 2004. Consensus recommendations are given graded according to the EFNS guidance regulations. To support the history taking, use of a structured questionnaire is recommended. When the drinking history is inconclusive, elevated values of carbohydrate-deficient transferrin and/or gammaglutamyl transferase can support a clinical suspicion. A first epileptic seizure should prompt neuroimaging (CT or MRI). Before starting any carbohydrate containing fluids or food, patients presenting with suspected alcohol overuse should be given prophylactic thiamine parenterally. After an alcohol withdrawal seizure (AWS), the patient should be observed in hospital for at least 24 h and the severity of withdrawal symptoms needs to be followed. For patients with no history of withdrawal seizures and mild to moderate withdrawal symptoms, routine seizure preventive treatment is not necessary. Generally, benzodiazepines are efficacious and safe for primary and secondary seizure prevention; diazepam or, if available, lorazepam, is recommended. The efficacy of other drugs is insufficiently documented. Concerning long-term recommendations for non-alcohol dependent patients with partial epilepsy and controlled seizures, small amounts of alcohol may be safe. Alcohol-related seizures require particular attention both in the diagnostic work-up and treatment. Benzodiazepines should be chosen for the treatment and prevention of recurrent AWS.     

EFNS guideline on the drug treatment of migraine – report of an EFNS task force

Migraine is one of the most frequent disabling neurological conditions with a major impact on the patients' quality of life. To give evidence-based or expert recommendations for the different drug treatment procedures of the different migraine syndromes based on a literature search and an consensus in an expert panel. All available medical reference systems were screened for all kinds of clinical studies on migraine with and without aura and on migraine-like syndromes. The findings in these studies were evaluated according to the recommendations of the EFNS resulting in level A,B, or C recommendations and good practice points. For the acute treatment of migraine attacks, oral non-steroidal anti-inflammatory drugs (NSAIDs) and triptans are recommended. The administration should follow the concept of stratified treatment. Before intake of NSAIDs and triptans, oral metoclopramide or domperidon is recommended. In very severe attacks, intravenous acetylsalicylic acid or subcutaneous sumatriptan are drugs of first choice. A status migrainosus can probably be treated by steroids. For the prophylaxis of migraine, betablockers (propranolol and metoprolol), flunarizine, valproic acid, and topiramate are drugs of first choice. Drugs of second choice for migraine prophylaxis are amitriptyline, naproxen, petasites, and bisoprolol.     

Left on the right or viceversa: a case of "alternating" constructional allochiria

We describe a patient with an ischemic right frontal lesion and mild left neglect who showed a systematic tendency to transpose drawings on one side of the page, which varied depending on the starting point (left or right) of his graphic productions. When not specifically cued, the patient started to draw in the ipsilesional (right) side and tended to show allochiria on the right, but occasionally, or under specific instructions, the patient started drawing from the left side and then showed a complete reversion of his spatial transpositions. To clarify the basic mechanisms underlying such a peculiar constructional phenomenon, we performed a series of experimental investigations, including extended copying tasks, a clock-marking test (to mark the position of single hours on a clock-face), and a line bisection task with progressive left-toright or right-to-left stimulus presentation. Findings suggested that "alternating" allochiria in copying and drawing from memory tasks is an epiphenomenon of a basic inability to move attention and action away from the starting point of graphic productions. The present case study, contrasted with observations on other brain-damaged patients, demonstrates that allochiria may have different neuro-cognitive bases and offers new insights for theoretical interpretations of unilateral spatial neglect.     

EFNS guideline on the drug treatment of migraine – revised report of an EFNS task force

Background:  Migraine is one of the most frequent disabling neurological conditions with a major impact on the patients' quality of life.
Objectives:  To give evidence-based or expert recommendations for the different drug treatment procedures in the particular migraine syndromes based on a literature search and the consensus of an expert panel.
Methods:  All available medical reference systems were screened for the range of clinical studies on migraine with and without aura and on migraine-like syndromes. The findings in these studies were evaluated according to the recommendations of the European Federation of Neurological Societies (EFNS) resulting in level A, B, or C recommendations and good practice points.
Recommendations:  For the acute treatment of migraine attacks, oral non-steroidal antiinflammatory drug (NSAID) and triptans are recommended. The administration should follow the concept of stratified treatment. Before intake of NSAID and triptans, oral metoclopramide or domperidone is recommended. In very severe attacks, intravenous acetylsalicylic acid or subcutaneous sumatriptan are drugs of first choice. Status migrainosus can be treated by cortoicosteroids, although this is not universally held to be helpful, or dihydroergotamine. For the prophylaxis of migraine, betablockers (propranolol and metoprolol) flunarizine, valproic acid, and topiramate are drugs of first choice. Drugs of second choice for migraine prophylaxis include amitriptyline, naproxen, petasites, and bisoprolol.     

EFNS guideline on neuroimaging in acute stroke. Report of an EFNS task force

Neuroimaging techniques are necessary for the evaluation of stroke, one of the leading causes of death and neurological impairment in developed countries. The multiplicity of techniques available has increased the complexity of decision making for physicians. We performed a comprehensive review of the literature in English for the period 1965–2005 and critically assessed the relevant publications. The members of the panel reviewed and corrected an initial draft, until a consensus was reached on recommendations stratified according to the European Federation of Neurological Societies (EFNS) criteria. Non-contrast computed tomography (CT) scan is the established imaging procedure for the initial evaluation of stroke patients. However, magnetic resonance imaging (MRI) has a higher sensitivity than CT for the demonstration of infarcted or ischemic areas and depicts well acute and chronic intracerebral hemorrhage. Perfusion and diffusion MRI together with MR angiography (MRA) are very helpful for the acute evaluation of patients with ischemic stroke. MRI and MRA are the recommended techniques for screening cerebral aneurysms and for the diagnosis of cerebral venous thrombosis and arterial dissection. For the non-invasive study of extracranial vessels, MRA is less portable and more expensive than ultrasonography but it has higher sensitivity and specificity for carotid stenosis. Transcranial Doppler is very useful for monitoring arterial reperfusion after thrombolysis, for the diagnosis of intracranial stenosis and of right-to-left shunts, and for monitoring vasospasm after subarachnoid hemorrhage. Currently, single photon emission computed tomography and positron emission tomography have a more limited role in the evaluation of the acute stroke patient.     

EFNS guideline on treatment of multiple sclerosis relapses: report of an EFNS task force on treatment of multiple sclerosis relapses.

Relapses, exacerbations or attacks of multiple sclerosis are the dominating feature of relapsing-remitting multiple sclerosis (MS), but are also observed in patients with secondary progressive MS. High-dose methylprednisolone is the routine therapy for relapses at present, but other treatments are also in current use. The objective of the task force was to review the literature on treatment of MS relapses to provide evidence-based treatment recommendations. Review was carried out on the literature with classification of evidence according to the EFNS guidelines for scientific task forces. Short-term, high-dose methylprednisolone treatment should be considered for the treatment of relapses of MS (level A recommendation). The optimal glucocorticoid treatment regimen, in terms of clinical efficacy and adverse events, remains to be established. A more intense, interdisciplinary rehabilitation programme should be considered as this probably further improves recovery after treatment with methylprednisolone (level B recommendation). Plasma exchange is probably efficacious in a subgroup of patients with severe relapses not responding to methylprednisolone therapy, and should be considered in this patient subgroup (level B recommendation). There is a need for further randomized, controlled trials in order to establish the optimal treatment regimen for relapses of MS.     

EFNS guideline on diagnosis and management of post-polio syndrome. Report of an EFNS task force

Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.     

Disorders of visual attention and the posterior parietal cortex

Traditionally, both the monkey and human posterior parietal cortex (PPC) have been considered to have a privileged role in spatial perception or action. Lesions to this region of the human brain, particularly of the right hemisphere, undoubtedly lead to spatially lateralised deficits such as visual extinction or neglect. However, although studies in monkeys have revealed much about the spatial functions of the parietal lobe, the monkey PPC may not be a good model system with which to understand fully the disorders of attention that follow damage to the human parietal cortex. Several lines of evidence, from functional imaging as well as investigations of patients with parietal damage, demonstrate that parts of the human inferior parietal lobe (IPL) have non-spatial functions. Here, we argue that it is important to distinguish spatially lateralised from spatial deficits. Both spatial and non-spatial impairments might, in principle, contribute to a spatially lateralised behavioural syndrome such as neglect. In this review, we discuss the evidence for such a proposal and suggest that a better understanding of human parietal syndromes may emerge from considering both the spatial and non-spatial functions of this region.     

Attention training in the cognitive rehabilitation of schizophrenic patients: a review of efficacy studies

OBJECTIVE: To review the literature on effects of attention and cognitive training on attentional functioning in schizophrenic patients. METHOD: Relevant efficacy studies were identified by literature searches (in Medline, Current Contents, Psyclit, and Psyndex databases) and through the reference lists of key papers. RESULTS: Re-examination of findings from nine methodologically adequate studies using computer-based or non-computer-based training procedures provided inconsistent results. On 16 of 35 outcome measures significant performance improvements were observed from pre-treatment to post-treatment but on the majority of the applied measures no performance improvement could be determined. In general, treatment effect sizes but also power of tests were low. For nearly all the outcome measures for which differential training effects were reported there also exist contradictory outcome results. CONCLUSION: There is inconclusive evidence that attention training is effective in schizophrenia. Longitudinal efficacy studies are needed in which different aspects of attention are systematically exercised and assessed.     

The effects of computer-based cognitive rehabilitation in patients with visuospatial neglect following stroke: a systematic review

Objectives: To identify studies concerning the effects of computer based cognitive rehabilitation (CBCR) on visuospatial neglect (VN) after stroke to summarize the current state of knowledge in this research field and make recommendations for future research.

Methods: Four electronic databases were systematically searched. Authors of relevant studies were contacted to detect unpublished data or articles not found by searching databases. Data was extracted from included studies using predefined coding schemes and characteristics and results of individual studies were summarized qualitatively.

Selection criteria: Studies were included if at least 50% of the included patients had a stroke, if the studies explored the effects of CBCR as a primary intervention for rehabilitation of VN and if they included neuropsychological outcome measures for the presence of VN.

Results: Seven studies were included. Six of the seven studies suggested positive effects of CBCR on VN after stroke. However, the study that did not find these effects was also the study with the strongest methodological quality. All included studies consisted of small samples, varied greatly in design and had various methodological limitations.

Conclusion: Because the existing literature is very sparse and studies have various methodological limitations, it is currently not possible to either support or reject the effects of CBCR on VN after stroke. Future studies should aim to compare CBCR with active and passive control conditions and include larger samples in randomized and blinded designs.     

Intensive cognitive rehabilitation therapy for chronic traumatic brain injury: a case study of neural correlates of functional improvement

Background: Some four million Americans are living with chronic disability related to traumatic brain injury (TBI). Yet, very little research has investigated training-induced neuroplasticity over many hours of treatment well into the chronic stage for the most commonly impaired functions (attention, executive functions (EFs), and prospective memory (PM)).

Aims: The purpose of the present study was to investigate whether a 30-h course of intensive evidence-based treatments of attention, EFs, and PM would show behavioural gains and associated brain structural and/or functional changes in an individual well into the chronic phase of recovery from TBI.

Methods & Procedures: An A-B single-participant quasi-experimental design with 1 month post-treatment follow-up was employed for cognitive rehabilitation of a 54-year-old man, 7.5 years after severe TBI. After an initial 4-week pre-treatment baseline phase, 30 hours of therapy was provided over 3 weeks, targeting attention, PM, and EFs, with follow-up testing at 1-month post-treatment. Treatments (selected from evidence-based practice guidelines) consisted of attention process training, metacognitive strategy instruction, and PM training. Neuroimaging (auditory oddball paradigm, resting state, and diffusion tensor imaging) was conducted weekly from the end of the A-phase, through the end of the B-phase, and then at 1-month follow-up.

Outcomes & Results: Results demonstrated gains to behavioural and quality of life measures for attention and EFs, with evidence of associated structural and functional neuroplasticity. There was no evidence of change to PM skills.

Conclusions: Intensive evidence-based treatment of attention and EFs improved functioning and quality of life in an individual well into the chronic phase of TBI, and may be associated with structural and functional neuroplasticity.     

Validation of an immersive virtual reality system for training near and far space neglect in individuals with stroke: a pilot study

Background: Unilateral spatial neglect (USN) is defined as impaired ability to attend and see on one side, and when present, it interferes seriously with daily life. These symptoms can exist for near and far spaces combined or independently, and it is important to provide effective intervention for near and far space neglect.

Objective: The purpose of this pilot study was to propose an immersive virtual reality (VR) rehabilitation program using a head-mounted display that is able to train both near and far space neglect, and to validate the immediate effect of the VR program in both near and far space neglect.

Methods: Ten USN patients underwent the VR program with a pre–post design and no control. In the virtual environment, we developed visual searching and reaching tasks using an immersive VR system. Behavioral inattention test (BIT) scores obtained pre- and immediate post-VR program were compared.

Results: BIT scores obtained pre- and post-VR program revealed that far space neglect but not near space neglect improved promptly after the VR program. This effect for far space neglect was observed in the cancelation task, but not in the line bisection task.

Conclusions: Positive effects of the immersive VR program for far space neglect are suggested by the results of the present pilot study. However, further studies with rigorous designs are needed to validate its clinical effectiveness.     

Guidelines on routine cerebrospinal fluid analysis. Report from an EFNS task force

A great variety of neurological diseases require investigation of cerebrospinal fluid (CSF) to prove the diagnosis or to rule out relevant differential diagnoses. The objectives were to evaluate the theoretical background and provide guidelines for clinical use in routine CSF analysis including total protein, albumin, immunoglobulins, glucose, lactate, cell count, cytological staining, and investigation of infectious CSF. The methods included a Systematic Medline search for the above-mentioned variables and review of appropriate publications by one or more of the task force members. Grading of evidence and recommendations was based on consensus by all task force members. It is recommended that CSF should be analysed immediately after collection. If storage is needed 12 ml of CSF should be partitioned into three to four sterile tubes. Albumin CSF/serum ratio ( Q _alb) should be preferred to total protein measurement and normal upper limits should be related to patients' age. Elevated Q _alb is a non-specific finding but occurs mainly in bacterial, cryptococcal, and tuberculous meningitis, leptomingeal metastases as well as acute and chronic demyelinating polyneuropathies. Pathological decrease of the CSF/serum glucose ratio or increased lactate concentration indicates bacterial or fungal meningitis or leptomeningeal metastases. Intrathecal immunoglobulin G synthesis is best demonstrated by isoelectric focusing followed by specific staining. Cellular morphology (cytological staining) should be evaluated whenever pleocytosis is found or leptomeningeal metastases or pathological bleeding is suspected. Computed tomography-negative intrathecal bleeding should be investigated by bilirubin detection.     

EFNS guideline on the treatment of tension‐type headache – Report of an EFNS task force

Background: Tension‐type headache (TTH) is the most prevalent headache type and is causing a high degree of disability. Treatment of frequent TTH is often difficult. Objectives: To give evidence‐based or expert recommendations for the different treatment procedures in TTH based on a literature search and the consensus of an expert panel. Methods: All available medical reference systems were screened for the range of clinical studies on TTH. The findings in these studies were evaluated according to the recommendations of the EFNS resulting in level A, B or C recommendations and good practice points. Recommendations: Non‐drug management should always be considered although the scientific basis is limited. Information, reassurance and identification of trigger factors may be rewarding. Electromyography (EMG) biofeedback has a documented effect in TTH, whilst cognitive‐behavioural therapy and relaxation training most likely are effective. Physical therapy and acupuncture may be valuable options for patients with frequent TTH, but there is no robust scientific evidence for efficacy. Simple analgesics and non‐steroidal anti‐inflammatory drugs are recommended for the treatment of episodic TTH. Combination analgesics containing caffeine are drugs of second choice. Triptans, muscle relaxants and opioids should not be used. It is crucial to avoid frequent and excessive use of analgesics to prevent the development of medication‐overuse headache. The tricyclic antidepressant amitriptyline is drug of first choice for the prophylactic treatment of chronic TTH. Mirtazapine and venlafaxine are drugs of second choice. The efficacy of the prophylactic drugs is often limited, and treatment may be hampered by side effects.     

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.