Pediatric stroke: what do we know and what do we need to know?

Stroke is a heterogeneous disorder and an important cause of mortality and chronic morbidity in children. Estimates of international incidence rates for childhood stroke have varied widely. Arterial ischemic stroke is reported to be more common than hemorrhagic stroke in children. The clinical presentation of stroke in children differs according to the child's age and stroke type and location. Several risk factors for ischemic and hemorrhagic stroke in children have been reported and include cardiac disorders, blood disorders, vasculopathies, viral infections, and arteriovenous malformations. Current treatment recommendations for stroke in children are based on small nonrandomized trials, adult stroke studies, case series, or consensus or individual expert opinion. Over half of children with stroke will develop lifelong cognitive or motor disability, and up to a third will have a recurrent stroke. International studies have provided important information on stroke in children, but major gaps in our knowledge of the disorder still exist. Currently, there is a need for prospective cohort studies in diverse populations, which utilize a consensus pediatric stroke classification system and a standard evaluation of risk factors and outcome, so that treatment and prevention strategies can be developed.     

Text level reading comprehension in aphasia: What do we know about therapy and what do we need to know?

Background: Reading comprehension difficulties are a common feature of aphasia, affecting the understanding of single words, sentences, paragraphs and extended text. Whilst there have been studies investigating treatment for single word reading, there are a limited number of studies of treatment methods targeting the reading of paragraphs and connected text.

Aims: This paper will describe a series of single case studies, each investigating the effectiveness of a different therapy approach for paragraph level reading comprehension. The studies raise a number of issues regarding the assessment of reading, choice of therapy and measurement of outcome; these will be discussed in relation to the studies and the wider evidence base.

Methods & Procedures: Four people with aphasia were involved in the studies. Participants presented with reading difficulties alongside other language difficulties. Each study involved a single case study, multiple baseline with control task design. Detailed assessment of reading comprehension was completed pre-therapy, post-therapy and at follow-up, approximately 2–4 weeks post-therapy. Participants were also asked about their pre-morbid reading abilities and preferences, their reading difficulties and their views about therapy and its impact.

Outcomes & Results: All participants showed some improvement in the accuracy of reading comprehension but there was extensive variability in the significance of gains, when gains were seen and on which assessments. Post-therapy, three of the four participants read the assessment passages and answered the questions more quickly. All participants reported some positive change in their reading, either in reading ability, reading behaviour or feelings about reading, but again individual variation was evident in the extent and type of change perceived.

Conclusions: These studies raise a number of important issues regarding the assessment of reading, therapy choice and measuring the outcome of therapy. These issues are discussed in relation to current literature, with an aim of informing future research investigating the assessment of, and therapy for, reading comprehension difficulties in people with aphasia. Reading is fundamental to everyday activities and developing the evidence base is of crucial importance in supporting people with aphasia to maximise their reading ability.     

Screening for depression in primary care: what do we still need to know?

The United States Preventive Services Task Force (USPSTF) recently issued the recommendation that primary care physicians screen adult patients for depression. A policy to screen primary care patients for depression has appeal as a strategy to reduce the personal and societal costs of undiagnosed and untreated depression. Such appeal may be justified if the evidence supports the screening policy in three areas: effectiveness, cost-effectiveness, and feasibility. The USPSTF recommendation leaves many issues in each of these areas unresolved and physicians are left the choice of two important program characteristics: screening instrument and screening interval. We discuss how uncertainties in the screening protocol and treatment process affect whether screening is an effective and cost-effective use of resources with respect to other health interventions. We suggest that targeting screening to groups at a higher risk for depression may lead to a more effective use of health care resources. A screening program may not be feasible even if effectiveness and cost-effectiveness are optimized. We discuss uncertainties in the USPSTF recommendation that affect the feasibility of implementing such a program in physicians' practices.     

Antiepileptic drug-induced mania in patients with epilepsy: what do we know?

Although mood disorders have a major impact on quality of life in patients with epilepsy, the nature of some aspects, such as mania, has received little attention. With the introduction of several new antiepileptic drugs into clinical practice, attention to psychiatric treatment-emergent adverse effects has been renewed. This article explores the relationship between antiepileptic drugs and mania in patients with epilepsy, with special emphasis on the clinical pathophysiology of this phenomenon.     

Cannabinoids for epilepsy: What do we know and where do we go?

Over the past decade there has been an increasing interest in using cannabinoids to treat a range of epilepsy syndromes following reports of some remarkable responses in individual patients. The situation is complicated by the fact that these agents do not appear to work via their attachment to endogenous cannabinoid receptors. Their pharmacokinetics are complex, and bioavailability is variable, resulting in difficulty in developing a suitable formulation for oral delivery. Drug interactions also represent another complication in their everyday use. Nevertheless, recent randomized, placebo‐controlled trials with cannabidiol support its efficacy in Dravet and Lennox‐Gastaut syndromes. Further placebo‐controlled studies are underway in adults with focal epilepsy using cannabidivarin. The many unanswered questions in the use of cannabinoids to treat epileptic seizures are briefly summarized in the conclusion.     

McArdle disease: what do neurologists need to know?

McArdle disease (also known as glycogen storage disease type V) is a pure myopathy caused by an inherited deficit of myophosphorylase, the skeletal muscle isoform of the enzyme glycogen phosphorylase. The disease exhibits clinical heterogeneity, but patients typically experience exercise intolerance, that is, reversible, acute crises (early fatigue and contractures, sometimes with rhabdomyolysis and myoglobinuria) triggered by static muscle contractions (e.g. lifting weights) or dynamic exercise (e.g. climbing stairs or running). In this Review, we discuss the main features of McArdle disease, with the aim of providing neurologists with up-to-date, useful information to assist their patients. The topics covered include diagnostic tools-for example, molecular genetic diagnosis, the classic ischemic forearm test and the so-called 'second wind' phenomenon-and current therapeutic options-for example, a carbohydrate-rich diet and carbohydrate ingestion shortly before strenuous exercise, in combination with medically supervised aerobic training of low to moderate intensity.     

ECT in the Asia Pacific region: what do we know?

OBJECTIVE: To review and describe the practice of ECT in the Asia Pacific region in the year 2000. METHOD: A survey of 34 defined countries within the Asia Pacific region was made prior to the 1st Asia Pacific ECT Conference held in Melbourne, Australia, 2001. RESULTS: Contact addresses for 23 of 34 countries (70%) were found with responses from 12 different countries (35%). Individual responses were received from less than 1% of the total mail out for the conference. The percentage of inpatients who received ECT was consistently less than or equal to 9%, except for Nepal where it was 25.6%. Except for Kiribati and the Solomon Islands, all devices delivered brief pulse, square wave currents. All of the 12 countries surveyed used anesthesia, preferred bilateral electrode placement and reported a response rate of at least 86%. Adverse events were uncommon, memory being the most commonly reported side effect. Community attitudes were generally negative. CONCLUSION: Despite the difficulties in attempting to generalize about this huge and diverse region, a number of seemingly universal findings appeared in accord with the world literature. These included the widespread use of ECT, its effectiveness and its relative safety despite equally widespread community reluctance.     

Temporal lobe epilepsy surgery: what do patients want to know?

Patients with pharmacoresistant temporal lobe epilepsy (TLE) contemplating brain surgery must make a complex treatment decision involving trade-offs. Patient decision aids, containing information on the risks and benefits of treatment interventions, increase patient knowledge and facilitate shared decision making between patients and physicians. We conducted five focus groups to describe the information patients need to make informed decisions about TLE surgery. Twenty patients who had undergone TLE surgery described the information used in their decision-making process, and evaluated the potential for a patient decision aid to assist other patients who are considering surgery. Thematic analysis revealed information needs that were both experiential (i.e., learning about other patients’ experiences through testimonials) and factual (i.e., individualized statistical information). Patients also made suggestions on how this information should be delivered to patients. These data will accelerate the development of a patient decision aid designed to assist TLE patients in their decision making about epilepsy surgery.     

Treating anxiety disorders in children and adolescents with epilepsy: What do we know?

Children with epilepsy are at significant risk of psychiatric disorders, which can in turn negatively impact social skills development, academic achievement, and quality of life. The most commonly reported psychiatric comorbidities in pediatric epilepsy are ADHD, depression, and anxiety. The prevalence rates of anxiety disorders in pediatric epilepsy range from 5 to 49%, and in the general population, anxiety disorders are the most common psychiatric disorder in childhood. For the purposes of this review, anxiety disorders will be examined in order to 1) examine rates of anxiety disorders in children and adolescents with epilepsy, 2) review treatment options for anxiety disorders in children with epilepsy, and 3) identify future avenues for the development of evidence-based practices for the treatment of anxiety disorders in youth with epilepsy.     

The chance of cure following surgery for drug-resistant temporal lobe epilepsy. What do we know and do we need to revise our expectations?

Although surgery is often seen as a curative treatment for patients with drug-resistant temporal lobe epilepsy, little information is available how many cases can be considered cured after surgery, i.e. are seizure-free for several years without taking antiepileptic drugs (AEDs). In our review, 13 retrospective and five prospective clinical observations published since 1980 provided data on long-term seizure control off AEDs in a total of 1658 patients. No randomized studies were found. Following temporal lobe surgery, approximately one in four adult patients and approximately one in three children or adolescents can currently shown to be seizure-free for 5 years without AEDs (25%, mean of eight studies in adults, 95% CI: 21–30%, and 31%, mean of three studies in children, 95% CI: 20–41%). The rate of seizure control off AEDs seemed to be stable after 2 years of follow-up. However, as 55% of patients free of disabling seizures preferred not to discontinue their medication completely as late as 5 years after surgery, it is impossible to know if they are cured or not. No features predictive of surgical cure were detected except for better cure outcome in children versus adults with hippocampal sclerosis and in patients with typical versus atypical Ammonshorn's sclerosis or tumor in one small study each. In conclusion, the available evidence on seizure outcome off AEDs after temporal lobe surgery is based on non-randomized studies and, in part, data were collected retrospectively. A randomized controlled trial is needed to determine if, in fact only one in three to four patients with temporal lobe epilepsy undergoing surgery can be considered cured.     

Recovery-based practice: do we know what we mean or mean what we know?

The concept of recovery is now widely promoted as the guiding principle for the provision of mental health services in Australia and overseas. While there is increasing pressure on service providers to ensure that services are recovery oriented, the way in which recovery-based practice is operationalized at the coalface presents a number of challenges. These are discussed in the context of five key questions that address (i) the appropriateness of recovery as a focus for service delivery, (ii) the distinction between recovery as a process and an outcome, (iii) the assessment of recovery initiatives, (iv) the alignment of recovery with current service delivery models, and (v) the risks associated with recovery-based practice. It is argued that these questions provide a framework for a debate that must extend beyond patients and providers of mental health services to the broader public, whose attitudes will ultimately determine the possibilities and limits of recovery-oriented practice.     

Childhood epilepsy: what is the evidence for what we think and what we do?

This article reviews the strength of the evidence that underlies the current approach to the management of childhood epilepsy. The authors reviewed published, peer-reviewed English literature accessed through PubMed and Cochrane reviews with evidence rated as Class 1 (strongest) to Class 4 (weakest). There is considerable inaccuracy in the diagnosis of seizures and epilepsy syndromes. Sound information supports the consensus that the diagnosis of epilepsy should await two unprovoked seizures. Population-based studies indicate that remission from childhood onset epilepsy occurs in at least 50% of children. It is easier to predict a good seizure outcome than a poor one. Absence of concomitant neurologic handicap and onset before about 12 years of age are the most consistent predictors of remission. Intractability is poorly defined and difficult to predict until several antiepilepsy drugs have been used and failed to control the seizures. Most epilepsy syndrome diagnoses do not yield an accurate prognosis. Social outcome appears unsatisfactory in about 50% of cases without intellectual handicap. Death is rare in childhood epilepsy. Those without severe neurologic handicaps have the same mortality as the general population. We identified only 27 published randomized trials of antiepilepsy drugs in children that compare the efficacy of antiepilepsy drugs, offer treatment of syndromes currently without successful treatment, or have negative effects. There is a pressing need for better definitions of seizures and epilepsy syndromes. The causes of poor social outcome are unclear. Intractability needs a clear definition and randomized trials comparing treatment regimes are sadly lacking.     

Brain temperature: what do we know?

This review article presents a summary of recent efforts to understand brain temperature and its regulation under different conditions. Brain temperature has a crucial influence on brain processes. Its regulation is the outcome of the balance of core and arterial blood temperatures, cerebral blood flow, brain metabolism, functional conditions, and external temperature. However, the relationship between these factors is not fully understood and several uncertainties remain. There are no satisfactory normative data on temperatures throughout the brain, but new technologies promise to fill this gap. Brain temperature changes with brain functional activation and under pathological conditions in ways that are not understood. A full understanding of brain temperature control is mandatory to optimize attempts at brain cooling during clinical conditions such as stroke and head injury.     

What do we know about the role of IncRNAs in multiple sclerosis?

Multiple sclerosis is a chronic,inflammatory and degenerative disease of the central nervous system of unknown aetiology although well-defined evidence supports...     

Stroke care in Spain. What do we have? What do we need?

The high level of scientific evidence which supports the recommendations for the care of acute stroke in Stroke Units (SU) with a good health care network, it does not correspond to the level of introduction in Spain. In this regard, the Cerebrovascular Diseases Study Group (GEECV) of the Spanish Society of Neurology has taken the initiative to conduct the "National Survey of Stroke Care" that will help to determine the real situation in acute stroke management in Spain just before the approval of the National Stroke Strategy (NSS) by the Ministry of Health and concludes that in the first semester of 2009 there were 39 SUs, unevenly distributed with higher concentration in Madrid and Barcelona. Although the approval of the NSS was a major achievement, much remains to be done to meet the objectives. We thank the GEECV?s initiative, which gives us an "X-ray" of the, not very satisfactory, state of stroke care in Spain in December 2008, and highlighting some achievements and the many shortcomings. Therefore, we must continue to improve, refine our data collection with records that include all available resources and all the stroke patients attended. We invite GEECV to carry out a second study to evaluate the impact of NSS and to serve as a stimulus to achieve a substantial improvement in stroke care in Spain, closer to the recommendations of the new PASI document.