Pregnancy and refractory epilepsy in Sturge-Weber syndrome: Challenges and outcomes.

Sturge-Weber Syndrome (SWS) is a rare neurocutaneous disorder caused by focal vascular dysplasia. It is associated with facial angiomas, ocular abnormalities, epilepsy, intellectual disability, and leptomeningeal angiomas. There is a paucity of literature regarding the management of epilepsy and pregnancy in women with SWS. The effects of pregnancy on this syndrome therefore remain largely undefined. Consensus on management is needed; as pregnancy has widespread effects on the vascular system and therefore may pose risk in women with SWS. This is a comprehensive review of the existing literature and case report of a woman with SWS, refractory epilepsy, and a successful pregnancy and delivery. Fifteen cases were found and, in addition to our case, 14 cases were reviewed, 11 of which discuss labor and delivery. Neurological complications were described in four cases and successful delivery without complication occurred in seven patient cases.

Active older adults goal setting outcomes for engaging in a physical activity app and the motivation characteristics of these goals (MOVEAGE-ACT).

Approximately 70% of older adults do not meet physical activity (PA) guidelines. While many interventions, are used in promoting PA, few target older adults or include substantial behavioural change techniques. Setting PA goals is often used but there is less research on goal setting outcomes, like improving health, preventing age effects, improving flexibility, goals that have been associated with increased likelihood of maintenance of PA. To understand the concept more fully in this cohort, the aim of this study was to identify older adults' goal setting outcomes - the purpose of engaging in a PA app and through analysis determine the motivation characteristics of these. A cross-sectional, qualitative online survey was completed by 24, 60+, community dwelling, mostly active, French and Irish older adults. Thematic template analysis was used, and the motivation of these outcomes was assessed using the Self-Determination Theory of Motivation. The themes were: improving/staying healthy or physically active, maintaining functional aspects of physical health, continuing to do the things I want, sustaining mental wellbeing, and preventing disease and aging. Individuals cited goal setting outcomes that were generic, specific or both, and goals related to maintenance of PA and prevention of aging decline, were cited most. The motivation characteristics of these goals in mostly active older adults were autonomous and internally driven. Interventions, including apps, for older adults that encourage them to set specific goal setting outcomes/purposes for PA, are likely to generate stronger internally driven motivation, enhance ownership and participation, and may therefore increase effectiveness.

Lennox-Gastaut Syndrome: Perspective of a Parent and a Physician.

This article is co-authored by a parent of a 32-year-old male patient with Lennox-Gastaut syndrome (LGS) and his epileptologist. It discusses the parent's experience of having a child with LGS from diagnosis through living day-to-day with the disease and the physician's perspective when treating this devastating epilepsy syndrome. The patient's mother, who is his legal representative, provided written consent for publication of this article.

Food Outlets Dietary Risk (FODR) assessment tool: study protocol for assessing the public health nutrition risks of community food environments.

BACKGROUND: Availability and accessibility of nutritious foods can vary according to the food outlets present within a neighbourhood or community. There is increasing evidence that community food environments influence food choice, diet and the risk of diet-related chronic disease, however contemporary community food environments assessments (e.g. unhealthy fast food outlets versus healthy supermarkets or fruit and vegetable shops) may be too simplistic to accurately summarise the complexities of their impacts on food choice. This study protocol describes the development of the Food Outlets Dietary Risk (FODR) assessment tool for use by local government in Perth, Western Australia. METHODS: Similar to food safety risk assessment, the FODR assessment tool rates the potential harmful public health nutrition impact of food outlets by identifying and characterising the issues, and assessing the risk of exposure. Scores are attributed to six public health nutrition attributes: 1) availability of nutrient-poor foods; 2) availability of nutritious foods; 3) acceptability and appeal; 4) accessibility; 5) type of business operation; and 6) complex food outlet considerations. Food retail outlets are then classified as having a low, medium, high or very high dietary risk based on their total score. DISCUSSION: A local government administered tool to rate the public health nutrition risk of food outlets requires data which can be collected during routine assessments or sourced from the internet. The ongoing categorical classification of foods available within food outlets as either unhealthy or nutritious will require nutrition scientists' input. An objective risk assessment of the dietary impact of food retail outlets can guide local government planning, policies and interventions to create supportive community food environments. It is intended that locally relevant data can be sourced throughout Australia and in other countries to apply the local context to the FODR assessment tool. Utility and acceptability of the tool will be tested, and consultation with environmental health officers and public health practitioners will inform future iterations.

A Qualitative Exploration of Electronic Image Sharing Among Young People: Navigating the Issues of Conformity, Trust, Intention, and Reputation.

This research sought to explore the main factors affecting young people's image-sharing behaviors, particularly in the context of peer relationships and norms. Ten focus groups were held with young people aged 13 to 14 years (Grade 9) with a total of 68 participants (39 females and 29 males) across 15 Australian schools contributing to the discussions. Focus groups were divided into either all-girls (n = 4), all-boys (n = 4), or mixed gender (n = 2) participants to observe gender-specific issues. Thematic analysis of the data revealed the considerable complexities that young people encounter daily when sharing images in the online environment, including how they navigate issues of conformity, trust, intention, and reputation. Gender-specific differences in young people's perceptions of risky image sharing were also observed. These findings have important implications for the design of school-based interventions to prevent and reduce harm from image-sharing behaviors, including the appointment and training of cyber ambassadors in schools to help positively influence peer-level norms about image sharing.

Stress as a seizure precipitant: Identification, associated factors, and treatment options.

Stress is a common and important seizure precipitant reported by epilepsy patients. Studies to date have used different methodologies to identify relationships between epilepsy and stress. Several studies have identified anxiety, depression, and childhood trauma as being more common in patients with epilepsy who report stress as a seizure precipitant compared to patients with epilepsy who did not identify stress as a seizure precipitant. In one survey study it was found that a majority of patients with stress-triggered seizures had used some type of stress reduction method on their own and, of those who tried this, an even larger majority felt that these methods improved their seizures. Additionally, small to moderate sized prospective trials, including randomized clinical trials, using general stress reduction methods have shown promise in improving outcomes in patients with epilepsy, but results on seizure frequency have been inconsistent. Based on these studies, we recommend that when clinicians encounter patients who report stress as a seizure precipitant, these patients should be screened for a treatable mood disorder. Furthermore, although seizure reduction with stress reduction methods has not been proven in a randomized controlled trial, other important endpoints like quality of life were improved. Therefore, recommending stress reduction methods to patients with epilepsy appears to be a reasonable low risk adjunctive to standard treatments. The current review highlights the need for future research to help further clarify biological mechanisms of the stress-seizure relationship and emphasizes the need for larger randomized controlled trials to help develop evidence based treatment recommendations for our epilepsy patients.

Singing for Lung Health-a systematic review of the literature and consensus statement.

There is growing interest in Singing for Lung Health (SLH), an approach where patients with respiratory disease take part in singing groups, intended to improve their condition. A consensus group was convened in early 2016 to address issues including: the specific features that make SLH distinct from other forms of participation in singing; the existing evidence base via a systematic review; gaps in the evidence base including the need to define value-based outcome measures for sustainable commissioning of SLH; defining the measures needed to evaluate both individuals' responses to SLH and the quality of singing programmes. and core training, expertise and competencies required by singing group leaders to deliver high-quality programmes. A systematic review to establish the extent of the evidence base for SLH was undertaken. Electronic databases, including Pubmed, OVID Medline and Embase, Web of Science, Cochrane central register of controlled trials and PEDro, were used. Six studies were included in the final review. Quantitative data suggest that singing has the potential to improve health-related quality of life, particularly related to physical health, and levels of anxiety without causing significant side effects. There is a significant risk of bias in many of the existing studies with small numbers of subjects overall. Little comparison can be made between studies owing to their heterogeneity in design. Qualitative data indicate that singing is an enjoyable experience for patients, who consistently report that it helps them to cope with their condition better. Larger and longer-term trials are needed.

Outpatient pharmacotherapy and modes of administration for acute repetitive and prolonged seizures.

Acute repetitive seizures (ARS) are a serious epilepsy phenomenon, generally described as closely grouped seizures over minutes to 2 days, representing an increase in seizure frequency compared with baseline. In some instances, ARS may not stop without treatment, and evolution into status epilepticus is a significant concern. Additionally, neuronal injury may occur after even brief repeated seizures. Given the substantial risks that may be involved with ARS, it is crucial to develop appropriate protocols for identification and management of this seizure phenomenon. This article focuses on pharmacotherapy and, in particular, different modes of administering medication for ARS in the outpatient setting. Our aim was to present a review of data from non-randomized and randomized, controlled trials to evaluate the efficacy, safety and tolerability of out-of-hospital ARS treatments. Several of the studies included patients with ARS, as well as patients with prolonged seizures. Prolonged seizures, or seizures lasting greater than 5 min, have similar risks and treatment options to those of ARS; therefore, this discussion also includes treatment trials and recommendations for prolonged seizures. All trials used benzodiazepines, a class of drugs that are ideal for the ARS and prolonged seizure populations because of their rapid onset of action and minimal adverse effects. Rectal diazepam is currently the only formulation approved by the US Food and Drug Administration (FDA) for out-of-hospital treatment. Oral benzodiazepines are appropriate only for mild ARS. Intramuscular diazepam autoinjection has shown success against ARS in clinical trials. Intranasal midazolam and diazepam are in testing. Other treatments have also been explored--specifically, buccal midazolam (approved in the European Union), sublingual lorazepam and intranasal lorazepam.

Perspectives of an international education initiative in children's palliative care.

Education is viewed as central to improving future palliative care for children and families across all countries. International education initiatives will ensure practitioners are aware of global health issues and can provide culturally sensitive care. Creative and innovative means of meeting such directives are required to achieve meaningful student learning. This paper focuses on one innovation, a children's palliative care workshop using case studies as a teaching method, with nursing students from the USA and nursing and midwifery students from the UK. Key learning points arising from student evaluation were recorded under three main themes, these were: differences across countries, similarities across countries, and making learning fun and memorable. Findings indicated that this joint learning activity was viewed positively by all students and has enabled them to learn with and from each other, potentially impacting on their future practice.

Developing self-regulation for dietary temptations: intervention effects on physical, self-regulatory and psychological outcomes.

We aimed to investigate whether a self-regulatory skills intervention can improve weight loss-related outcomes. Fifty-five participants (M BMI = 32.60 ± 4.86) were randomized into self-regulation training and advice groups and received two training workshops and weekly practice tasks. The self-regulation training group was trained to use six self-regulatory skills: Delayed gratification, thought control, goal setting, self-monitoring, mindfulness, and coping. The advice group received dietary and physical activity advice for weight loss. Physical, self-regulatory, and psychological measures were taken at baseline, end of intervention (week 8) and at follow-up (week 12). Using intention-to-treat analysis, weight, waist circumference, body fat and body mass index (BMI) were significantly reduced at follow-up for both groups. There were significant increases in all six self-regulatory skills and the psychological measures of self-efficacy, self-regulatory success, and physical self-worth for both groups. Results indicate that self-regulatory skills training might be as effective as dietary and physical activity advice in terms of weight loss and related outcomes.

An ecological momentary assessment of lapse occurrences in dieters.

PURPOSE: The aim of this study is to investigate the factors related to dietary lapse occurrence in a community sample of dieters. METHODS: An ecological momentary assessment (EMA) methodology, via mobile phone-based diaries, was employed to record dietary lapse occurrences in a group of dieters (N = 80; M age = 41.21 ± 15.60 years; M BMI = 30.78 ± 7.26) over 7 days. RESULTS: Analyses indicated that lapses were positively associated with the strength of dietary temptation, presence of others, coping responses, and the environment (exposure to food cues) in which the dieters were in; lapses were more likely to occur in the evening and were negatively associated with the use of coping mechanisms. Additionally, lapse occurrence was found to mediate the relationships among the above predictors of lapse and the self-efficacy to resist future dietary temptations. CONCLUSIONS: Results provide an insight into the occurrence of lapses in dieters and have implications for interventions focusing on weight loss maintenance and relapse prevention.

Multiple-goal management: an examination of simultaneous pursuit of a weight-loss goal with another goal.

This study investigated the characteristics contributing to successful goal attainment of a weight-loss and a non-weight-loss goal simultaneously. Weight-loss and non-weight-loss goals, self-efficacy, persistence, temptations, intergoal interference and facilitation were measured via an online questionnaire (N=103, Mage=25.36±6.9, 80% women). Weight-loss self-efficacy, persistence, temptations and intergoal facilitation significantly predicted weight-loss goal attainment. Those who were more successful in attaining both their goals simultaneously had higher self-efficacy and persistence and experienced less temptation towards both goals. This study provides an insight into the characteristics necessary for successful simultaneous management of a weight-loss goal with another goal.

Macroglossia in amyotrophic lateral sclerosis.

IMPORTANCE: We encountered 2 patients with amyotrophic lateral sclerosis (ALS) with tongue enlargement and protrusion outside the oral cavity (macroglossia). To our knowledge, the relationship between macroglossia and ALS has not been reported in the literature. The objective of this article was to describe the clinical characteristics, imaging, and pathology of macroglossia in ALS and to develop a hypothesis regarding its pathophysiology. OBSERVATIONS: Two patients developed progressive weakness at age 54 and 40 years. Both patients exhibited dysarthria, dysphagia, tongue atrophy, neck extensor weakness, and weakness of jaw closure during a 1-year period. Both required tracheostomy and mechanical ventilation and afterward developed macroglossia. A 3-dimensional-reconstructed sagittal computed tomographic image confirmed tongue protrusion outside the oral cavity with focal compression and showed the transition from the atrophied part of the tongue in the oropharynx to the edematous part outside the mouth. Tongue biopsy demonstrated fatty replacement and fascicles of degenerative muscle. CONCLUSIONS AND RELEVANCE: We are unaware of previous reports of macroglossia in ALS/motor neuron disease. Given the paucity of case material, we speculated that this is an extremely rare complication of ALS. Based on this series, we propose a pathophysiological mechanism by reviewing imaging and tongue biopsy.

Weight maintenance: self-regulatory factors underpinning success and failure.

OBJECTIVES: To investigate the differences in the contributing factors involved in weight maintenance success and failure. DESIGN: Semi-structured interviews were conducted with both successful and unsuccessful weight maintainers. Eighteen participants were recruited (16 women), nine of who had lost 10% of their body weight and maintained this weight for a minimum of 12 months (Maintainers), and nine individuals who met the above criteria for weight loss but had subsequently regained their weight (Regainers). A thematic analysis was employed to compare the differences between the two groups. RESULTS AND CONCLUSIONS: Two main themes highlighted the differences between the two groups, these were: goal regulation and self-control. Within these overarching themes, successful weight maintenance was related to the following subthemes: long-term, realistic goal setting, consistent use of routines and self-monitoring, avoiding deprivation and effective coping skills. Unsuccessful maintenance was related to short-term unrealistic goal setting, inconsistent routines and self-monitoring, experiencing deprivation and poor coping skills. These factors are explained in terms of the interrelationships that they have on one another and their subsequent impact on weight maintenance success or failure.

Chiropractic in the United States: trends and issues.

Chiropractic is the best established of the alternative health care professions. Although marginalized for much of the 20th century, it has entered the mainstream of health care, gaining both legitimacy and access to third-party payers. However, the profession's efforts to validate the effectiveness of spinal manipulative therapy, its principal modality, have yielded only modest and often contrary results. At the same time, reimbursement is shrinking, the number of practitioners is growing, and competition from other healing professions is increasing. The profession's efforts to establish a role in primary care are meeting resistance, and its attempts to broaden its activities in alternative medicine have inherent limitations. Although patients express a high level of satisfaction with chiropractic treatment and politicians are sympathetic to it, this may not be enough as our nation grapples to define the health care system that it can afford.

MRI and neuropsychological improvement in Huntington disease following ethyl-EPA treatment.

A 6-month randomized, placebo-controlled pilot study of the ethyl-ester of eicosapentaenoic acid (ethyl-EPA) was carried out in seven in-patients with advanced (stage III) Huntington's disease (three on ethyl-EPA, four on placebo; no significant difference in age or sex between the groups). After 6 months all the patients treated with ethyl-EPA improved on the orofacial component of the Unified Huntington's Disease Rating Scale while all the patients on placebo deteriorated on this scale (p < 0.03). Following subvoxel registration of follow-up 3D MRI brain scans with baseline scans, subtraction images showed that while the placebo was associated with progressive cerebral atrophy, the ethyl-EPA was associated with a reverse process. We conclude that treatment with ethyl-EPA is associated with beneficial motor and MRI changes.

Economic and demographic trends signal an impending physician shortage.

It is widely believed that the United States is producing too many physicians. We have approached this issue by developing a new model for workforce planning based on assessments of the macrotrends that underlie the supply and use of physician services. These trends include economic expansion, population growth, physicians' work effort, and the provision of services by nonphysician clinicians. Contrary to earlier predictions, this model projects that the United States soon will have a shortage of physicians and that if the pace of medical education remains unchanged, the shortage will become more severe. A dialogue focused on that eventuality is imperative.