Care of pharmaco-resistant absence seizures in childhood.

In childhood absence epilepsy, pharmaco-resistance occurs in 20-30% of patients. In that situation, glucose transporter type 1 deficiency has to be ruled out, especially if absences started before the age of four years and if neurological signs are present. If ethosuximide, valproate and lamotrigine have failed in monotherapy or in association, there are currently no valuable therapeutic options. The same rules apply for epilepsy with myoclonic absences. Importantly, arguments supporting that making the patient seizure-free will improve eventual associated cognitive deficits such as attention deficit are very weak. Therefore, limiting the cognitive side effects of the anti-epileptic drugs has always to be a priority when faced with typical refractory absences in childhood. In epilepsy with eyelid myoclonia, the majority of patients are pharmaco-resistant. However, absence seizures, if present, tend to be very brief, and seizures are limited in many patients to eyelid myoclonia that eventually do not affect their quality of life and are well attenuated by wearing blue lenses. Atypical absences occurring in the course a developmental and/or epileptic encephalopathy are often pharmaco-resistant. In that situation, characterizing the type of epilepsy syndrome and searching for a specific genetic or structural etiology are needed to offer the best therapeutic options to the patient.

Work stress and burnout among emergency physicians: a systematic review of last 10 years of research.

AIM OF THE STUDY: First, to provide a synthesis and analysis of available scientific literature regarding the level of work stress and burnout among emergency physicians. Second, to identify the effect of the specific work situation-related factors. METHODS: A systematic search was performed in NCBI PubMed and Embase. Comparative primary studies, both systematic review and cross-sectional, quantifying burnout in emergency physicians were included. Only studies published between 2011 and 2022 were retained. Synonym sets were compiled for the search key for 'burnout & stress', 'emergency', 'physician' and 'burnout & posttraumatic stress disorder'. RESULTS: Thirty-five papers were retained for further research. Emergency physicians scored significantly higher for all dimensions of burnout compared to other healthcare professions. Significant correlations for burnout were found with work characteristic and organizational factors. Critical incidents and aggression were identified as the most important acute work characteristics and organizational factors impacting emergency physician's mental wellbeing including the development of posttraumatic stress disorder. Moreover, personal factors such as age, personality, and coping strategies also play an important role in the development of burnout as well as work-related trauma. CONCLUSION: Available studies show that emergency physicians report higher scores of emotional exhaustion and depersonalization when compared to other healthcare professionals. Work characteristics contribute to this, but work-related traumatic incidents and aggression are important determinants. Personal characteristics such as age, personality type D, previous experiences and coping strategies seem to be determining factors likewise. Emergency physicians showed a high risk for developing burnout and work stress-related problems.

Long-term outcome of developmental and epileptic encephalopathies.

Developmental and epileptic encephalopathies are conditions where there is developmental impairment related to both the underlying etiology independent of epileptiform activity and the epileptic encephalopathy. Usually they have multiple etiologies. Therefore, long-term outcome is related to both etiology-related factors and epilepsy-related factors-age at onset of epilepsy, type(s) of seizure(s), type of electroencephalographic abnormalities, duration of the epileptic disorder. This paper focuses on long-term outcome of six developmental and epileptic encephalopathies with onset from the neonatal period to childhood: early epileptic encephalopathy with suppression bursts, West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, epilepsy with myoclonic atonic seizures and epileptic encephalopathy with continuous spike and waves during slow-wave sleep including Landau-Kleffner syndrome. For each syndrome, definition, main etiologies if multiple, and long-term outcome are discussed.

A legal framework on advanced practice nursing in Belgium: what do we and don't we know?

OBJECTIVES: Presenting the Belgian new framework for Advanced Practice Nursing (APN) - 'Verpleegkundig Specialist [VS]'/"Infirmier de pratique avancée [IPA]" outlined in the Law of 22 April 2019, followed by a discussion of the lack of clarity, the current challenges and future opportunities. METHODS: The framework was analyzed by an expert in healthcare legislation and discussed by academics in Nursing Science and members of the board of directors of the Belgian Society of APN. RESULTS: Relevant paragraphs within this new law are"Article 46 §1. No one is allowed to carry the title of 'VS/IPA' who does not possess a bachelor in nursing mentioned in article 45 and who does not meet the requirements specified in this article. At the minimum, a master's degree in Nursing Sciences is also required. §2. Additional to the scope of practice of nursing as mentioned in article 46, the 'VS/IPA' perform, in the context of complex nursing care, medical interventions in order to maintain, improve or restore the health of the patient. Care is provided in the context of a specific target group of patients and in close concertation with the physician and potential other healthcare professionals. CONCLUSION: Although the legal recognition of the title of VS/IPA is a major breakthrough that will innovate healthcare, clarification is needed: How do VS/IPA distinguish themselves from other nursing functions, what is complex nursing care, which medical interventions can be performed, what is meant by specific target group of patients, what does 'in close concertation with the physician' entail, and will advisory power be possible?

Systematic review of international clinical guidelines for the promotion of physical activity for the primary prevention of cardiovascular diseases.

BACKGROUND: Cardiovascular diseases are the world's leading cause of morbidity and mortality. An active lifestyle is one of the cornerstones in the primary prevention of cardiovascular disease. An initial step in guiding primary prevention programs is to refer to clinical guidelines. We aimed to systematically review clinical practice guidelines on primary prevention of cardiovascular disease and their recommendations regarding physical activity. METHODS: We systematically searched Trip Medical Database, PubMed and Guidelines International Network from January 2012 up to December 2020 using the following search strings: 'cardiovascular disease', 'prevention', combined with specific cardiovascular disease risk factors. The identified records were screened for relevance and content. We methodologically assessed the selected guidelines using the AGREE II tool. Recommendations were summarized using a consensus-developed extraction form. RESULTS: After screening, 27 clinical practice guidelines were included, all of which were developed in Western countries and showed consistent rigor of development. Guidelines were consistent about the benefit of regular, moderate-intensity, aerobic physical activity. However, recommendations on strategies to achieve and sustain behavior change varied. Multicomponent interventions, comprising education, counseling and self-management support, are recommended to be delivered by various providers in primary health care or community settings. Guidelines advise to embed patient-centered care and behavioral change techniques in prevention programs. CONCLUSIONS: Current clinical practice guidelines recommend similar PA lifestyle advice and propose various delivery models to be considered in the design of such interventions. Guidelines identify a gap in evidence on the implementation of these recommendations into practice.

Opsoclonus in a child with neuroborreliosis: Case report and review of the literature.

Opsoclonus consists of massive erratic rapid eye jerks. They may occur in isolation or in association with myoclonus and ataxia, i.e., opsoclonus-myoclonus syndrome (OMS). We report the case of a 9-year-old girl who suffered from headaches for several days and was shown to have opsoclonus and left peripheral facial palsy. Work-up excluded the diagnosis of neuroblastoma, but CSF analysis showed aseptic meningitis, and serology for Borrelia burgdorferi (Lyme) was positive. The outcome was favorable with complete regression of symptoms after treatment with ceftriaxone 2g/day for 3 weeks. Although rare, the diagnosis of Lyme neuroborreliosis must be raised in the presence of isolated opsoclonus, particularly if the clinical picture is incomplete and if other features, such as peripheral facial palsy and pleocytosis in the CSF, are present.

SolCos model-based individual reminiscence for older adults with mild to moderate dementia in nursing homes: a randomized controlled intervention study.

WHAT IS KNOWN ON THE SUBJECT?: To stimulate reminiscence of older adults with dementia performed individually or through group sessions is a well-known practice in nursing homes resulting in effects on behaviour and well-being as an alternative for medication. Robust scientific proof of the effectiveness of individual reminiscence therapy performed in nursing homes is sparse. WHAT THIS PAPER ADDS TO EXISTING KNOWLEDGE?: We have provided individual standardized reminiscence therapy to residents with dementia. The therapy was developed and tested in a previous study and performed in this study by trained nursing home volunteers. In comparison with a control group who received usual care, residents who received the reminiscence therapy showed significant less depressive symptoms. Moreover, residents were, in general, attentive, open and collaborative during the sessions and volunteers experienced the sessions as useful and pleasant. WHAT ARE THE IMPLICATIONS FOR PRACTICE?: Individual reminiscence therapy can be learned and used by nursing home volunteers to improve care in nursing homes. ABSTRACT: Aim To investigate the effect of a standardized individualized intervention based on the SolCos transformational reminiscence model on depressive symptoms (primary outcome), cognition and behaviour (secondary outcomes) for older people with mild to moderate dementia, performed by trained nursing home volunteers as facilitators. Background Because of limited pharmacological treatment options for older adults with dementia relevant physical, sensory, psychological or social interventions offer alternative opportunities. Method Randomized controlled trial (ISRCTN74355073) was set up in two nursing homes with 29 and 31 residents in the intervention and the control groups respectively. Eighteen nursing home volunteers were trained to perform the reminiscence therapy. Various assessment scales were measured pre- and post-sessions. Results Linear regression analysis showed an impact on depressive symptoms. However, no impact was identified on cognition and behaviour. Facilitators experienced the sessions as useful and pleasant, and study participants were, in general, attentive, open and collaborative. Discussion Study results showed that organizing standardized individual reminiscence therapy with nursing home volunteers was feasible and study participants' attention and participation were overall good. Further study initiatives to explore the potential of individual reminiscence therapy within a person-centred framework are recommended in order to improve care in nursing homes.

Effect of movement rate on corticokinematic coherence.

AIMS OF THE STUDY: This study investigates the effect of movement rate on the coupling between cortical magnetoencephalographic (MEG) signals and the kinematics of repetitive active finger movements, i.e., the corticokinematic coherence (CKC). MATERIAL AND METHODS: CKC was evaluated in ten right-handed healthy adults performing repetitive flexion-extension of the right-hand fingers in three different movement rate conditions: slow (∼1 Hz, duration: 11 min), medium (∼2 Hz, duration: 5 min) and fast (∼3 Hz, duration: 3 min). Neuromagnetic signals were recorded with a whole-scalp-covering MEG (Elekta Oy) and index acceleration was monitored with a 3-axis accelerometer. Coherent sources were estimated on the time-course of the cross-correlogram using equivalent current dipole (ECD) modeling. RESULTS: Significant coherence was found at movement frequency or its first harmonics in all subjects and movement conditions. ECDs clustered at the primary sensorimotor cortex contralateral to hand movements. Movement rate had no effect on the coherence levels and the location of coherent sources. CONCLUSIONS: This study demonstrates that the movement rate does not affect coherence levels and CKC source location during active finger movements. This finding has direct implications for CKC functional mapping applications and studies investigating the pathophysiology of central nervous disorders affecting proprioceptive pathways.

[Systematic follow-up of infants born preterm]. / Le suivi systématique des grands prématurés.

Advances in pediatric medicine have enabled a decrease in perinatal mortality, especially among infants born preterm (< 32 weeks gestational age) or low birth weight (< 1.500 g). However, this population is exposed to a greater risk of neurological sequelae. This is why the creation of specific follow-up program are mandatory to screen at-risk children to offer them a support able to minimize the impact of prematurity on their future neurological development.

Pathophysiology of sleep-dependent memory consolidation processes in children.

Cognitive impairments are often associated with abnormal sleep activity in developmental disorders and pathologies of childhood. Besides, accumulated evidence indicates that post-training sleep benefits to the consolidation of recently learned information in healthy adults and children. Although sleep-dependent consolidation effects in children are clearly established for declarative memories, they remain more debated in the procedural memory domain. Nowadays, recent experimental data suggest close interactions between the development of sleep-dependent plasticity markers, cortical maturation and cognition in children. In the present review, we propose that studying sleep and memory consolidation processes in developmental disorders and acquired childhood pathologies can provide novel, enlightening clues to understand the pathophysiological mechanisms subtending the disruption of long-term cerebral plasticity processes eventually leading to cognitive and learning deficits in children.

SBAR improves nurse-physician communication and reduces unexpected death: a pre and post intervention study.

BACKGROUND: The Joint Commission International Patient Safety Goal 2 states that effective communication between health care workers needs to improve. The aim of this study was to determine the effect of SBAR (situation, background, assessment, recommendation) on the incidence of serious adverse events (SAE's) in hospital wards. METHOD: In 16 hospital wards nurses were trained to use SBAR to communicate with physicians in cases of deteriorating patients. A pre (July 2010 and April 2011) and post (June 2011 and March 2012) intervention study was performed. Patient records were checked for SBAR items up to 48 h before a SAE. A questionnaire was used to measure nurse-physician communication and collaboration. RESULTS: During 37,239 admissions 207 SAE's occurred and were checked for SBAR items, 425 nurses were questioned. Post intervention all four SBAR elements were notated more frequently in patient records in case of a SAE (from 4% to 35%; p<0.001), total score on the questionnaire increased in nurses (from 58 (range 31-97) to 64 (range 25-97); p<0.001), the number of unplanned intensive care unit (ICU) admissions increased (from 13.1/1000 to 14.8/1000 admissions; relative risk ratio (RRR)=50%; 95% CI 30-64; p=0.001) and unexpected deaths decreased (from 0.99/1000 to 0.34/1000 admissions; RRR=-227%; 95% CI -793 to -20; NNT 1656; p<0.001). There was no difference in the number of cardiac arrest team calls. CONCLUSION: After introducing SBAR we found increased perception of effective communication and collaboration in nurses, an increase in unplanned ICU admissions and a decrease in unexpected deaths.

Work engagement supports nurse workforce stability and quality of care: nursing team-level analysis in psychiatric hospitals.

Research in healthcare settings reveals important links between work environment factors, burnout and organizational outcomes. Recently, research focuses on work engagement, the opposite (positive) pole from burnout. The current study investigated the relationship of nurse practice environment aspects and work engagement (vigour, dedication and absorption) to job outcomes and nurse-reported quality of care variables within teams using a multilevel design in psychiatric inpatient settings. Validated survey instruments were used in a cross-sectional design. Team-level analyses were performed with staff members (n = 357) from 32 clinical units in two psychiatric hospitals in Belgium. Favourable nurse practice environment aspects were associated with work engagement dimensions, and in turn work engagement was associated with job satisfaction, intention to stay in the profession and favourable nurse-reported quality of care variables. The strongest multivariate models suggested that dedication predicted positive job outcomes whereas nurse management predicted perceptions of quality of care. In addition, reports of quality of care by the interdisciplinary team were predicted by dedication, absorption, nurse-physician relations and nurse management. The study findings suggest that differences in vigour, dedication and absorption across teams associated with practice environment characteristics impact nurse job satisfaction, intention to stay and perceptions of quality of care.

Impact of a standardized nurse observation protocol including MEWS after Intensive Care Unit discharge.

BACKGROUND: Analysis of in-hospital mortality after serious adverse events (SAE's) in our hospital showed the need for more frequent observation in medical and surgical wards. We hypothesized that the incidence of SAE's could be decreased by introducing a standard nurse observation protocol. AIM: To investigate the effect of a standard nurse observation protocol implementing the Modified Early Warning Score (MEWS) and a color graphic observation chart. METHODS: Pre- and post-intervention study by analysis of patients records for a 5-day period after Intensive Care Unit (ICU) discharge to 14 medical and surgical wards before (n=530) and after (n=509) the intervention. RESULTS: For the total study population the mean Patient Observation Frequency Per Nursing Shift (POFPNS) during the 5-day period after ICU discharge increased from .9993 (95% C.I. .9637-1.0350) in the pre-intervention period to 1.0732 (95% C.I. 1.0362-1.1101) (p=.005) in the post-intervention period. There was an increased risk of a SAE in patients with MEWS 4 or higher in the present nursing shift (HR 8.25; 95% C.I. 2.88-23.62) and the previous nursing shift (HR 12.83;95% C.I. 4.45-36.99). There was an absolute risk reduction for SAE's within 120h after ICU discharge of 2.2% (95% C.I. -0.4-4.67%) from 5.7% to 3.5%. CONCLUSION: The intervention had a positive impact on the observation frequency. MEWS had a predictive value for SAE's in patients after ICU discharge. The drop in SAE's was substantial but did not reach statistical significance.

White matter abnormalities are related to microstructural changes in preterm neonates at term-equivalent age: a diffusion tensor imaging and probabilistic tractography study.

BACKGROUND AND PURPOSE: Preterm infants have a high risk of brain injury and neurodevelopmental impairment, often associated with WMA on conventional MR imaging. DTI can provide insight into white matter microstructure. The aim of this study was to investigate the association between WMA on conventional MR imaging and DTI parameters in specific fibers in preterm neonates at term-equivalent age. MATERIALS AND METHODS: Seventy preterm neonates (39 boys and 31 girls) were included in the study. WMA were classified as no, mild, moderate, or severe. Probabilistic tractography provided tract volumes, FA, MD, λ(//), and λ(⊥) in the CST, SLF, TRs, and corpus callosum. Data were compared by using MANOVA, and adjustment for multiple comparisons was performed. RESULTS: Important associations were found between WMA and microstructural changes. Compared with neonates with no WMA (n = 41), those with mild WMA (n = 27) had significantly increased λ(⊥) and MD in the left ATR, the left sensory STR, the bilateral motor STR, and for λ(⊥) also in the right CST; FA decreased significantly in the left sensory STR. Diminished tract volumes and altered diffusion indices were also observed in the 2 neonates with moderate WMA. CONCLUSIONS: Altered DTI indices in specific tracts, with λ(⊥) as most prominent, are associated with mild WMA in preterm neonates at term-equivalent age.

A novel NDUFV1 gene mutation in complex I deficiency in consanguineous siblings with brainstem lesions and Leigh syndrome.

Although deficiency of complex I of the mitochondrial respiratory chain is a frequent cause of encephalopathy in children, only a few mutations have been reported in each of its subunits. In the absence of families large enough for conclusive segregation analysis and of robust functional testing, it is difficult to unequivocally show the causality of the observed mutations and to delineate genotype-phenotype correlations, making additional observations necessary. We observed two consanguineous siblings with an early-onset encephalopathy, medulla, brainstem and mesencephalon lesions on brain magnetic resonance imaging and death before 8 months of age, caused by a complex I deficiency. We used a homozygosity mapping approach and identified a missense mutation in the NDUFV1 gene. The mutation, p.Arg386His, affects a highly conserved residue, contiguous to a cysteine residue known to coordinate an Fe ion. This observation adds to our understanding of complex I deficiency disease. It validates the important role of Arg386 and therefore supports the current molecular model of iron-sulfur clusters in NDUFV1.

Impact of focal interictal epileptiform discharges on behaviour and cognition in children.

It is hypothesised that focal interictal epileptiform discharges (IED) may exert a deleterious effect on behaviour and cognition in children. This hypothesis is supported by the abnormally high prevalence of IED in several developmental disorders, like specific language impairment, and of cognitive and behavioural deficits in epileptic children after excluding confounding factors such as underlying structural brain lesions, drug effects, or the occurrence of frequent or prolonged epileptic seizures. Neurophysiological and functional neuroimaging evidence suggests that IED may impact cognition through either transient effects on brain processing mechanisms, or through more long-lasting effects leading to prolonged inhibition of brain areas distant from but connected with the epileptic focus (i.e. remote inhibition effect). Sustained IED may also impair sleep-related learning consolidation processes. Nowadays, the benefits of anti-epileptic treatment aimed at reducing IED are not established except in specific situations like epileptic encephalopathies with continuous spike and waves during slow-wave sleep. Well-designed pharmacological studies are still necessary to address this issue.

Epileptic syndromes: differential treatment in infants, children, and adolescents.

This paper proposes therapeutic guidelines for the management of some epileptic syndromes in infants, children, and adolescents, based on available medical literature and clinical practice in the French Community of Belgium. The guidelines address both epileptic encephalopathies (West syndrome, Lennox-Gastaut syndrome, and Dravet syndrome) and idiopathic epilepsies (typical absence seizures, epilepsy with centro-temporal spikes and juvenile myoclonic epilepsy).

Gender differences in language and motor-related fibers in a population of healthy preterm neonates at term-equivalent age: a diffusion tensor and probabilistic tractography study.

BACKGROUND AND PURPOSE: Sex differences in white matter structure are controversial. In this MR imaging study, we aimed to investigate possible sex differences in language and motor-related tracts in healthy preterm neonates by using DTI and probabilistic tractography. MATERIALS AND METHODS: Thirty-eight preterm neonates (19 boys and 19 girls, age-matched), healthy at term-equivalent age and at 12 months were included. TBV was measured individually. Probabilistic tractography provided tract volumes, relative tract volumes (volume normalized to TBV), FA, MD, and λ(⊥) in the SLF, in the TRs, and in the CSTs. Data were compared by using independent t tests, and Bonferroni corrections were performed to adjust for multiple comparisons. RESULTS: We showed that healthy preterm boys had larger TBV than girls. However, girls had statistically significantly larger relative tract volumes than boys bilaterally in the parieto-temporal SLF, and in the left CST. Moreover, in the left parieto-temporal SLF, a trend toward lower MD and λ(⊥) was observed in females. CONCLUSIONS: Structural sex differences were found in preterm neonates at term-equivalent age in both sides of the parieto-temporal SLF and in the left CST. Further studies are necessary to investigate whether these structural differences are related to later sex differences in language skills and handedness or to the effect of prematurity.

[Attention deficit disorder in childhood]. / Le déficit attentionnel chez l'enfant.

Attention deficit disorder, eventually associated with hyperactivity (ADD +/- H), is nowadays viewed as a syndrome often of unknown etiology but probably not unique, with important genetic influence and associated environmental factors. The cognitive model proposing ADHD as a result of impaired inhibitory control which makes the child less flexible to changing circumstances suffers from poor sensibility and specificity. As studies aimed to make genotype-phenotype correlations were disappointing, recent genetic researches tend to correlate the genotype to an endophenotype defined from neuro-imaging data with encouraging preliminary results. Treatment with methylphenidate has long been considered as a first choice for disabling forms of ADHD, but recent data do not show evidence for superiority of methylphenidate compared to non pharmacological approach at long-term. Evaluation and treatment of each suspected case of ADHD need to be tailored with special concern for associated conditions as psycho-affective troubles or learning difficulties.