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Competing Interests: L. Le Goff déclare être investigatrice dans des essais cliniques menés par Roche et par Généthon et avoir reçu un financement de Biogen pour mener à bien une étude académique. Je déclare avoir été pris en charge (transport, hôtel, repas), à l’occasion de déplacement pour congrès ou formations, par Biogen, Allergan et Ipsen.É. Mamimoue, A. Pelluet, V. Des Portes et P. Fourneret déclarent n'avoir aucun lien d'intérêts.

Competing Interests: L. Le Goff déclare être investigatrice dans des essais cliniques menés par Roche et par Généthon et avoir reçu un financement de Biogen pour mener à bien une étude académique. Je déclare avoir été pris en charge (transport, hôtel, repas), à l’occasion de déplacement pour congrès ou formations, par Biogen, Allergan et Ipsen.É. Mamimoue, A. Pelluet, V. Des Portes et P. Fourneret déclarent n'avoir aucun lien d'intérêts.

Aim: The aim was to describe and to analyze the ethics of decision-making in situations involving children with intellectual disability and cancer, from the referent-doctor's point-of-view, in pediatric oncology units in France., Methods: Pediatricians working in pediatric oncology units were interviewed through an online questionnaire and a semi-directive interview was systematically proposed. We analyzed the ethical issues that arose during the process of decision-making and we made suggestions in order to address them., Results: Sixteen doctors reported twenty-one clinical cases. Of these cases, one third of the children had a change in their oncologic treatment, with a risk of pejorative outcome on the prognosis. Despite the fact that ethical issues appeared in 80 % of the cases, there were few consultations with ethical committees. Decision-making process showed no difference compared to children without intellectual disability, thus raising ethical issues in the medical team. Our study showed discrepancy between frequently reported ethical issues, high consensus rate regarding treatment decision and lack of consultation with ethical committees., Discussion: We propose three steps to guide the decision-making process in situations involving children with intellectual disability and cancer: 1/deeper understanding of the child through reinforced interactions with their caregivers, 2/better cross-boundary discussions, to improve the effectiveness of the multidisciplinary staff, and 3/more systematic ethical committees consultation., (Copyright © 2021 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Pediatric epilepsies. Epilepsies are a heterogeneous group of diseases characterized by the recurrence of epileptic seizures. The incidence is higher in children than in adults. Epilepsies are much more than the simple recurrence of seizures. Co-morbidities are common as well as daily-life activity restrictions. In children, special attention must be paid to schooling and behavioral difficulties in order to set up early intervention. Epilepsies with focal- onset seizures are the most common in children. Few syndromes account for the majority of pediatric epilepsies: epilepsy with centro-temporal spikes, childhood absence epilepsy, juvenile myoclonic epilepsy and juvenile absence epilepsy. An appropriate syndromic diagnosis allows to conduct appropriate investigations, propose adequate treatment and explain the prognosis., Competing Interests: Auvin déclare des liens ponctuels (interventions et/ou prise en charge lors de congrès) avec Arvelle Therapeutics, Biomarin, Eisai, Nutricia, UCB, Vitaflo et Zogenix. B. Dozières- Puyravel déclare des liens ponctuels (interventions et/ou prise en charge lors de congrès) avec Biomarin, Eisai, UCB et Zogenix.

Disabled child, care and ethical aspects. The child's doctor occupies a privileged place in the life of a child with a disability. At all times, he must be an adviser, favouring a global approach and support to ensure optimal autonomy. Discovery of a neurodevelopmental disorder justifies a systematic search for the various causes known to date. Identification and knowledge of a precise diagnosis is an essential element in constructing the life plan for a disabled child. The announcement of the diagnosis is an integral part of the care system, it only makes sense when combined with tailor-made and step by step support. This requires that the doctor has a good knowledge of the main childcare structures, guidance agencies, and available financial aid. Multidisciplinary consultations enable a global approach to support a child with a disability. More children with disabilities become adults as medical care progresses. Transition from "children" to "adult" consultations represents a major challenge. However, some cases could be life-threatening. The decision on whether to continue the various therapies have to be considered and discussed with the child and his family, with reference to the notion of "unreasonable obstinacy". Drafting an individual certificate of "remarkable patient" will best help the implementation of end-of-life support measures., Competing Interests: B. Chabrol déclare n’avoir aucun lien d’intérêts.

The objective of this study was to investigate the adaptive functioning of adults who had a slight to moderate intellectual deficiency, in regard of age and intellectual quotient (IQ). Cognitive and adaptive functioning were evaluated using the WAIS and VINELAND scales in 16 adults who accepted to participate in this study. We found a correlation between global IQ and each domain of the adaptive score, mostly communication skills. We also found that there was an age effect on socialization skills. Most skills were learned during infancy and adolescence, especially communication skills, which are highly stable at different ages and highly correlated with IQ. However, some abilities are still acquired in adulthood, mostly socialization skills, especially in persons with the lowest IQ. These data are of particular interest for people caring for disabled adults., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Febrile seizures (FS) are the most common seizures seen in the paediatric population in the out-of-hospital and emergency department settings, and they account for the majority of seizures seen in children younger than 5 years old. An FS is a seizure accompanied by fever, without central nervous system infection, occurring in children between 6 months and 5 years old. Five criteria have been used and taught to classify any FS as simple or complex FS. These factors do not bear the same significance for clinical practice, in particular, the decision to perform a lumbar puncture for cerebrospinal fluid analysis to rule out an intracranial infection. Moreover, epidemiological studies have illustrated that some factors are predictive of febrile seizure recurrence while others are predictive of epilepsy occurrence. On this basis, a workshop was organized to provide an answer to three clinical practice questions: when should a lumbar puncture be performed in a child who has experienced a seizure during a fever episode, is the prescription of a rescue drug required with a risk of a prolonged febrile seizure recurrence, when should a neurological consultation be requested (risk of later epilepsy)? Based on a review of the literature and on a 1-day workshop, we report here the conclusion of the working group. A lumbar puncture is required in any child with meningitis symptoms or septic signs or behaviour disturbance. A lumbar puncture should be discussed based on the clinical symptoms and their progression over time when a child has experienced a focal FS or repetitive FSs without signs of meningitis or sepsis or behaviour disturbance. The lumbar puncture is not necessary in case of simple FS without signs of meningitis, including in infants between 6 and 12 months old. An early clinical evaluation (at least 4 h after the first clinical assessment) could be helpful, in particular in infants younger than 12 months of age. A rescue drug might be prescribed when there is a high risk of prolonged FS (i.e., risk higher than 20%): age at FS<12months OR a history of a previous febrile status epilepticus OR if the first FS was a focal seizure OR abnormal development/neurological exam/MRI OR a family history of nonfebrile seizure. A neurological consultation should be requested for any child who has experienced a prolonged FS before the age of 1 year, for children who have experienced prolonged and focal FS or repetitive (within 24h) focal FS, for children who have experienced multiple complex (focal or prolonged or repetitive) FS, for children with an abnormal neurological exam or abnormal development experiencing a FS. Although childhood febrile seizures in most cases are benign, witnessing such seizures is always a terrifying experience for the child's parents. Most parents feel that their child is dying or could have severe brain injury related to the episode. Therefore, the group also suggests a post-FS visit with the primary care physician., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

The prenatal finding of a head circumference (HC) below the 3rd percentile (p) remains, in the same way as short femur or increased nuchal translucency with normal karyotype, one the most difficult situations for the praticionner in the setting of prenatal diagnosis. Microcephaly is a gateway to possible cerebral pathologies, but the main objective is to identify serious prenatal situations. A standardized HC measurement, the use of adapted reference tools and charts, longitudinal following of cephalic biometrics in high-risk situations, and systematic central nervous system analysis can increase the diagnostic performance of ultrasound which is often disappointing for microcephaly. The early distinction between associated or isolated microcephaly makes it possible to quickly orient the prenatal management and counseling. Fetal MRI and genetic counseling are fundamental in this context, making it possible to specify at best the etiological diagnosis and to provide assistance to the neuropediatrician in the establishment of an often uncertain prognosis. The recent increase in cases of microcephaly concomitant with the epidemic of the ZIKA virus is an additional argument to improve our practices and the daily apprehension of HC<3rd p., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant neuropathy. It is characterized by recurrent sensory and motor nerve palsies, usually precipitated by minor trauma or compression. Even though rare in childhood, this disorder is probably underdiagnosed given its wide spectrum of clinical symptoms. We review three separate cases of HNPP diagnosed in children with various phenotypes: fluctuating and distal paresthesias disrupting learning at school, cramps related to intensive piano practice, and discrete muscle weakness with no functional complaint. Family history should be carefully reviewed to identify potential undiagnosed HNPP cases, as in our three reports. Electrophysiological study is essential for the diagnosis, with a double advantage: to confirm the presence of focal abnormalities in clinically symptomatic areas and to guide molecular biology by revealing an underlying demyelinating polyneuropathy. The diagnosis of HNPP is confirmed by genetic testing, which in 90% of cases shows a 1.5-Mb deletion of chromosome 17p11.2 including the PMP22 gene. Patients are expected to make a full recovery after each relapse. However, it is very important for both the patient and his or her family to establish a diagnosis in order to prevent recurrent palsy brought on by situations involving prolonged immobilizations leading to nerve compression., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Childhood absence epilepsy (CAE) is a common pediatric epilepsy syndrome accounting for 10% of all pediatric epilepsies. The aim of this review is to provide an updated overview of this epilepsy syndrome to pediatricians. Most of the patients can be initially managed in private practice or in general pediatric settings. Absence seizures are the only seizure type observed at the time of diagnosis in these patients. An electroencephalogram recording and a clinical evaluation lead to the diagnosis. The underlying mechanisms are not yet fully understood. CAE is considered a self-limited epilepsy syndrome since most of the patients will become seizure free. Only a few patients (5-10%) have resistant CAE. However, CAE, as well as any pediatric epilepsy syndrome, should not be considered a "benign" epilepsy. Attention deficit is observed in about one third of the patients. There is also an increased risk of academic difficulties related to specific cognitive disorders. Therefore, the early detection of children at risk of developing neuropsychological problems can be helpful for preventing school underachievement and poor psychosocial outcome. Recently, several studies including a large randomized controlled trial indicated that ethosuximide should be considered as a first-line treatment and valproate as a second-line treatment. Cognitive side effects should be an important factor in the selection of the antiepileptic drug and should be specifically assessed during the follow-up. This review concludes by discussing the criteria that might lead to referring the patient to a specialist., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy. Acute chest syndrome (ACS) is the foremost reason for admission to the intensive care unit and the leading cause of premature death. Guidelines on its management have recently been published. Asthma appears to be a different comorbidity and may increase the risk of vaso-occlusive crisis, ACS, and early death. Its management is not specific in SCD, but systemic steroids must be used carefully. Pulmonary hypertension (PH) is a major risk factor of death in adult patients. In children, no association between PH and death has been shown. Elevated tricuspid regurgitant velocity was associated with lower performance on the 6-min walk test (6MWT) but its long-term consequences are still unknown. These differences could be due to different pathophysiology mechanisms. Systematic screening is recommended in children. Regarding lung functions, although obstructive syndrome appears to be rare, restrictive pattern prevalence increases with age in SCD patients. Adaptation to physical exercise is altered in SCD children: they have a lower walking distance at the 6MWT than controls and can experience desaturation during effort, but muscular blood flow regulation maintains normal muscular strength. Sleeping disorders are frequent in SCD children, notably Obstructive sleep apnea syndrome (OSAS). Because of the neurological burden of nocturnal hypoxia, OSAS care is primordial and mainly based on adenotonsillectomy, which has been shown to reduce ischemic events. The high morbidity and mortality related to pulmonary impairments in SCD require a careful pulmonary assessment and follow-up. Mainly based on clinical examination, follow-up aims to the diagnosis of SCD-related respiratory complications early in these children., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Although the clinical picture and the natural progression of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) differ, borderline forms exist. Classical orthopaedic treatment is based on self-rehabilitation (by the parents and the patient), physical therapy, posture alignment with orthotics, ergotherapy to set up technical aides, notably positioning in an electric wheelchair to provide more satisfactory autonomy. The functional aspect predominates over pure orthopaedics. Although surgical indications have evolved for the lower limbs, pelvic-spinal arthrodesis for treatment of scoliosis remains the reference treatment, but the methods have evolved since the advent of corticotherapy for DMD. Corticotherapy slows the progression of motor deficits, the age at which walking ability is lost is delayed (shifting from 10 years to 13-14 years depending on the studies), scoliosis progresses later, respiratory insufficiency is better controlled, and therefore survival is prolonged (between 20 and 40 years). However, although this functional aspect seems to respond better to the progress in overall treatment, it also results from a multidisciplinary approach to the disease. Nevertheless, assessment is required, not at a time t as reflected by the scales currently in use, but during daily activities as proposed by qualitative and quantitative monitoring seeking to model nyctohemeral functional motor skills. The principle is to characterize the type of activity (sitting, standing, lying down, walking), its duration, its intensity (walking speed), its frequency (number of activity changes, number of walking episodes), and their sequence (temporal sequence, organization of activity variation). The goal is to identify the variety of functional motor skills and their occurrence over time to determine whether treatment contributes a functional benefit and whether this benefit is put into practice daily., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

We report on the case of a child who presented with recurrent, multiple, and voluminous bladder diverticula. Bladder diverticula are defined as a herniation of the mucosa through the bladder muscle or the detrusor. Causes are numerous and diverticula can be classified into primary congenital diverticula (para-ureteral - or Hutch diverticula - and posterolateral diverticula); secondary diverticula (resulting from chronic mechanical obstruction or from neurological disease; and diverticula secondary to connective tissue or muscle fragility. The latter is seen in disease entities such as prune belly syndrome, Ehlers-Danlos syndrome, cutis laxa syndrome, OHS (occipital horn syndrome), Menkes disease, and Williams-Beuren syndrome. In this patient, the cause of these diverticula was OHS, a genetic, recessive X-chromosome-linked syndrome, responsible for abnormal tissue caused by a disorder in copper metabolism. This case reminds us of the importance of pushing the diagnostic workup when presented with multiple and/or large bladder diverticula, and in particular to search for rare malformation syndromes after exclusion of an obstacle., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Aim: The purpose of this study was to determine the institutional trajectory and future of young children in child welfare., Materials and Methods: A catamnestic study - based on data from the child welfare office in Maine and Loire, France, from 1994 to 2001 - was conducted by a child psychiatrist and a psychologist. Medical, judicial, and educational data (development, health, pathways in child protection services) were collected and analyzed regarding the status of these children 15 years later, adding information gathered by interviewing the child welfare and foster family consultant., Results: We included 128 children admitted to the child welfare office before 4 years of age. Admission to the child welfare system suffers from care delays (a mean of 13.1 months between the first child protection referral and placement) with an average entry age of 17 months and frequent cases of child abuse (e.g., seven Silverman syndrome cases). The physical and mental health status of these children was poor (poorly monitored pregnancies, prematurity, low birth weight). More than one third of the children had growth failure at admission, with catch-up in half of the cases. The average length of stay in the child welfare system was 13.2±4.6 years. At the end of the follow-up, there were specific measures to safeguard vulnerable adults: "young adult" (24 cases), "major protection" (eight cases) and "disabled living allowance" (nine cases). One hundred and sixteen children suffered from psychiatric disorders at entry and 98 at the end. The general functioning of children as assessed by the Children's Global Assessment Scale (CGAS) showed a statistically significant improvement. One out of two young adults showed problems integrating socially with chaotic pathways: many foster placements, unsuccessful return to the family, and academic failures., Conclusion: The clinical situations of children in the child welfare office and their long-term progression confirm the importance of this public health problem. Although the measures can greatly improve their physical and psychological recovery, with evidence of thriving, this remains limited: only a few of these children are well integrated socially and academically., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Vitamin D deficiency is common in the general population and even more frequent in patients with chronic diseases. The prevention of rickets with native vitamin D supplementation is one of the oldest and most effective prophylactic measures ever reported in medicine, leading to an almost complete eradication of vitamin D-deficient rickets in developed countries. We report on two children with vitamin D abnormalities: the first, 10-year-old child developed rickets without any vitamin D supplementation despite different risk factors (autism, ethnicity, nutritional problems, chronic antiepileptic therapies). In contrast, the second, 8-month-old child received double doses of native vitamin D from birth for several months and was referred for acute and symptomatic hypercalcemia. As such, vitamin D supplementation must follow specific rules: neither too much nor too little! We also discuss the emergence of "new" genetic diseases such as mutations in the 24-hydroxylase (CYP24A1) gene inducing neonatal hypercalcemia and nephrocalcinosis: we believe that before prescribing conventional vitamin D supplementation as recommended by the national guidelines, pediatricians should quickly rule out a potential genetic abnormality in phosphate/calcium metabolism (namely a history of lithiasis or hypercalcemia) that would lead to further biological investigations., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Background: There is a paucity of epidemiologic studies of prolonged seizures (persisting for more than 5 minutes) in the Democratic Republic of Congo (DRC) and in Rwanda., Objective: We sought to analyze the clinical presentation, causes, pharmacologic management, and shortterm course of these seizures., Methods: We enrolled 436 children, aged five months to ten years, who presented with prolonged seizures at the pediatric emergency departments of nine hospitals. Findings: Overall, 57.8% of the children were younger than three years; 7% had pre-existing psychomotor delay. Although 21% had had previous seizures, only 13% were receiving antiepileptic therapy. On presentation, 63.5% of the patients had fever and 26% were in status epilepticus. The seizures were focal in 21% of the cases. Malaria was the most common cause, involving 63% of the cases. The recurrence rate was 38%, and the mortality rate 4%., Conclusion: Prolonged seizures in DRC and Rwanda are frequently associated with fever, most commonly caused by malaria. The immediate use of long-acting antiepileptic drug could improve their outcomes.

Mycoplasma pneumoniae infection is common in children. Extrapulmonary symptoms usually reveal as neurological symptoms, mainly as encephalitis with significant morbidity and mortality. Various other neurological presentations have also been reported. We describe a cohort of nine children with neurological manifestations due to M. pneumoniae infection, including five cases of encephalitis, one of polyradiculoneuritis, one of ophthalmoplegia, one of optic neuritis, and one of myositis. Progression was variable from ad integrum recovery to severe brain damage. Diagnosis is usually confirmed by PCR and/or serological follow-up, but the latter is still insufficiently used in practice to systematically affirm the diagnosis. Therapeutic management is not clearly defined and long-term progression can be uncertain despite early antibiotic and/or anti-inflammatory treatments., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Epilepsy surgery raises hopes, but still remains reserved for a small number of cases of epilepsy resistant to medical treatments. It requires the involvement of multidisciplinary medical and allied health teams with expertise in this field. From the patient's admission through to their discharge, the nurse and the electroencephalogram technician have an essential role to play., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

An epileptic seizure in a child is a major source of anxiety and turns the family's everyday life upside down. Through therapeutic education, the nurse guides the families towards the autonomous management of the seizures, antiepileptic treatments, adaptations to daily life and potential comorbidities., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Antiepileptics are a group of drugs with various pharmacological properties and mechanisms of action. They are grouped together due to the fact that they are used to treat epilepsy. There are around twenty molecules in this group. Particular care needs to be taken when prescribing them for children as they carry risks., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Scurvy is the clinical manifestation of a deficiency in vitamin C, which is present in fresh fruits and vegetables. It is historically linked to the era of great maritime expeditions. Manifestations are misleading in children, in contrast with adults: bone disease and hemorrhagic syndrome are the earliest and most frequent manifestations due to a collagen biosynthesis defect. Scurvy is an old, potentially fatal disease but is easily curable with ascorbic acid. It can be prevented with vitamin C treatment in pediatric populations with unusual eating habits. We describe two cases of pediatric scurvy in two 7-year-old boys who had dietary restrictions stemming from developmental disorders., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Moya-Moya disease is a rare arterial occlusive disease affecting the internal carotid artery and its branches. It is found in both pediatric and adult populations, and it may lead to severe clinical presentations such as stroke and intracranial hemorrhage. Several surgical procedures have been developed to improve its clinical outcome. Imaging techniques have a key role in management of Moya-Moya disease, as they are necessary for diagnosis, choice of treatment and follow-up. Although catheter angiography remains the diagnostic gold standard, and nuclear-medicine techniques best perform hemodynamic studies, less invasive imaging techniques have become efficient in serving these purposes. Conventional MRI and MR angiography, as well as MR functional and metabolic studies, are now widely used in each stage of disease management, from diagnosis to follow-up. CT scan and Doppler sonography may also help assess severity of disease and effects of treatment. The aim of this review is to clarify the utility, efficiency and latest developments of each imaging modality in management of Moya-Moya disease., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Monitoring of blood glucose is usually reported to reduce the risk of hypoglycemia in term newborns with high risk factors and for prematurity in neonatal intensive care unit patients. Differential diagnosis has rarely been discussed. In the eutrophic term newborn, hypoglycemia remains rare and an etiological diagnosis must be made. Intensive management of neonatal hypoglycemia is required to prevent neurodevelopmental defects. Without evident cause or if hypoglycemia persists, a systematic review of possible causes should be made. We report isolated glucocorticoid deficiency diagnosed in an infant at 10 months of age. This boy had neonatal hypoglycemia and mild jaundice that had not been investigated. During his first 9 months of life, he presented frequent infections. At 10 months of age, febrile seizures occurred associated with shock, hypoglycemia, hyponatremia, mild hyperpigmentation, and coma. He was diagnosed with hypocortisolemia and elevated ACTH levels. Brain injury was revealed by MRI after resuscitation, with hypoxic-ischemic and hypoglycemic encephalopathy. The molecular studies demonstrated the presence of p.Asp107Asn and previously unreported frameshift p.Pro281GlnfsX9 MC2R gene mutations. A substitutive hormone therapy was provided and during a follow-up of 12 months no adrenal crisis was noted. We report an unusual case of familial glucocorticoid deficiency with severe neurological injury. This case demonstrates the importance of an appropriate etiological diagnosis in neonatal hypoglycemia., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Context and Objectives: Guidelines recommend that any epileptic seizure that lasts more than 5 min (prolonged seizures) be treated immediately with benzodiazepines in order to prevent progression to status epilepticus. However, there is very little published information on how such seizures are managed outside of the hospital setting, for example in schools or at home. This paper presents the initial results, specific to France, of the Practices in Emergency and Rescue medication For Epilepsy managed with Community-administered Therapy (PERFECT) Initiative, which was set up in 2011 to gain a better understanding of how prolonged convulsive seizures are managed and rescue medication is administered in out-of-hospital settings across Europe., Methods: The PERFECT Initiative was conducted in six countries (France, Germany, Italy, Spain, Sweden, the United Kingdom) and consists of three phases. This paper reports on the first two phases of this study and focuses on findings for France. Phase I of the PERFECT initiative consisted of a review of existing clinical guidelines, guidance to schools, and relevant policy and legal frameworks in France, from both published and web-based sources. Phase II consisted in a telephone survey of 20 pediatricians, pediatric neurologists, and neurologists who treat children with prolonged convulsive seizures in France in order to obtain their views on how prolonged seizures are managed outside of hospitals. Phase III is ongoing and consists of a survey of children with prolonged seizures and their caregivers., Results: Existing clinical guidelines pertain mainly to the hospital setting and contain very little informations on how prolonged seizures should be managed outside the hospital. Guidance to schools is unclear as to whether teachers are allowed to administer rescue medication to children and legal frameworks are full of contradictions. As a result, whether or not children who experience prolonged seizures receive their rescue medication during school hours depends mostly on the resources and training available in each school. Healthcare professionals stated that lack of information on prolonged seizures and their management, as well as a lack of up-to-date guidance specific to schools were considerable obstacles to a systematic approach to managing prolonged seizures outside of the hospital., Discussion: In France and elsewhere, there has been growing recognition of substantial gaps in the provision of care to children with epilepsy in recent years. However, the issues that arise for children who require rescue medication for prolonged seizures outside of hospitals are conspicuously absent from policy documents or clinical guidelines. This lack of information, combined with insufficient training in schools on epilepsy, and inadequate guidelines create a situation in which schools are likely to call an ambulance every time a child has a prolonged seizure, therefore risking delays in treatment, and putting the child at risk of status epilepticus and clinical sequelae, not to mention stigmatization at school., Conclusion: There is a need for more explicit guidance covering educational and healthcare settings, clearer information to parents and schools, and more systematic training to be made available to caregivers in order to ensure that all children at risk of prolonged seizures receive their prescribed rescue medication as quickly as possible, regardless of where their seizure occurs., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objectives: Neurological causes are common diagnoses for apparent life-threatening events in infants. The objective of this study was to evaluate the relevancy of electroencephalography performed after an apparent life-threatening event., Material and Methods: A retrospective study was conducted in a children's hospital over a 1-year period. The charts of infants under 2 years of age who were admitted following an apparent life-threatening event were reviewed. Clinical and biological data were collected and electroencephalograms - divided into normal and abnormal - were reviewed. To evaluate the follow-up state of the patients, parents were invited to complete an evaluation form an average 13 months after the event. The yield for electroencephalography was established according to the ratio of positive results contributing to the diagnosis of the cause of the apparent life-threatening event., Results: A total of 47 patients met the inclusion criteria. Fifteen had had an EEG, 32 had not. The rate of abnormal neurological signs described by parents during the apparent life-threatening event was higher in the EEG group compared to the group without EEG (53% vs. 22%, P=0.05). In the follow-up, 35% of the children presented a second event, which was described as being similar or less impressive and occurred in the 1st month after the event (91%). Of the eight abnormal electroencephalograms, six had no specific abnormalities and two contributed to the diagnosis of epileptic seizure. Therefore, the diagnostic yield of electroencephalography in this study was 13% (2/8)., Conclusions: The yield of electroencephalography performed after an apparent life-threatening event is low. Neurological history and repeated physical examinations still remain the major diagnostic tools before resorting to electroencephalography., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

The management of mucopolysaccharidoses (MPS) requires a multidisciplinary approach and all medical and paramedical specialties may be involved because of the multiorgan nature of the disease. For this reason, patients should ideally be managed in a reference center and their management should be coordinated by an experienced physician who has access to the various specialists through a multi-disciplinary approach. The management is focused on assessments of the numerous features of the disease, study their progression and the effectiveness and pitfalls of specific and non-specific treatments. The main concerns of reeducation and rehabilitation should be to preserve global function through specific goals to be set along with the child and its family. This is a very demanding approach for the patient and family in which education plays a fundamental role in order to ensure optimal management., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)