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Objectives: To evaluate physicians' opinions concerning continuous deep sedation until death (CDSUD) and implementation of Claeys–Leonetti; a law intended to be applicable to all patients, but without a specific framework for children thus giving rise to ethically and legally complex situations. The secondary objective was to identify if physicians' characteristics could influence their opinions. Study Design: This was a national, multicenter, noninterventional cross-sectional survey from January 30, 2020, until March 1, 2020. The target population consisted of French physicians involved in children's end-of-life situations. The validated questionnaire explored respondents' characteristics and their opinions on four hypothetical pediatric clinical cases. Results: Analysis was conducted on 391 respondents. The oncological situation was more easily recognized as end of life compared with the neurological pathology (77% vs. 40.4%). Dependence on mechanical ventilation was another major factor influencing physicians in identifying end-of-life situations. Physicians clearly recognized the difference in intention between CDSUD and euthanasia. They accepted to implement CDSUD more easily in newborns. The withdrawal of artificial nutrition and hydration gave rise to divergent opinions. Respondents were in favor of adolescents' decision-making autonomy and their access to drafting advance directives. The child's best interest prevailed in case of objection by parents, except in situations outside the law's framework or in cases of disagreement within the health care team. Conclusion: Results of our study showed differences in the interpretation of the law concerning the CDSUD application framework and provide elements for reflection, which may ultimately contribute to the development of specific guidelines in CDSUD in children at the end of life. [ABSTRACT FROM AUTHOR]

Purpose: Several weeks after COVID-19 infection, some children report the persistence or recurrence of functional complaints. This clinical presentation has been referred as "long COVID" in the adult population, and an [18F]-FDG brain PET hypometabolic pattern has recently been suggested as a biomarker. Herein, we present a retrospective analysis of 7 paediatric patients with suspected long COVID who were explored by [18F]-FDG brain PET exam. Metabolic brain findings were confronted to those obtained in adult patients with long COVID, in comparison to their respective age-matched control groups. Methods: Review of clinical examination and whole-brain voxel-based analysis of [18F]-FDG PET metabolism of the 7 children in comparison to 21 paediatric controls, 35 adult patients with long COVID and 44 healthy adult subjects. Results: Despite lower initial severity at the acute stage of the infection, paediatric patients demonstrated on average 5 months later a similar brain hypometabolic pattern as that found in adult long COVID patients, involving bilateral medial temporal lobes, brainstem and cerebellum (p-voxel < 0.001, p-cluster < 0.05 FWE-corrected), and also the right olfactory gyrus after small volume correction (p-voxel = 0.010 FWE-corrected), with partial PET recovery in two children at follow-up. Conclusion: These results provide arguments in favour of possible long COVID in children, with a similar functional brain involvement to those found in adults, regardless of age and initial severity. [ABSTRACT FROM AUTHOR]

Introduction/objectives We report the case of a child prospectively followed in our institution for a severe, neonatal onset epilepsy presenting with severe attacks of apnea that were not initially recognized as seizure since they were not associated with any abnormal movement and since interictal EEG was normal. Recording of attacks using prolonged video-EEG recording allowed to confirm the diagnosis of epileptic seizures. Results Using whole exome sequencing we found a de novo heterozygous, missense mutation of FHF1 (p.Arg52His, NM_004113), a mutation that has been very recently described in 7 patients with an early onset epileptic encephalopathy. The initial workup showed a partial deficit of the complex II of the respiratory chain in muscle and liver. The prospective follow-up demonstrated that 2 drugs seemed to be more effective than the others: sodium blocker carbamazepine, and serotonin reuptake blocker fluoxetine. GABAergic drugs seemed to be ineffective. No drug aggravated the epilepsy. Discussion This case report contributes to the description of an emerging phenotype for this condition. [ABSTRACT FROM AUTHOR]

Abstract: Palliative care are intended to children with life limiting conditions. In France, for the majority of pediatric team, the idea of continuity of cares is essential for the announcement of the diagnosis to the final state. The author describes here different situations according to the initial disease of these children. In all cases, the choices of artificial feeding depend of pluridisciplinary medical teams and their experience, and of family opinion. Ethical consideration must be present in this decision. [Copyright &y& Elsevier]

Background Anti-N-methyl- d -aspartate receptor (NMDAR) encephalitis is a frequent and severe cause of encephalitis in children with potential efficient treatment (immunotherapy). Suggestive clinical features are behavioural troubles, seizures and movement disorders. Prompt diagnosis and treatment initiation are needed to guarantee favourable outcome. Nevertheless, diagnosis may be challenging because of the classical ancillary test (magnetic resonance imaging (MRI), electroencephalogram, standard cerebro-spinal fluid analysis) have limited sensitivity. Currently, immunological analyses are needed for the diagnostic confirmation. In adult patients, some studies suggested a potential role of cerebral 18 FluoroDeoxy-Glucose Positron Emission Tomography (FDG-PET) in the evaluation of anti-NMDAR encephalitis. Nevertheless, almost no data exist in paediatric population. Method We report retrospectively clinical, ancillary tests and cerebral FDG-PET data in 6 young patients (median age = 10.5 years, 4 girls) with immunologically confirmed anti-NMDAR encephalitis. Results Our patients presented classical clinical features of anti-NMDAR encephalitis with severe course (notably four patients had normal MRI). Our series shows the feasibility and the good sensitivity of cerebral FDG-PET (6/6 patients with brain metabolism alteration) in paediatric population. We report some particular features in this population: extensive, symmetric cortical hypometabolism especially in posterior areas; asymmetric anterior focus of hypermetabolism; and basal ganglia hypermetabolism. We found also a good correlation between the clinical severity and the cerebral metabolism changes. Moreover, serial cerebral FDG-PET showed parallel brain metabolism and clinical improvement. Conclusion Our study reveals the existence of specific patterns of brain metabolism alteration in anti-NMDAR encephalitis in paediatric population. [ABSTRACT FROM AUTHOR]

• Young children with USCP and equinovalgus gait present reduced plantar flexor extensibility. • With age, plantar flexor extensibility deteriorates while selective motor control improves. • Moderate correlation exists between extensibility and stretch response of the plantar flexors. • TA and PL/GM markedly co-contract during the entire swing phase of gait • Inappropriate overactivity was greater in PL than GM during swing phase. Children with spastic cerebral palsy gradually lose muscle extensibility but the interplay between the muscular and neurological components of the condition is unclear especially in the pathophysiology of equinovalgus gait. This study aimed to quantify the muscular and neurological disorders in young children with unilateral cerebral palsy, and to investigate the role of the peroneus longus (PL) in equinovalgus gait. Design, setting and population: This was an observational study with prospective assessments of 31 children (median age: 2.9 years, range: 2–6) from outpatient clinic in a tertiary teaching hospital. Clinical measures of plantar flexor extensibility (X V1), stretch response (X V3), and active ankle dorsiflexion angle (X A) were obtained as well as walking velocity and electromyography of tibialis anterior (TA), gastrocnemius medialis (GM) and PL during walking. We found reduced extensibility of the triceps surae on the paretic side (effect size r = 0.73, p < 0.001 for soleus and r = 0.68, p < 0.001 for gastrocnemius) and a correlation between reduced triceps surae extensibility and earlier stretch response (ρ = 0.5, p = 0.004). During the swing phase, there was major co-contraction between TA and GM/PL, and significantly larger activation of PL compared to GM (r = 0.46, p = 0.011). Both GM and PL activation decreased with age. Our results suggest gradual deterioration of the muscular disorder and a link between the muscular and neurological disorders, although plantar flexor co-contraction improved with age. The PL was more activated than the GM and may be considered an intervention target to treat equinovalgus gait. [ABSTRACT FROM AUTHOR]

Abstract: We investigated the consequences of a progressive damage to the muscular system on the organization of anticipatory postural adjustments (APA) in children with Duchenne muscular dystrophy (DMD). We used a bimanual load-lifting task requiring the stabilization of the forearm position despite its voluntary or imposed unloading. Eight children with DMD from 4 to 11 years of age were compared to eight typically developing (TD) children. Elbow angle and multiple surface EMGs were recorded and assessed the use of APA. The muscle weakness did not impair (1) the proprioceptive afference and the motor efference constituting the unloading reflex; and (2) the use of an anticipatory function in children with DMD. However, APA used for the forearm stabilization were less efficient in the group of children with DMD. We conclude that in DMD the muscular weakness could be a restraint to the efficiency of APA with respect to TD children. [Copyright &y& Elsevier]