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Background: A large number of studies have shown time perception impairment and reaction time (RT) variability in children with attention deficit hyperactivity disorder (ADHD), and have discussed the causes of such difficulties. However, very few studies have investigated time knowledge (i.e., the correct representation and use of time units) in children with ADHD., Methods: We evaluated time knowledge in 33 children with ADHD, aged 8-12 years, who had consulted a reference center for learning disabilities in Paris, matched for age and gender with 33 typically developing (TD) children. We used a simple questionnaire-based survey and neuropsychological tests for cognitive and attentional skills., Results: The acquisition of time knowledge was delayed in children with ADHD compared with TD children (P<0.01). At the end of primary school, children with ADHD obtained time knowledge scores that were close to those of TD children at the beginning of primary school. In children with ADHD, time knowledge was significantly related to the working memory index (P<0.05), but not to ADHD presentation (with or without hyperactivity)., Conclusion: This study shows time knowledge impairment in children with ADHD, and paves the way for new screening tests and rehabilitation focused on time knowledge and time-related skills, in order to improve patient care and autonomy., (Copyright © 2020 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Pediatric MS is better understood after a series of epidemiological studies including the French cohort following almost 500 children since more than 7 years. Pediatric MS may have an onset as early as 2 years but symptoms are different than those at adolescence. Its evolution towards a motor handicap is slower than in adults but cognitive impairment have to be evaluated carefully. Interferon beta treatment can be use, if needed, even before the age of 10. Several environmental factors might increase its risk, especially early EBV infection or passive smoking. HBV vaccination does not increase significantly its risk of occurrence.

Lambert-Eaton myasthenic syndrome is a paraneoplasic syndrome which can reveal a primitive tumor. Frequently, the first diagnosis is myasthenia gravis. This disease is extremely rare in children. Only 10 cases have been reported in the last 35 years. We report 2 new observations occurring in very young patients, aged 2 and 3 years, with a ganglioneurobastoma as primitive tumor.

Aim: We studied the use of premixed nitrous oxide and oxygen in 80 patients with neurologic diseases., Patients and Methods: Mean ages ranged 10 +/- 5 yrs. Twenty-three patients (29%) were mentally retardated among which 17 of them presented with severe epilepsy. Painful procedures consisted of: lumbar punctures (80%), intravenous access (7), gastric endoscopy (6), skin biopsy (4), gastrostomy tube management (3). High-risk children were continuously monitored using ECG, non invasive blood pressure and transcutaneous oxygen saturation. We studied acceptation of the inhalation, vital signs, satisfaction of children, parents, medical and nursing staffs; side effects were compared with a group of healthy children undergoing venous access before induction of anesthesia., Results and Discussion: Acceptation increased with age. No significant changes in vital signs variables were observed. Satisfaction rate regarding the method was 88% for all children, parents, physicians and nurses. No serious undesirable event (as respiratory depression, seizure, inhalation of gastric content) occurred in these patients. The more frequent side-effects were: drowsiness during and after inhalation (35 and 9% respectively in the handicapped patients); nausea and vomiting (8%), headaches (3%), were more frequent than reported in literature but there were 25% of meningitis among our patients., Conclusion: Premixed nitrous oxide and oxygen was effective for reducing procedural pain and anxiety in children with neurological disorders, even in severely handicapped patients, with minor side-effects.

Unlabelled: The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol., Patients and Methods: All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination., Results: Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis., Conclusion: Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.

Background: Symptomatic forms of hyperphenylalaninemia are rare in France since neonatal screening began., Case Report: A child born in Algeria from consanguinous parents was referred at 2 years of age for a severe epileptic encephalopathy with hypotonia. Amino acid chromatography revealed hyperphenylalaninemia due to a dihydropteridine reductase deficiency. Dietary restriction of phenylalanine and oral administration of amine precursors, L-dopa and 5-hydroxytryptophan had poor efficiency on epilepsy and psychomotor delay., Conclusion: Diagnosis of hyperphenylalaninemia must be evoked in any children with progressive encephalopathy born in country where neonatal screening is not performed.