[Familial and non-familial benign infantile seizures: A homogeneous entity?]. / Convulsions infantiles bénignes familiales et non familiales : une entité homogène ?

Among the epileptic syndromes occurring during infancy, which are mostly non-idiopathic and associated with a poor prognosis, benign infantile convulsions are characterized by a favourable evolution. This work aims to analyse and compare the clinical, EEG and outcome characteristics of familial benign infantile convulsions (FBIC) and non-familial benign infantile convulsions (NFBIC). This is a retrospective study, conducted between 1988 and 2008, in 40 infants who presented benign infantile seizures during the two first years of life. All of them had no personal history, normal psychomotor development, normal neurological examinations, no abnormalities on biological and radiological investigations and a favourable outcome. In 14 cases, there was a familial history of familial benign infantile convulsions. However, among the 26 cases with non-familial benign infantile convulsions, 11 children had a familial history of other epileptic syndrome. That may suggest a genetic familial susceptibility. In the two groups, the clinical features and the electroencephalography were similar. The seizures had short duration and occurred most often in clusters. Twenty-nine children had secondarily generalized partial seizures and 11 infants had generalized seizures but a focal onset cannot be excluded. The antiepileptic drugs allowed rapid resolution of seizures. One child necessitated a prolonged antiepileptic treatment. In the other cases, seizures cured in the first year without recurrence of seizures after treatment discontinuation. The evolution was characterised in five children by a later occurrence of dystonia. This subgroup was described as infantile convulsion and choreoathetosis syndrome (ICCA). Benign infantile convulsions are probably an underestimated epileptic syndrome. The diagnosis is relatively easy in the familial forms with dominant autosomal transmission. In contrast, in sporadic forms, the diagnosis can be confirmed only by the evolution. The good prognosis must be tempered by the subsequent onset of dystonia consisted in the ICCA syndrome and justifies a prolonged follow-up.

Prospective follow-up of a cohort of preterm infants<33 WG receiving ketamine for tracheal intubation in the delivery room: Neurological outcome at 1 and 2 years.

OBJECTIVE: Although ketamine analgesia is effective in reducing pain and facilitating the tracheal intubation of newborns in the delivery room, no data on the neurological effects of this treatment are available. This study compared the neurodevelopmental outcomes at 2 years of age in a cohort of preterm newborns having received ketamine prior to tracheal intubation at birth (the ketamine group) and in a control group. METHODS: We included newborns delivered at less than 33 weeks gestational age (WGA) having undergone tracheal intubation at birth. The Ages and Stages Questionnaire (ASQ) was completed at 1 and 2 years of age. The development quotient (DQ) was calculated from the revised Brunet-Lezine score assessed at a corrected age of 2 years. RESULTS: There were no statistically significant differences between the ketamine group (n=54 at 1 year and n=51 at 2 years) and the control group (n=16 at 1 and 2 years) in terms of the mean±standard deviation DQ at the age of 2 (98±12 vs. 103±9, respectively; P=0.17) and the ASQ score at the age of 2 (221±44 vs. 230±39, respectively; P=0.55). DISCUSSION: This prospective cohort of 51 preterm newborns having received ketamine at birth did not reveal any differences in terms of neurological development at the age of 2 (relative to a control group and the literature data). These preliminary results must be confirmed in a randomized trial with longer follow-up.

[Experience with levetiracetam in the treatment of childhood refractory epilepsy]. / Utilisation du lévétiracétam (LEV) dans le traitement des épilepsies réfractaires de l'enfant : expérience du service de neuropédiatrie du CHU d'Amiens en France.

INTRODUCTION: In young children presenting drug-resistant epilepsy, the number of approved antiepileptic drugs is limited. Levetiracetam (LEV) is one of the most recent antiepileptic drugs (AED) introduced on the market and data on its effectiveness and tolerance in children are scarce. PATIENTS AND METHODS: The objective of this retrospective study was to report our experience with the use of levetiracetam as an adjuvant therapy in a population of 42 children presenting a drug-resistant epilepsy. The study was conducted over a 5-year-period (from 1 January 2004 to 30 June 2007). RESULTS: The patients' mean age was 10.8 years (range, 2.1-19 years). The mean duration of epilepsy was 6.6 years (range, 1.5-19 years). After the administration of LEV, 10 patients (23.8%) became seizure-free and 16 (38.1%) had more than 50% seizure reduction. A reduction of less than 50% was observed in 13 patients (31%). Three patients (7.1%) presented an increase in seizure frequency. The effectiveness of LEV was similar in partial and generalized epilepsy. LEV was well tolerated by these patients. The main adverse effects were anorexia, asthenia, and behavioral disorders, and drowsiness was encountered in 17% of the patients. All persistent adverse events were noted. In children under 4 years of age, LEV was particularly well tolerated. CONCLUSION: This study confirms the effectiveness and tolerance of LEV used as an adjuvant therapy in children presenting drug-resistant epilepsy, particularly in the very young ones.

[Infratentorial ischemic stroke in children: Three case reports]. / Accidents vasculaires cérébraux de la fosse postérieure chez l'enfant : à propos de trois cas.

Ischemic stroke is rare in children, most of which occur in the supratentorial brain, and infratentorial infarcts are very rare. Some clinical manifestations may be similar but others such as ataxia and cranial nerve palsy are more specific. Vertebral artery dissection is the most frequent cause of stroke in the vertebrobasilar territory, but the cause most often remains unknown in children. We report three cases of infratentorial stroke in children. The first observation concerns a 4-year-old boy brought to medical attention because left hemicorporal motor deficit associated with ataxia following a minor cranial traumatism. While computed tomography (CT) of the brain was normal, magnetic resonance imaging (MRI) revealed an area of signal alteration on the diffusion-weighted image within the right protuberance. The second observation is a 15-year-old girl who developed sudden-onset ataxia. The CT scan and MRI of the brain revealed an acute bilateral cerebellar stroke. MRI angiography showed an anatomical variant of the left vertebral artery that did not participate in the Willis polygon. In these two observations, no other abnormalities were detected except they were homozygotous for MTHFR mutation in the first observation and minor alpha-thalassemia for the second one. The outcome in these two children was good without sequelae after a 6-month follow-up. The third observation is a 6-year-old girl who suddenly exhibited cephalalgia, ataxia, and left visual impairment. The brain MRI revealed left occipital and cerebellar strokes due to vertebral artery dissection. The authors recommend the systematic search for vertebral artery dissection in cases of infratentorial stroke.

Maturation of response time and attentional control in ADHD: evidence from an attentional capture paradigm.

Inattention and hyperactivity/impulsivity are the core symptoms of Attention Deficit/Hyperactivity Disorder (ADHD). Slowness, although less known, has been also recently reported in children with ADHD and may contribute to their learning difficulties. Slow response time and greater response time variability have been highlighted by several computerized tasks. The goal of the present work was to evaluate the age-related response time in ADHD children and in a group of matched control children during an attentional capture paradigm. The study population included 75 children with ADHD (aged between 6 and 13) and 75 age- and gender-matched typical developing children (Control group). The children with ADHD made more errors than children on the control group. The response times and the response time variability decreased with age in both groups and were significantly greater in ADHD than in controls. The distractor effect was similar in both groups. The maturation of response times and response time variability with age is quite similar in children with ADHD and typical developing children but whatever the age-class, children with ADHD were slower and exhibited greater response time variability than control children that could explain the variation during day-time of attention capacities in ADHD.