[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.

Quantitative autoradiographic study of somatostatin and neurotensin binding sites in medulla oblongata of SIDS.

Quantitative autoradiography analysis of neurotensin (NT) and somatostatin (SS) binding sites was performed on coronal sections of the medulla oblongata from 2 fetuses, 6 controls and 7 victims of Sudden Infant Death Syndrome (SIDS). Throughout the first postnatal year, mean SS binding site density was similar in controls and SIDS in all structures of the medulla oblongata. The density of neurotensin binding sites was significantly higher in the nucleus of tractus solitarius (NTS) of SIDS than in controls, but there was no significant differences in the other areas of the medulla oblongata. Our findings suggest an immature developmental pattern of increased NT binding sites the NTS of SIDS. This alteration may be related to an abnormal central cardiorespiratory and arousal control which is thought to be present in SIDS.

A familial syndrome with micrognathia, cleft palate, short neck and stature, vertebral anomalies and mental retardation.

We report on a male adult and his son both affected with hitherto undescribed multiple congenital anomalies and mental retardation. Both have peculiar facies, cleft palate, short sature, congenital brevicollis and vertebral abnormalities. Chromosomal analysis is normal and an autosomal dominant mode of inheritance is most likely. Genetic aspects and clinical manifestations are compared with those of previous reports of Robin sequence or cleft palate, short stature and vertebral dysostosis.

Neonatal thermoregulation.

The human being is a homeotherm. Homeothermy is a result of thermoregulation which includes many physiological processes. Thermoregulation maintains an equilibrium between heat production (thermogenesis) and heat loss (thermolysis). There are three principal modes of heat production: 1. Voluntary muscle activity. 2. Involuntary tonic or rhythmic muscle activity known as "shivering". 3. Non-shivering thermogenesis (NST) essential for newborns. Heat loss occurs in two stages: 1. The flow of heat from the center of the body to its surface. 2. The flow of heat from the body surface to the environment by conduction, convection, radiation or water evaporation. Even in the very small premature baby, we find that metabolic and vasomotor control responses are developed. To protect the newborn from stress resulting from hypo or hyperthermia, one should take into consideration the concept of the neutral temperature range which is also called the "Thermoneutral Zone" in (TNZ) or "Thermal Neutrality". Curves, proposed in 1971 by Hey are essential for keeping newborns in the TNZ.

[Cardio-respirography in neonatal medicine: value, interpretation, indications]. / L'enregistrement cardiorespirographique en médecine néonatale: intérêt, interprétation, indications.

The development of cardiorespirography with data processing enables to perform polygraphic recordings. However the standardized results supplied by this new generation of equipment cannot be used directly and need to be validated and analized by the clinician. The confrontation of the cardiac and respiratory curves allows a semiological analysis of the cardiac and respiratory functions and of their relationships in the various syndromic contexts encountered in the neonate (bradycardia and other cardiac arythmia, central or obstructive apnea, thoracic hypoampliation, periodic breathing).

[Standardized oculocardiac reflex in ex-premature near full term (93 cases)]. / Réflexe oculocardiaque standardisé chez l'ex-prématuré à proximité du terme (93 cas).

UNLABELLED: The aim of the study was to present data investigating vagal reactivity in a population of premature infants reaching term, using the oculo-cardiac reflex. PATIENTS AND METHODS: Ninety three premature infants, free of any disease, near full term at the moment of testing, were prospectively investigated at a time close to discharge from neonatal unit. After an all-night polygraphic recording, a standardized oculo-cardiac reflex test was performed during quiet sleep. Data were classified in relation to both chronological and postconceptional ages. Simple liner regression analyses were performed on the selected variables. RESULTS: The results showed heterogeneity of the vagal response in this population: longest asystole (1049 ms +/- 540; 95th percentile = 1894 ms); maximal percentage of deviation between two successive RR intervals (88% +/- 90; 95th percentile = 200%); and duration between the beginning of decrease in heart rate and return to mean heart rate (14 s +/- 10; 95th percentile = 30 s). CONCLUSION: Our healthy premature infants at time of discharge exhibited a wider range of vagal reactivity than previously reported for the full term newborns. Considering our findings, we recommend caution before proceeding with treatment of vagal bradycardia in a similar premature infant population.

[Typhoid fever in children in Tananarive (Madagascar). Comments on 97 cases]. / La fièvre typhoïde chez l'enfant à Tananarive (Madagascar). Commentaires à propos de 97 observations.

97 cases of typhoïd fever in child are reported. This disease remains frequent and even severe in tropical zone where its evolution is of endemoepidemic type. Established fever, in spite of an appropriate treatment, is one of the main features of this disease. A certain number of clinical signs call for it; although classical, some others are less significant. Complications are frequent, without any correlation between the date of hospitalizing and the beginning of the disease. They are sometimes severe but mortality rate during hospitalization is quite low. Leukoneutropenia and thrombopenia are both exceptional. Blood culture and serology are always the background of any diagnosis, the latter appearing to the authors much more valuable than the former. Sulfamethoxazole-trimethoprime is an antibiotic to be recommended in first instance. Precocity of treatment has no influence on the time of apyrexia is appearing. If hopes put on oral vaccine could be confirmed, so it might be used on a large scale for children living in endemia zones.

[Role of home monitoring in the context of sudden infant death prevention. Current methods indications, monitoring]. / La place du monitorage à domicile dans le contexte de la prévention de la mort subite du nourrisson. Moyens actuels, indications, surveillance.

Some infants are cared with a home monitoring system during their first year of life. An international clinical consensus has been obtained and has proposed this technique mainly for infants who have presented an apparent life threatening event or for ex-premature with bradycardia or apnea, rather than for siblings of sudden infant death syndrome or other infants. In any case, this monitoring must be held after a complete clinical evaluation of the infant and after a real education of the parents about the use of the device. Many types of devices are used. The most efficient is the cardio-respiratory monitoring. Some of them include a processor and record the alarms. The need to see or to call the medical team to decode them allows close collaboration between the family and the clinical team. Knowledge of the alarms and the circumstances in which they have occurred help the medical team to propose the withdrawal of the home monitoring. Thus, sometimes preventive, sometimes prophylactic, this device will provide us for an optimal help.

[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.

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.

[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.

Visual sensory processing deficit in the occipital region in children with attention-deficit / hyperactivity disorder as revealed by event-related potentials during cued continuous performance test.

AIMS OF THE STUDY: Recent studies described several changes of attention-related components of late frontal event-related potentials (ERPs) during Go/NoGo paradigm in children with attention-deficit/hyperactivity disorder (ADHD). We aimed to determine whether ERP components corresponding to earlier encoding of visual incoming information are also modulated by attentional disorders. METHODS: We recorded high-resolution EEG in 15 children meeting DSM-IV criteria for ADHD, comprising 15 age-matched control groups during an equiprobable Go/NoGo task in a cued continuous performance test (CPT-AX) paradigm. Both P100 and N200 ERP components were measured in response to both Go and NoGo stimuli. We analyzed both components with SwLORETA in order to localize their brain sources. RESULTS: A low rate of Go correct response and high rate of omission errors were observed in ADHD children. When compared to controls, these displayed delayed P100 and N200 latency, and lower P100-NoGo amplitude. In addition, the P100 latency was delayed for NoGo compared to Go condition. The source of P100 was located in occipital area. A sizable decrease in early electrical activity was found in ADHD, especially in the NoGo condition. CONCLUSION: Our results suggest an early deficit in visual sensory integration within the occipital cortex in children with ADHD.

Vasomotor reactivity in premature neonates at term.

In order to assess the specific sympathetic reactivity in premature infants at term, we designed a study to evaluate the peripheral vasomotor response of such infants when exposed to auditory challenges. Testing was performed in 29 premature neonates at term in both quiet and active sleep during a morning session. Two types of noises were used (click and continuous tones) at three frequencies (250, 1,000 and 6,000 Hz) and at three intensities (60, 85 and 110 dBA). Vasomotor response was studied by analyzing with Mathlab software the variability of the plethysmographic wave of the oxymetric pulse. No behavioral awakening was observed in response to any stimulation. When a tachycardia or a bradycardia reaction to the stimuli was observed, all neonates responded with a vasoconstriction. The global mean of the vasoconstrictive response was 18.45%. The overall ANOVA on the vasomotor response revealed significant effects for sleep stages (t: 1.98; p < 0.05), for frequency (t: 3.3; p < 0.001) and for intensity of noise (t: 3.01; p < 0.03) but no significant response with heart rate variability. From these results, we could conclude that the assessment of the vasomotor response is a very sensitive procedure to determine the reactivity of the autonomic nervous system in neonates, and could be used to study such vegetative responses in other stressful situations with good accuracy.

[Home cardio-respiratory monitoring with alarm record system in infants at risk of life-threatening events]. / Surveillance cardiorespiratoire à domicile avec alarmes enregistrées chez des nourrissons à risque de malaises.

In few infants, home monitoring is useful to prevent recurrent apparently life-threatening events. Some devices have an alarm record system. We report our experience of home monitoring with such a device in 22 infants. 43.3% of the recorded events were considered as false alarms and 56.7% as true alarms. Among the alarms relative to abnormal respiratory events (38%), more than half occurred after two min of very low impedance thoracic signal. Among the true alarms relative to cardiac abnormalities (18.7%) more than half occurred during high amplitude fluctuations of the thoracic impedance signal and were relative to obstructive apnea or hypertonic vagal reactivity. Three infants presented an apparent life threatening event during an alarm, and two of them were hospitalized. These results indicate that it is important to define precisely the significance of the alarms during the survey of home monitoring of infants at risk for sudden infant death.

[Treatment of acute renal failure by continuous hemofiltration in newborn infants and infants]. / Traitement de l'insuffisance rénale aiguë par hémofiltration continue chez le nouveau-né et le nourrisson.

During acute oligoanuric renal failure in neonates and infants, arteriovenous or veno-venous continuous hemofiltration is a simple, useful technique when peritoneal dialysis or hemodialysis cannot be used. It allows fluid removal, optimal calorie intake and mild blood purification, when waiting for diuresis to stabilize. It does not give any control over hypercatabolism; the use of continuous dialysis increases epuration.

[Standardization of oculo-cardiac reflex in infants under 3 months of age]. / Standardisation du réflexe oculo-cardiaque chez les nourrissons de moins de 3 mois.

The authors present a standardization of the oculocardiac reflex carried out in early-age infants (under 3 months). This standardization is easy to perform. It takes into account the fall of the cardiac frequency, the asystoly and the range of bradycardia until its normalisation. Thus, it allows inter or intra individual comparison and can be applied to the study of infants at risk for sudden infant death syndrome.

[Epiglottitis and pulmonary edema in children]. / Epiglottite et oedème pulmonaire chez l'enfant.

A case of acute epiglottitis complicated by pulmonary edema, after intubation in a 30 month-old child is reported. The pathophysiology of this complication and its treatment associating oxygen therapy and positive end expiratory pressure are discussed.