[Indications of electroencephalogram in the newborn]. / Indications de l'électroencéphalogramme en période néonatale. Recommandations du groupe de neurophysiologie clinique de l'enfant.

The electroencephalogram (EEG), an easy-to-use and non invasive cerebral investigation, is a useful tool for diagnosis and early prognosis in newborn babies. In newborn full term babies manifesting abnormal clinical signs, EEG can point focal lesions or specific aetiology. EEG background activity and sleep organization have a high prognostic value. Tracings recorded over long period can detect seizures, with or without clinical manifestations, and differentiate them from paroxysmal non epileptic movements. The EEG should therefore be recorded at the beginning of the first symptoms, and if possible before any seizure treatment. When used as a neonatal prognostic tool, EEG background activity is classified as normal, abnormal (type A and type B discontinuous and hyperactive rapid tracing) or highly abnormal (inactive, paroxysmal, low voltage plus theta tracing). In such cases, the initial recording must be made between 12 and 48 h after birth, and then between 4 and 8 days of life. Severe EEG abnormalities before 12 h of life have no reliable prognostic value but may help in the choice of early neuroprotective treatment of acute cerebral hypoxia-ischemia. During presumed hypoxic-ischemic encephalopathy, unusual EEG patterns may indicate another diagnosis. In premature newborn babies (29-32 w GA) with neurological abnormalities, EEG use is the same as in term newborns. Without any neurological abnormal sign, EEG requirements depend on GA and the mother's or child's risk factors. Before 28 w GA, when looking for positive rolandic sharp waves (PRSW), EEG records are to be acquired systematically at D2-D3, D7-D8, 31-32 and 36 w GA. It is well known that numerous and persistent PRSW are related to periventricular leukomalacia (PVL) and indicate a bad prognosis. In babies born after 32 GA with clinically severe symptoms, an EEG should be performed before D7. Background activity, organization and maturation of the tracing are valuable diagnosis and prognosis indicators. These recommendations are designed (1) to get a maximum of precise informations from a limited number of tracings and (2) to standardize practices and thus facilitate comparisons and multicenter studies.

[SPECT-identified hypoperfusion of the left temporomesial structures in a Kleine-Levin syndrome]. / Hypoperfusion temporomésiale gauche en TEMP dans un syndrome de Kleine-Levin.

A 13-year-old boy developed typical features of Kleine-Levin syndrome. Routine investigations and MRI were normal. SPECT, performed both during an attack and during a symptom-fee period, demonstrated clear hypoperfusion of the left mesiotemporal structures. The possible implications of this finding are discussed.

[Epilepsy presenting as life-threatening events in infants]. / Les malaises du nourrisson d'origine épileptique.

OBJECTIVE: This study was aimed at assessing the frequency and features of epilepsy presenting as life-threatening events in infancy. PATIENTS AND METHODS: Fifteen cases were collected over eight years. Subtle symptoms suggestive of epilepsy were rare (9/15) in the retrospective analysis; the clinical context and cerebral imaging were occasionally contributive (5/15); interictal EEG was useful but inconstantly contributive, including when the epileptic nature of the episodes had become clinically probable (10/15). Though it had no clear relationships with epileptic episodes but contributed to mislead diagnosis, gastroesophageal reflux was frequently associated. Uncovered epilepsies were not homogeneous, ranging from severe epileptic encephalopathy to benign epilepsy of infancy. CONCLUSION: Epilepsy presenting as life-threatening events is sometimes a diagnostic challenge. When an infant develops recurrent critical episodes during a several-week follow-up, with neurological and general investigations not providing more information, the immediate success of antiepileptic treatment might support diagnosis.

[Electroencephalography of the premature and term newborn. Maturational aspects and glossary]. / Electroencéphalographie du nouveau-né prématuré et à terme. Aspects maturatifs et glossaire.

From the first publication of C. Dreyfus-Brisac and N. Monod, a strong tradition combined with tremendous development of neonatal EEG has taken place in France. After 3 years of collaborative work, 12 clinical neurophysiologists trained at the Port-Royal medical school in Paris detail in this paper the currently available neonatal EEG recording techniques. They have synthesized the criteria of maturational state analysis and have defined the normal and pathological neonatal EEG patterns, including descriptions already present in the French as well as the English literature. In this review one may find a complete description of neonatal EEG patterns according to the states of vigilance and to gestational age. Furthermore, definitions of all normal and pathological patterns are provided in a glossary. Both chapters are illustrated by numerous figures. This detailed terminology in neonatal EEG should allow a better homogeneity in EEG reports, and could lead to multicentric studies on normal, unusual or pathological patterns, according to etiology. Although based on analogic EEG data, this work can equally be applied to digitized EEG tracings.

[Evaluation of the attention span of 10,000 school children 8-11 years of age]. / Evaluation de la capacité d'attention chez 10,000 enfants scolarisés âgés de 8 à 11 ans.

AIM: This study was conducted in order to evaluate the attention capacity during the school day and week, taking school life and others factors into account in 10,000 children (8 to 11 years old) in public Parisian schools. MATERIAL AND METHODS: The test used to measure attention was a paper and pencil test validated from the Zazzo test. The children answered a questionnaire before each test to obtain information concerning the time and day of the test, and concerning also the different life styles of children, particularly quantity and quality of sleep and composition of breakfast. The evaluation was performed four times in the morning and four times in the afternoon every day, to measure exactly the fluctuations of attention. This study lasted four weeks. The last evaluation week took place after the summer time change. RESULTS: The results showed the existence of an ultradian fluctuation of attention during the day, with the lowest level between 08.00 a.m. and 09.30 a.m. and the highest level between 04.00 p.m. and 04.30 p.m. The attention levels on Monday and Thursday, after a day without school, were not lowered. Furthermore, after a 2 days weekend the attention level was higher than after a 1 day and half weekend. The summer time change also worsened the attention capacity of children all along the next week. Finally, there was a statistical relationship between children attention capacity and their sleep practice. CONCLUSION: These results confirm the ultradian fluctuation of attention capacity and show the importance of the regularity in the child's life style with respect to their mental functioning.

Haemorrhagic shock and encephalopathy syndrome: neurological course and predictors of outcome.

UNLABELLED: The haemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease. The aetiology of this syndrome is unknown, and, despite intensive treatment, the outcome is often fatal or associated with severe neurological sequelae. OBJECTIVE: To assess the neurological features and potential prognostic markers of the disease. DESIGN: Retrospective study. SETTING: Division of Neuropaediatrics in a children's university hospital. PATIENTS AND METHODS: Fourteen patients fulfilling the HSES criteria out of 42 children admitted with fever and shock to the Paediatric Intensive Care Unit between 1986 and 1994, were analysed for clinical, biological, neuroradiological, EEG and neuropathological findings. RESULTS: The patients (age range from 2 to 33 months) were found at night or in the morning either comatous (n = 3) or convulsing (n = 11). All but one were healthy before admission, although eight had had a brief prodromal infectious disease. All were febrile (mean body temperature 39.9 degrees C +/-0.9 degrees). Seasonal clustering during the winter months was observed. Coma and seizures with frequent status epilepticus were the main neurological manifestations. All children recovered from their multiple organ failure within a few days. Seven died (50%); four survivors had neurological sequelae (29%) with a developmental quotient (DQ) of 50% or less in three and a DQ of 75% in one and three infants (21%) had normal outcomes. Computed tomography (CT) displayed a diffuse area of low density mainly in the cerebral cortex and intraventricular and parenchymal haemorrhages. Magnetic resonance imaging (MRI) showed haemorrhagic cortical lesions. Postmortem examination of the brain conducted in three patients showed necrotic and haemorrhagic lesions, mainly in cortical areas. Comparison of the children with adverse outcome (death or neurological sequelae) with those with normal outcome revealed that predictors of poor outcome were status epilepticus (p = 0.003) and coma for more than 24 h (p = 0.01). Infants without disseminated intravascular coagulation, without a biphasic course and without brain hypodensities or haemorrhages on CT scans performed at least 4 days after onset had a normal neurodevelopmental outcome. CONCLUSION: The central nervous system appeared to be the main target of the HSES lesions. The most common outcome was brain death or severe brain damage. Further studies with a larger sample are necessary to determine whether the prognostic indicators we identified are reliable.

[[Emergency pediatric EEG in mental confusion, behavioral disorders and vigilance disorders: a retrospective study]. / L'EEG d'urgence en pédiatrie dans les troubles de la vigilance, la confusion mentale et les troubles du comportement: étude rétrospective.

Recording of electroencephalogram (EEG) is of value to estimate vigilance states in children as in adults. In order to determine the diagnostic and prognostic value of emergency EEG in case of mental confusion, behavioral disorders and vigilance disorders in childhood, we conducted a retrospective study in 397 children (aged 2 months to 16 years). EEG was recorded less than 24 hours after an emergency consultation for acute confusion or acute behavioral disorder (n = 106) or after admission to the intensive care unit for comatose stage (n = 291). EEG gave diagnostic indications mainly in case of convulsive pathology or hepatic encephalopathy. In comatose children, we established a 4-stage EEG scale of increasing severity. This classification was compared to EEG scales already published in the literature and appeared very similar to that from Pampiglione and Harden, established in 150 children after cardiac arrest. A highly poor prognostic value was associated with burst-suppression post-anoxic patterns and with isoelectric records signaling brain death. Our classification of emergency EEG patterns is mainly helpful in these two situations, but does not exclude strict and repeated clinical and EEG follow-up in other cases, as a relatively preserved initial EEG may later deteriorate.

[Intraventricular hemorrhage and parenchymatous ischemia in the newborn at term. Report of five cases]. / Hémorragie intraventriculaire et ischémie parenchymateuse chez le nouveau-né à terme à propos de cinq cas.

Although intraventricular hemorrhage associated with cerebral ischemia without severe perinatal asphyxia is rare in full-term newborns, it can be severe, have early or late onset depending on the etiology and be of poor prognosis. Five full-term neonates (37 to 41 weeks of gestational age) without criteria of severe perinatal asphyxia were admitted to the intensive care unit for seizures: four were between seven and 11 days of age and one was only 12 h old. Clinical or electroclinical seizures recorded by continuous EEG monitoring were numerous, leading to status epilepticus in three babies. They were unilateral (at the level of the left hemisphere) in one infant and have not been recorded in the fourth case. Past-ictal EEG abnormalities were numerous rolandic or temporal slow or fast sharp waves of variable polarity. Cranial CT scans showed uni- or bilateral intraventricular hemorrhage with dilatation and subcortical or periventricular ischemic lesions with hemorrhage. Four out of the five infants died during the neonatal period.

Congenital unilateral perisylvian syndrome: radiological basis and clinical correlations.

DESIGN: Advances in neuroimaging have allowed correlations between radiological patterns and clinical features of brain malformations. This paper reports clinical, prognosis, and electroencephalographic features of six children with a previously unrecognised neuroimaging picture of unilateral widening and verticalisation of the sylvian fossa associated with an abnormal ipsilateral perisylvian cortex. RESULTS: All children had reduced hemisphere size and thalamostriatal hypoplasia ipsilateral to the cleft and hemiplegia. Cognitive development was mostly impaired. Epilepsy occurred in two patients and was mainly characterised by partial seizures. Studies with EEG showed hemispheric slowing of background activity homolateral to the perisylvian dysplasia. Occurrence of the malformation among their siblings was not found. CONCLUSION: Similar brain malformations occasionally reported in older patients confirm the clinical picture, sporadic occurrence, and prognosis found, allowing the validation of a unilateral perisylvian syndrome.

Flumazenil in the treatment of hepatic encephalopathy in children with fulminant liver failure.

OBJECTIVE: To evaluate the effect of flumazenil on hepatic encephalopathy complicating fulminant liver failure in children. DESIGN: Uncontrolled prospective study. SETTING: Pediatric Intensive Care Unit, tertiary care center. PATIENTS: 9 children with fulminant liver failure and hepatic encephalopathy awaiting emergency liver transplantation. INTERVENTIONS: Changes in hepatic encephalopathy grade and in electroencephalogram were recorded during the injection of a bolus of flumazenil (0.01 mg/kg/i.v. bolus) followed by a continuous infusion of flumazenil (0.01 mg/kg/h). MEASUREMENTS AND MAIN RESULTS: Before flumazenil, 7 children had grade 2 and 2 had grade 3 hepatic encephalopathy. Flumazenile injection mediated an arousal effect in 1 child in whom encephalopathy improved from grade 3 to grade 2. This effect lasted 30 min. No clinical response was observed in other children. An improvement of EEG anomalies was observed lasting 3 min in one child. Despite continuous infusion of flumazenil, encephalopathy worsened in all children. CONCLUSION: The effect of intravenous administration of flumazenil on hepatic encephalopathy in children with fulminant liver failure is inconsistent. Its efficacy is transient. The therapeutic value of flumazenil in children with fulminant liver failure awaiting a liver graft is minimal at this dosage.

Confirmation of linkage of benign familial neonatal convulsions to D20S19 and D20S20.

Benign familial neonatal convulsions (BFNC) is an idiopathic form of epilepsy beginning within the first six months of life. Its genetic origin and autosomal dominant mode of inheritance have been suspected since its first description. Recently, the BFNC gene has been localised within chromosome 20q in one large pedigree. For the first time, we confirm here (with D20S19 and D20S20) the close linkage of BFNC to chromosome 20q in six French pedigrees. In addition, the existence in these families of several cases of febrile convulsions (FC), another epileptic syndrome with an autosomal dominant genetic component, led us to study the possibility of a genetic background identical to BFNC. Our results suggest the existence of different susceptibility genes for BFNC and FC.

[Atypical electroencephalographic activity in terminal phase subacute sclerosing panencephalitis]. / Activité électroencéphalographique atypique lors d'une panencéphalite sclérosante subaiguë en phase terminale.

The authors describe the evolution of the EEG during a 3-yr period in a case of subacute sclerosing panencephalitis (SSPE) revealed by epilepsy in a 9 yr-old boy. During the first 2 yr, the EEG was markedly abnormal (diffuse spike-waves, delta rhythms). During the static period, it looked like a continuous non-convulsive status epilepticus. The typical periodic abnormal activity only appeared at the terminal stage, and after a Valium test. Then slow wave complexes persisted on a dysrhythmic background. This type of EEG has never been described; our study stressed the efficacy of the Valium test in this particular case.

The distribution of slow-wave sleep across the night: a comparison for infants, children, and adults.

This study describes the temporal distribution of slow-wave sleep (SWS) (defined as the visually scored stages 3 + 4) across the night for 16 infants aged between 20 weeks and 1 year, 17 children between 1 and 6 years, and 17 adults between 20 and 36 years. In all three groups the amounts of SWS peaked during the first nonrapid eye movement (NREM) episode. SWS decreased across the night for adults and children, but not for infants. In infants the amounts of SWS remained at a fairly constant level from the second cycle onward, although many cycles were observed with zero SWS. The latter was evident from the very low tendency for SWS to appear in consecutive NREM/REM cycles. Rather, SWS was observed in alternate cycles. In children this phenomenon was less prominent but still well visible, and the tendency for SWS to appear in consecutive cycles had increased. In adults SWS occurred predominantly in consecutive cycles. The results suggest that whereas REM recurrence time increases twofold from infancy to adulthood, SWS recurrence time remains of similar length in infants, children, and adults.

[Disorders in sleep organization in pediatric diseases]. / Troubles de l'organisation du sommeil en pathologie pédiatrique.

Interactions between sleep and general pathology in pediatrics are numerous and little known. Sleep organization evolves with age and stages of development. The most important physiological sleep changes occur during the first year of life. The main symptom of sleep disorders is excessive daytime sleepiness associated with dyssomnia, behavioral and learning disorders. Sleep disorders in childhood are often difficult to evidence, sleep investigations being still heavy and expensive.

[Serious acute hepatocellular insufficiency in children: EEG prognostic features]. / Insuffisance hépato-cellulaire aiguë grave de l'enfant: aspects EEG pronostiques.

Fulminant Hepatic Failure (FHF) in children leads to 60 to 90% of mortality without age influence, depending on the studies. Twenty-two children with this disease had daily EEG, 15 died, seven survived. The neurological electroclinical study evidenced a stereotyped evolution of the EEG anomalies which were not age-related, but correlated to clinical stages of coma and to severe coagulation disorders; 5/22 children with clinical and/or EEG convulsive disorders died. One child exhibited triphasic slow waves and died from paracetamol intoxication. A prospective study of a larger population could help to recognize factors of decision for hepatic transplantation.