Intracerebral inflammatory pseudotumour in a 16-month-old boy.

A 16-month-old boy presenting with a previous two-month history of absences, was admitted for partial seizures and vomiting. Magnetic resonance imaging revealed a left temporo-occipital intraparenchymatous, well circumscribed lesion. Complete macroscopic resection was performed.

[Physiopathology of periventricular leukomalacia].

The term "periventricular leukomalacia" (PVL) usually covers necrotic and/or gliotic lesions from perinatal origin occurring in the periventricular ring of telencephalic white matter. However focal white matter necrosis is often associated to a diffuse white matter disease and to brainstem and grey matter lesions, making up the basis of a true encephalopathy. PVLs are diagnosed in 4% to 10% of infants born before 33 weeks of gestation.

Goldenhar syndrome and neuroblastoma: a chance association?

Unlabelled: Oculoauriculovertebral dysplasia, also called Goldenhar syndrome, includes several anomalies: epibulbar dermoids or lipodermoids, microtia, mandibular hypoplasia, vertebral, skeletal, cardiac and kidney anomalies, among others. Tumours have also been observed in patients with oculoauriculovertebral dysplasia. We report the first case of oculoauriculovertebral dysplasia associated with a neuroblastoma.

[Management of meconium peritonitis: a remarkable case of idiopathic meconium peritonitis diagnosed antenatally].

Meconium peritonitis is an inflammatory peritonitis induced by meconium extruding into the peritoneal cavity through a perforation of the fetal intestine. Antenatal diagnosis is possible. Prognosis depends on the pathology having caused perforation.

White matter damage and intraventricular hemorrhage in very preterm infants: the EPIPAGE study.

Objective: To evaluate the prevalence of cranial ultrasound abnormalities in very preterm infants as a function of gestational age, plurality, intrauterine growth restriction, and death before discharge.

Study Design: A prospective, population-based cohort of 2667 infants born between 22 and 32 weeks of gestation in 1997 in nine regions of France, transferred to a neonatal intensive care unit, for whom at least one cranial ultrasound scan was available.

Results: The frequencies of white matter damage (WMD), major WMD, cystic periventricular leukomalacia (PVL), periventricular parenchymal hemorrhagic involvement, and intraventricular hemorrhage with ventricular dilatation were 21%, 8%, 5%, 3%, and 3%, respectively.

Effect of enteral supplementation with glutamine on mesenteric blood flow in premature neonates.

Background And Aims: This study investigated the effects of enterally supplied glutamine on mesenteric blood flow in premature neonate.

Methods: Twenty-five neonates, aged at least 14 days and free of acute illness participated in a prospective, randomised, double-blind study. All were fed with total enteral nutrition enriched with glutamine (0.

[Mechanisms of action of tobacco smoke on the developing brain].

Maternal smoking is associated with an increased risk of intra-uterine growth retardation and conduct disorder in their children, and is a major risk factor for sudden infant death syndrome. Among the numerous compounds present in cigarette smoke, carbon monoxide and nicotine have been largely studied. They can both affect the fetal brain by inducing intra-uterine hypoxia or by acting directly on the developing brain.

Glucocorticoid treatment in an ischaemic-like excitotoxic model of periventricular leucomalacia in mice.

Objective: To determine the effects of fluorinated glucocorticoids on the occurrence and extent of ischaemic-like excitotoxic grey and white matter cerebral injuries in an animal model.

Design: A study of the influence of single or repeated doses of glucocorticoids when creating excitotoxic lesions in mice pups, mimicking some aspects of periventricular leucomalacia and cortical-subcortical stroke as observed in human neonates.

Sample: Four hundred and sixty-seven mouse pups out of more than 30 litters.

[Conjunctivo-corneal complex choristoma. Report of an anatomoclinical study].

Complex choristomas are very rare ocular tumors. We report a case of a 3-month-old infant who was referred to us because of a left congenital conjunctivo-corneal protruding mass. Total excision removing 2/3 of the limbus was performed and replaced simultaneously by a limbal autograft.

X-linked lissencephaly with absent corpus callosum and ambiguous genitalia (XLAG): clinical, magnetic resonance imaging, and neuropathological findings.

X-linked lissencephaly with absent corpus callosum and ambiguous genitalia is a newly recognized syndrome responsible for a severe neurological disorder of neonatal onset in boys. Based on the observations of 3 new cases, we confirm the phenotype in affected boys, describe additional MRI findings, report the neuropathological data, and show that carrier females may exhibit neurological and magnetic resonance imaging abnormalities. In affected boys, consistent clinical features of X-linked lissencephaly with absent corpus callosum and ambiguous genitalia are intractable epilepsy of neonatal onset, severe hypotonia, poor responsiveness, genital abnormalities, and early death.

Isolated mild fetal cerebral ventriculomegaly: a retrospective analysis of 26 cases.

We retrospectively studied 26 fetuses with isolated mild cerebral ventriculomegaly diagnosed between 1992 and 1998 and defined by a lateral ventricular atrial diameter of 10-15 mm without any other cerebral anomaly. Our objectives were to determine maternal risk factors, to evaluate complementary investigations, to assess developmental prognosis and to propose possible management. During pregnancy 10/26 patients had regressive ventriculomegalies, ten remained borderline at birth and six were confirmed postnatally.

Environmental factors and disturbances of brain development.

Foetal and neonatal brain is under the influence of environmental factors from maternal and extra-maternal origin. Based on the available data, these environmental factors can be classified into three arbitrary groups: (i) factors and maternal status with a demonstrated deleterious effect on the foetal brain (i.e.

Cerebral white matter damage in the preterm infant: pathophysiology and risk factors.

Based on clinical, epidemiologic, and experimental studies, the aetiology of white matter damage, specifically periventricular leukomalacia (PVL), is multifactorial and involves pre- and perinatal factors possibly including genetic factors, hypoxic-ischaemic insults, infection, excess cytokines, free radical production, increased excitatory amino acid release, and trophic factor deficiencies. The article summarizes research findings about the aetiology of white matter damage and cerebral palsy in preterm infants. The information is organized according to specific antecedents, for which we present epidemiological and neurobiological data.

Fetal and neonatal cerebral infarcts.

Focal arterial infarction in the full-term newborn is an important cause of acquired cerebral lesions in the perinatal period. Clinical motor seizures, most often unifocal, are the nearly constant disclosing symptom confirmed by focal EEG abnormalities. A multifactorial physiopathology is usual, including genetic and perinatal environmental factors.

Central role of microglia in neonatal excitotoxic lesions of the murine periventricular white matter.

Periventricular leukomalacia (PVL) is the main cause of neurologic handicap in pre-term infants. The understanding of cellular and molecular mechanisms leading to white matter damage is critical for development of innovative therapeutic strategies for PVL. The pathogenesis of PVL remains unclear but possibly involves glutamate excitotoxicity as an important molecular pathway.

Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction.

Disorders of neuronal migration in cerebral cortex are associated with neurological impairments, including mental retardation and epilepsy. Their causes and pathophysiology remain largely unknown, however. In patients with Zellweger disease, a lethal panperoxisomal disorder, and in mice lacking the Pxr1 import receptor for peroxisomal matrix proteins, the absence of peroxisomes leads to abnormal neuronal migration.

[Grand prematurity, risk of neuropsychic handicaps and neuroprotection].

Increased survival of very preterm infants and extremely preterm infants does not imply an increase in neurodevelopmental sequelae. However, preterm infants remain at high risk of severe cerebral palsy with neuromotor dysfunction and mental retardation. Necrotic or hemorrhagic lesions occurring in the periventricular ring of telencephalic white matter are the most threatening events for the developing brain of these infants.

Activity-dependent neurotrophic factor-14 requires protein kinase C and mitogen-associated protein kinase kinase activation to protect the developing mouse brain against excitotoxicity.

Activity-dependent neurotrophic factor (ADNF) is a newly identified compound that prevents in vitro neuronal death when present in fentomolar concentrations. ADNF-14, a 14 amino acid peptide derived from ADNF, has the same effects on growth as the parent molecule. However, the transduction pathways and target cells for these highly potent trophic factors are still unknown.

Fetal lactic dehydrogenase variation in normal pregnancy and in cases of severe intra-uterine growth restriction.

Physiological and pathological fetal levels of lactic dehydrogenase (LDH), including its five different iso-enzymes are still poorly known. Our objectives were to compare total LDH levels and its five iso-enzymes between a control group of healthy fetuses and a group of fetuses with severe intra-uterine growth restriction (IUGR), and to determine the biochemical associations and the prognostic value of elevated LDH activity in fetuses with IUGR. Total LDH levels, haematologic values and liver enzyme activities were measured in 108 healthy fetuses from 17 to 37 weeks of gestation and in 44 fetuses with severe IUGR.