[Acute encephalitis in children]. / Encéphalites aiguës de l'enfant.

Acute encephalitis is mainly of viral origin. Two groups of are considered: i) primary encephalitis, such as Herpes simplex encephalitis with intra-thecal synthesis of antibodies, and ii) post-viral infection encephalitis or acute disseminated encephalitis with immune dysregulation. The most common clinical presentation (fever, consciousness disturbance and seizures) is not specific and may reveal bacterial meningitis or cerebral abscess which require a specific treatment. Acyclovir has allowed consistant advances in the treatment of herpes encephalitis. Vaccination against selected viral infection, such as measle vaccine, is the only way to prevent acute disseminated encephalitis.

[Kocher-Debre-Semelaigne syndrome. Apropos of a case]. / Le syndrome de Kocher-Debré-Semelaigne. A propos d'une observation.

We report a new case of Kocher-Debré-Semelaigne syndrome in a child and describe this condition on the basis of a review of the literature. Affected individuals exhibit both hypothyroidism and pseudohypertrophic myopathy and consequently have an athletic appearance that contrasts with physical and psychological sluggishness.

[A purely cutaneous form of periarteritis nodosa in children. A new case with discussion of the literature]. / Forme cutanée pure de périartérite noueuse de l'enfant. Une nouvelle observation avec discussion de la littérature.

We report a new case of strictly cutaneous periarteritis nodosa in a girl aged 5 1/2 years at onset of the disease. No visceral involvement has developed during the seven years follow-up. Our patient is the fourth published pediatric case of strictly cutaneous periarteritis nodosa.

[Infantile spasms and lissencephaly. Apropos of 2 cases]. / Spasmes infantiles et lissencéphalie. A propos de 2 observations.

Lissencephaly is a rare brain anomaly characterized by a lack of cerebral sulci and gyri. We report 2 cases of lissencephaly syndrome with infantile spasms diagnosed by CT-Scan. We discuss the relationship between lissencephaly and infantile spasms, and attempt to estimate the frequency of infantile spasms in lissencephaly and the frequency of this cerebral abnormality among other etiologies of infantile spasms. The prognosis of infantile spasms is indeed variable, according to the presence of underlying cerebral abnormality.

[Periosteal reaction in periarteritis nodosa in children]. / Réaction périostée au cours d'une périartérite noueuse de l'enfant.

Bone involvement has exceptionally been reported in children with periarteritis nodosa. A 5-year old girl was admitted to hospital for fever and arthralgias. Three months later, myalgias and painful subcutaneous nodules developed on the legs and ankles. Skin biopsy yielded a diagnosis of periarteritis nodosa. X-ray films revealed a bilateral periosteal reaction with images of laminae in the tibia and fibula. After a 4-year remission under corticosteroid therapy, the periosteal reaction persisted.

[Rheumatic manifestations of distal tubular acidosis. Apropos of a case]. / Manifestations rhumatismales de l'acidose tubulaire distale. A propos d'un cas.

In a 13 year-old boy treated for polyarthritis, nephrocalcinosis and an inability to acidify urine were observed. Distal renal tubular acidosis may be associated with articular manifestations and is reversible with the usual treatment with alkali as are the other manifestations of the disease.

[Monotherapy with carbamazepine in childhood epilepsies]. / Monothérapie par la carbamazépine dans les épilepsies de l'enfant.

68 epileptic children were treated with carbamazepine (CBZ) monotherapy for a mean period of 10 months. The seizures disappeared in 43% and decreased in another 26.5% of cases. The treatment was particularly efficient in partial benign epilepsies (disappearance: 64.7%, reduction: 29.4%) and in partial unclassifiable epilepsies (disappearance: 50%, reduction: 25%). It is sometimes useful to achieve blood levels over 4 micrograms/ml; but over 8 micrograms/ml the frequency of side-effects becomes greater than improvement of fit frequency. The dose-concentration relation is well correlated to age, so that blood level measurements do not need to be systematically performed. In order to obtain mean blood levels of 7 micrograms/ml 3 to 4 hours after morning intake, mean daily dose should range from: 20 mg/kg/24 h before 5 years to 10 mg/kg/24 h after 10 years.