[Moyamoya disease: advantage of early diagnosis and survival treatment. Review of three cases]. / Maladie de Moya-Moya: intérêt d'un diagnostic et d'un traitement chirurgical précoces. A propos de trois observations.

A diagnosis of moyamoya disease was made in three children aged five, eight and 13 years (including two Turkish sisters). Clinical presentation was recurrent episodes of cerebral ischemia and stroke. CT scans and MRI showed infarcts in various distributions. Angiography revealed anterior bilateral stenosis of the circle of Willis and development of Moyamoya collateral pathways. In one case there was coagulopathy with protein C deficiency. To increase transdural collateral flow, revascularisation with encephalo-duro-arterio-synangiosis was attempted in all three children. Outcome was clinically and angiographically satisfactory and none of the children developed further neurological complications. The current state of study on Moyamoya disease is also presented.

[Platelet serotonin concentration in children under 5 years of age]. / Etude de la concentration en sérotonine plaquettaire chez les enfants de moins de 5 ans.

High platelet serotonin concentrations have been reported in children with early infantile autism. However, as yet there are no reference values regarding platelet serotonin in normal infants and young children so that it remains difficult to define the exact significance of this finding. We report here with the platelet serotonin concentration found in 57 infants and children (20 girls, 37 boys) ranging in age from 10 days to 5 years old. Our results show that mean platelet serotonin concentrations in infants and young children are significantly greater than mean values obtained in older children (+11%) and neonates in the umbilical cord (+64%). No significant variations were found relating to sex, leucocyte count and platelet count. There therefore appears to be a physiological elevation of platelet serotonin concentration in infants and young children, and this has to be taken into consideration in the interpretation of the elevated values found in cases of infantile autism.

[Post-puberty anorexia nervosa]. / L'anorexie mentale postpubertaire.

Post-puberty anorexia nervosa requires a thorough clinical study to facilitate its diagnosis and its early treatment. Longitudinal studies have demonstrated that good results usually depend on the speed of medical intervention (preferably with admission to hospital) and to the duration of treatment. The fact that certain cases result in death is too often due to excessive intensive care. Infusions must be avoided at all costs and should be replaced by forcible feeding if absolutely necessary. Finally, a better approach to, and a better understanding of adolescents should contribute to the prevention of anorexia nervosa.

[Sleep disorders in infants and children]. / Les troubles du sommeil du nourrisson et de l'enfant.

Sleep disorders are common in childhood, and almost always benign. Simple explanations and parental reassurance are generally sufficient for the alleviation or tolerance of the disorder. However, severe forms exist for which a therapeutic support is necessary and often very efficient.

[Evaluation of the treatment with fenfluramine of autism in children]. / Evaluation du traitement par la fenfluramine dans l'autisme infantile.

The effects of fenfluramine were studied in a group of 44 children with the autistic syndrome and in 26 non autistic children with behavior abnormalities, mostly hyperkinetic children, as a control group. The mean daily dosage was 0.65 mg/kg/day. There were 75% positive results in the autistic children and 77% in the control group. The clinical improvement appears to be mainly related to the control of the hyperactive behavior in the autistic children. Platelet serotonin levels were studied in both groups, showing a clear cut decrease during fenfluramine therapy with no significant differences between the 2 groups and no correlation between the clinical effects and the magnitude of the decrease.

Prevention of recurrent febrile convulsions--a randomized therapeutic assay: sodium valproate, phenobarbital and placebo.

The purpose of this study was to compare three different modes of treatment in the prevention of relapses of febrile convulsions (Phenobarbital = PH, Sodium Valproate = SV, Placebo = PO) in a randomized therapeutic trial. The patients included in the study had shown their first generalized convulsive seizure during a bout of fever (greater than or equal to 38.5 degrees C) and were aged between 6 months and 4 years. They were subsequently followed up as outpatients, and Phenobarbital and sodium valproate levels were measured regularly to ascertain compliance with the treatment and to adjust the dosage accordingly. The patients' families were questioned with respect to the occurrence of feverish bouts and convulsive seizures during the interval between visits, as well as possible adverse reactions. An EEG was carried out yearly. Results were as follows: - 69 patients - 35 boys and 34 girls - with an average age of 24 months were divided into 3 groups according to treatment: 21 cases on PH, 22 cases on SV, and 26 cases on PO. - they were followed up for an average duration of 21 months. - the average number of feverish bouts per child and per year was evaluated at 2.5, no statistically significant difference being noticeable between the various modes of prophylaxis. - 15 relapses of febrile seizures were noted in 14 children, over an average duration of 23 months; on average, relapses occurred after 9 months; among the 14 children who had relapsed, one had been treated with SV, 4 with PH and 9 with PO, leading to estimated relapse rates of 4%, 19%, and 35% respectively. There is a statistically significant difference in the relapse rates between the treated groups (SV and PH) and the Placebo group, and a particularly significant difference between Sodium Valproate and Placebo.(ABSTRACT TRUNCATED AT 250 WORDS)

[Maternal and perinatal antecedents of encephalopathies: their contribution to a trial classification of encephalopathies of unknown origin]. / Antécédents maternels et périnatals des encéphalopathes: leur apport à un essai de classification des encéphalopathies d'étiologie inconnue.

We had a sample of 135 mentally retarded children, whose case history (maternal, obstetrical and perinatal) was known before their mental retardation was suspected. This analysis made it possible to pinpoint characteristic anamnesic giving rise to mental retardation by comparison with a check population. It appears that these factors differ according to the supposed origin of mental retardation (genetic, embryonic, foetal, perinatal and postnatal periods) therefore, they can be considered "explicative" of these origins. A multivariate analysis of these factors indicates that the majority of cases of mental retardation with unknown origin are due to disorders during foetal life, which had not been detected.

The pattern of urinary catecholamines and their metabolites in Duchenne myopathy, in relation to disease evolution.

In this report we have tried to determine whether or not catecholamines are involved in the progressive muscular dystrophy. Catecholamines and their metabolites were studied in urines of children with Duchenne disease or other forms of myopathy (limb-girdle and facio-scapulo humeral myopathies). Catecholamine deaminated metabolites were normal in either form of myopathy; in contrast, Duchenne patients, contrarily to other children, eliminated excessive amounts of most amines (catecholamines and methoxylated amines) in relation to age and degree of disease evolution. Our results indicate that catecholamines are not the primary factors involved in the pathogenesis of Duchenne myopathy, but are rather secondary to some disease effects. It is suggested that the high excretion of catecholamines and their methoxylated amine metabolites observed in severely affected Duchenne boys might be related to thermoregulatory process or/and to alterations in some enzymatic systems.

[Weight, height, skull and chest circumferences, biparietal diameter in the newborn infant as a function of gestational age, weight, height and weight gain of the mother]. / Poids, taille, perimetres cranien et thoracique, diametre biparietal du nouveau-ne en fonction de l'age gestationnel, du poids, de la taille et de la prise ponderale de la mere.

The weights, heights, skull and chest circumferences and biparietal diameters were measured in 7,905 newborns in 1972, in the 5 maternity hospitals of Lyon. These data were studied with respect to the gestational ages of the children and according to the usual weights, heights and weight gains of the mothers. The evolution of these parameters was compared to each other; tentative explanations are given.

[Congenital adrenal hypoplasia of cytomegalic type. Recessive, sex-linked form. Apropos of 3 cases]. / Hypoplasie surrenalienne congenitale de type cytomegalique. Forme recessive liee au sexe. A propos de trois observations

Three personal cases, and the cases of literature of congenital adrenal hypoplasia with cytomegaly allow an histological definition. Clinical and biological findings are described. In 2 cases, a diffused hypertrophy of the cells responsible for corticotrope or melanotrope secretion was discovered; it confirmed the peripheral adrenal origin of the condition, as well as high plasmatic level of ACTH, in the third case. Sex-bound cytomegalic adrenal hypoplasia is differenciated from other cortico-adrenal insufficiencies with cytomegaly.