[Evaluation of the effect of long term valproic acid treatment on plasma levels of carnitine, ammonia and amino acids related to the urea cycle in pediatric epileptic patients]. / Valoración del efecto del tratamiento prolongado con ácido valproico sobre los niveles plasmáticos de carnitina, amonio y aminoácidos relacionados con el ciclo de la urea en pacientes epilépticos pediátricos.

INTRODUCTION: Valproic acid (VPA) is an antiepileptic drug widely used in paediatrics. In spite of being a safe and effective anticonvulsant, VPA has been involved in the onset of changes in the metabolism of ammonia and carnitine, although few prospective studies have been made of this. OBJECTIVES: To evaluate the effect of long-term VPA administration, particularly on the metabolism of carnitine, ammonia and plasma amino-acids and the possible clinical repercussions of this in a group of epileptic patients studied prospectively and retrospectively. MATERIAL AND METHODS: A study was made of 102 epileptic children on long term anticonvulsant treatment mainly with VPA. These patients were divided into two groups: group I (n = 25) were studied prospectively (basal sample, after one, six and twelve months of treatment) and group II (n = 77) or long term treatment group (a single sample extraction). In each epileptic patient and in 56 children from a control group (group III) studies were made of free plasma carnitine, ammonia and amino-acids related to the urea cycle and the plasma levels of each anticonvulsant drug. RESULTS: It was observed that in group I there was a fall in plasma carnitine concentrations with time and a progressive rise which was statistically significant (p = 0.001) in plasma levels, mainly of ammonia, glutamine, glycine and ornithine, from the basal levels to those after a year of treatment in practically 100% of the children studied. In group II children on antiepileptic drugs, mainly VPA, were seen to have lower plasma carnitine levels than those in the control group and higher serum ammonia, glutamine and glycine levels than the healthy population not treated with anticonvulsants. These differences were statistically significant (p = 0.001). No relationship was found between the parameters studied and the plasma levels of the drug, type of epilepsy or presence of side effects. CONCLUSIONS: These changes show the negative effects of VPA on the metabolism of carnitine and ammonia. It would therefore seem advisable to monitor these parameters in epileptic children on long term antiepileptic treatment.

[Evaluation of usefulness of the IHS criteria in the diagnosis of functional headaches in pediatric age]. / Valoración de la utilidad de los criterios de la IHS en el diagnóstico de las cefaleas funcionales en la edad pediátrica.

Following the criteria of the IHS, we studied 117 children (63 female and 54 male, mean age 9.35 +/- 1.65 years), selecting those classified as migraine (77) or tension-type headaches (30), total 107. We analysed the frequency of clinical manifestations with each type of headache (type and localization of pain, severity of the headache--intensity, duration and degree of disability--and associated symptoms) and their contribution to the differential diagnosis of each entity (evaluated as the presence--sensitivity--or absence--specificity--of each clinical manifestation in each group). Our study shows that throbbing headache (49%) and a unilateral location (31%) as well as nausea and vomiting (33.6%), although constituting diagnostic criteria of migraine, according to the IHS, have acceptable sensibility (91-95%), but only slight specificity (34-40%) for infantile migraine. On the other hand, we found that a greater intensity of headache (22%), and the presence of abdominal pain (15%) as associated symptom, although not considered diagnostic criteria, are also parameters of high sensitivity (100-92%) respectively, and also of greater specificity (30-70%) than the anterior criteria. We conclude that the criteria of IHS for the diagnosis of headache in infancy may be valid although some considerations are necessary. The new IHS criteria for the headache diagnosis in adults [1] may be useful in pediatric age, but the experience on this use is spare, consequently the increment on the use of this classification and the future changes, could produce a best operativity in the diagnosis of headache in childrens.

[Mitochondrial disease due to the deficit of Q-cytochrome C oxidoreductase coenzyme in the respiratory chain. Report of a new case]. / Enfermedad mitocondrial por déficit del coenzima Q-citocromo-C-óxido reductasa de la cadena respiratoria. Aportación de un nuevo caso.

Reference has been made in the literature of the variability in the clinical presentation of deficiency of complex III of the respiratory chain, identifying up to the moment, four groups, the first of which is characterized by hipotonia and wearness starting at variable ages. We report a new case of mitochondrial myopathy due to deficiency of this complex and included within this first group, and consider the importance of defining the clinical and histochemical characteristics of this polymorphous entity.