Aysima Özçelik

Effects of epilepsy and antiepileptic drugs on nitric oxide, lipid peroxidation and xanthine oxidase system in children with idiopathic epilepsy

The aim of this study is to investigate the effects of epilepsy, valproic acid and oxcarbazepine on nitric oxide levels, lipid peroxidation and xanthine oxidase levels in newly diagnosed epileptic children and healthy controls. A total of 49 patients with newly diagnosed idiopathic epilepsy and 15 healthy children were enrolled in this study. Of these 49 patients, 16 children were treated with valproate and 16 treated with oxcarbazepine. Nitric oxide, malondialdehyde and xanthine oxidase levels prior to antiepileptic drug therapy were measured in the serum. Blood samples were drawn before antiepileptic drug therapy and after 3 and 6 months of the antiepileptic drug treatment. Nitric oxide levels were statistically higher in the newly diagnosed epileptic patients. In oxcarbazepine group, the nitric oxide and malondialdehyde levels were found to be decreased. No statistically significant differences were noted in nitric oxide, malondialdehyde and xanthine oxidase levels in valproic acid treated group. Oxcarbazepine which is a frequently used new antiepileptic drug in childhood epilepsy may modify nitric oxide levels and lipid peroxidation. These results suggest that decreased lipid peroxidation would play a role in the mechanism of antiepileptic effects by oxcarbazepine treatment.

3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family.

A family with 3-methylcrotonyl-CoA carboxylase deficiency with different clinical features is described. A 15-month-old boy, who was the index patient, was admitted to the hospital with atonic seizure. His brother had delayed language development and their uncle had been followed with diagnosis of epilepsy for the last 5 years. Urinary organic acid analysis displayed elevated 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, analysis of acylcarnitines showed elevated 3-hydroxyisovalerylcarnitine and decreased free carnitine levels in both the patients and their uncle. Methylcrotonyl-CoA carboxylase activity in cultured fibroblasts displayed a low residual activity of 2.2% of the median control value while propionyl-CoA carboxylase activity was normal in the index patient. Mutation analysis revealed a large homozygous deletion of 2264 bp (c.873+4524_6787de12264) in the MCCA gene, which has not been described to date. Adult-onset afebrile seizures have not been reported in the literature. Our cases are an example of this wide phenotypic variability within a single family.

Attitudes of Parents and Physicians Toward Febrile Seizures

Objectives Although febrile seizures are common in children, attitudes may change among parents. The management of a child may differ depending on the specialty of the attending physician. This study was carried out to analyze attitudes of Turkish parents and physicians toward febrile seizures. Material and Methods 308 children with febrile seizure who were admitted to the Department of Pediatric Neurology at Dr Sami Ulus Childrens Hospital and Gazi University in Turkey between January 2006 and March 2007 were enrolled. Results Prior to seizure, approximately half of the parents took appropriate steps in reducing fever. The data also showed that there was a wide variation of treatment practice depending on the specialty of the attending physician. Discussion Educational level and economic status are important variables affecting attitudes of parents toward fever and febrile seizure. The management of the child with a febrile seizure differs even within the same specialty in Turkish physicians.

Successful surgical treatment of medically refractory epilepsy after chemotherapy in a child with leukemia: a case report.

Introduction:Mesial temporal sclerosis associated with acute lymphoblastic leukemia has been rarely reported. Case Report:We report a case of a 15-year-old boy with acute lymphoblastic leukemia who developed medically refractory temporal lobe epilepsy in a long time period after chemotherapy, and successfully treated with surgical resection. Conclusions:To our knowledge, this is the first description of a leukemic child with medically refractory temporal lobe epilepsy who achieved seizure-freedom postoperatively during the long-term follow-up. The clinical history and details of the underlying pathophysiological background and treatment of this complication are discussed in this case report.

A Pediatric Case Presented with Posterior Reversible Encephalopathy Due to Cystinosis: Case Report

52 ystinosis is an inherited autosomal recessive disorder, which the defect involves cystinosin, the membrane transport protein playing role in amino acid cystine metabolism. Thus, cystine accumulates in lysosomes and elicits the symptoms in childhood like end stage renal disease, secondary Fanconi syndrome (proximal renal tubular acidosis).1 Neurologic complications consisting of cystinosis encephalopathy, myopathy, stroke like episode with coma, hemiplegia and myopathy are seen later. A Pediatric Case Presented with Posterior Reversible Encephalopathy Due to Cystinosis: Case Report