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Journal of Pediatric Epilepsy is an English multidisciplinary peer-reviewed international journal publishing articles on all topics related to epilepsy and seizure disorders in childhood. These topics include the basic sciences related to the condition itself, the differential diagnosis, natural history and epidemiology of seizures, and the investigation and practical management of epilepsy (including drug treatment, neurosurgery and non-medical and behavioral treatments).
Journal of Pediatric Epilepsy provides an in-depth update on new subjects, and current comprehensive coverage of the latest techniques in the diagnosis and treatment of childhood epilepsy.
Journal of Pediatric Epilepsy encourages submissions from all authors throughout the world.
The following articles will be considered for publication: editorials, original and review articles, short report, rapid communications, case reports, letters to the editor, and book reviews. The aim of the journal is to share and disseminate knowledge between all disciplines that work in the field of epilepsy in childhood.
Abstract: Epileptic encephalopathies are age-dependent conditions, typically drug resistant, characterized by severe cognitive impairment due to the epileptic activity itself. Corticosteroid therapy with adrenocorticotrophic hormone, hydrocortisone, prednisone or methylprednisolone is one of the therapeutic approaches tried in these children. Although the first use of steroids as antiepileptic drugs dates back to 1950, there is no wide agreement about the ideal treatment regimen in epileptic encephalopathies other than West syndrome; no evidence from class I and II studies are available at the moment. In this study, we present a literature review on steroid therapy in childhood epilepsy with special regard to effectiveness,…tolerability and possible mechanisms of action.
Abstract: Lennox-Gastaut syndrome (LGS) is a pediatric epileptic encephalopathy, which is characterized by uncontrolled seizures, diffuse slow spike and wave discharges on encephalogram, and cognitive impairment. This is a severe form of childhood epilepsy, pharmacoresistant in most cases, with a peak incidence between the ages of 3 and 5 years. Mental retardation is common attributed to increased frequency of seizures. Rufinamide approval by Food and Drug Administration gave new hope to patients and their caregivers. Rufinamide is a third generation anticonvulsant, which is structurally different from other anticonvulsants. Clinical trials of rufinamide have shown a decreased frequency of seizures including atonic…seizures and drop attacks in patients with LGS. In this current paper, we discuss the role of rufinamide as a new option in the management of this childhood epileptic encephalopathy.
Abstract: Many studies have reported cognitive and behavioral abnormalities with recurrent seizures in adults. Similar evidence from the pediatric population is scarce and controversial. We aimed to investigate the effect of recurrent seizures on the developing brain. Included were 42 children with epilepsy (mean age 14.1 ± 1.72 yr) and 30 healthy children for comparison. Patients had recurrent untreated epilepsy (generalized or focal). Negative attitudes and misconceptions about epilepsy are common in developing countries, often resulting in neglect of medical services and treatment. Epilepsy is greatly misunderstood by the public, and is attributed to spiritual causes such as the devil or…mistaken as odd behavior or daydreaming. Another misconception is that medications will harm the child. Intelligence quotient (IQ) and cognition were examined using Wechsler Intelligence Scale for Children and Stanford Binet Subsets Test version 4 (SBST4). Serum levels of neuron-specific enolase (NSE) and S100ß proteins, sensitive markers of neuronal and glial cell damage were measured. Compared to controls, patients had lower mean score of full scale IQ on the Wechsler Intelligence Scale for Children (P = 0.045), particularly performance IQ scores (P < 0.01), and comprehension, pattern analysis, quantitation, bead memory and memory for sentences of SBST4 (P = 0.045; P = 0.013, P = 0.007, P = 0.002; P = 0.035), but not for NSE or S100ß. Severe epileptogenic (EEG) records were observed in children with lower IQ. Serum concentrations of NSE and S100ß were lower in children with higher seizure frequency but did not reach significance compared to controls. Significant correlation was observed between full scale IQ and duration of illness (r = −0.430, P = 0.035), number of seizures (r = −0.580, P = 0.005) and severity of electroencephalography changes (r = −0.450, P = 0.052), but not with S100ß or NSE levels. Lower intelligence and poor cognitive performance were common with recurrent childhood epilepsy. Functional brain abnormalities without structural brain injury may likely be the cause. It seems that serum levels of NSE and S100ß are not sensitive markers for structural or minimal brain damage in children with untreated epilepsy. Thus, early recognition and optimal seizure control is necessary to prevent the subsequent damaging effects on the brain due to prolonged and recurrent seizures and subclinical epileptiform activity. Looking for additional, more specific markers of brain injury is necessary, elevated serum levels of NSE and S100ß were not detected.
Abstract: Epilepsy is the most common neurological disorder worldwide. One-third of epileptic patients do not respond after treatment with first- or second-line antiepileptic drugs. Pregabalin is a novel antiseizure drug with established anxiolytic and analgesic efficacy. In this study, we evaluated the efficacy and tolerability of pregabalin as an adjunctive therapy in a group of children with intractable epilepsy. From October 2011 to September 2012, 67 children with refractory epilepsy who visited the pediatric neurology clinic of Mofid Children’s Hospital were enrolled in this study. The patients were treated with pregabalin. Reduction in seizure frequency and severity were compared after 1…and 6 mo of treatment initiation. During follow-up, >50% reduction in seizure frequency or severity was observed as a response to the drug. Of the 60 children who reached the last stage, 29 (48.3%) were boys and 31 (51.7%) were girls. The age of the children ranged between 6 mo and 16 yr, with a mean age of 71 ± 42.9 mo. Pregabalin reduced seizure frequency up to 2.41 ± 2.38 (48% decline) and 2.75 ± 2.38 (40.86% decline) after 1 and 6 mo of treatment initiation, respectively. There was a significant difference between seizure frequency at 1 (P < 0.001) and 6 mo (P < 0.001) after pregabalin initiation compared with the initial attacks. Increased appetite, frequent urination, hallucinations, and headache were the most common side effects in our patients, with a complication rate of 18.33%. Thus, pregabalin seems to be effective and well tolerated for seizure control in children with intractable epilepsies.
Abstract: Felbamate (FBM) use in treatment of pediatric and adult epilepsy has sharply declined since early reports of FBM-associated hematologic and hepatic injury. We retrospectively reviewed the efficacy and safety profile of FBM in children and young adults with intractable epilepsy treated with this antiepileptic drug as adjunctive therapy in our pediatric epilepsy clinic from 2001–2010. We identified 101 patients 2–22 yr (mean 12.8 ± 1.8 yr) with various seizure types and epilepsy syndromes. FBM dosing was titrated according to tolerability and seizure control. Mean duration of FBM treatment was 43.7 ± 30.4 mo. Patients had periodic serum drug levels, serum…hematologic and metabolic panels and body-mass index measurements during treatment and follow-up. No patient had serious adverse effects, including aplastic anemia or hepatic failure. Using subjective seizure counts, FBM also seemed to be effective in seizure control; 22 (22%) patients were reported as seizure-free; 59 (59%) patients were said by parents to have had fewer seizures, and 20 (20%) patients were estimated as having unchanged seizure frequency. Weight loss, though typically mild, was the major adverse effect. In children older than 3 yr, 46 (53%) of 86 lost weight during FBM administration and 40 (47%) children gained weight. No patients required discontinuation of FBM due to adverse effects. FBM was well-tolerated and without serious adverse effects in our patients. FBM seemed to be effective in reducing seizure frequency. It should be considered a viable current option for treatment of intractable seizures in children, though weight should be monitored in these patients.
Keywords: Epilepsy, felbamate, adverse drug effects
Abstract: Glucose transporter 1 deficiency syndrome (GLUT1-DS1) is a rare and complex congenital metabolic encephalopathy characterized by infantile seizures, movement disorder, delayed development and acquired microcephaly. GLUT1-DS1 is most often caused by a de novo heterozygous mutation of the gene encoding the GLUT1 transporter, SLC2A1. We present an otherwise classical case of GLUT1-DS1 presenting at 6 wk of age with seizures. The infant had unexplained neutropenia during the months preceding molecular diagnosis. Mutational analysis of the SLC2A1 gene identified a de novo novel heterozygous deletion of 26 nucleotides between exon 5 and 6. As expected, the treatment with ketogenic diet…remedied the seizures, but surprisingly it also corrected the neutropenia. We cannot rule out that this might be a phenomenon of neutropenia as an unexplained association in this single patient, but this novel observation has led us to hypothesize that in a subset of susceptible individuals with GLUT1-DS1, disturbed myelopoieses may be an accompanying phenomenon, which may be explained by deficient energy flux in hematological progenitor cells.
Abstract: Agenesis of corpus callosum is a rare malformation of the brain. In Aicardi syndrome, it is associated with retinal lacunae and infantile spasms. Once Aicardi syndrome is diagnosed, one should look for other central nervous system malformations. We report a child who presented with developmental delay, chorioretinal lacunae and infantile spasms, due to Aicardi syndrome, associated with a type 2b interhemispheric cyst diagnosed on magnetic resonance imaging.