Maria Luiza Giraldes de Manreza

A double-blind controlled clinical trial of oxcarbazepine versus phenytoin in children and adolescents with epilepsy

In many countries oxcarbazepine (OXC) has been registered for use as first-line and add-on treatment for patients with partial seizures with or without secondarily generalized seizures (PS) and generalized tonic-clonic seizures without partial onset (GTCS). Its use as monotherapy in children and adolescents with newly diagnosed epilepsy was investigated in this double-blind, randomized, parallel-group comparison with phenytoin (PHT). A total of 193 patients aged 5-18 years with either PS or GTCS were enrolled. After a retrospective baseline assessment, patients were randomized to OXC or PHT in a 1:1 ratio. The double-blind treatment phase comprised two periods: an 8-week flexible titration period; followed by 48 weeks maintenance treatment. In the efficacy analyses, there were no statistically significant differences between OXC and PHT. Forty-nine (61%) patients in the OXC group and 46 (60%) in the PHT group were seizure-free during the maintenance period. In total, 24 patients in the OXC group discontinued treatment prematurely (two for tolerability reasons) compared with 34 in the PHT group (14 for tolerability reasons). The number of premature discontinuations due to adverse experiences was statistically significantly lower in the OXC group than in the PHT group. Moreover, the odds of an individual discontinuing prematurely (regardless of reason) were almost twice as high in the PHT group. This trial provides further support for the efficacy and safety of OXC as first-line treatment in children and adolescents with PS and GTCS. In addition, the results show that OXC in these patients has significant advantages over PHT in terms of tolerability and treatment retention.

Pediatric multiple sclerosis report of 14 cases

We present clinical data from 14 multiple sclerosis (MS) patients who have been admitted to our hospital between January 1980 and May 1992, whose age of onset ranged from 2-15 years. Our patients could be classified as having a clinically definite form of the disease. Initial symptoms varied from minor, such as motor or sensory impairment, bladder dysfunction, to the worst clinical presentation, suggesting diffuse encephalopathy. All the patients had a relapsing-remitting course. We report the paraclinical and laboratory examinations that were done in these patients. Over the period 1980 to 1992 these patients had 39 attacks. CSF analysis was performed in the phase of activity of the disease on 23 occasions and was normal in 12. At least one brain CT scan was performed in 9 patients and showed white matter abnormalities in 6. Cranial magnetic resonance imaging was done in 6 patients and were abnormal in 5. Visual evoked potential (EP) was abnormal in 7 of 8 patients; brainstem acoustic EP was abnormal in 4 of 8 patients and somatosensory EP in 4 of 8. MS is not so rare in childhood and although its diagnosis is essentially a clinical one, paraclinical investigations are of great value in the identification of demyelinating disorders in childhood.

Recurrence after a first unprovoked cryptogenic/idiopathic seizure in children: a prospective study from São Paulo, Brazil.

Summary:  Purpose: To evaluate the recurrence risk after a first unprovoked seizure in a large population of children and adolescents of a developing country.

Impact of benign childhood epilepsy with centrotemporal spikes (BECTS) on school performance

UNLABELLED BECTS represents the vast majority of childhood focal epilepsy. Owing to the age peculiarity of children who suffer from this disease, i.e., school-going age of between 6 and 9 years, the condition is often referred to as a school disorder by parents and teachers. OBJECTIVE The aim of this study was to evaluate the academic performance of children with BECTS, according to the clinical and electroencephalographic ILAE criteria, and compare the results of neuropsychological tests of language and attention to the frequency of epileptic discharges. METHODS The performances of 40 school children with BECTS were evaluated by applying a school performance test (SBT), neuropsychological tests (WISC and Trail-Making), and language tests (Illinois Test Psycholinguistic Abilities-ITPA--and Staggered Spondaic Word-SSW). The same tests were applied in the control group. RESULTS Children with BECTS, when compared to those in the control group, showed lower scores in academic performance (SPT), digits and similarities subtests of WISC, auditory processing subtest of SSW, and ITPA--representational and automatic level. The study showed that epileptic discharges did not influence the results. CONCLUSION Children with BECTS scored significantly lower scores in tests on academic performance, when compared with those in the control group probably due to executive dysfunction.

Prevalence of epilepsy in children from a Brazilian area of high deprivation.

This study assessed the prevalence rate of epilepsy and its causes in children and adolescents in one area of high deprivation in São Paulo, São Paulo, in Southeast Brazil. Between July 2005 and June 2006, 4947 families from a population of 22,013 inhabitants (including 10,405 children and adolescents between the ages of 0 and 16 years) living in the shantytown of Paraisópolis, were interviewed. In the first phase, a validated questionnaire was administered, to identify the occurrence of seizures. In the second phase, clinical history, neurologic examination, electroencephalography, and structural neuroimaging were performed. The diagnosis of epilepsy, including etiology, seizure types, and epileptic syndrome classification, was according to criteria of the International League Against Epilepsy. The screening phase identified 353 presumptive cases. In the second phase, 101 of these cases (33.8%) received the diagnosis of epilepsy. Crude prevalence of epilepsy was 9.7/1000 and prevalence of active epilepsy was 8.7/1000. Partial seizures were the most frequent seizure type (62/101). Symptomatic focal epilepsy was the most common form, and hypoxic-ischemic encephalopathy the most common etiology, reflecting the socioeconomic conditions of this specific population. Adequate public policies regarding perinatal assistance could help reduce the prevalence of epilepsy.

Treatment of febrile seizures with intermittent clobazam

Fifty children, 24 female and 26 male, with ages varying from 6 to 72 months (mean = 23.7 m.) that experienced at least one febrile seizure (FS) entered a prospective study of intermittent therapy with clobazam. Cases with severe neurological abnormalities, progressive neurological disease, afebrile seizures, symptomatic seizures of other nature, or seizures during a central nervous system infection were excluded. Seizures were of the simple type in 25 patients, complex in 20 and unclassified in 5. The mean follow-up period was 7.9 months (range = 1 to 23 m.), and the age at the first seizure varied from 5 to 42 months (mean = 16.8 m.). Clobazam was administered orally during the febrile episode according to the childs weight: up to 5 kg, 5 mg/day; from 5 to 10 kg, 10 mg/day; from 11 to 15 kg, 15 mg/day, and over 15 kg, 20 mg/day. There were 219 febrile episodes, with temperature above 37.8 degrees C, in 40 children during the study period. Twelve children never received clobazam and 28 received the drug at least once. Drug efficacy was measured by comparing FS recurrence in the febrile episodes that were treated with clobazam with those in which only antipyretic measures were taken. Ten children (20%) experienced a FS during the study period. Of the 171 febrile episodes treated with clobazam there were only 3 recurrences (1.7%), while of the 48 episodes treated only with antipyretic measures there were 11 recurrences (22.9%), a difference highly significant (p < 0.0001). Adverse effects occurred in 10/28 patients (35.7%), consisting mainly in vomiting, somnolence and hyperactivity. Only one patient had recurrent vomiting which lead to drug interruption. These effects did not necessarily occurred in every instance the drug was administered, being present in one febrile episode and not in the others. We conclude that clonazepam is safe and efficacious in preventing FS recurrence. It may be an alternative to diazepam in the intermittent treatment of FS recurrence.

Occipital intermittent rhythmic delta activity in absence epilepsy

Occipital intermittent rhythmic delta activity (OIRDA) is considered good prognostic factor in typical absences (TA). We report electroclinical evolution in 14 patients with TA and OIRDA, which performed video-EEG. Seven patients were female; 9 had childhood absence epilepsy and the others did not present electroclinical characteristics for syndromic classification according to ILAEs classification (1989). Pyknolepsy was referred to in 13; TA was the only seizure type in 13; one had generalized tonic-clonic seizures (GTCS) and three had myoclonic jerks during TA. VPA monotherapy controlled seizures in 11, diVPA and ESM, in one each. After seizure control EEG normalized in 10 while in three, spike-wave complexes (SWC) persisted, accompanied by OIRDA in one. Finally in another, seizures were not controlled and SWC and OIRDA persisted. In conclusion, we observed in this series of TA and OIRDA with onset before 10 years, pyknolepsy as common finding and few GTCS. VPA controlled seizures in most cases and EEG normalized in 76.92%. We suggest that OIRDA could be considered good prognostic factor in TA associated with SWC and of epileptiform nature leading to appropriate investigation.

The role of mirror focus in the surgical outcome of patients with indolent temporal lobe tumors

PURPOSE To review the clinical and neurophysiological data of 21 patients with epilepsy due to temporal lobe tumors and who had undergone evaluation and surgery at the Hospital das Clínicas da Universidade de São Paulo. The aim of this study was to investigate whether the occurrence of a mirror focus was influenced either by certain clinical factors or if the surgical outcome was influenced by the presence of a mirror focus. METHOD We included these 21 patients who had undergone at least one interictal electroencephalogram in the pre- and post-surgical periods. They had had a minimum follow-up of one year. RESULTS Eight patients had mirror focus (Group 1) and 13 did not (Group 2). The mean age at seizure onset, duration of epilepsy disorder and total number of seizures did not vary statistically between the two groups of patients. Generalized tonic-clonic seizures occurred more frequently in the mirror focus group. All, but one patient, with a mirror focus were seizure free at follow- up. The mirror focus disappeared in all eight patients in the post-surgical electroencephalogram. In this group, the patient who was not seizure - free had a seizure recorded in his post-surgical electroencephalogram with seizure onset ipsilateral to the resected tumor. The patients who were not seizure-free had either been submitted to an incomplete resection of the tumor or showed evidence of associated cortical dysplasia. CONCLUSION The occurrence of mirror focus is not a contraindication to surgery even when interictal epileptiform activity predominates contralaterally to the tumor and neither when seizures appear to arise from the mirror focus on scalp EEG. Good surgical outcome is expected despite EEG findings that may conflict with tumor location.

Follow-up study of macrocephalic children with enlargement of the subarachnoid space

Eighteen macrocephalic children with enlargement of the subarachnoid space (ESAS), with or without mild ventricular dilatation, were followed prospectively to a mean age of 56 months. All were born at term, with uneventful neonatal period and negative tests for congenital infections. There were 17 boys and 1 girl and the mean follow-up period was 46 months (8-58 months). The initial neurologic evaluation, between ages of 2 to 33 months, disclosed abnormalities in 2 cases. At the follow-up one was still abnormal and the other had a normal neurological examination. Another child, who had a normal neurological examination at the age of 5 months, at the age of 7 years and 7 months had an IQ of 77. Thus the abnormality rate at follow-up was 11%. The OFC returned to the normal range in 45% of the children at the follow-up period. There were no cases of intracranial hypertension. One infant had subdural taps performed at the age of 13 months that disclosed a fluid with the same characteristics as the CSF. All the children had a CT-scan performed at the beginning of the study that revealed a large subarachnoid space; in 77% it was associated with mild ventricular dilatation. Eleven had CT-scans repeated, during the study period, which showed resolution of the process in 3 cases, improvement in 2, and unchanged in 6. We conclude that enlargement of the subarachnoid space in macrocephalic children is often a benign entity. ESAS and macrocephaly will still be present in the majority of children in the long-term follow-up.

Vigabatrin in refractory childhood epilepsy. The Brazilian multicenter study

Children, 47, with various types of severe drug-resistant epilepsy were entered into a prospective, add-on, open trial with vigabatrin. Patients with West syndrome and idiopathic generalized epilepsies were excluded. Seven children had the drug withdrawn, five because of increase in seizure frequency and two because of adverse effects. Drug efficacy, measured according to seizure type, showed a 100% decrease in seizure frequency in 18.6% of partial seizures and 17.3% of the generalized seizures. There was a higher than 50% decrease in 39.5% of partial and 60.8% of generalized seizures, and less than 50% decrease or increase in seizure frequency in 41.8% and 21.8% of partial and generalized seizures, respectively. Vigabatrin mean dosage during phase 3 was 63.6 mg/kg per day (S.D. = 30.5), ranging from 19.3 to 110.5 mg/kg per day. Parametric statistical analysis (Students t-test) of seizure frequency between phases 1 and 3 showed a significant decrease in seizure frequency for partial (P = 0.022), and generalized seizures (P < 0.0001). Drug-related adverse effects were observed in 18/47 cases (38.3%), consisting mainly of irritability, hyperactivity, dizziness, somnolence and gastrointestinal symptoms.