Hemispheric lateralization in benign focal epilepsy in childhood with centrotemporal spikes (BECTS).

Benign focal epilepsy in childhood with centrotemporal spikes (BECTS) is one of the most common forms of epilepsy. In adults there is a higher percentage of lateralized epileptic discharges in the left cerebral hemisphere; however, in children this pattern does not seem to have the same distribution. The objective of this study was to evaluate the lateralization of interictal spikes in children with BECTS in relation to the sex of the child and the age of onset of epilepsy. We studied the electroencephalograms (EEGs) of 114 children with a clinical diagnosis of BECTS according to ILAE. The results obtained from two EEGs, performed at intervals of 6 and 12 months, were correlated with the age of onset of the epileptic seizures and the sex of the child. There was no association between the onset of epileptic seizures and the age of the child (p=0.461). When we analyzed the relationship between laterality and sex we did not observe any difference in the first EEG (p = 0.767) results; however, in the results of the second EEG there was a difference (p = 0.002). In males, left and bilateral interictal spikes were predominant, and in females the right hemisphere showed predominant spikes and there were continuous spike-and-wave discharges during slow sleep (CSWSS). The analysis between laterality and a child's age did not show predominant interictal spikes in the hemispheres, except in males where there were predominant multifocal and generalized spikes, but not lateralization (p=0.011). The conclusion was that in BECTS the lateralization of interictal spikes was not consistent as described in adult patients, but there was a slight left hemispheric predominance in boys and right hemispheric predominance in girls.

A pilot study of topiramate in children with Lennox-Gastaut syndrome.

We conducted an open, add-on study with topiramate (TPM) as adjunctive therapy in Lennox-Gastaut syndrome (LGS), to assess the long-term efficacy and safety and to evaluate quality of life (QL) measurements in the chronic use of TPM. We studied 19 patients (11 male; age ranging from 4 to 14 years) with uncontrolled seizures receiving 2-3 anti-epileptic drugs. Patients were followed up to 36 months of treatment. A questionnaire was used to query parents about QL. Seven patients completed the study at 36 months and seizure frequency was reduced > or = 75% in 4, and < 50% in 3 patients. Two children became seizure free for more than 24 months. Most side effects were CNS related, with the most frequent being somnolence and anorexia. These were generally transient. One patient dropped-out due to powder in the urine. None of the patients required hospitalization. At 36 months, patients' alertness (2/7), interaction with environment (5/7), ability to perform daily activities (5/7), and verbal performance (6/7) improved on TPM. We conclude that TPM may be useful as adjunctive therapy in the treatment of LGS. The efficacy of TPM was maintained in long-term treatment in more than 40% of patients, long term safety was confirmed and QL improved on TPM.

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.

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 child's 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.

[Continuous spike-wave activity during sleep. Electroencephalographic and clinical features]. / Espícula-onda contínua durante o sono. Aspectos clínicos e eletrencefalográficos.

Seventeen children were retrospectively evaluated. They exhibited continuous spike-wave activity during slow wave sleep (CSWS). Five of these had only speech problems and seizures (Landau-Kleffner syndrome) (group 1). The other cases had developmental milestones acquisition delay and/or mental retardation (group 2). Epileptic seizures were present in 11 of these, tetraparesis was observed in 5, hemiparesis in 2, microcephaly in 2 and behavior disturbances in 4 cases. The electroencephalogram showed in all cases diffuse CSWS. Group 1 showed diffuse activity, at times accentuated in the centrotemporal region (4/5). Group 2 had widespread discharges, including multifocal activity (5/12), sometimes with anterior predominance (7/12). We concluded that CSWS is a non specific electrographic pattern observed in some types of epilepsy in childhood that have different clinical presentation. It has however some topographic differentiation, depending upon the lesional sites.

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 (Student's 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.

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.

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.

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

Trata-se de estudo prospectivo de 18 crianças com macrocefalia por aumento do espaço sub-aracnóideo, com ou sem dilataçäo ventricular, seguidas até idade em média de 56 meses. Todas nasceram a termo, sem intercorrências perinatais e com testes negativos para TORCH. O tempo médio de seguimento foi 46 meses. Havia 17 meninos e apenas uma menina no grupo estudado. A porcentagem de anormalidades neurológicas no seguimento foi de 11%. Durante o seguimento, o perímetro cefálico retornou aos níveis da normalidade em 45% das crianças. Nenhum caso desenvolveu hipertensäo intracraniana durante o estudo. Todas as crianças realizaram TAC de crânio como parte da avaliaçäo inicial e, além do aumento do espaço sub-encefálico, 77% delas apresentavam discreta dilataçäo ventricular. No seguimento, 11 realizaram TAC de controle que revelaram resoluçäo completa do processo em 3 casos, melhora em 2 e permaneceram inalteradas em 6. Concluimos que o aumento do espaço sub-aracnóideo em crianças macrocefálicas é entidade que apresenta bom prognóstico neurológico na maioria dos casos e que a macrocefalia e o aumento do sub-aracnódeo continuaräo presentes, na maioria das crianças, no seguimento a longo prazo

Neurocisticercose na infancia: aspectos clinicos e do diagnostico. / Neurocysticercosis in childhood: clinical aspects and diagnosis

Foram analisados o quadro clinico e o exame neurologico relativos a 100 observacoes de criancas de zero a 13 anos, portadoras de neurocisticercose internadas na Clinica Neuropediatrica do Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo no periodo de 1945 a 1980. As principais sindromes clinicas observadas, por ordem de frequencia foram: hipertensao intra-craniana, convulsiva,disturbios psiquicos, meningoencefalitica, meningitica, de cefaleia e de nervos cranianos. A associacao da sindrome de hipertensao intra-craniana e convulsiva foi o achado mais frequente.Ressalta-se a frequencia da sindrome H-H. O exame neurologico mostrou maior incidencia de comprometimento do nervo otico