Occipital intermittent rhythmic delta activity in absence epilepsy

Atividade occipital delta rítmica intermitente (AODRI) é considerada fator de bom prognóstico em crises de ausência típica (AT). Neste estudo relatamos a evolução eletroclínica de 14 pacientes com AT e AODRI que realizaram vídeo-EEG. Sete pacientes eram do sexo feminino; nove tinham epilepsia ausência da infância e os outros não apresentavam características eletroclínicas para classificação sindrômica de acordo com a classificação da ILAE (1989). Picnolepsia foi relatada em 13; AT foi o único tipo de crise em 13; um tinha crises generalizadas TCG e três, abalos mioclônicos durante AT. Monoterapia com VPA controlou as crises em 11, diVPA e ESM, em um cada. Após o controle das crises, o EEG normalizou em 10; em três, complexos de espícula-onda (CEO) persistiram, acompanhados por AODRI em um. Finalmente em outro, as crises não foram controladas, persistindo CEO e AODRI. Concluindo, observamos nesta série de AT e AODRI, com início antes dos 10 anos, picnolepsia freqüente e poucas crises TCG. VPA controlou as crises na maioria dos casos e o EEG normalizou em 76,92%. Sugerimos que AODRI possa ser considerada um fator de bom prognóstico em AT associada a CEO e de possível natureza epileptiforme, levando por sua vez, à investigação apropriada.

Syndromic classification of patients with typical absence seizures

The aim of this study is to compare ILAE classification (1989) and Panayiotopoulos' criteria (1997) for absence epilepsies. We studied 455 typical absences (ILAE, 1981) by video-EEG in 43 patients with normal neurological and neuroradiological examinations and interictal EEG with spike-wave complexes higher than 2.5Hz. Syndromic diagnosis was possible in 60.5 percent and 67.4 percent of the patients using ILAE classification and Panayiotopoulos' proposal, respectively. According to ILAE criteria 19 patients had childhood absence epilepsy (CAE), five juvenile absence epilepsy (JAE), one juvenile myoclonic epilepsy (JME) and one epilepsy with specific modes of seizure precipitation. According to Panayiotopoulos' proposal, 10 had CAE, 14 JAE, one JME, three myoclonic absence epilepsy and one eyelid myoclonia with absences. We conclude that Panayiotopoulos' criteria and ILAE classification for absence epilepsies, which did not allow for the classification of 32.6 percent and 39.5 percent of cases, respectively, were still insufficient to classify all patients under specific diagnosis

Benign focal epilepsy of childhood with centrotemporal spikes (BECTS): clinical characteristics of seizures according to age at first seizure

BECTS is characterized by the presence of simple partial motor seizures in the face and/or oropharynx, with or without sensory symptoms and often with secondary generalization. These seizures tend to occur more often during sleep or drowsiness. According to some authors, generalized seizures prevail over other types particularly among children aged five or less. The purpose of this study is to determine the characteristics of the first epileptic episode among children with BECTS, grouped by age as of their first epileptic seizure, as well as to analyze how such seizures change over the course of clinical evolution. A total of 61 children were examined, 16 of whom below the age of 5 and 45 above. With regard to the first and recurrent epileptic episodes, our final assessment showed that partial seizures occurred more frequently than generalized tonic-clonic seizures in both groups. Although no conclusive relation could be established between the type of partial seizure (i.e. simple versus complex) and the children's age as of their first epileptic episode, it was possible to correlate the type of epileptic seizure with their clinical evolution, in which case simple partial seizures proved to be more frequent than complex partial seizures. It should be noted that the number of children under the age of five was relatively small, which evinces the need for further studies. It should also be borne in mind that the reported frequency of generalized seizures in these children's first epileptic episode may be due to their parents' lack of attention and familiarity with this pathology and their attendant difficulty in characterizing its clinical symptoms

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 = 75 per cent in 4, and < 50 per cent in 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 per cent of patients, long term safety was confirmed and QL improve on TPM.

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, sympromatic 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 Celsius, 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 percent) experienced a FS during the study period. Of the 171 febrile episodes treated with clobazam there were only 3 recurrences (1.7 percent), while of the 48 episodes treated only with antipyretic measures there were 11 recurrences (22.9 percent), a difference highly significant (p<0.0001). Adverse effects occurred in 10/28 patients (35.7 percent), consisting maily in vomiting, somnolene and hyperactivity. Only one patient had recurrent vomiting which lead to drug interruption. These effects did not necessarily ocurred 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 deazepam in the intermittent treatment of FS recurrence.

Espícula-onda contínua durante o sono. Aspectos clínicos e eletrencefalográficos / Continuous spike-wave activity during sleep and eletroencephalographical aspects

Dezessete crianças com espícula-onda contínua durante o sono foram estudadas retrospectivamente. Cinco apresentavam distúrbio da fala após desenvolvimentos normal da linguagem e crise epilépticas (síndrome de Landau e Kleffner - grupo 1). Doze crianças tinham atraso do desenvolvimento neuropsicomotor e/ou deficiência mental (grupo 2). Crises epilépticas estavam presentes em 11 pacientes deste grupo, tetraparesia em 5, hemiparesia em 2, microcefalia em 2, distúrbios de comportamento em 4 casos. O eletrencefalograma mostrou em todos os casos espícula-onda contínua durante o sono. Pacientes do grupo 1 apresentavam atividade epileptiforme difusa com acentuaçäo das descargas nas regiöes temporais em 4 de 5 casos; e os do grupo 2, descargas difusas, incluindo atividade multifocal (5/12), por vezes com predomínio anterior (7/12). Concluímos que espícula-onda contínua durante o sono é um padräo eletrográfico inespecífico de certos tipos de epilepsia na infância com diferentes manifestaçöes clínicas, que mostra no entanto certa diferenciaçäo topográfica, de acordo com os prováveis sítios lesionais.