Epileptic features of patients with unilateral and bilateral schizencephaly.

The extent of cortical maldevelopment might correlate with the severity of the clinical manifestation, such as cognitive delay or motor dysfunction. The objective of this study was to investigate the clinical features of epilepsy in patients with unilateral and bilateral schizencephaly. We studied 44 consecutive patients with schizencephaly diagnosed by magnetic resonance imaging (MRI). The epileptic features were analyzed in detail: frequency of epilepsy, median age at onset of epilepsy, semiology of seizures, characteristic features of electroencephalographic (EEG) abnormalities, epileptic syndromes, and antiepileptic drug treatment. We also verified the presence of motor disabilities. Data were analyzed according to the presence of unilateral or bilateral clefts and to the presence of open-lip versus closed-lip schizencephaly. We used the chi-square test and Fisher exact test for statistical analysis. Twenty-four patients had a unilateral cleft (group 1) and 20 patients had bilateral clefts (group 2). Ages ranged from 1 to 37 years (mean 10.6 years). Epilepsy was present in 15 (63%) patients in group 1 and in 11 (55%) patients of group 2; a history of status epilepticus occurred in 13% of group 1 and in 27% in group 2; and a history of clusters of seizures occurred in 40% of group 1 and in 45% of group 2. Eight (53%) patients in group 1 and 6 (54%) patients in group 2 were in monotherapy. Ten (67%) patients in group 1 and 7 (64%) patients in group 2 had seizures controlled with antiepileptic drugs. The frequency of EEG abnormalities was similar between groups (75% and 85%, groups 1 and 2, respectively). Statistical analysis showed no difference between the two groups in the variables mentioned above. However, motor disability was significantly more frequent and more severe in group 2. Regarding the type of schizencephaly (open lip versus closed lip), there was no difference in the frequency of patients with epilepsy, and severe motor deficit was more frequently found in bilateral and open-lip schizencephaly. The extent of the cortical maldevelopment in patients with schizencephaly does not correlate with the severity of the clinical and EEG features of epilepsy, unlike the cognitive and motor manifestations. In addition, the type of schizencephaly (open lip versus closed lip) does not correlate with the presence of epilepsy or seizure control, unlike motor deficit.

Intrapartum complications associated with malformations of cortical development.

Birth asphyxia can be an early sign of cerebral palsy, the effect of an antenatal anomaly rather than its cause. The objective of this study was to evaluate the occurrence of intrapartum complications in patients with malformation of cortical development. The data were retrospectively assessed and compared with a control group of children with idiopathic epilepsy. Intrapartum complications were reported in 22 (31.5%) patients, as opposed to only 2 in the control group (p < 0.001). Patients with cortical malformations frequently present intrapartum complications, which could lead to the misdiagnosis of hypoxic-ischemic encephalopathy.

Gangliocytoma, a surgically treatable cause of drug resistant early onset partial epilepsy

Paciente de 20 anos de idade apresentava crises parciais simples sensitivo-motoras com início na perna direita desde os 4 anos. As crises ocorriam pelo menos uma vez por semana e eram refratárias a drogas antiepilépticas. Crises secundariamente generalizadas eram infrequentes. EEGs interictais mostravam espículas centro-parietais máximas na linha média. EEGs letais registraram atividade teta rítmica nas regiões parasagitais, sem lateralização. Ressonãncia magnética mostrou uma lesão na porção medial do lobo parietal esquerdo, isointensa ao córtex em imagens ponderadas em T1 e hiperintensa em imagens T2. Foi realizada a ressecção cirúrgica completa da lesão, guiada por eletrocorticografia. Histopatologia demonstrou um gangliocitoma, uma causa pouco frequente, cirurgicamente tratável, de epilepsia parcial resistente ao tratamento medicamentoso

Recurrent abdominal pain: when should an epileptic seizure be suspected?

Recurrent episodes of abdominal pain are common in childhood. Among the diagnostic possibilities are migraine and abdominal epilepsy (AE). AE is an infrequent syndrome with paroxystic episodes of abdominal pain, awareness disturbance, EEG abnormalities and positive results with the introduction of antiepileptic drugs. We present one 6 year-old girl who had short episodes of abdominal pain since the age of 4. The pain was followed by cry, fear and occasionally secondary generalization. MRI showed tumor in the left temporal region. As a differential diagnosis, we report a 10 year-old boy who had long episodes of abdominal pain accompanied by blurring of vision, vertigo, gait ataxia, dysarthria, acroparesthesias and vomiting. He received the diagnosis of basilar migraine. In our opinion, AE is part of a large group (partial epilepsies) and does not require a special classification. Pediatric neurologists must be aware of these two entities that may cause abdominal pain.

Recurrent abdominal pain: when an epileptic seizure should be suspected?

Recurrent episodes of abdominal pain are common in childhood. Among the diagnostic possibilities are migraine and abdominal epilepsy (AE). AE is an infrequent syndrome with paroxystic episodes of abdominal pain, awareness disturbance, EEG abnormalities and positive results with the introduction of antiepileptic drugs. We present one 6 year-old girl who had short episodes of abdominal pain since the age of 4. The pain was followed by cry, fear and occasionally secondary generalization. MRI showed tumor in the left temporal region. As a differential diagnosis, we report a 10 year-old boy who had long episodes of abdominal pain accompanied by blurring of vision, vertigo, gait ataxia, dysarthria, acroparesthesias and vomiting. He received the diagnosis of basilar migraine. In our opinion, AE is part of a large group (partial epilepsies) and does not require a special classification. Pediatric neurologists must be aware of these two entities that may cause abdominal pain