Childhood epilepsy due to neurocysticercosis: a comparative study.

PURPOSE: To assess the clinical profile of pediatric patients with epilepsy and neurocysticercosis (NC), and compare them with a group of pediatric patients with benign partial epilepsy to determine clinical differences, response to treatment, and prognosis. METHODS: We studied 28 patients (16 girls) with probable or definitive diagnosis of NC and epilepsy and 32 patients (16 girls) with partial benign epilepsy (BE). All patients had normal neurologic examination. We compared NC and BE patients looking for differences in demographics (age at first seizure, gender, family history); clinical presentation (type, frequency, duration, and total number of seizures, duration of epilepsy, status epilepticus, cluster, and postictal deficit); treatment [duration, number of antiepileptic drugs (AEDs), maximal dose, drug association, number of seizure-free patients, time to obtain control and recurrence after medication discontinuation]; complementary examinations (the first and the last EEG). RESULTS: The mean follow-up was 5.4 years for the 28 NC patients and 4.6 years for the 32 BE patients (p=0.98). We did not find statistical differences between NC and BE in gender, family history, types of seizures, frequency and length of seizures, previous status epilepticus, seizure clustering, and presence of postictal deficits. However, we found that NC compared with BE patients had significant longer AED treatment, more seizures after AED introduction, tried more AEDs and at maximal dose, and in 20%, required polytherapy. The recurrence rate in NC was 54.4% and this was not significantly associated with number of lesions and disease activity seen on CT scans or the presence of EEG abnormalities. CONCLUSIONS: NC presents with a mild form of epilepsy in terms of seizure severity; however, it is more challenging in regard to drug management and has a less favorable long-term prognosis in terms of seizure remission. The number of lesions or disease activity seen on computed tomography (CT) as well as EEG abnormalities have no prognostic value in childhood epilepsy due to NC.

Predisposition to metabolic acidosis induced by topiramate.

RATIONALE: Metabolic acidosis induced by topiramate is a well documented but infrequent adverse event. The objective was to demonstrate the lowering of carbon dioxide serum levels, which is usually asymptomatic but may facilitate the occurrence of metabolic acidosis in patients using topiramate. METHODS: We evaluated, prospectively, the carbon dioxide serum levels of 18 patients seen at the epilepsy clinic of our university hospital, before and 3 months after introducing topiramate. RESULTS: Five patients were female and 13 were male, age ranging from 2 to 16 years old (mean=9. 3). Carbon dioxide mean serum levels were 25 and 21.2 mmol/L (normal = 22 to 30), before and 3 months after introducing topiramate, respectively. Dose ranged from 2.08 to 11.76 mg/kg/day (mean=6. 7mg/kg/day). Adverse events were anorexia, nausea and somnolence. CONCLUSION: We conclude that the lowering of carbon dioxide serum levels induced by topiramate is mostly asymptomatic, but may facilitate the occurrence of metabolic acidosis. Since patients in use of topiramate have refractory epilepsy, they may need epilepsy surgery, and must be carefully monitored for the risk of metabolic acidosis during surgery.

[Familial partial epilepsies]. / Epilepsias parciais familiares.

OBJECTIVE: To investigate the clinical and genetic characteristics of familial partial epilepsies. METHOD: Family history of seizures was questioned in all patients followed in our epilepsy clinics, from October 1997 to December 1998. Those with positive family history were further investigated and detailed pedigrees were obtained. All possibly affected individuals available underwent clinical evaluation. Seizures and epilepsy syndromes were classified according to the ILAE recommendations. Whenever possible, EEG and MRI were performed. RESULTS: Positive family history was identified in 32 unrelated patients. A total of 213 possibly affected individuals were identified, 161 of whom have been evaluated. The number of affected subjects per family ranged from two to 23. Temporal lobe epilepsy (TLE) was identified in 22 families (68%), frontal lobe epilepsy in one family (3%), partial epilepsy with centrotemporal spikes in five families (15%), and other benign partial epilepsies of childhood in four families (12%). Most of the affected individuals in the TLE families (69%) had clinical and/or EEG characteristics of typical TLE. However, the severity of epilepsy was variable, with 76% of patients with spontaneous seizure remission or good control with medication and 24% with refractory seizures, including 7 patients that underwent surgical treatment. In the other 10 families, we identified 39 possibly affected subjects, 23 of whom were evaluated. All had good seizure control (with or without medication) except for one patient with frontal lobe epilepsy. Pedigree analysis suggested autosomal dominant inheritance with incomplete penetrance in all families. CONCLUSION: Family history of seizures is frequent among patients with partial epilepsies. The majority of our families had TLE and its expression was not different from that observed in sporadic cases. The identification of genes involved in partial epilepsies may be usefull in classification of syndromes, to stablish prognosis and optimal treatment.

De novo psychogenic seizures after epilepsy surgery: case report.

The occurrence of de novo psychogenic seizures after epilepsy surgery is rare, and is estimated in 1.8% to 3.6%. Seizures after epilepsy surgery should be carefully evaluated, and de novo psychogenic seizures should be considered especially when there is a change in the ictal semiology. We report a patient with de novo psychogenic seizures after anterior temporal lobe removal for refractory temporal lobe epilepsy. Once psychogenic seizures were diagnosed and psychiatric treatment was started, seizures stopped.

Dyskinesia induced by phenytoin.

UNLABELLED: Phenytoin is an effective antiepileptic drug, although, it can be associated with many side effects, including dyskinesia. OBJECTIVE: To describe the clinical characteristics of phenytoin induced dyskinesia. METHODS: We investigated the occurrence of involuntary movements in patients followed at our adult and pediatric epilepsy clinics during the period of one year. RESULTS: Three patients presented with phenytoin-induced dyskinesia: one adult with axial and orofacial dyskinesia, and two children with choreoathetosis. They did not have other signs of phenytoin intoxication and had complete recovery after phenytoin withdrawal. CONCLUSION: Phenytoin induced dyskinesia may occur during either chronic or initial treatment and with normal serum phenytoin levels. However, it occurs most often in patients on polytherapy, usually after increasing dosage and with toxic serum levels. Other signs of phenytoin intoxication may be present in these patients, but often the dyskinesia is the only side effect, which may delay the diagnosis and treatment. The clinical characteristics of the involuntary movements vary and may be focal or generalized, most often characterized by choreoathetosis and dyskinesias. These may last for hours, days or even years, but frequently disappear completely after phenytoin withdrawal.

[First epileptic crisis. Analysis of risk factors for recurrence]. / Crise epiléptica única. Análise dos fatores de risco para recorrência.

We studied children who presented with a first seizure and came to the outpatient clinic for childhood from September/1989 to July/1996. The following risk factors were assessed: age, sex, type and etiology of the seizure, sleep state at time of seizure, family history of seizures, electroencephalogram (EEG) and computerized tomography (CT). Our purpose was to assess the risk factors in univariable and multivariable analysis. We found two groups of patients according to the etiology: (a) idiopathic cases (IC) and (b) symptomatic cases (SC). This last group had only two patients and was excluded from the analysis. We studied only the IC group with 86 patients. Seizure recurrence was observed 29 children (33%), during 2.4 years. Only patients with abnormal EEG and family history for unprovoked seizures had a higher risk of recurrence (p < 0.003). After the first unprovoked seizure estimate of recurrence by survival curves was 18% at first semester, 27% at first year, 35% at second year and 38% at third year.

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.

[Cerebrovascular disease in children: I. Epileptic manifestations]. / Doença cerebrovascular na infância. I. Manifestações epilépticas.

Seizures may occur as a complication of cerebrovascular disease (CVD) and its prevalence, clinical presentation, risk factors and evolution have been reported by few authors. We evaluated 39 children with CVD and analyzed the association with seizures. Seizures occurred in 24 (61.5%) patients and were classified as partial (29.2%), generalized (54.2%) and secondarily generalized (16.6%). Infants had a significantly higher prevalence of seizures (p = 0.0362) than children at other ages. Cortical localization was associated with a significantly higher prevalence of seizures (p = 0.0101). There were no differences between ischemic and hemorrhagic strokes. Fourteen patients had no seizures after the acute phase of the CVD, the 2 previously epileptic patients had their seizures controlled with antiepileptic drugs, 3 developed epilepsy, 2 died during the acute phase and in 3 patients there was not enough time yet to make a clear diagnosis of epilepsy.

[Cerebrovascular disease in children: II. Clinical aspects in 42 cases]. / Doença cerebrovascular na infância. II. Aspectos clínicos em 42 casos.

We report the findings recorded in 42 children suffering cerebrovascular disease and assisted at the Hospital das Clínicas FCM-UNICAMP, over a 8 years period (January 1990 until April 1998). The ischemic type was the most common, and involvement of the middle cerebral artery, sudden onset of clinical manifestation with seizures and motor disability were more common in early aged children. Motor sequelae predominated in the follow-up of these children.

[Feelings and behaviors of parents of children with epilepsy]. / Sentimentos e reações de pais de crianças epilépticas.

PURPOSE: To assess the efficacy of support groups in identifying parents feelings and behaviors facing the diagnosis of epilepsy in their children. METHODS: Protocols were applied to 18 parents before and after the sessions. Each protocol consisted of questions concerning feelings and beliefs toward epilepsy as well as children-parent interactions. RESULTS: The following feelings were observed: disappointment (94.4%), fear (72.2%), frightening (27.8%), sadness (33.3%), anxiety (27.8%) and rejection (38.9%). These feelings were associated with overprotection (83.3%) and a lack of limits (38.9%). Parents reported feeling of safety after seizure control and 77.8% associate major of difficulties to the lack of information and the inadequate beliefs involved. After support sessions, 94.4% of the parents reported less anxiety. CONCLUSION: Support groups dispel misconception, clarify child parent relationships and prevent behavioral difficulties.

[Brain abscess in childhren: A ten cases report] / Abscesso cerebral na infância: relato de 10 casos.

OBJECTIVES: Considering that the brain abscess is rare in infants, with a high mortality rate, the objective of this paper is to report the clinical evolution of ten children with the diagnosis of brain abscess in the Pediatric Nursery of the Hospital das Clínicas of the Medical School of the Campinas State University (UNICAMP).METHODS: The data of the patients with diagnosis of brain abscess recorded between January 1986 and July 1995 were reviewed. The following data were analyzed: age, sex, clinical manifestations, physical examination, radiological data, etiological agent, treatment, complications and clinical evolution of the patients.RESULTS: The age of the patients varied from 2 to 13 years (median 3 years); 6 of them were female. The neurological manifestations predominated, and 2 patients had history of prior otorhinolaryngological infection (chronic otitis media and sinusitis). Two patients had congenital cyanogenic cardiopathy (Fallot tetralogy and Pulmonary Stenosis with Interventricular Communication). The diagnosis and follow-up were made with computed tomography of the brain. In six cases there were one sole abscess located more frequently in the frontal lobe. The treatment in majority of the cases was broad-spectrum antibiotic association and surgical drainage. Five patients had neurological sequelae (seizure, hydrocephalus and paresis); one death occurred.CONCLUSIONS: Although rare, the brain abscess has to be remembered in patients that have neurological alterations associated to risk factors, as otorhinolaryngological infections and congenital cyanotic cardiopathy, being mandatory the realization of computed tomography of the brain to confirm the diagnosis.

Brain single photon emission computed tomography imaging in Landau-Kleffner syndrome.

Five right-handed children with Landau-Kleffner syndrome (LKS) who had disease onset between the ages of 3 and 9 years were studied with EEG and single-photon emission computed tomography (SPECT) before and, in four cases, after 6 months of corticosteroid treatment. EEG findings included both focal and generalized spikes as well as spike-wave discharges with bilateral temporal predominance. These increased markedly during sleep in 1 child, and continuous spike-and-wave complexes appeared during slow-wave sleep in another patient. Neuropsychological testing demonstrated verbal auditory agnosia. Magnetic resonance imaging (MRI) was performed in 4 children and was normal. Brain SPECT imaging demonstrated abnormal perfusion in the left temporal lobe in all patients. The response to corticosteroid therapy was mixed. Our findings reinforce the concept that LKS is a functional disease affecting the language-dominant brain areas. We conclude that SPECT imaging may be of diagnostic assistance in the evaluation of this syndrome of unknown etiology.

[Basal ganglia calcifications in children]. / Calcificações dos gânglios da base na infância.

We report the study of four children with bilateral basal ganglia calcifications (BGC) visualized on CT scan. Epilepsy was the clinical manifestation of three patients whose laboratory investigation revealed abnormal calcium metabolism. The first aim of this paper is to call attention to a treatable entity that can cause epileptic syndromes in infancy and childhood. The second purpose is to review the literature comparing with our fourth child who presented encephalopathy with BGC.

[Intermittent prophylaxis in febrile convulsions with oral diazepam]. / Profilaxia intermitente na convulsão febril com diazepam via oral.

Intermittent prophylaxis with oral diazepam is presented as an optional treatment for febrile seizures. This proposition is justified by the severe side effects of the currently used chronic anticonvulsant drug therapy in febrile seizures (phenobarbital and valproate). Nineteen patients aged between 3 months and 5 years were treated. They had either simple or complex febrile seizures. Sixteen patients had at least one prognostic factor for recurrence of febrile seizures: first febrile seizure before 15 months of age, positive family history for epilepsy or febrile seizures, occurrence of a complex febrile seizure or abnormal neurological examination. Three patients had none (cases 8, 12 and 13). We recommended 2.5mg b.i.d. for children younger than 12 months, 5mg b.i.d. for children older than 12 months and younger than 3 years, and 7.5 b.i.d. for children older than 3 years. The results showed that only one patient had febrile convulsions while taking adequate diazepam dosage. Transient side effects occurred in 36.8% of the cases.