Letícia Pereira de Brito Sampaio

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.

Neurobiochemical mechanisms of a ketogenic diet in refractory epilepsy

A ketogenic diet is an important therapy used in the control of drug-refractory seizures. Many studies have shown that children and adolescents following ketogenic diets exhibit an over 50% reduction in seizure frequency, which is considered to be clinically relevant. These benefits are based on a diet containing high fat (approximately 90% fat) for 24 months. This dietary model was proposed in the 1920s and has produced variable clinical responses. Previous studies have shown that the mechanisms underlying seizure control involve ketone bodies, which are produced by fatty acid oxidation. Although the pathways involved in the ketogenic diet are not entirely clear, the main effects of the production of ketone bodies appear to be neurotransmitter modulation and antioxidant effects on the brain. This review highlights the impacts of the ketogenic diet on the modulation of neurotransmitters, levels of biogenic monoamines and protective antioxidant mechanisms of neurons. In addition, future perspectives are proposed.

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.

Ketogenic diet for epilepsy treatment

The ketogenic diet (KD), a high-fat, low-carbohydrate, and adequate-protein diet is an established, effective nonpharmacologic treatment option for intractable childhood epilepsy. The KD was developed in 1921 and even though it has been increasingly used worldwide in the past decade, many neurologists are not familiar with this therapeutic approach. In the past few years, alternative and more flexible KD variants have been developed to make the treatment easier and more palatable while reducing side effects and making it available to larger group of refractory epilepsy patients. This review summarizes the history of the KD and the principles and efficacy of the classic ketogenic diet, medium-chain triglyceride(s) (MCT) ketogenic diet, modified Atkins diet, and low glycemic index treatment.

Ketogenic diet in epileptic children: impact on lipoproteins and oxidative stress

Objectives: Ketogenic diet (KD) is an important therapy used in the control of drug-refractory seizures. The major goal of this review is to update the knowledge about the adverse effects of KD on lipoproteins, lipid profile, and cardiometabolic risk. Methods: Articles on the effect of the KD on plasma lipoproteins of children and adolescents with refractory epilepsy, which were published in the past 15 years and indexed in the PubMed and MedLine databases, were included. Results: Dyslipidemia was recurrent in children, and adolescents treated with KD. Evidence suggests that hypercholesterolemia promotes structural modifications in low-density lipoprotein particles. Such modifications possibly favor oxidative processes and contribute to changes in the size of lipoproteins, particularly related to small and denser LDL. However, oxidative modifications in LDL of children on KD are not described in the literature. Discussion: The positive effects of KD on the health of children and adolescents with refractory epilepsy are unquestionable. Conversely, this positive role is associated with significant and negative changes in lipid metabolism. Moreover, the positive effects are possibly related to oxidative reactions and unbalance of antioxidants that can contribute to an increased cardiometabolic risk. Therefore, this review invites clinicians and researchers to investigate the lipid and oxidative metabolism in their clinical practice and trials, respectively.

Cefepime-induced encephalopathy in patient without renal failure.

This case report describes neurotoxicity in an older patient on cefepime 2 g twice a day. The 81-year-old male patient developed non-convulsive status epilepticus during cefepime treatment with 1 g twice a day. There was recovery 30 days after discontinuation of cefepime.

T21. Status epilepticus cessation during pyridoxine infusion in an infant with delayed diagnosis of ALDH7A1 mutation

Introduction Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive disorder leading to neonatal intractable seizures and epileptic encephalopathy. Clinical seizures usually begin in the first hours of life, with poor response to anticonvulsants, evolving to refractory status epilepticus. Diagnostic assessment includes cerebrospinal fluid analysis, gene testing and clinical response to parenteral pyridoxine. Early recognition and treatment is highly desired to avoid unfavorable neurodevelopmental outcome. Electroencephalographic monitoring commonly reveals pretreatment multifocal epileptiform activity and burst suppression pattern; after pyridoxine injection, the incidence of sharp waves decreases and periods of suppression can occur. Methods Case report of a 11-month-old girl with first seizure with 4 h after birth and delayed genetic confirmation of pyridoxine-dependent epilepsy due to the gene ALDH7A1. EEG monitoring was performed during pyridoxine infusion. Results A 11 month-old girl, with unremarkable gestational and delivery history, started seizures 4 h after birth. Initially treated with phenobarbital, she evolved to convulsive status epilepticus (CSE), leading to orotracheal intubation and continuous sedation for 5 days. At 3 months, she had a new CSE, being hospitalized for 4 months and started on sodium valproate, vigabatrin, clobazam and levetiracetam. Epileptic seizures remained weekly. Phenobarbital and cannabidiol were added, with no further benefit. At 9 months, seizures became daily and she had a new CSE. In the ICU, it was started midazolam, ketamine, topiramate and ketogenic diet. After 1 month, she was discharged and referred to a tertiary hospital. An exome sequencing revealed 2 copies in homozygosis in the gene ALDH7A1, variant Chr5:125.894.936 C > T, previously associated with PDE. While being admitted for pyridoxin treatment, she started a new CSE. EEG showed diffuse spikes, polyspikes and sharp waves at intervals of 1–2 s. Immediately after intravenous injection of pyridoxine 200 mg, epileptiform activity became progressively less frequent, alternating with periods of attenuattion lasting from 2 to 8 s, with no longer SE after 3 min. After 3 days, EEG showed moderate diffuse disorganization, with no epileptiform activity. Discharge was after 10 days. Outpatient EEG monitoring showed left centrotemporal sharp waves and mild disorganization of background activity. Conclusion This case illustrates a clinical scenario of seizures starting in the neonatal period, with recurrent convulsive status epilepticus and little response to anticonvulsants and ketogenic diet. The delayed diagnosis can be explained by rarity of the condition and limited availability of gene testing in public health system in medium and low-income countries. It remarks the importance of empirical pyridoxine treatment in neonates with early beginning of refractory seizures and status epilepticus (SE). EEG evolution and SE cessation documents pyridoxine responsiveness.