Pediatric peri-insular hemispherotomy and functional hemispherectomy for severe medically refractory epilepsy: comparison of two techniques.

Purpose: Since it was first described in the 1970s, functional hemispherotomy has been an essential tool in treating disabling, medically refractory epilepsy resulting from diffuse unilateral hemispheric disease. We report our experience with 23 patients who underwent hemispherotomy, both using the functional hemispherotomy (FH) as well as a modified peri-insular hemispherotomy (PIH) technique. We present the surgical technique for the latter, review outcomes following disconnection surgery and discuss the differences between the techniques when it comes to complications and postoperative results.

Vagus nerve stimulation in pediatric patients with drug-resistant epilepsy: a step-by-step video.

Vagus nerve stimulation (VNS) is a neuromodulatory treatment involving chronic intermittent electrical stimulation of the left vagus nerve, administered through a programmable pulse generator implanted subcutaneously in the chest. This generator connects to a bipolar lead, with electrodes wrapped around the vagus nerve in the neck. Primarily used as an adjunct therapy for patients with refractory epilepsy who cannot undergo or have not benefitted from resective surgery, VNS is generally well tolerated with few severe side effects.

Surgical Anatomy and Technique of Peri-Insular Hemispherotomy in Pediatric Epilepsy.

Background And Objectives: Hemispherotomy is a highly complex procedure that demands a steep learning curve. An incomplete brain disconnection often results in failure of seizure control. The purpose of this article was to present a step-by-step guide to the surgical anatomy of this procedure.

[Benefit of surgery in a case of negative motor focal epileptic seizures secondary to parietal cortical dysplasia].

Focal atonic seizures are recognized rarely as ictal phenomena, they can correspond to both generalized epilepsy and focal epilepsy. The areas of the brain involved in the management of this type of seizure are: the negative motor area and the primary motor and primary somatosensory cortices, although the neurophysiology that generates them is still unclear. We present the case of a patient with focal atonic seizures in the left upper limb, refractory to drug treatment.

Rasmussen syndrome: an atypical presentation in ten patients.

The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017. Six patients were boys and four were girls.

[Vagus nerve stimulation: treatment of 158 pediatric patients with a long-term follow-up].

Aim: To describe a series of patients with drug resistant epilepsy treated with vagus nerve stimulation in a national pediatric hospital, evaluating efficacy, safety and tolerability.

Patients And Methods: A retrospective analysis of 158 pediatric patients with epilepsy resistant to pharmacological and non pharmacological treatment including surgery that were treated with vagus nerve stimulation between 2001-2015. Patients with progressive encephalopathies, and congenital heart disease were excluded.

Type II focal cortical dysplasia: electroclinical study and surgical outcome in 31 pediatric patients.

Objective: The aim of this study was to analyze the electroclinical features and surgical outcome of 31 pediatric patients with focal cortical dysplasia (FCD) type II.

Material And Methods: We conducted a retrospective, descriptive study of 31 patients with FCD type II followed between 1998 and 2011. We included patients with FCD type II confirmed by histopathological examination with abnormal magnetic resonance imaging and at least 1 year of follow-up.

Rasmussen syndrome: an Argentinean experience in 32 patients.

Purpose: The aim of this study is to analyze the electroclinical features, treatment, and evolution of patients with Rasmussen syndrome (RS).

Materials And Methods: We conducted a retrospective, descriptive study in 32 consecutive patients with RS followed between 1990 and 2012.

Results: Twenty boys and 12 girls were included in the study.

Vagus nerve stimulation: effectiveness and tolerability in 64 paediatric patients with refractory epilepsies.

Aim: We discuss the effectiveness, tolerability, and safety of vagus nerve stimulation (VNS) as adjunctive therapy in 64 paediatric patients with refractory epilepsies.

Materials And Methods: Sixty-four patients (34 male and 30 female) implanted with VNS for refractory epilepsy were analysed. Electroclinical features were compatible with Lennox-Gastaut syndrome in 46 patients, focal epilepsies in 10 patients, Dravet syndrome in three patients, epilepsy with myoclonic-astatic seizures in three patients, and West syndrome in two.

[Intracranial hemorrhage as initial presentation of biliary atresia: two cases report].

Biliary atresia in infants occasionally presents as intracranial, nasal or gastrointestinal bleeding, instead of the classical triad of jaundice, acholia and choluria. We present two female infants aged four and two months, who were hospitalized with convulsive episode, cephalohematoma and drowsiness. Computed tomography findings were subdural hemorrhage in one patient and intraventricular and parenchymal bleeding in the other one.

Hemispherectomy in pediatric patients with epilepsy: a study of 45 cases with special emphasis on epileptic syndromes.

Objective: In this study we report the clinical outcomes of hemispherectomy for epilepsy in pediatric patients with special emphasis on the epileptic syndromes and their etiologies.

Material And Methods: We retrospectively studied 45 patients with medically refractory epilepsy with hemispheric lesions who underwent hemispherectomy at the "Hospital de Pediatría Prof. Dr.

Mesial temporal lobe epilepsy with hippocampal sclerosis: study of 42 children.

Purpose: We present the electroclinical features, treatment, and evolution of patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS).

Material And Methods: We analyzed the charts of forty-two patients who met the diagnostic criteria of MTLE-HS. The mean follow-up after seizure onset was 10.

Vagus nerve stimulation: effectiveness and tolerability in patients with epileptic encephalopathies.

Purpose: We discuss the effectiveness, tolerability, and safety of vagus nerve stimulation (VNS) as adjunctive therapy in 26 patients with refractory epileptic encephalopathies (EEs).

Material And Methods: Twenty-six patients (17 male and 9 female) with electroclinical features compatible with Lennox-Gastaut syndrome (LGS) in 20 patients, Dravet syndrome (DS) in 3 patients, and epilepsy with myoclonic-astatic seizures (EMAS) in 3 patients implanted with the NCP system were analyzed.

Results: In our series of patients with LGS, 17 cases showed a significant improvement in seizure control, with a reduction in seizure frequency of at least 50%.

Cytogenetic findings in a rare pediatric mixed glioneuronal tumor and review of the literature.

Objective: The aim of the present study was to report the chromosomal abnormalities findings in rare pediatric mixed glioneuronal tumor (GNT), which could not be classified according to the WHO classification.

Methods: Cytogenetic studies were performed using G-banding and fluorescence in situ hybridization (FISH) techniques.

Results: Cytogenetic analyses showed a deletion of 1p as primary genetic event and gain of chromosome 7 as secondary change.

New proteins configure a brain drug resistance map in tuberous sclerosis.

Epileptogenic cortical tubers, characterized by dysplastic neurons and balloon cells, is a frequent feature of tuberous sclerosis. In severe tuberous sclerosis-affected individuals, seizures are refractory to medication. Multidrug resistance proteins (multidrug resistance protein-1 [MDR-1] and multidrug resistance-associated protein-1 [MRP-1]) have been found to be highly expressed in epileptogenic cortical tubers.

Multidrug resistance proteins in tuberous sclerosis and refractory epilepsy.

Tuberous sclerosis is an autosomal dominant syndrome characterized by seizures that are refractory to medication in severely affected individuals. The mechanism involved in drug resistance in tuberous sclerosis is unknown. The proteins MDR-1 (multidrug resistance) and MRP-1 (multidrug resistance-associated protein-1) are linked to chemotherapy resistance in tumor cells.

Symptomatic supratentorial arachnoid cysts in children.

This study was undertaken to evaluate the clinical and radiologic long-term outcome of symptomatic primary arachnoid cysts in pediatric patients. Thirty-three children, ranging from 2 months to 17 years of age (mean age, 6 years) were treated. Craniotomy and fenestration of the cyst were used for temporal fossa and midline cysts in 24 patients (73%); later, two patients required shunt placement.

Extratemporal epilepsy in children: candidate selection and surgical treatment.

From June 1988 to June 1998, 60 children with extratemporal epilepsies (EE), most of whom were symptomatic, underwent surgery. All patients were studied by means of CT scanning, MRI and scalp EEG. Video-telemetry was used in 40 cases.