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Purpose: Hemispherectomy is an effective treatment option for pharmacoresistant epilepsy. Nevertheless, when high cortical functions are at risk during the presurgical evaluation, especially for older children, and for the left hemisphere, despite good seizure outcome, the anticipated decrease of cognitive functions may prevent a decision to perform surgery. The objective of this study is to report the cognitive outcome, based on verbal and performance intelligence skills, in a series of older children and adolescents who underwent left hemispherectomy, analyzing the risks (residual cognitive deficit) and benefits (seizure reduction) of surgery., Methods: We retrospectively analyzed pre- and postoperative clinical and neuropsychological data from our patients who underwent left hemispherectomy, aged between 6 and 18 years., Results: We included 15 patients, with a mean follow-up of 3.1 years, 12 patients (80%) were Engel I, and the other three were classified as Engel II, III, and IV. Nine patients were tested by Wechsler Scales of Intelligence; postsurgically all but one kept the same intellectual levels; verbal intelligence quotient (VIQ) remained unchanged in 13 and improved in one, whereas performance intelligence quotient (PIQ) decreased in four patients. Both Total Vineland and communication scores of Vineland Adaptive Behavior Scales were obtained in six patients: in all, scores were classified as deficient adaptive functioning pre- and postoperatively, remaining unchanged., Conclusion: The evaluation of the remaining intellectual abilities after left hemispherectomy in older children and adolescents is useful to discuss the risks and benefits of this surgery, enabling better and safer decisions regarding surgical indications and timing.

Objective: The objective of this study was to analyze the impact of pediatric epilepsy surgery on the quality of life (QOL), determining whether patients improve, worsen, or maintain their preoperative patterns, as it relates to the burden of caregivers, as well as evaluating potential related factors, from both the children and caregivers perspectives., Material and Methods: This is a retrospective study of children and adolescents who underwent epilepsy surgery and were evaluated through clinical data, videoelectroencephalogram (V-EEG), neuroimaging findings, neuropsychological testing, and aspects of QOL. These assessments were performed prior to surgery and after six months and two years of follow-up. Quality of life was assessed with epilepsy-specialized questionnaires, namely Questionnaire health-related quality of life for children with epilepsy (QVCE-50), Autoquestionnaire Qualité de Vie Enfant Image Scale (AUQUEI), Quality of life in epilepsy inventory for adolescents (QOLEI-AD-48); and burden of caregivers with Burden Interview - ZARIT scale. Postoperative changes in QVCE-50 were quantified using measures of the analysis of variance (ANOVA MR) for comparison of the difference between the three times of the scale and domains., Results: Fifty patients were enrolled. Of these, 27 (54%) were male, with a mean age at surgery of 8.2 years (range: 1-18 years). Thirty-five patients (70%) were Engel I and one was Engel II (2%) at six months of follow-up, whereas 28 (56%) were Engel I and 32 (64%) were Engel I or II at two years of follow-up. Preoperatively, 21 (42%) presented with moderate or severe intellectual disability. Postoperative cognitive evaluations at the two-year follow-up showed 18 (36%) maintained similar deficits. The QVCE-50 showed postoperative improvement in the two-year follow-up period, but not at six months after surgery. Postoperative improvements were associated mainly with better seizure outcome. Autoperception evaluations were limited because of the clinical and cognitive severity of patients. The burden of caregivers was quoted as mild to moderate and remained unchanged postoperatively., Conclusions: Children and adolescents with surgically treated epilepsy reach a good seizure outcome, stabilize in intellectual and adaptive functions, and have an increase in QOL, from the caregiver's perspective. Nevertheless, their burden remains unchanged. Seizure outcome is the main factor for improvement in the QOL. The upgrading of structured questionnaires and QOL instruments specific to pediatric epilepsy can be helpful to assess patient- and caregiver-reported surgical outcomes, allowing for better planning of therapeutic approaches., Competing Interests: Declaration of competing interest None of the authors has any conflict of interest to disclose. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: To assess the effects of a memory rehabilitation program in patients with left temporal lobe epilepsy following surgery., Methods: Twenty-four patients agreed to participate and 18 completed the study; nine received memory rehabilitation while nine had no input and were designated as controls. Verbal learning efficiency, naming abilities, memory subjective ratings, ecological activity measures and a language fMRI paradigm were used as outcome measures., Results: Improved verbal learning and naming test performance, increase in memory strategy use and improved self-perception were observed following the rehabilitation. Changes in fMRI activation patterns were seen in the rehabilitation group over the long term., Conclusion: The findings support the potential role of a cognitive rehabilitation program following left temporal lobe surgery.

Background and Purpose: Hemispheric dysplasia (HD) and hemimegalencephaly (HME) are both brain malformations with early clinical manifestation including developmental delay and intractable epilepsy. Sometimes the differentiation of these conditions is not simple. HME is an anomaly of cortical development caused by a combination of neural proliferation and cell migration dysfunction, showing lobar or hemispheric enlargement. On the other hand, HD shows no brain hypertrophy, and even brain atrophy, eventually., Patients and Methods: To compare both conditions, we reviewed clinical, MRI, and histopathology of 23 patients with developmental delay and refractory epilepsy treated with hemispheric surgery., Results: Histologically, both groups presented polymicrogyria, focal cortical dysplasia, gray matter (GM) heterotopia, pachygyria, and agyria. The white matter (WM) showed different degrees of gliosis and myelin impairment. Even though with no specificity in histopathology, the degree of lesion was more impressive on HME. The combination of WM dysmyelination and hypertrophy leads to the so called hamartomatous appearing. Although not all HME showed brain enlargement and some HD might show no size changes or atrophy, the size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions., Conclusion: Brain MRI was the best diagnostic tolls because it allowed together high contrast resolution, whole brain coverage and spatial distribution analysis. HD and HMD showed brain asymmetry tendency, but in opposite directions. The size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions.

The purpose of this study was to shed light on cortical audiotactile integration and sensory substitution mechanisms, thought to serve as a basis for the use of a sensor glove in the preservation of the cortical map of the hand after peripheral nerve injuries. Fourteen subjects were selected and randomly assigned either to a training group, trained to replace touch for hearing with the use of a sensor glove, or to a control group, untrained. Training group volunteers had to identify textures just by the sound. In an fMRI experiment, all subjects received three types of stimuli: tactile only, combined audiotactile stimulation, and auditory only. Results indicate that, for trained subjects, a coupling between auditory and somatosensory cortical areas is established through associative areas. Differences in signal correlation between groups point to a pairing mechanism, which, at first, connects functionally the primary auditory and sensory areas (trained subjects). Later, this connection seems to be mediated by associative areas. The training with the sensor glove influences cortical audiotactile integration mechanisms, determining BOLD signal changes in the somatosensory area during auditory stimulation., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Introduction: Epilepsy surgery may be a promising alternative therapy for seizure control in patients with refractory seizures, resistant to medication. Cognitive outcome is another important factor in favor of the surgical decision., Aim: To investigate the correlation between seizure outcome and cognitive outcome after epilepsy surgery in a pediatric population., Patients and Methods: A total of 59 pediatric patients were retrospectively assessed with the WISC-III (Full Scale, Verbal Scale and Performance Scale) before and, at least, 6 months after surgery. Patients were divided into two groups according whether or not improvement of seizure control after surgery. Data collected for each child included: epileptic syndrome, etiology, age at epilepsy onset, duration of epilepsy and seizure frequency., Results: Comparison using a MANOVA test revealed significant differences across pre-operative Full Scale, Verbal Scale and Performance Scale (p = 0.01) with seizure reduction group performing better than no seizure reduction group. Seizure improvement group achieved significant Performance Scale improvement (p = 0.01) and no seizure improvement group showed significant Verbal Scale worsened after surgery (p = 0.01)., Conclusions: Our results suggest that the success of the epilepsy surgery in childhood when the seizure control is achieved may also provide an improvement in the Performance Scale whereas the seizure maintenance may worsen the Verbal Scale.

Introduction: A multimodal approach of the human immunodeficiency virus (HIV) encephalopathy using quantitative magnetic resonance (MR) techniques can demonstrate brain changes not detectable only with conventional magnetic resonance imaging (MRI). The aim of this study was to compare conventional MRI and MR quantitative techniques, such as magnetic resonance spectroscopy (MRS) and relaxometry and to determine whether quantitative techniques are more sensitive than conventional imaging for brain changes caused by HIV infection., Methods: We studied prospectively nine HIV positive children (mean age 6 years, from 5 to 8 years old) and nine controls (mean age 7.3 years; from 3 to 10 years), using MRS and relaxometry. Examinations were carried on 1.5-T equipment., Results: HIV-positive patients presented with only minor findings and all control patients had normal conventional MR findings. MRS findings showed an increase in choline to creatine (CHO/CRE) ratios bilaterally in both frontal gray and white matter, in the left parietal white matter, and in total CHO/CRE ratio. In contrast, N-acetylaspartate to creatine (NAA/CRE) ratios did not present with any significant difference between both groups. Relaxometry showed significant bilateral abnormalities, with lengthening of the relaxation time in HIV positive in many regions., Conclusion: Conventional MRI is not sensitive for early brain changes caused by HIV infection. Quantitative techniques such as MRS and relaxometry appear as valuable tools in the diagnosis of these early changes. Therefore, a multimodal quantitative study can be useful in demonstrating and understanding the physiopathology of the disease.

Purpose: In the present study, we evaluated the preoperative demographic, clinical, and neuropsychological variables that could predict postoperative seizure outcome in a group of pediatric epileptic patients., Materials and Methods: We studied 40 consecutive pediatric patients, ages ranging from 6 to 16 years, that underwent resective surgery for the treatment of medically intractable epilepsy at the Clinical Hospital of Ribeirão Preto School of Medicine. We performed ictal electroencephalography (EEG), interictal EEG, magnetic resonance imaging (MRI), and a preoperative neuropsychological assessment in the presurgical workup., Results: The following factors were correlated with seizure outcome: (1) duration of epilepsy, (2) surgery localization, (3) localized Neuropsychological (NPS) Evaluation, (4) ictal EEG, (5) interictal EEG, and (6) MRI. Mental retardation, NPS tests, and the other demographic variables failed to correlate with seizure reduction., Conclusions: The identification of predictor variables of epilepsy surgery outcome could improve the epileptic prognosis and guarantee the children's full potential development.

Background and Purpose: Rasmussen encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. The progression of the symptoms to significant neurological impairment usually occurs within months to a few years. RE causes are unknown, although evidence of an autoimmune process has been extensively described in the literature. Antiepileptic drugs are usually not effective to control seizures or cerebral atrophy; despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, for intractable seizures in RE patients with advanced disease, epilepsy surgery in the form of hemispheric disconnection has been considered the treatment of choice. This work describes the clinical and electrographic analyses, as well as the post-operative evolution of patients with RE., Materials and Methods: This work includes all the patients with RE evaluated from January 1995 to January 2008 by the Ribeirão Preto Epilepsy Surgery Program (CIREP), taking variables such as gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery, when done; duration of epilepsy; surgery complications; follow-up duration; anatomo-pathological findings; post-surgery seizure; language and cognitive outcome; and anti-epileptic drug treatment after surgery into account., Results: Twenty-five patients were evaluated; thirteen were female. Mean age of epilepsy onset was 4.4+/-2.0 years. There were no differences between patients with slow and fast evolution with respect to age of epilepsy onset (p=0.79), age at surgery (p=0.24), duration of epilepsy (0.06), and follow-up (p=0.40). There were no correlations between the presence of bilateral EEG abnormalities or the absence of spikes and post-operative seizure outcome (p=0.06). Immunomodulatory therapy was tried in 12 patients (48%). Twenty-three patients underwent surgery. The mean follow-up was 63.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (six patients), occasional hemigeneralized tonic-clonic seizures (three patients), and frequent tonic-clonic seizures (three patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. Eight patients presented post-operative cognitive decline, while only two patients had cognitive improvement. Comparing pre- and post-operative language deficits, 66.7% of the 12 patients with language disturbance did not improve after surgery., Conclusions: This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Patients were divided into two groups: fast evolution and slow evolution to hemiparesis and epilepsia partialis continua. These groups may represent different RE substrates. Fourteen patients achieved satisfactory seizure control, three patients had partial response to surgery, and five patients had maintenance of the pre-operative condition. All patients with left-side involvement presented with some language disturbance, which did not improve after surgery in 66.6% of patients. Cognitive evaluation showed that the majority of the patients did not have any significant improvement, and 38.1% had cognitive deterioration after surgery.

Functional MRI produces a more accurate localization of the language areas for epilepsy surgery purpose, but requires the patient's cooperation. We report a 34 year-old woman with mental retardation who underwent two different verbal fluency tasks, category and word naming. We found a strong activation of Broca's area in the most difficult task. We suggest that a multi-task fMRI study could be successful in patients with cognitive delay.

Background: Hemispheric brain lesions are commonly associated with early onset of catastrophic epilepsies and multiple seizure types. Hemispheric surgery is indicated for patients with unilateral intractable epilepsy. Although described more than 50 years ago, several new techniques for hemispherectomy have only recently been proposed aiming to reduce operatory risks and morbidity., Materials and Methods: We present the clinical characteristics, presurgical workup, and postoperative outcome of a series of pediatric patients who underwent hemispherectomy for medically intractable epileptic seizures. Thirty-nine patients with medically intractable epilepsy underwent surgery from 1996 to 2005., Results and Discussion: We analyzed demographic data, interictal and ictal EEG findings, age at surgery, surgical technique and complications, and postsurgical seizure outcome. There were 74.4% males. Tonic and focal motor seizures occurred in 30.8 and 20.5% of the patients. Most frequent etiologies were Rasmussen encephalitis (30.8%) and malformation of cortical development (23.1%). Postsurgical outcomes were Engel classes I and II for 61.5% of the patients. In general, 89.5% of the patients exhibited at least a 90% reduction in seizure frequency. All patients had acute worsening of hemiparesis after surgery. Basically, two surgical techniques have been employed, both with similar results, although a trend has been noted toward one of the procedures which produced consistently complete disconnection. Patients with hemispheric brain lesions usually have abnormal neurological development and intractable epilepsy. When video-EEG monitoring and magnetic resonance imaging show unilateral disease, the patient may evolve with a good surgical outcome. We showed that a marked reduction in seizure frequency may be achieved, with acceptable neurological impairments.

Cognitive impairment has long been recognized in people with medically refractory epilepsies. Mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE-HS), the most common surgically remediable epileptic syndrome, has been associated with a cellular prion protein (PrPc) gene (Prnp) variant allele at codon 171. The polymorphism consisting of a methionine-for-valine substitution at codon 129 has been associated with early cognitive deterioration in elderly people and patients with Down syndrome. The same variant allele in homozygosis (V129V) has been associated to a lower long-term memory in healthy humans. PrPc mediates several processes related to neuroplasticity, and its role in cognitive processes remains unknown. In this study, we evaluated the genetic contribution of Prnp alleles to cognitive performance in patients with MTLE-HS. Cognitive performance, measured with 19 neuropsychological tests, of patients with refractory MTLE-HS with the normal Prnp genotypes was compared with that of patients with the variant alleles at codons 129 and 171. With the effects of clinical, demographic, electrophysiological, and neuroimaging variable interactions controlled by multiple linear regression analysis and adjustment for multiple test comparisons, the presence of Prnp variant alleles was found not to be significantly associated to cognitive performance of patients with MTLE-HS. The presence of variant alleles at codons 129 and 171 is not associated to cognitive performance of patients with refractory MTLE-HS.

Purpose: To analyze the patterns of brain activation in polymicrogyric cortex in epilepsy patients by blood oxygenation level-dependent (BOLD) functional MRI (fMRI), during language and motor activation tasks., Methods: Five patients with extratemporal epilepsy associated with polymicrogyria were scanned in a 1.5-T Siemens scanner with BOLD fMRI sequences, by following language and motor paradigms. Functional images were processed by using Brain Voyager software., Results: Activation of polymicrogyric cortex in the brain area that corresponds to the motor strip was present in all patients. Language studies revealed activation of polymicrogyric cortex over Broca's area in three cases., Conclusions: Functionality was shown in polymicrogyric areas. Therefore if surgical resection is indicated, a careful functional planning is necessary because of functionality of the polymicrogyric cortex.

Background and Purpose: Temporal lobe epilepsy (TLE) encompasses 10-20% of the cases of intractable epilepsy in pediatric patients. Mesial temporal sclerosis (MTS) can still be encountered in adolescent patients, but is rare in children under 5 years of age. In this paper we report on the surgical outcome of a series of TLE patients ranging in age from 1 to 18 years at the time of operation., Patients and Methods: Thirty-five patients (37 surgeries) with medically intractable TLE were operated upon between January 1996 and December 2002. The following variables were analyzed: age at surgery, age at epilepsy onset, history of an initial precipitating injury, etiology, seizure semiology, interictal and ictal EEG findings, surgical complications, and post-surgical seizure outcome., Results: There were 68.6% females and 31.3% males, and complex partial seizures (CPS) occurred in 86.5%. The most common etiology was MTS (40%) followed by isolated cortical developmental abnormalities (22.9%). In the age group up to 5 years, cortical development abnormalities predominated, and 71% of these children had multifocal interictal EEG. Patients older than 10 years had more frequently MTS (78.6%) and focal temporal interictal EEG abnormalities. Post-surgical seizure outcome showed that 88.5% of patients were in Engel classes I and II., Conclusions: Adolescents with TLE had clinical features, electrographic findings, and seizure outcome similar to those observed in adult patients. However, younger children up to 5 years of age had distinct ictal semiology and different etiological, electrophysiological and outcome profiles, clearly suggesting that they behave as a special subgroup within the TLE.