RESUMO
Stigma has been related to epilepsy since ancient times. Despite the importance of this issue, only a few interventions focusing on the reduction of epilepsy-related stigma may be found in the literature. Thus, the aim of this study is to evaluate the effectiveness of two interventions focused on the reduction of epilepsy-related stigma in children aged 9-11years. The first group of children involved in the study (n1=89) completed the 23-item Czech version of the SSE (Stigma Scale of Epilepsy) questionnaire and an 11-item multiple-choice knowledge test, then watched a video and completed the same questionnaire and test immediately after the intervention. The same procedure was used for the second group (n2=93) where a story was read by an instructor. Both groups were retested 6months later using the same methods. Both interventions resulted in long-term decrease of epilepsy-related stigma - the average value on SSE decreased from 55.15 points at baseline testing to 43.28 points in the 6-month follow-up for the case of the video (p<0.001) and from 48.68 points to 36.97 points for the case of the story (p<0.001). Knowledge about epilepsy was also significantly improved with the average result in the knowledge test increasing from 6.58 to 9.09 points in case of the video (p<0.001) and from 6.88 to 8.99 points in case of the story (p<0.001). The results showed that both aforementioned interventions were significant and effective ways to reduce epilepsy-related stigma in the given age group.
Assuntos
Epilepsia/psicologia , Epilepsia/terapia , Narração , Educação de Pacientes como Assunto/métodos , Estigma Social , Gravação em Vídeo/métodos , Criança , Comportamento de Escolha , República Tcheca/epidemiologia , Epilepsia/epidemiologia , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Masculino , Inquéritos e Questionários , Resultado do TratamentoRESUMO
Dravet syndrome is a severe epilepsy syndrome characterized by infantile onset of therapy-resistant, fever-sensitive seizures followed by cognitive decline. Mutations in SCN1A explain about 75% of cases with Dravet syndrome; 90% of these mutations arise de novo. We studied a cohort of nine Dravet-syndrome-affected individuals without an SCN1A mutation (these included some atypical cases with onset at up to 2 years of age) by using whole-exome sequencing in proband-parent trios. In two individuals, we identified a de novo loss-of-function mutation in CHD2 (encoding chromodomain helicase DNA binding protein 2). A third CHD2 mutation was identified in an epileptic proband of a second (stage 2) cohort. All three individuals with a CHD2 mutation had intellectual disability and fever-sensitive generalized seizures, as well as prominent myoclonic seizures starting in the second year of life or later. To explore the functional relevance of CHD2 haploinsufficiency in an in vivo model system, we knocked down chd2 in zebrafish by using targeted morpholino antisense oligomers. chd2-knockdown larvae exhibited altered locomotor activity, and the epileptic nature of this seizure-like behavior was confirmed by field-potential recordings that revealed epileptiform discharges similar to seizures in affected persons. Both altered locomotor activity and epileptiform discharges were absent in appropriate control larvae. Our study provides evidence that de novo loss-of-function mutations in CHD2 are a cause of epileptic encephalopathy with generalized seizures.
Assuntos
Proteínas de Ligação a DNA/genética , Epilepsias Mioclônicas/genética , Animais , Criança , Transtornos Cognitivos/genética , Transtornos Cognitivos/patologia , Estudos de Coortes , Epilepsias Mioclônicas/patologia , Exoma , Feminino , Técnicas de Silenciamento de Genes , Haploinsuficiência , Humanos , Deficiência Intelectual/genética , Deficiência Intelectual/patologia , Larva/genética , Masculino , Canal de Sódio Disparado por Voltagem NAV1.1/genética , Fenótipo , Convulsões Febris/genética , Convulsões Febris/patologia , Adulto Jovem , Peixe-ZebraRESUMO
BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system (CNS). Its immunopathogenesis has been proposed to include early cerebrospinal fluid (CSF) lymphocytosis, subsequent CNS disease restriction and B cell mechanism predominance. There are limited data regarding T cell involvement in the disease. To contribute to the current knowledge, we investigated the complex system of chemokines and cytokines related to B and T cell functions in CSF and sera samples from anti-NMDAR encephalitis patients at different time-points of the disease. One patient in our study group had a long-persisting coma and underwent extraordinary immunosuppressive therapy. METHODS: Twenty-seven paired CSF/serum samples were collected from nine patients during the follow-up period (median 12 months, range 1-26 months). The patient samples were stratified into three periods after the onset of the first disease symptom and compared with the controls. Modified Rankin score (mRS) defined the clinical status. The concentrations of the chemokines (C-X-C motif ligand (CXCL)10, CXCL8 and C-C motif ligand 2 (CCL2)) and the cytokines (interferon (IFN)γ, interleukin (IL)4, IL7, IL15, IL17A and tumour necrosis factor (TNF)α) were measured with Luminex multiple bead technology. The B cell-activating factor (BAFF) and CXCL13 concentrations were determined via enzyme-linked immunosorbent assay. We correlated the disease period with the mRS, pleocytosis and the levels of all of the investigated chemokines and cytokines. Non-parametric tests were used, a P value <0.05 was considered to be significant. RESULTS: The increased CXCL10 and CXCL13 CSF levels accompanied early-stage disease progression and pleocytosis. The CSF CXCL10 and CXCL13 levels were the highest in the most complicated patient. The CSF BAFF levels remained unchanged through the periods. In contrast, the CSF levels of T cell-related cytokines (INFγ, TNFα and IL17A) and IL15 were slightly increased at all of the periods examined. No dynamic changes in chemokine and cytokine levels were observed in the peripheral blood. CONCLUSIONS: Our data support the hypothesis that anti-NMDAR encephalitis is restricted to the CNS and that chemoattraction of immune cells dominates at its early stage. Furthermore, our findings raise the question of whether T cells are involved in this disease.
Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato/líquido cefalorraquidiano , Quimiocinas/líquido cefalorraquidiano , Citocinas/líquido cefalorraquidiano , Adolescente , Adulto , Encefalite Antirreceptor de N-Metil-D-Aspartato/terapia , Fator Ativador de Células B/líquido cefalorraquidiano , Linfócitos B/metabolismo , Quimiocina CXCL10/líquido cefalorraquidiano , Quimiocina CXCL13/líquido cefalorraquidiano , Criança , Coma/líquido cefalorraquidiano , Coma/etiologia , Progressão da Doença , Feminino , Humanos , Imunoterapia , Masculino , Troca Plasmática , Esteroides/uso terapêutico , Linfócitos T/metabolismo , Resultado do Tratamento , Adulto JovemRESUMO
Interictal epileptiform discharges (spikes, IEDs) are electrographic markers of epileptic tissue and their quantification is utilized in planning of surgical resection. Visual analysis of long-term multi-channel intracranial recordings is extremely laborious and prone to bias. Development of new and reliable techniques of automatic spike detection represents a crucial step towards increasing the information yield of intracranial recordings and to improve surgical outcome. In this study, we designed a novel and robust detection algorithm that adaptively models statistical distributions of signal envelopes and enables discrimination of signals containing IEDs from signals with background activity. This detector demonstrates performance superior both to human readers and to an established detector. It is even capable of identifying low-amplitude IEDs which are often missed by experts and which may represent an important source of clinical information. Application of the detector to non-epileptic intracranial data from patients with intractable facial pain revealed the existence of sharp transients with waveforms reminiscent of interictal discharges that can represent biological sources of false positive detections. Identification of these transients enabled us to develop and propose secondary processing steps, which may exclude these transients, improving the detector's specificity and having important implications for future development of spike detectors in general.
Assuntos
Encéfalo/fisiopatologia , Eletroencefalografia/métodos , Epilepsia/fisiopatologia , Reconhecimento Automatizado de Padrão/métodos , Adolescente , Adulto , Algoritmos , Criança , Dor Crônica/diagnóstico , Dor Crônica/fisiopatologia , Eletrodos Implantados , Epilepsia/diagnóstico , Dor Facial/diagnóstico , Dor Facial/fisiopatologia , Reações Falso-Negativas , Reações Falso-Positivas , Feminino , Humanos , Masculino , Análise de Componente Principal , Sensibilidade e Especificidade , Processamento de Sinais Assistido por Computador , Adulto JovemRESUMO
A 4-year-old girl with intractable epilepsy due to left-side hemispheric cortical dysplasia underwent a hemispherotomy. She was seizure-free after the surgery. EEG showed persistent abundant epileptiform activity over the left (disconnected) hemisphere, including ictal patterns that neither generalised nor had clinical correlates. Antiepileptic medication was completely withdrawn four years following the surgery. One week after the withdrawal, she developed episodes of intense left-sided hemicranias (ipsilateral to the surgery) with vomiting and photophobia that did not resemble her habitual seizures and were unresponsive to non-steroidal anti-inflammatory drugs. Video-EEG showed association of the headache attacks with ictal patterns over the disconnected hemisphere. Brain MRI revealed increased signal changes in the left hemisphere. Attacks responded promptly to i.v. midazolam and carbamazepine at a low dose. Mechanisms underlying peri-ictal headache originating in the disconnected hemisphere are discussed. [Published with video sequences].
Assuntos
Epilepsia/complicações , Cefaleia/complicações , Convulsões/complicações , Anticonvulsivantes/uso terapêutico , Encéfalo/patologia , Carbamazepina/uso terapêutico , Pré-Escolar , Eletroencefalografia , Epilepsia/patologia , Epilepsia/cirurgia , Feminino , Lateralidade Funcional , Cefaleia/patologia , Hemisferectomia , Humanos , Midazolam/uso terapêutico , Procedimentos Neurocirúrgicos , Convulsões/patologiaRESUMO
PURPOSE: Variable predictors of postsurgical seizure outcome have been reported in children with tuberous sclerosis complex (TSC). We analyzed a large surgical series of pediatric TSC patients in order to identify prognostic factors crucial for selection of subjects for epilepsy surgery. METHODS: Thirty-three children with TSC who underwent excisional epilepsy surgery at Miami Children's Hospital were retrospectively reviewed. A total of 29 clinical, neuropsychological, electroencephalography (EEG), magnetic resonance imaging (MRI), and surgical variables were analyzed and related to seizure outcomes. Univariate Barnard's exact test, Wilcoxon's rank-sum test, and multivariate statistical Cox's model were used to examine the significance of associations between the variables and seizure outcome. KEY FINDINGS: Eighteen patients (55%) have been seizure-free 2 years after (final) surgery; postoperative complications occurred in five subjects (15%). Complete removal of epileptogenic tissue detected by both MRI and intracranial EEG, regional scalp interictal EEG patterns, and agreement of interictal and ictal EEG localization were the most powerful predictors of seizure-free outcome. Other significant predictors included occurrence of regional scalp ictal EEG patterns, fewer brain regions affected by tubers, presence of preoperative hemiparesis, and one-stage surgery. Remaining factors such as age at seizure onset, incidence of infantile spasms or other seizure types, duration of epilepsy, seizure frequency, mental retardation, as well as types and extent of resections did not influence outcome. SIGNIFICANCE: Perioperative features rather than preoperative variables are the most important determinants of postsurgical seizure outcome in patients with TSC. Our findings may assist in the surgical management of these patients.
Assuntos
Eletroencefalografia , Epilepsia/cirurgia , Esclerose Tuberosa/cirurgia , Pré-Escolar , Eletroencefalografia/métodos , Epilepsia/complicações , Feminino , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética/métodos , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Esclerose Tuberosa/complicaçõesRESUMO
PURPOSE: To assess the predictive value of ictal single-photon emission computed tomography (SPECT) for outcome after excisional epilepsy surgery in a large population of children with focal cortical dysplasia (FCD). METHODS: One hundred seventy-three ictal SPECT studies in 106 children with histologically proven FCD were retrospectively analyzed. The extent and location of ictal hyperperfusion and completeness of surgical removal were assessed. Completeness of resection of epileptogenic regions defined by ictal SPECT, electroencephalography (EEG), and magnetic resonance imaging (MRI) were compared and correlated with postoperative seizure outcome. In addition, subcortical activation of the cerebellum, basal ganglia, and thalamus were analyzed. KEY FINDINGS: The extent of hyperperfusion was focal or lobar in 58%, whereas multilobar activations occurred in only 32%; hemispheric or bilateral findings were rare. Favorable postsurgical seizure outcome was achieved in 67% patients with nonlocalized SPECT findings, 45% with nonresected ictal hyperperfusion, 36% with partially resected ictal hyperperfusion, and 86% when the zone of ictal hyperperfusion was completely resected (p = 0.000198). The favorable postsurgical outcome after complete removal of the SPECT hyperperfusion zone surpassed the 75% rate of seizure freedom in patients with removal of MRI/EEG-defined epileptogenic region. A similar predictive value of ictal SPECT for seizure outcome was found in nonoperated patients and subjects who were undergoing reoperation. Subcortical activation conferred no predictive value. SIGNIFICANCE: Ictal SPECT helps to define the epileptogenic zone in a high proportion of children with FCD undergoing surgical evaluation. Complete removal of both SPECT and MRI/EEG-defined regions is a strong predictor of surgical success and has important implications for surgical planning.
Assuntos
Eletroencefalografia/métodos , Imageamento por Ressonância Magnética/métodos , Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Malformações do Desenvolvimento Cortical/patologia , Tomografia Computadorizada de Emissão de Fóton Único/métodos , Adolescente , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Circulação Cerebrovascular/fisiologia , Criança , Pré-Escolar , Epilepsia/diagnóstico por imagem , Epilepsia/patologia , Feminino , Humanos , Lactente , Masculino , Malformações do Desenvolvimento Cortical/cirurgia , Procedimentos Neurocirúrgicos , Estudos Retrospectivos , Adulto JovemRESUMO
Epilepsy is both a disease of the brain and the mind. Brain diseases, structural and/or functional, underlie the appearance of epilepsy, but the notion of epilepsy is larger and cannot be reduced exclusively to the brain. We can therefore look at epilepsy from two angles. The first perspective is intrinsic: the etiology and pathophysiology, problems of therapy, impact on the brain networks, and the "mind" aspects of brain functions - cognitive, emotional, and affective. The second perspective is extrinsic: the social interactions of the person with epilepsy, the influence of the surrounding environment, and the influences of epilepsy on society. All these aspects reaching far beyond the pure biological nature of epilepsy have been the topics of two International Congresses of Epilepsy, Brain, and Mind that were held in Prague, Czech Republic, in 2010 and 2012 (the third Congress will be held in Brno, Czech Republic on April 3-5, 2014; www.epilepsy-brain-mind2014.eu). Here, we present the first of two papers with extended summaries of selected presentations of the 2012 Congress that focused on epilepsy, behavior, and art.
Assuntos
Arte , Comportamento/fisiologia , Encéfalo/fisiopatologia , Epilepsia , Transtornos Mentais/complicações , Arte/história , Epilepsia/história , Epilepsia/patologia , Epilepsia/psicologia , História do Século XVI , História do Século XVII , História do Século XVIII , História do Século XIX , História do Século XX , História Antiga , História Medieval , Humanos , Transtornos Mentais/históriaRESUMO
AIMS: To identify variables that influence the extent of ictal single-photon emission computed tomography (SPECT) findings in paediatric patients with focal cortical dysplasia (FCD). METHODS: We visually evaluated 98 ictal SPECT studies from 67 children treated surgically for intractable epilepsy caused by FCD. SPECT findings were classified as "non-localised", "well-localised", and "extensive" and compared with parameters of injected seizures (seizure type and duration, injection time, and scalp EEG ictal pattern), presence of structural pathology on MRI, type of surgery performed after SPECT study, and histological findings. RESULTS: A shorter injection time and duration of injected seizure was associated with more localised SPECT hyperperfusion. SPECT findings were not significantly influenced by type of injected seizure. Widespread ictal scalp EEG patterns were associated with extensive SPECT findings. Larger zones of hyperperfusion were more common in patients with lesional MRI and patients undergoing multilobar resections. SPECT studies demonstrating good localisation were more common in patients with mild malformations of cortical development. CONCLUSION: Early ictal SPECT radiotracer injection is crucial for successful localisation of the epileptogenic zone. Seizure duration, type of scalp EEG findings, and presence of structural pathology on MRI may influence the extent of ictal SPECT hyperperfusion, which was associated with certain types of epilepsy surgery as well as histopathological findings.
Assuntos
Epilepsia/diagnóstico por imagem , Tomografia Computadorizada de Emissão de Fóton Único , Criança , Pré-Escolar , Eletroencefalografia/métodos , Epilepsia/patologia , Epilepsia/fisiopatologia , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Valor Preditivo dos Testes , Tomografia Computadorizada de Emissão de Fóton Único/métodosRESUMO
AIMS: To assess the practical localising value of subtraction ictal single-photon emission computed tomography (SISCOM) coregistered with MRI and (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) in patients with extratemporal epilepsy and normal MRI. METHODS: We retrospectively studied a group of 14 patients who received surgery due to intractable epilepsy and who were shown to have focal cortical dysplasia, undetected by MRI, based on histological investigation. We coregistered preoperative SISCOM and PET images with postoperative MRI and visually determined whether the SISCOM focus, PET hypometabolic area, and cerebral cortex, exhibiting prominent abnormalities on intracranial EEG, were removed completely, incompletely, or not at all. These results and histopathological findings were compared with postoperative seizure outcome. RESULTS: Two patients underwent one-stage multimodal image-guided surgery and the remaining 12 underwent long-term invasive EEG. SISCOM findings were localised for all but 1 patient. FDG-PET was normal in 3 subjects, 2 of whom had favourable postsurgical outcome (Engel class I and II). Complete resection of the SISCOM focus (n=3), the area of PET hypometabolism (n=2), or the cortical regions with intracranial EEG abnormalities (n=7) were predictive of favourable postsurgical outcome. Favourable outcome was also encountered in: 4 of 8 patients with incomplete resection and 1 of 2 with no resection of the SISCOM focus; 4 of 7 patients with incomplete resection and 1 of 2 with no resection of the PET hypometabolic area; and 2 of 7 patients with incomplete resection of the area corresponding to intracranial EEG abnormality. No correlation between histopathological FCD subtype and seizure outcome was observed. CONCLUSION: Complete resection of the dysplastic cortex localised by SISCOM, FDG-PET or intracranial EEG is a reliable predictor of favourable postoperative seizure outcome in patients with non-lesional extratemporal epilepsy.
Assuntos
Encéfalo/diagnóstico por imagem , Epilepsia/diagnóstico por imagem , Adolescente , Adulto , Encéfalo/patologia , Encéfalo/cirurgia , Mapeamento Encefálico/métodos , Criança , Eletroencefalografia , Epilepsia/patologia , Epilepsia/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Cintilografia , Estudos Retrospectivos , Cirurgia Assistida por Computador , Resultado do TratamentoRESUMO
We report a girl with a drug-resistant frontal lobe epilepsy caused by focal cortical dysplasia, who exhibited uncommon seizures. The seizures consisted of shoulder or whole body shuddering after a short psychic aura and face grimacing. Consciousness was fully preserved. The seizures resembled "wet dog shake" seizures described in rat models of epilepsy or shuddering attacks in infants. EEG findings were inconclusive, however, MRI showed a clear dysplastic lesion in the right frontal mesial and polar structures. The patient underwent an extended lesionectomy guided by neuronavigation and intraoperative electrocorticography. Focal cortical dysplasia type Ib was histologically confirmed and the patient has been seizure-free for the three years following resection. [Published with video sequences].
Assuntos
Epilepsia do Lobo Frontal/fisiopatologia , Lobo Frontal/fisiopatologia , Malformações do Desenvolvimento Cortical/fisiopatologia , Convulsões/fisiopatologia , Criança , Eletroencefalografia , Epilepsia do Lobo Frontal/patologia , Epilepsia do Lobo Frontal/cirurgia , Feminino , Lobo Frontal/patologia , Lobo Frontal/cirurgia , Humanos , Malformações do Desenvolvimento Cortical/patologia , Malformações do Desenvolvimento Cortical/cirurgia , Procedimentos Neurocirúrgicos , Convulsões/patologia , Convulsões/cirurgia , Resultado do TratamentoRESUMO
Sturge-Weber syndrome is a neurocutaneous disorder classically characterized by the presence of facial port-wine stain and ipsilateral leptomeningeal angiomatosis. It is often associated with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo-occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo-occipital area as well as myoclonic and myoclonic-astatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge-Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony. [Published with video sequences].
Assuntos
Encéfalo/cirurgia , Epilepsias Mioclônicas/cirurgia , Síndrome de Sturge-Weber/cirurgia , Encéfalo/fisiopatologia , Criança , Eletroencefalografia , Epilepsias Mioclônicas/fisiopatologia , Humanos , Masculino , Síndrome de Sturge-Weber/fisiopatologia , Resultado do TratamentoRESUMO
Epilepsy is a serious health disorder affecting both paediatric and adult population worldwide. Due to difficulties in identifying its aetiology, initial management is often guided by empiric therapy measures. Symptomatic control requires the use of antiepileptic drugs (AEDs), many of which have the potential for adverse drug interactions. Children are especially susceptible to drug interactions and frequently exhibit atypical adverse events, which may require special care. Aim. To demonstrate a case of a 15 year old girl suffering from refractory epilepsy with underlying focal cortical dysplasia (FCD), whose seizure deterioration was most probably associated with drug-drug interactions between prescribed common antiepileptic drugs, namely valproic acid, phenobarbital or the prodrug primidon and carbamazepine.
Assuntos
Anticonvulsivantes/efeitos adversos , Epilepsia/tratamento farmacológico , Adolescente , Encéfalo/anormalidades , Carbamazepina/efeitos adversos , Interações Medicamentosas , Quimioterapia Combinada , Epilepsia/complicações , Feminino , Humanos , Fenobarbital/efeitos adversos , Primidona/efeitos adversos , Ácido Valproico/efeitos adversosRESUMO
The aim of the study was to analyze the lateralizing value of proton magnetic resonance spectroscopy ((1)H MRS) in histopathologically different subgroups of mesial temporal lobe epilepsies (MTLE) and to correlate results with clinical, MRI and seizure outcome data. A group of 35 patients who underwent resective epilepsy surgery was retrospectively studied. Hippocampal (1)H MR spectra were evaluated. Metabolite concentrations were obtained using LCModel and NAA/Cr, NAA/Cho, NAA/(Cr+Cho), Cho/Cr ratios and coefficients of asymmetry were calculated. MRI correctly lateralized 89% of subjects and (1)H MRS 83%. MRI together with (1)H MRS correctly lateralized 100% of patients. Nineteen subjects had "classical" hippocampal sclerosis (HS), whereas the remaining 16 patients had "mild" HS. Nineteen patients had histopathologically proven malformation of cortical development (MCD) in the temporal pole; 16 subjects had only HS. No difference in (1)H MRS findings was found between patients in different histopathological subgroups of MTLE. Our results support the hypothesis that (1)H MRS abnormalities do not directly reflect histopathological changes in MTLE patients. Subjects with non-lateralized (1)H MRS abnormalities did not have a worse postoperative seizure outcome. We found no significant impact of contralateral (1)H MRS abnormality on post-surgical seizure outcome.
Assuntos
Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/patologia , Imageamento por Ressonância Magnética/métodos , Espectroscopia de Ressonância Magnética/métodos , Adolescente , Adulto , Eletroencefalografia/métodos , Epilepsia do Lobo Temporal/cirurgia , Feminino , Hipocampo/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
We performed a retrospective, multicenter, open-label study to evaluate the efficacy of vagus nerve stimulation (VNS) in all patients in the Czech Republic who have received this treatment for at least 5 years (n=90). The mean last follow-up was 6.6+/-1.1 years (79+/-13 months). The median number of seizures among all patients decreased from 41.2 seizures/month in the prestimulation period to 14.9 seizures/month at 5 years follow-up visit. The mean percentage of seizure reduction was 55.9%. The responder rate in these patients is in concordance with the decrease of overall seizure frequency. At 1 year after beginning the stimulation, 44.4% of patients were responders; this percentage increased to 58.7% after 2 years. At the 5 years last follow-up 64.4% of patients were responders, 15.5% experienced > or = 90% seizure reduction, and 5.5% were seizure-free. A separate analysis of patients younger than 16 years of age showed lower efficacy rates of VNS in comparison to the whole group. Complications and chronic adverse effects occurred in 13.3% of patients. VNS is an effective and safe method to refractory epilepsy in common clinical practice.
Assuntos
Epilepsia/terapia , Estimulação do Nervo Vago/métodos , Adolescente , Criança , Pré-Escolar , República Tcheca/epidemiologia , Humanos , Estudos Longitudinais , Medição da Dor , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
[This corrects the article DOI: 10.3389/fneur.2018.00184.].
RESUMO
BACKGROUND: Diffusion tensor imaging (DTI) is a powerful tool for investigating brain anatomical connectivity. The aim of our study was to compare brain connectivity among children with autism spectrum disorders (ASD), developmental dysphasia (DD), and healthy controls (HC) in the following tracts: the arcuate fasciculus (AF), inferior frontal occipital fasciculus (IFOF), inferior longitudinal fasciculus (ILF), and uncinate fasciculus (UF). METHODS: Our sample consisted of 113 children with a mean age 8.7±2.2 years (77 boys, 36 girls), divided into three subgroups: ASD (n=39), DD (n=36), and HC (n=38). The International Classification of Diseases, 10th ed. was used to make clinical diagnoses. DTI images were collected using a 1.5 T Phillips Achieva MR imaging system. RESULTS: Detailed analyses of fractional anisotropy (FA) revealed significant differences among the ASD, DD, and HC groups in the left AF (p=0.014) and right AF (p=0.001), the left IFOF (p<0.001) and right IFOF (p<0.001), the left ILF (p<0.001) and right ILF (p<0.001), but not in the UF. Post-hoc analyses revealed three patterns of FA differences among the groups: (1) in the right AF, right IFOF, and right ILF, FA was significantly lower in the ASD group compared to the DD and HC groups; however, there was no difference in FA between DD and HC; (2) in the left AF and left IFOF, FA was significantly lower in the ASD than in the HC group, but there were no differences between DD vs HC nor DD vs ASD; and (3) in the left ILF, no difference in FA was seen between ASD and DD, but FA in both was significantly lower than in the HC. CONCLUSION: Microstructural white matter properties differed between ASD vs DD and HC subjects. The tract where FA impairment in ASD and DD subjects was the most similar was the left ILF.
RESUMO
BACKGROUND: We aimed first to describe trends in cognitive performance over time in a large patient cohort (n = 203) from a single tertiary centre for paediatric epilepsy surgery over the period of 16 years divided in two (developing-pre-2011 vs. established-post-2011). Secondly, we tried to identify subgroups of epilepsy surgery candidates with distinctive epilepsy-related characteristics that associate with their pre- and post-surgical cognitive performance. Thirdly, we analysed variables affecting pre-surgical and post-surgical IQ/DQ and their change (post- vs. pre-surgical). METHODS: We analysed IQ/DQ data obtained using standardized neuropsychological tests before epilepsy surgery and one year post-surgically, along with details of patient's epilepsy, epilepsy surgery and outcomes in terms of freedom from seizures. Using regression analysis, we described the trend in post-operative IQ/DQ. Cognitive outcomes and the associated epilepsy- and epilepsy surgery-related variables were compared between periods before and after 2011. Using multivariate analysis we analysed the effect of individual variables on pre- and post-operative IQ/DQ and its change. RESULTS: Epilepsy surgery tends to improve post-surgical IQ/DQ, most significantly in patients with lower pre-surgical IQ/DQ, and post-surgical IQ/DQ strongly correlates with pre-surgical IQ/DQ (Rho = 0.888, p < 0.001). We found no significant difference in pre-, post-surgical IQ/DQ and IQ/DQ change between the periods of pre-2011 and post-2011 (p = 0.7, p = 0.469, p = 0.796, respectively). Patients with temporal or extratemporal epilepsy differed in their pre-surgical IQ/DQ (p = 0.001) and in IQ/DQ change (p = 0.002) from those with hemispheric epilepsy, with no significant difference in post-surgical IQ/DQ (p = 0.888). Groups of patients with different underlying histopathology showed significantly different pre- and post-surgical IQ/DQ (p < 0.001 and p < 0.001 respectively) but not IQ/DQ change (p = 0.345).Variables associated with severe epilepsy showed effect on cognitive performance in multivariate model. DISCUSSION: Post-surgical IQ/DQ strongly correlates with pre-surgical IQ/DQ and greatest IQ/DQ gain occurs in patients with lower pre-surgical IQ/DQ scores. Cognitive performance was not affected by changes in paediatric epilepsy surgery practice. Pre- and post-operative cognitive performances, as well as patients' potential for cognitive recovery, are highly dependent on the underlying aetiology and epileptic syndrome.
RESUMO
PURPOSE: We assessed trends in spectrum of candidates, diagnostic algorithm, therapeutic approach and outcome of a pediatric epilepsy surgery program between 2000 and 2017. METHODS: All pediatric patients who underwent curative epilepsy surgery in Motol Epilepsy Center during selected period (n = 233) were included in the study and divided into two groups according to time of the surgery (developing program 2000-2010: n = 86, established program 2011-2017: n = 147). Differences in presurgical, surgical and outcome variables between the groups were statistically analyzed. RESULTS: A total of 264 resections or hemispheric disconnections were performed (including 31 reoperations). In the later epoch median age of candidates decreased. Median duration of disease shortened in patients with temporal lobe epilepsy. Number of patients with non-localizing MRI findings (subtle or multiple lesions) rose, as well as those with epileptogenic zone adjacent to eloquent cortex. There was a trend towards one-step procedures guided by multimodal neuroimaging and intraoperative electrophysiology; long-term invasive EEG was performed in fewer patients. Subdural electrodes for long-term invasive monitoring were almost completely replaced by stereo-EEG. The number of focal resections and hemispherotomies rose over time. Surgeries were more often regarded complete. Histopathological findings of resected tissue documented developing spectrum of candidates. 82.0% of all children were seizure-free two years after surgery; major complications occurred in 4.6% procedures; both groups did not significantly differ in these parameters. CONCLUSION: In the established pediatric epilepsy surgery program, our patients underwent epilepsy surgery at younger age and suffered from more complex structural pathology. Outcomes and including complication rate remained stable.
Assuntos
Epilepsia/patologia , Epilepsia/cirurgia , Hemisferectomia/métodos , Neurologia/tendências , Adolescente , Córtex Cerebral/cirurgia , Criança , Pré-Escolar , Eletroencefalografia/métodos , Feminino , Hemisferectomia/efeitos adversos , Humanos , Masculino , Neuroimagem/métodos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
At present, more and more researchers are applying tested mathematical-engineering methods into different domains of life. One of these areas is helping people with different forms of disabilities. Research in this area is focused on searching for the relation between clinical and electrophysiological symptoms of children with developmental dysphasia. Sleep EEG and speech analyses are the primary areas under discussion, while the finding of methods acceptable for improvement of the diagnosis and determination of therapeutic procedures is the research topic. It is possible to reduce fundamentally, or to cure optimally these disorders in advanced diagnosis. Therefore it is important to search for new methods and to combine what has been used separately till now.