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We sought to perform a systematic review and individual participant data meta-analysis to identify predictors of treatment response following thalamic neuromodulation in pediatric patients with medically refractory epilepsy. Electronic databases (MEDLINE, Ovid, Embase, and Cochrane) were searched, with no language or data restriction, to identify studies reporting seizure outcomes in pediatric populations following deep brain stimulation (DBS) or responsive neurostimulation (RNS) implantation in thalamic nuclei. Studies featuring individual participant data of patients with primary or secondary generalized drug-resistant epilepsy were included. Response to therapy was defined as >50% reduction in seizure frequency from baseline. Of 417 citations, 21 articles reporting on 88 participants were eligible. Mean age at implantation was 13.07 ± 3.49 years. Fifty (57%) patients underwent DBS, and 38 (43%) RNS. Sixty (68%) patients were implanted in centromedian nucleus and 23 (26%) in anterior thalamic nucleus, and five (6%) had both targets implanted. Seventy-four (84%) patients were implanted bilaterally. The median time to last follow-up was 12 months (interquartile range = 6.75-26.25). Sixty-nine percent of patients achieved response to treatment. Age, target, modality, and laterality had no significant association with response in univariate logistic regression. Until thalamic neuromodulation gains widespread approval for use in pediatric patients, data on efficacy will continue to be limited to small retrospective cohorts and case series. The inherent bias of these studies can be overcome by using individual participant data. Thalamic neuromodulation appears to be a safe and effective treatment for epilepsy. Larger, prolonged prospective, multicenter studies are warranted to further evaluate the efficacy of DBS over RNS in this patient population where resection for curative intent is not a safe option.
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Estimulação Encefálica Profunda , Epilepsia Resistente a Medicamentos , Tálamo , Humanos , Epilepsia Resistente a Medicamentos/terapia , Epilepsia Resistente a Medicamentos/fisiopatologia , Estimulação Encefálica Profunda/métodos , Criança , Tálamo/fisiopatologia , Adolescente , Resultado do Tratamento , Feminino , MasculinoRESUMO
OBJECTIVE: The risk of hydrocephalus following hemispherectomy for drug resistant epilepsy (DRE) remains high. Patients with pre-existing hydrocephalus pose a postoperative challenge, as maintaining existing shunt patency is necessary but lacks a clearly defined strategy. This study examines the incidence and predictors of shunt failure in pediatric hemispherectomy patients with pre-existing ventricular shunts. METHODS: We performed a retrospective chart review at our center to identify pediatric patients diagnosed with DRE who were treated with ventricular shunt prior to their first hemispherectomy surgery. Demographic and perioperative data were obtained including shunt history, hydrocephalus etiology, epilepsy duration, surgical technique, and postoperative outcomes. Univariate analysis was performed using Fisher's exact test and Pearson correlation, with Bonferroni correction to a = 0.00625 and a = 0.01, respectively. RESULTS: Five of nineteen (26.3%) patients identified with ventriculoperitoneal shunting prior to hemispherectomy experienced postoperative shunt malfunction. All 5 of these patients underwent at least 1 shunt revision prior to hemispherectomy, with a significant association between pre- and post-hemispherectomy shunt revisions. There was no significant association between post-hemispherectomy shunt failure and valve type, intraoperative shunt alteration, postoperative external ventricular drain placement, hemispherectomy revision, lateralization of shunt relative to resection, postoperative complications, or postoperative aseptic meningitis. There was no significant correlation between number of post-hemispherectomy shunt revisions and age at shunt placement, age at hemispherectomy, epilepsy duration, or shunt duration prior to hemispherectomy. CONCLUSIONS: Earlier shunt revision surgery may portend a subsequent need for shunt revision following hemispherectomy. These findings may guide neurosurgeons in counseling patients with pre-existing ventricular shunts prior to hemispherectomy surgery.
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Epilepsia Resistente a Medicamentos , Epilepsia , Hemisferectomia , Hidrocefalia , Criança , Humanos , Hemisferectomia/efeitos adversos , Estudos Retrospectivos , Hidrocefalia/cirurgia , Derivação Ventriculoperitoneal/efeitos adversos , Epilepsia/cirurgia , Epilepsia Resistente a Medicamentos/cirurgia , Reoperação , Complicações Pós-Operatórias/etiologiaRESUMO
The aim of this report is to present a unique case of hemimegalencephaly and concomitant tuberous sclerosis complex (TSC1 mutation) with severe neonatal-onset epilepsy, which successfully underwent an anatomical hemispherectomy at 6.5 weeks of age for refractory seizures. Genetic testing confirmed a rare pathogenic, sporadic, heterozygous c.2041 + 1G > A gene mutation in intron 16 of the TSC1 gene, diagnostic for tuberous sclerosis. Post-operatively, the infant remained seizure free for at least 1 year. Following recurrence of her seizures, she has continued on multiple anti-seizure medications and everolimus therapy. We review the pathological and molecular features of this condition and highlight the ethics of intervention and steps taken toward safe neurosurgical intervention in this very young infant.
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Epilepsia , Hemimegalencefalia , Hemisferectomia , Esclerose Tuberosa , Epilepsia/cirurgia , Feminino , Hemimegalencefalia/complicações , Hemimegalencefalia/diagnóstico por imagem , Hemimegalencefalia/genética , Humanos , Lactente , Recém-Nascido , Esclerose Tuberosa/complicações , Esclerose Tuberosa/cirurgiaRESUMO
Epilepsy is a neurological condition characterised by recurrent and persistent seizures. For paediatric patients, achieving early seizure freedom can have positive impacts on cognition, development, social integration and mental health, leading to improved quality of life. In general, one third of patients with epilepsy are refractory to medication; for these patients, epilepsy surgery may offer the only chance for improved seizure control. Epilepsy surgery as a therapeutic intervention has become increasingly accepted in the past few decades, with more diverse options available (including neuromodulatory and minimally invasive techniques). In this context, we discuss here the pre-operative workup for paediatric patients with medically refractory epilepsy and provide an updated review on current and emerging surgical therapies for this condition. We also discuss the clinical, neuropsychological, quality of life and economic impacts of epilepsy surgery.
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Epilepsia Resistente a Medicamentos , Epilepsia , Humanos , Criança , Qualidade de Vida , Resultado do Tratamento , Epilepsia/cirurgia , Epilepsia/tratamento farmacológico , Convulsões , Cognição , Epilepsia Resistente a Medicamentos/cirurgia , EletroencefalografiaRESUMO
Extramedullary leukemia in pediatric acute myeloid leukemia can manifest as a myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, in a variety of sites, or as leukemic blasts in the cerebrospinal fluid. Isolated MS of the central nervous system is rare. We report a case of acute myeloid leukemia with central nervous system-MS presenting as a posterior fossa mass mimicking a primary intracranial tumor.
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Neoplasias Encefálicas/diagnóstico , Neoplasias Infratentoriais/diagnóstico , Leucemia Mieloide Aguda/diagnóstico , Sarcoma Mieloide/diagnóstico , Neoplasias Encefálicas/complicações , Criança , Diagnóstico Diferencial , Humanos , Neoplasias Infratentoriais/complicações , Leucemia Mieloide Aguda/complicações , Masculino , Prognóstico , Sarcoma Mieloide/complicaçõesRESUMO
BACKGROUND: Epilepsy and seizure awareness is gradually improving across Canada. With the strategic proposal for a Comprehensive Epilepsy Program in Manitoba (including formation of a new Pediatric Epilepsy Monitoring Unit (EMU)), a provincial strategy has been recommended outlining a path towards improved access to epilepsy care. We sought to qualify the current state of clinician knowledge and comfort towards diagnosis and management of this condition. METHODS: A qualitative online survey (Survey of Epilepsy and Seizure Awareness in Manitoba: An Evaluation (SESAME)), comprised of 36 short-answer questions, was delivered to primary care and specialist physicians in Manitoba. RESULTS: One hundred and eight subjects responded across varying medical disciplines. One hundred and one (93.5%) had previously managed patients with epilepsy, and 87 (80.6%) had previously ordered an electroencephalogram (EEG). A total of 63 (59.4%) had referred to a neurologist, with a lower proportion (30, 28.3%) referring specifically to an epileptologist. Only 36 respondents (33.3%) had heard of the International League Against Epilepsy (ILAE) guidelines. A total of 61 (56.5%) were unaware of invasive EEG techniques. Most (85, 78.7%) understood a role for surgery in treating epilepsy, with 12 (11.1%) unaware of surgical therapies beyond vagal nerve stimulation (VNS). Finally, less than half (44.2%) had heard about the Comprehensive Epilepsy Program in Manitoba, with nearly two-thirds (62.8%) indicating that they would like to have more information on epilepsy management. CONCLUSIONS: The SESAME successfully identified strong awareness towards epilepsy, with identifiable lapses in knowledge that will benefit from a formal provincial-wide educational curriculum.
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Eletroencefalografia/métodos , Epilepsia/diagnóstico , Epilepsia/terapia , Estimulação do Nervo Vago/métodos , Gerenciamento Clínico , Feminino , Pesquisas sobre Atenção à Saúde , Humanos , Masculino , Manitoba , Monitorização FisiológicaRESUMO
PURPOSE: This study aims to compare stereotactic radiosurgery (SRS) planning of epilepsy that complies with Radiosurgery or Open Surgery for Epilepsy (ROSE) guidelines in GammaKnife, non-coplanar conformal (NCC) plan in Eclipse, dynamic conformal arc (DCA) plan in Brainlab, and a volumetric modulated arc therapy (VMAT) plan in Eclipse. METHODS: Twenty plans targeting Mesial temporal lobe epilepsy (MTLE) was generated using GammaKnife, Eclipse with 20 NCC beams, Brainlab with 5 DCA, and Eclipse VMAT with 4 arcs observing ROSE trial guidelines. Multivariate analysis of variance and Wilcoxon signed-rank test were used to compare dosimetric data of the plans and perform pairwise comparison, respectively. RESULTS: The plans obeyed the recommended prescription isodose volume (PIV) within 5.5-7.5 cc and maximum doses to brainstem, optic apparatus (OA) of 10 and 8 Gy, respectively, for a prescription dose of 24 Gy. The volumes of the target were in the range 4.0-7.4 cc. Mean PIV, maximum dose to brainstem, OA were 6.5 cc, 10 Gy, 7.9 Gy in GammaKnife; 7.2 cc, 6.1 Gy, 4.5 Gy in Eclipse NCC; 7.2 cc, 6.4 Gy, 5.7 Gy in Brainlab DCA; and 5.2 cc, 8.4 Gy, 6.1 Gy in Eclipse VMAT plans, respectively. Multivariate analysis of variance showed significant differences among the 4 SRS planning techniques (P-values < 0.01). CONCLUSIONS: Among the 4 SRS planning methods, VMAT with least PIV and acceptable maximum doses to brainstem and OA showed highest compliance with ROSE trial. Having the most conformal dose distribution and least dose inhomogeneity, VMAT scored higher than GK, Eclipse NCC, and Brainlab DCA plans.
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Epilepsia do Lobo Temporal/cirurgia , Guias de Prática Clínica como Assunto/normas , Garantia da Qualidade dos Cuidados de Saúde/normas , Radiocirurgia/normas , Planejamento da Radioterapia Assistida por Computador/métodos , Planejamento da Radioterapia Assistida por Computador/normas , Humanos , Órgãos em Risco/efeitos da radiação , Dosagem Radioterapêutica , Radioterapia de Intensidade Modulada/métodosRESUMO
We report on a unique case of a giant, recurrent, supratentorial neurenteric cyst causing intractable eplipsy. Following resection, the patient developed a delayed reactive cerebritis with focal edema and worsened seizures that fully resolved with medical management. At last follow-up, over 18 months later, the patient has no evidence for cyst recurrence and remains seizure-free. we conclude that complete resection of these lesions not only requires fenestration, but also microsurgical stripping of the cyst wall. Moreover, post-operative management inclues monitoring for worsened seizures as a consequence of intracranial exposure to the cystic contents and subsequent reactive cerebritis.
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Epilepsia Resistente a Medicamentos/etiologia , Defeitos do Tubo Neural/complicações , Defeitos do Tubo Neural/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , RecidivaAssuntos
Fluoresceína/administração & dosagem , Corantes Fluorescentes/administração & dosagem , Neoplasias Infratentoriais/diagnóstico por imagem , Neoplasias Infratentoriais/cirurgia , Pré-Escolar , Feminino , Fluoresceína/metabolismo , Corantes Fluorescentes/metabolismo , Humanos , Neoplasias Infratentoriais/metabolismo , Injeções IntravenosasRESUMO
Mesial temporal lobe epilepsy (MTLE) describes recurrent seizure activity originating from the depths of the temporal lobe. MTLE patients who fail two trials of medication now require testing for surgical candidacy at an epilepsy center. For these individuals, temporal lobectomy offers the greatest likelihood for seizure-freedom (up to 80-90%); unfortunately, this procedure remains largely underutilized. Moreover, for select patients unable to tolerate open surgery, novel techniques are emerging for selective ablation of the mesial temporal structures, including stereotactic radiosurgery (SRS). We present here a review of SRS as a potential therapy for MTLE, when open surgery is not an option.
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Epilepsia do Lobo Temporal/cirurgia , Radiocirurgia , Epilepsia do Lobo Temporal/patologia , Humanos , Seleção de PacientesRESUMO
We report here the unusual case of an 8-year-old child with left hemispheric focal epilepsy secondary to a perinatal infarction who presented with new onset absence seizures and eventual nonconvulsive status epilepticus that was refractory to medical management. Following review at our multidisciplinary Epilepsy Surgery conference, the patient underwent disconnective surgical hemispherotomy with immediate cessation of his seizures; and has remained seizure-free at 4 months following surgery. In this context, we present here an overview of hemispherectomy and related procedures, including peri-insular disconnective hemispherotomy, and we discuss the efficacy of surgery for challenging hemispheric epilepsies.
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Paralisia Cerebral/complicações , Hemisferectomia , Estado Epiléptico/etiologia , Estado Epiléptico/cirurgia , Criança , Deficiências do Desenvolvimento/etiologia , Hemisferectomia/métodos , Humanos , Masculino , Estado Epiléptico/diagnóstico , Resultado do TratamentoRESUMO
OBJECTIVE: Ictal Single Photon Emission Computed Tomography (SPECT) and stereo-electroencephalography (SEEG) are diagnostic techniques used for the management of patients with drug-resistant focal epilepsies. While hyperperfusion patterns in ictal SPECT studies reveal seizure onset and propagation pathways, the role of ictal hypoperfusion remains poorly understood. The goal of this study was to systematically characterize the spatio-temporal information flow dynamics between differently perfused brain regions using stereo-EEG recordings. METHODS: We identified seizure-free patients after resective epilepsy surgery who had prior ictal SPECT and SEEG investigations. We estimated directional connectivity between the epileptogenic-zone (EZ), non-resected areas of hyperperfusion, hypoperfusion, and baseline perfusion during the interictal, preictal, ictal, and postictal periods. RESULTS: Compared to the background, we noted significant information flow (1) during the preictal period from the EZ to the baseline and hyperperfused regions, (2) during the ictal onset from the EZ to all three regions, and (3) during the period of seizure evolution from the area of hypoperfusion to all three regions. CONCLUSIONS: Hypoperfused brain regions were found to indirectly interact with the EZ during the ictal period. SIGNIFICANCE: Our unique study, combining intracranial electrophysiology and perfusion imaging, presents compelling evidence of dynamic changes in directional connectivity between brain regions during the transition from interictal to ictal states.
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Eletroencefalografia , Convulsões , Tomografia Computadorizada de Emissão de Fóton Único , Humanos , Tomografia Computadorizada de Emissão de Fóton Único/métodos , Masculino , Feminino , Adulto , Convulsões/fisiopatologia , Convulsões/diagnóstico por imagem , Eletroencefalografia/métodos , Adolescente , Adulto Jovem , Eletrocorticografia/métodos , Encéfalo/fisiopatologia , Encéfalo/diagnóstico por imagem , Pessoa de Meia-Idade , Criança , Epilepsia Resistente a Medicamentos/fisiopatologia , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgiaRESUMO
BACKGROUND AND OBJECTIVES: Stereoelectroencephalography (SEEG) is an important method for invasive monitoring to establish surgical candidacy in approximately half of refractory epilepsy patients. Identifying factors affecting lead placement can mitigate potential surgical risks. This study applies multivariate analyses to identify perioperative factors affecting stereotactic electrode placement. METHODS: We collected registration and accuracy data for consecutive patients undergoing SEEG implantation between May 2022 and November 2023. Stereotactic robotic guidance, using intraoperative imaging and a novel frame-based fiducial, was used for planning and SEEG implantation. Entry-point (EE), target-point (TE), and angular errors were measured, and statistical univariate and multivariate linear regression analyses were performed. RESULTS: Twenty-seven refractory epilepsy patients (aged 15-57 years) undergoing SEEG were reviewed. Sixteen patients had unilateral implantation (10 left-sided, 6 right-sided); 11 patients underwent bilateral implantation. The mean number of electrodes per patient was 18 (SD = 3) with an average registration mean error of 0.768 mm (SD = 0.108). Overall, 486 electrodes were reviewed. Univariate analysis showed significant correlations of lead error with skull thickness (EE: P = .003; TE: P = .012); entry angle (EE: P < .001; TE: P < .001; angular error: P = .030); lead length (TE: P = .020); and order of electrode implantation (EE: P = .003; TE: P = .001). Three multiple linear regression models were used. All models featured predictors of implantation region (157 temporal, 241 frontal, 79 parietal, 9 occipital); skull thickness (mean = 5.80 mm, SD = 2.97 mm); order (range: 1-23); and entry angle in degrees (mean = 75.47, SD = 11.66). EE and TE error models additionally incorporated lead length (mean = 44.08 mm, SD = 13.90 mm) as a predictor. Implantation region and entry angle were significant predictors of error (P ≤ .05). CONCLUSION: Our study identified 2 primary predictors of SEEG lead error, region of implantation and entry angle, with nonsignificant contributions from lead length or order of electrode placement. Future considerations for SEEG may consider varying regional approaches and angles for more optimal accuracy in lead placement.
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Hemispherectomy is an effective procedure used in the treatment of drug-resistant hemispheric epilepsy, especially in the pediatric population. A number of resective and disconnective techniques are used, and selection of surgical strategy is paramount to achieving successful results. Notably, disconnective (or functional) hemispherotomy maximizes the benefits of safe, surgical disconnection while minimizing hemispheric tissue resection, thereby avoiding some of the perioperative factors contributing to morbidity in traditional anatomical hemispherectomy procedures. In this video, the authors outline the principal surgical steps of disconnective hemispherotomy and highlight important technical factors leading to optimal outcomes in patients with refractory, oftentimes catastrophic, hemispheric epilepsy. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID2436.
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Objective.For medically-refractory epilepsy patients, stereoelectroencephalography (sEEG) is a surgical method using intracranial electrode recordings to identify brain networks participating in early seizure organization and propagation (i.e. the epileptogenic zone, EZ). If identified, surgical EZ treatment via resection, ablation or neuromodulation can lead to seizure-freedom. To date, quantification of sEEG data, including its visualization and interpretation, remains a clinical and computational challenge. Given elusiveness of physical laws or governing equations modelling complex brain dynamics, data science offers unique insight into identifying unknown patterns within high-dimensional sEEG data. We apply here an unsupervised data-driven algorithm, dynamic mode decomposition (DMD), to sEEG recordings from five focal epilepsy patients (three with temporal lobe, and two with cingulate epilepsy), who underwent subsequent resective or ablative surgery and became seizure free.Approach.DMD obtains a linear approximation of nonlinear data dynamics, generating coherent structures ('modes') defining important signal features, used to extract frequencies, growth rates and spatial structures. DMD was adapted to produce dynamic modal maps (DMMs) across frequency sub-bands, capturing onset and evolution of epileptiform dynamics in sEEG data. Additionally, we developed a static estimate of EZ-localized electrode contacts, termed the higher-frequency mode-based norm index (MNI). DMM and MNI maps for representative patient seizures were validated against clinical sEEG results and seizure-free outcomes following surgery.Main results.DMD was most informative at higher frequencies, i.e. gamma (including high-gamma) and beta range, successfully identifying EZ contacts. Combined interpretation of DMM/MNI plots best identified spatiotemporal evolution of mode-specific network changes, with strong concordance to sEEG results and outcomes across all five patients. The method identified network attenuation in other contacts not implicated in the EZ.Significance.This is the first application of DMD to sEEG data analysis, supporting integration of neuroengineering, mathematical and machine learning methods into traditional workflows for sEEG review and epilepsy surgical decision-making.
Assuntos
Eletrocorticografia , Humanos , Masculino , Feminino , Adulto , Eletrocorticografia/métodos , Eletroencefalografia/métodos , Algoritmos , Eletrodos Implantados , Rede Nervosa/fisiopatologia , Epilepsia Resistente a Medicamentos/cirurgia , Epilepsia Resistente a Medicamentos/fisiopatologia , Epilepsia/fisiopatologia , Epilepsia/cirurgia , Epilepsia/diagnóstico , Técnicas Estereotáxicas , Adulto JovemRESUMO
BACKGROUND AND OBJECTIVES: We developed, tested, and validated a novel, noninvasive, Leksell G frame-based fiducial attachment, for use in stereotactic registration for stereoelectroencephalography (sEEG). Use of the device increased the number of fixed reference points available for registration, while obviating the need for additional scalp incisions. We report here on our experience and safety profile of using the device. METHODS: We collected registration data using the fiducial device across 25 adult and pediatric patients with epilepsy consecutively undergoing robotic-guided sEEG for invasive epilepsy monitoring, treated between May 2022 and July 2023. ROSA One Brain was used for trajectory planning and electrode implantation. Postoperative clinical and radiographic data were computed and quantified, including mean registration error for all patients. Entry point, target point (TP), and angular errors were measured. Descriptive statistics and correlation coefficients for error were calculated. RESULTS: Twenty-five patients underwent robotic-guided sEEG implantation (11 patients, bilateral; 10 patients, left unilateral; 4 patients, right). The mean number of electrodes per patient was 18 ± 3. The average mean registration error was 0.77 ± 0.11 mm. All patients were implanted with Ad-Tech depth electrodes. No clinically relevant complications were reported. Analysis of trajectory error was performed on 446 electrodes. The median entry point error was 1.03 mm (IQR 0.69-1.54). The median TP error was 2.26 mm (IQR 1.63-2.93). The mean angular error was 0.03 radians (IQR 0.02-0.05). There was no significant correlation between root mean square error and lead error. Root mean square error did not appreciably change over time, nor were there any significant changes in average angular, entry point, or TP error metrics. CONCLUSION: A novel, noninvasive, Leksell G frame-based fiducial attachment was developed, tested, and validated, facilitating O-arm-based stereotactic registration for sEEG. This simple innovation maintained an excellent accuracy and safety profile for sEEG procedures in epilepsy patients, with the added advantages of providing additional reference points for stereotactic registration, without requiring additional scalp incisions.
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Eletrodos Implantados , Eletroencefalografia , Procedimentos Cirúrgicos Robóticos , Técnicas Estereotáxicas , Humanos , Feminino , Masculino , Procedimentos Cirúrgicos Robóticos/métodos , Procedimentos Cirúrgicos Robóticos/instrumentação , Adulto , Eletroencefalografia/métodos , Eletroencefalografia/instrumentação , Criança , Adolescente , Marcadores Fiduciais , Adulto Jovem , Pessoa de Meia-Idade , Epilepsia/cirurgia , Pré-EscolarRESUMO
BACKGROUND: Granule cells in the hippocampus project axons to hippocampal CA3 pyramidal cells where they form large mossy fiber terminals. We have reported that these terminals contain the gap junction protein connexin36 (Cx36) specifically in the stratum lucidum of rat ventral hippocampus, thus creating morphologically mixed synapses that have the potential for dual chemical/electrical transmission. METHODOLOGY: Here, we used various approaches to characterize molecular and electrophysiological relationships between the Cx36-containing gap junctions at mossy fiber terminals and their postsynaptic elements and to examine molecular relationships at mixed synapses in the brainstem. RESULTS: In rat and human ventral hippocampus, many of these terminals, identified by their selective expression of vesicular zinc transporter-3 (ZnT3), displayed multiple, immunofluorescent Cx36-puncta representing gap junctions, which were absent at mossy fiber terminals in the dorsal hippocampus. In rat, these were found in close proximity to the protein constituents of adherens junctions (i.e., N-cadherin and nectin-1) that are structural hallmarks of mossy fiber terminals, linking these terminals to the dendritic shafts of CA3 pyramidal cells, thus indicating the loci of gap junctions at these contacts. Cx36-puncta were also associated with adherens junctions at mixed synapses in the brainstem, supporting emerging views of the structural organization of the adherens junction-neuronal gap junction complex. Electrophysiologically induced long-term potentiation (LTP) of field responses evoked by mossy fiber stimulation was greater in the ventral than dorsal hippocampus. CONCLUSIONS: The electrical component of transmission at mossy fiber terminals may contribute to enhanced LTP responses in the ventral hippocampus.
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OBJECTIVE: Hemispherectomy surgery is an effective procedure for pediatric patients with intractable hemispheric epilepsy. Hydrocephalus is a well-documented complication of hemispherectomy contributing substantially to patient morbidity. Despite some clinical and operative factors demonstrating an association with hydrocephalus development, the true mechanism of disease is incompletely understood. The aim of this study was to investigate a range of clinical and surgical factors that may contribute to hydrocephalus to enhance understanding of the development of this complication and to aid the clinician in optimizing peri- and postoperative surgical management. METHODS: A retrospective chart review was conducted on all pediatric patients younger than 21 years who underwent hemispherectomy surgery at the Cleveland Clinic between 2002 and 2016. Data collected for each patient included general demographic information, neurological and surgical history, surgical technique, pathological analysis, presence and duration of perioperative CSF diversion, CSF laboratory values obtained while an external ventricular drain (EVD) was in place, length of hospital stay, postoperative aseptic meningitis, and in-hospital surgical complications (including perioperative stroke, hematoma formation, wound breakdown, and/or infection). Outcomes data included hemispherectomy revision and Engel grade at last follow-up (based on the Engel Epilepsy Surgery Outcome Scale). RESULTS: Data were collected for 204 pediatric patients who underwent hemispherectomy at the authors' institution. Twenty-eight patients (14%) developed hydrocephalus requiring CSF diversion. Of these 28 patients, 13 patients (46%) presented with hydrocephalus during the postoperative period (within 90 days), while the remaining 15 patients (54%) presented later (beyond 90 days after surgery). Multivariate analysis revealed postoperative aseptic meningitis (OR 7.0, p = 0.001), anatomical hemispherectomy surgical technique (OR 16.3 for functional/disconnective hemispherectomy and OR 7.6 for modified anatomical, p = 0.004), male sex (OR 4.2, p = 0.012), and surgical complications (OR 3.8, p = 0.031) were associated with an increased risk of hydrocephalus development, while seizure freedom (OR 0.3, p = 0.038) was associated with a decreased risk of hydrocephalus. CONCLUSIONS: Hydrocephalus remains a prominent complication following hemispherectomy, presenting both in the postoperative period and months to years after surgery. Aseptic meningitis, anatomical hemispherectomy surgical technique, male sex, and surgical complications show an association with an increased rate of hydrocephalus development while seizure freedom postsurgery is associated with a decreased risk of subsequent hydrocephalus. These findings speak to the multifactorial nature of hydrocephalus development and should be considered in the management of pediatric patients undergoing hemispherectomy for medically intractable epilepsy.
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Epilepsia Resistente a Medicamentos , Hemisferectomia , Hidrocefalia , Meningite Asséptica , Humanos , Masculino , Criança , Epilepsia Resistente a Medicamentos/cirurgia , Hemisferectomia/efeitos adversos , Estudos Retrospectivos , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , ConvulsõesRESUMO
BACKGROUND: Radiofrequency thermocoagulation (RFTC) during intracranial stereoelectroencephalography (sEEG) was first described as a safe technique for creating lesions of epileptic foci in 2004. Since that time, the method has been applied as a diagnostic and/or palliative intervention. Although widely practiced in European epilepsy surgical programs, the technique has not been popularized in the United States given the lack of Food and Drug Administration (FDA)-approved technologies permitting safe usage of in situ sEEG electrodes for this purpose. OBSERVATIONS: The authors present a case report of a young female patient with refractory left neocortical temporal lobe epilepsy undergoing sEEG electrode implantation, who underwent sEEG-guided RFTC via a stereotactic temperature-sensing pallidotomy probe. Although used as a diagnostic step in her workup, the patient has remained seizure-free for nearly 18 months. LESSONS: The use of in situ sEEG electrodes for RFTC remains limited in the United States. In this context, this case highlights a safe alternative and temporizing approach to performing diagnostic sEEG-guided RFTC, using a temperature-sensing pallidotomy probe to create small, precise stereotactic lesions. The authors caution careful consideration of this technique as a temporary work-around solution while also highlighting the rising need for new FDA-approved technologies for safe RFTC through in situ temperature-sensing sEEG electrodes.