RESUMO
The relationship between clinically accessible epileptic biomarkers and neuronal activity underlying the transition to seizure is complex, potentially leading to imprecise delineation of epileptogenic brain areas. In particular, the pattern of interneuronal firing at seizure onset remains under debate, with some studies demonstrating increased firing and others suggesting reductions. Previous study of neocortical sites suggests that seizure recruitment occurs upon failure of inhibition, with intact feedforward inhibition in non-recruited territories. We investigated whether the same principle applies in limbic structures. We analysed simultaneous electrocorticography (ECoG) and neuronal recordings of 34 seizures in a cohort of 19 patients (10 male, 9 female) undergoing surgical evaluation for pharmacoresistant focal epilepsy. A clustering approach with five quantitative metrics computed from ECoG and multiunit data was used to distinguish three types of site-specific activity patterns during seizures, which at times co-existed within seizures. Overall, 156 single units were isolated, subclassified by cell-type and tracked through the seizure using our previously published methods to account for impacts of increased noise and single-unit waveshape changes caused by seizures. One cluster was closely associated with clinically defined seizure onset or spread. Entrainment of high-gamma activity to low-frequency ictal rhythms was the only metric that reliably identified this cluster at the level of individual seizures (P < 0.001). A second cluster demonstrated multi-unit characteristics resembling those in the first cluster, without concomitant high-gamma entrainment, suggesting feedforward effects from the seizure. The last cluster captured regions apparently unaffected by the ongoing seizure. Across all territories, the majority of both excitatory and inhibitory neurons reduced (69.2%) or ceased firing (21.8%). Transient increases in interneuronal firing rates were rare (13.5%) but showed evidence of intact feedforward inhibition, with maximal firing rate increases and waveshape deformations in territories not fully recruited but showing feedforward activity from the seizure, and a shift to burst-firing in seizure-recruited territories (P = 0.014). This study provides evidence for entrained high-gamma activity as an accurate biomarker of ictal recruitment in limbic structures. However, reduced neuronal firing suggested preserved inhibition in mesial temporal structures despite simultaneous indicators of seizure recruitment, in contrast to the inhibitory collapse scenario documented in neocortex. Further study is needed to determine if this activity is ubiquitous to hippocampal seizures or indicates a 'seizure-responsive' state in which the hippocampus is not the primary driver. If the latter, distinguishing such cases may help to refine the surgical treatment of mesial temporal lobe epilepsy.
Assuntos
Epilepsia do Lobo Temporal , Neocórtex , Humanos , Masculino , Feminino , Eletroencefalografia/métodos , Convulsões , Epilepsia do Lobo Temporal/cirurgia , Neurônios/fisiologiaRESUMO
OBJECTIVE: Stereotactic laser amygdalohippocampotomy (SLAH) is an appealing option for patients with temporal lobe epilepsy, who often require intracranial monitoring to confirm mesial temporal seizure onset. However, given limited spatial sampling, it is possible that stereotactic electroencephalography (stereo-EEG) may miss seizure onset elsewhere. We hypothesized that stereo-EEG seizure onset patterns (SOPs) may differentiate between primary onset and secondary spread and predict postoperative seizure control. In this study, we characterized the 2-year outcomes of patients who underwent single-fiber SLAH after stereo-EEG and evaluated whether stereo-EEG SOPs predict postoperative seizure freedom. METHODS: This retrospective five-center study included patients with or without mesial temporal sclerosis (MTS) who underwent stereo-EEG followed by single-fiber SLAH between August 2014 and January 2022. Patients with causative hippocampal lesions apart from MTS or for whom the SLAH was considered palliative were excluded. An SOP catalogue was developed based on literature review. The dominant pattern for each patient was used for survival analysis. The primary outcome was 2-year Engel I classification or recurrent seizures before then, stratified by SOP category. RESULTS: Fifty-eight patients were included, with a mean follow-up duration of 39 ± 12 months after SLAH. Overall 1-, 2-, and 3-year Engel I seizure freedom probability was 54%, 36%, and 33%, respectively. Patients with SOPs, including low-voltage fast activity or low-frequency repetitive spiking, had a 46% 2-year seizure freedom probability, compared to 0% for patients with alpha or theta frequency repetitive spiking or theta or delta frequency rhythmic slowing (log-rank test, p = .00015). SIGNIFICANCE: Patients who underwent SLAH after stereo-EEG had a low probability of seizure freedom at 2 years, but SOPs successfully predicted seizure recurrence in a subset of patients. This study provides proof of concept that SOPs distinguish between hippocampal seizure onset and spread and supports using SOPs to improve selection of SLAH candidates.
Assuntos
Epilepsia do Lobo Temporal , Humanos , Estudos Retrospectivos , Resultado do Tratamento , Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/cirurgia , Epilepsia do Lobo Temporal/complicações , Convulsões/diagnóstico , Convulsões/cirurgia , Convulsões/complicações , Eletroencefalografia , Lasers , Imageamento por Ressonância MagnéticaRESUMO
OBJECTIVE: One of the clinical hallmarks of tuberous sclerosis complex (TSC) is radiologically identified cortical tubers, which are present in most patients. Intractable epilepsy may require surgery, often involving invasive diagnostic procedures such as intracranial electroencephalography (EEG). Identifying the location of the dominant tuber responsible for generating epileptic activities is a critical issue. However, the link between cortical tubers and epileptogenesis is poorly understood. Given this, we hypothesized that tuber voxel intensity may be an indicator of the dominant epileptogenic tuber. Also, via tuber segmentation based on deep learning, we explored whether an automatic quantification of the tuber burden is feasible. METHODS: We annotated tubers from structural magnetic resonance images across 29 TSC subjects, summarized tuber statistics in eight brain lobes, and determined suspected epileptogenic lobes from the same group using EEG monitoring data. Then, logistic regression analyses were performed to demonstrate the linkage between the statistics of cortical tuber and the epileptogenic zones. Furthermore, we tested the ability of a neural network to identify and quantify tuber burden. RESULTS: Logistic regression analyses showed that the volume and count of tubers per lobe, not the mean or variance of tuber voxel intensity, were positively correlated with electrophysiological data. In 47.6% of subjects, the lobe with the largest tuber volume concurred with the epileptic brain activity. A neural network model on the test dataset showed a sensitivity of .83 for localizing individual tubers. The predicted masks from the model correlated highly with the neurologist labels, and thus may be a useful tool for determining tuber burden and searching for the epileptogenic zone. SIGNIFICANCE: We have proven the feasibility of an automatic segmentation of tubers and a derivation of tuber burden across brain lobes. Our method may provide crucial insights regarding the treatment and outcome of TSC patients.
Assuntos
Epilepsia , Esclerose Tuberosa , Eletroencefalografia/métodos , Epilepsia/diagnóstico por imagem , Epilepsia/etiologia , Humanos , Imageamento por Ressonância Magnética , Redes Neurais de Computação , Esclerose Tuberosa/diagnósticoRESUMO
Electrocortical stimulation mapping (ESM) is often performed in patients undergoing stereoelectroencephalography (SEEG) prior to epilepsy surgery, with the goal of identifying functional cortex and preserving it postoperatively. ESM may also evoke a patient's typical seizure semiology. The purpose of this study was to determine whether the sites at which typical auras are evoked during ESM are associated with other known clinical and electrophysiologic biomarkers of the epileptogenic zone: the seizure onset zone (SOZ), the early spread zone (ES), and high-frequency oscillations (HFOs). We found that the sites at which auras were provoked were not consistently associated with known biomarkers (pâ¯=â¯0.09). We conclude that evoked auras during ESM may reflect electrical spread rather than true epileptogenicity, and that a larger study is needed to assess their potential value as independent epileptic biomarkers.
Assuntos
Eletroencefalografia , Epilepsia , Biomarcadores , Mapeamento Encefálico , Epilepsia/diagnóstico , Humanos , ConvulsõesRESUMO
BACKGROUND: Surgical site infections (SSIs) are one of the most common complications following pediatric complex tethered spinal cord release. This patient population is similar in some ways to the neuromuscular scoliosis population, in which higher-than-expected rates of gram-negative SSIs have been identified. METHODS: We conducted a single-center retrospective chart review of all patients who underwent complex tethered spinal cord release over a 10-year period between 2007 and 2017. RESULTS: A total of 69 patients were identified, with 10 documented SSIs (14%). 50% of the SSIs were polymicrobial or included at least 1 gram-negative organism. Among the organisms isolated, 3 were fully or -partially resistant to cefazolin, the most common antibiotic prophylaxis in this population. CONCLUSION: Among children undergoing complex tethered spinal cord release, gram-negative and polymicrobial infections are a significant cause of SSIs. Although further multicenter data are needed, these findings suggest that standard antibiotic prophylaxis with cefazolin may not be sufficient.
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Infecções por Bactérias Gram-Positivas/epidemiologia , Defeitos do Tubo Neural/epidemiologia , Defeitos do Tubo Neural/cirurgia , Procedimentos Neurocirúrgicos/efeitos adversos , Infecção da Ferida Cirúrgica/epidemiologia , Criança , Pré-Escolar , Feminino , Infecções por Bactérias Gram-Positivas/diagnóstico , Humanos , Lactente , Masculino , Defeitos do Tubo Neural/diagnóstico , Procedimentos Neurocirúrgicos/tendências , Projetos Piloto , Prevalência , Estudos Retrospectivos , Infecção da Ferida Cirúrgica/diagnósticoRESUMO
PURPOSE: Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. METHODS: A multidisciplinary, retrospective cohort study from seven children's hospitals was performed including asymptomatic children with MMC < 21 years old, managed with or without prophylactic untethering prior to scoliosis surgery. Patients were divided into three groups for analysis: (1) untethering at the time of scoliosis surgery (concomitant untethering), (2) untethering within 3 months of scoliosis surgery (prior untethering), and (3) no prophylactic untethering. Baseline data, intra-operative reports, and 90-day post-operative outcomes were analyzed to assess for differences in neurologic outcomes, surgical complications, and overall length of stay. RESULTS: A total of 208 patients were included for analysis (mean age 9.4 years, 52% girls). No patient in any of the groups exhibited worsened motor or sensory function at 90 days post-operatively. However, comparing the prophylactic untethering groups with the group that was not untethered, there was an increased risk of surgical site infection (SSI) (31.3% concomitant, 28.6% prior untethering vs. 12.3% no untethering; p = 0.0104), return to the OR (43.8% concomitant, 23.8% prior untethering vs. 17.4% no untethering; p = 0.0047), need for blood transfusion (51.6% concomitant, 57.1% prior untethering vs. 33.8% no untethering; p = 0.04), and increased mean length of stay (LOS) (13.4 days concomitant, 10.6 days prior untethering vs. 6.8 days no untethering; p < 0.0001). In multivariable logistic regression analysis, prophylactic untethering was independently associated with increased adjusted relative risks of surgical site infection (aRR = 2.65, 95% CI 1.17-5.02), unplanned re-operation (aRR = 2.17, 95% CI 1.02-4.65), and any complication (aRR = 2.25, 95% CI 1.07-4.74). CONCLUSION: In this study, asymptomatic children with myelomeningocele who underwent scoliosis surgery developed no neurologic injuries regardless of prophylactic untethering. However, those who underwent prophylactic untethering were more likely to experience SSIs, return to the OR, need a blood transfusion, and have increased LOS than children not undergoing untethering. Based on these data, prophylactic untethering in asymptomatic MMC patients prior to scoliosis surgery does not provide any neurological benefit and is associated with increased surgical risks.
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Transfusão de Sangue/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Meningomielocele/cirurgia , Procedimentos Cirúrgicos Profiláticos , Escoliose/cirurgia , Doenças da Medula Espinal/cirurgia , Infecção da Ferida Cirúrgica/epidemiologia , Adolescente , Doenças Assintomáticas , Perda Sanguínea Cirúrgica/estatística & dados numéricos , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Modelos Logísticos , Masculino , Meningomielocele/complicações , Análise Multivariada , Defeitos do Tubo Neural/cirurgia , Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias/epidemiologia , Reoperação/estatística & dados numéricos , Escoliose/etiologia , Doenças da Medula Espinal/etiologiaRESUMO
Cerebral vasospasm is associated with significant morbidity, and most commonly occurs following subarachnoid hemorrhage. Rarely, vasospasm can follow tumor resection and traumatic brain injury. We present the first reported case of a young child who developed diffuse vasospasm following open fenestration of an arachnoid cyst and was promptly treated, with full recovery of neurologic function. Although vasopasm after arachnoid cyst fenestration is rare, it can be included in the differential for a new focal neurologic deficit.
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Cistos Aracnóideos/diagnóstico por imagem , Cistos Aracnóideos/cirurgia , Craniotomia/efeitos adversos , Complicações Pós-Operatórias/diagnóstico por imagem , Vasoespasmo Intracraniano/diagnóstico por imagem , Pré-Escolar , Humanos , Masculino , Complicações Pós-Operatórias/etiologia , Vasoespasmo Intracraniano/etiologiaRESUMO
A 22-year-old Hispanic man with sickle cell trait presented with blurred vision, double vision, and pain with OD movement. MRI demonstrated an extra-axial mass centered around the temporal bone with extension into the middle cranial fossa and lateral aspect of the extra-conal right orbit, and mass effect on the lateral rectus muscle. Biopsy of the lesion was consistent with renal medullary carcinoma. CT chest/abdomen/pelvis confirmed a primary tumor in the right kidney. No additional metastases were found. Renal medullary carcinoma is a rare, highly aggressive malignancy, which almost exclusively affects young men of African descent with sickle cell trait or sickle cell disease. The authors present the second confirmed case of renal medullary carcinoma metastatic to the orbit, with ocular symptoms prior the typical presenting symptoms of flank pain and hematuria.Renal medullary carcinoma is a highly aggressive malignancy, most commonly seen in African American patients with sickle cell disease. Involvement of the orbit is rare and visual symptoms may precede systemic diagnosis.
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Carcinoma Medular/secundário , Neoplasias Renais/patologia , Neoplasias Orbitárias/secundário , Humanos , Masculino , Adulto JovemRESUMO
Inactivating mutations of the TSC1/TSC2 complex (TSC1/2) cause tuberous sclerosis (TSC), a hereditary syndrome with neurological symptoms and benign hamartoma tumours in the brain. Since TSC effectors are largely unknown in the human brain, TSC patient cortical tubers were used to uncover hyperphosphorylation unique to TSC primary astrocytes, the cell type affected in the brain. We found abnormal hyperphosphorylation of catenin delta-1 S268, which was reversible by mTOR-specific inhibitors. In contrast, in three metastatic astrocytoma cell lines, S268 was under phosphorylated, suggesting S268 phosphorylation controls metastasis. TSC astrocytes appeared epithelial (i.e. tightly adherent, less motile, and epithelial (E)-cadherin positive), whereas wild-type astrocytes were mesenchymal (i.e. E-cadherin negative and highly motile). Despite their epithelial phenotype, TSC astrocytes outgrew contact inhibition, and monolayers sporadically generated tuberous foci, a phenotype blocked by the mTOR inhibitor, Torin1. Also, mTOR-regulated phosphokinase C epsilon (PKCe) activity induced phosphorylation of catenin delta-1 S268, which in turn mediated cell-cell adhesion in astrocytes. The mTOR-dependent, epithelial phenotype of TSC astrocytes suggests TSC1/2 and mTOR tune the phosphorylation level of catenin delta-1 by controlling PKCe activity, thereby regulating the mesenchymal-epithelial-transition (MET). Thus, some forms of TSC could be treated with PKCe inhibitors, while metastasis of astrocytomas might be blocked by PKCe stimulators.
Assuntos
Cateninas/genética , Hamartoma/genética , Proteína Quinase C-épsilon/genética , Serina-Treonina Quinases TOR/genética , Proteínas Supressoras de Tumor/genética , Astrócitos/efeitos dos fármacos , Astrócitos/patologia , Adesão Celular/efeitos dos fármacos , Linhagem Celular Tumoral , Inibição de Contato/efeitos dos fármacos , Transição Epitelial-Mesenquimal/genética , Hamartoma/patologia , Humanos , Naftiridinas/administração & dosagem , Metástase Neoplásica , Fosforilação/efeitos dos fármacos , Proteína 1 do Complexo Esclerose Tuberosa , Proteína 2 do Complexo Esclerose Tuberosa , delta CateninaRESUMO
OBJECTIVE: Selective laser amygdalohippocampotomy (SLAH) using magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) is emerging as a treatment option for drug-resistant mesial temporal lobe epilepsy (MTLE). SLAH is less invasive than open resection, but there are limited series reporting its safety and efficacy, particularly in patients without clear evidence of mesial temporal sclerosis (MTS). METHODS: We report seizure outcomes and complications in our first 30 patients who underwent SLAH for drug-resistant MTLE between January 2013 and December 2016. We compare patients who required stereoelectroencephalography (SEEG) to confirm mesial temporal onset with those treated based on imaging evidence of MTS. RESULTS: Twelve patients with SEEG-confirmed, non-MTS MTLE and 18 patients with MRI-confirmed MTS underwent SLAH. MTS patients were older (median age 50 vs 30 years) and had longer standing epilepsy (median 40.5 vs 5.5 years) than non-MTS patients. Engel class I seizure freedom was achieved in 7 of 12 non-MTS patients (58%, 95% confidence interval [CI] 30%-86%) and 10 of 18 MTS patients (56%, 95% CI 33%-79%), with no significant difference between groups (odds ratio [OR] 1.12, 95% CI 0.26-4.91, P = .88). Length of stay was 1 day for most patients (range 0-3 days). Procedural complications were rare and without long-term sequelae. SIGNIFICANCE: We report similar rates of seizure freedom following SLAH in patients with MTS and SEEG-confirmed, non-MTS MTLE. Consistent with early literature, these rates are slightly lower than typically observed with surgical resection (60%-80%). However, SLAH is less invasive than open surgery, with shorter hospital stays and recovery, and severe procedural complications are rare. SLAH may be a reasonable first-line surgical option for patients with both MTS and SEEG confirmed, non-MTS MTLE.
Assuntos
Eletroencefalografia/métodos , Epilepsia do Lobo Temporal/cirurgia , Hipocampo/patologia , Hipocampo/cirurgia , Terapia a Laser/métodos , Técnicas Estereotáxicas , Adulto , Idoso , Eletroencefalografia/tendências , Epilepsia do Lobo Temporal/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Terapia a Laser/tendências , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Esclerose , Técnicas Estereotáxicas/tendências , Lobo Temporal/patologia , Lobo Temporal/cirurgia , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Surgical intervention during infancy for both syndromic and nonsyndromic patients with craniosynostosis remains the criterion standard of treatment with the 2 main options being open vault remodeling versus minimally invasive surgery. Although open cranial vault remodeling was initially considered a high-risk procedure, many advances have improved its safety. Despite this, there is a paucity of literature on the long-term outcomes of contemporary open craniosynostosis repair. METHODS: A retrospective review of all patients who underwent primary open cranial vault repair for craniosynostosis by a single surgeon (J.A.A.) at New York-Presbyterian Hospital from 1995 to 2015 was performed. RESULTS: For primary open repair, 81 patients (46 males, 35 females) were analyzed, and affected sutures included unicoronal (28), bicoronal (7), metopic (24), sagittal (11), lambdoid (2), and multisuture (9). Fourteen patients (17.3%) were syndromic. Mean (SD) operative patient age was 13.81 (16.24) months: 34 (42%) were 0 to 6 months; 26 (32%), 7 to 12 months; and 21 (26%), 12 months of age or older. There were no intraoperative complications. Mean (SD) estimated blood loss for the plastic surgery portion of all cases was 74.53 (72.34) mL, and total estimated blood loss was 174.93 (182.23) mL. Mean (SD) hospital length of stay was 4.31 (1.59) days. One syndromic patient was readmitted for a wound infection (1.2%) that was successfully treated with antibiotics, and 2 syndromic patients (2.5%) had reoperation for fronto-orbital readvancement. CONCLUSIONS: This 20-year experience demonstrates the safety of modern open craniosynostosis repairs at a large academic medical center with low rates of mortality (0%), complications (1.2%), and reoperations (2.5%).
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Craniossinostoses/cirurgia , Procedimentos Ortopédicos/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Complicações Pós-Operatórias/epidemiologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
INTRODUCTION: Syrinx has been reported in 25-85 % of children with Chiari malformation type I (CMI), and it is most commonly cervical in location. As a result, cervical MRI is routinely included in an evaluation for CMI. Isolated thoracic syrinx without involvement of the cervical cord in this population is uncommon but clinically important because its presence may influence the decision to operate, surgical techniques employed, or interpretation of follow-up imaging. The purpose of this study was to determine the incidence of isolated thoracic syrinx in a large group of children evaluated for CMI. METHODS: We retrospectively reviewed all patients under 21 years of age who were evaluated for CMI at Columbia University/Morgan Stanley Children's Hospital of New York from 1998 to 2013. All patients underwent MRI of the entire spine as part of the CMI evaluation, regardless of whether surgery was planned. The proportion of patients exhibiting isolated thoracic syrinx was determined. Presenting signs, symptoms, and imaging findings were then studied in an attempt to identify any clinical features associated with isolated thoracic syrinx. RESULTS: We identified 266 patients evaluated over the study period. One-hundred thirty-two patients (50 %) presented with a syrinx, and 12 patients (4.5 % of all patients evaluated and 9.1 % of all patients with a syrinx) had an isolated thoracic syrinx. Demographic variables, clinical presentation, and extent of tonsillar ectopia showed great heterogeneity in this group, and no factor was consistently associated with isolated thoracic syrinx. CONCLUSIONS: Isolated thoracic syrinx is an uncommon but clinically significant finding in children with CMI. Our data demonstrate that the presence of a CMI-related thoracic syrinx cannot be reliably predicted clinically and is therefore likely to be missed in patients who do not undergo complete spinal cord imaging. MRI of the entire spinal cord should be considered for all children undergoing initial evaluation for CMI.
Assuntos
Malformação de Arnold-Chiari/complicações , Siringomielia/complicações , Siringomielia/epidemiologia , Vértebras Torácicas/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Imageamento por Ressonância Magnética , Masculino , Estudos RetrospectivosRESUMO
Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) as a primary treatment for hydrocephalus is gaining popularity in North America, particularly among the infant population. Unfortunately, despite considerable experience with ETV/CPC at several centers, treatment failures still exist. Early reports have suggested that greater than 90 % cauterization of the choroid plexus is associated with improved clinical outcomes. However, individual patient anatomy and smaller overall ventricular size can limit the amount of choroid plexus cauterization that is technically possible through a single frontal burr hole. Furthermore, the degree of cauterization achieved by surgeons using this technique is difficult to quantify objectively. In this report, we describe the case of an infant who failed initial ETV/CPC but then had successful resolution of hydrocephalus after additional choroid plexus cauterization performed through bilateral occipital burr holes. The child remains shunt-free over a year after treatment, suggesting that this three-pronged CPC approach (the "bowling ball" technique) may be successful in some young children with persistent hydrocephalus after ETV/CPC from a single frontal burr hole.
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Cauterização/métodos , Plexo Corióideo/cirurgia , Hidrocefalia/cirurgia , Terceiro Ventrículo/cirurgia , Ventriculostomia/métodos , Adolescente , Plexo Corióideo/diagnóstico por imagem , Feminino , Humanos , Hidrocefalia/diagnóstico por imagem , Imageamento por Ressonância Magnética , Terceiro Ventrículo/diagnóstico por imagemRESUMO
OBJECT: Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature. METHODS: The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes. RESULTS: The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003). CONCLUSIONS: Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.
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Anemia Falciforme/complicações , Veias Cerebrais/cirurgia , Doença de Moyamoya/cirurgia , Procedimentos Neurocirúrgicos/métodos , Adolescente , Anemia Falciforme/fisiopatologia , Isquemia Encefálica/cirurgia , Angiografia Cerebral , Criança , Estudos de Coortes , Feminino , Humanos , Ataque Isquêmico Transitório/etiologia , Ataque Isquêmico Transitório/cirurgia , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Masculino , Doença de Moyamoya/diagnóstico por imagem , Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/cirurgia , Resultado do Tratamento , Adulto JovemRESUMO
Despite abundant evidence of functional networks in the human brain, their neuronal underpinnings, and relationships to real-time behavior have been challenging to resolve. Analyzing brain-wide intracranial-EEG recordings with video monitoring, acquired in awake subjects during clinical epilepsy evaluation, we discovered the tendency of each brain region to switch back and forth between 2 distinct power spectral densities (PSDs 2-55Hz). We further recognized that this 'spectral switching' occurs synchronously between distant sites, even between regions with differing baseline PSDs, revealing long-range functional networks that would be obscured in analysis of individual frequency bands. Moreover, the real-time PSD-switching dynamics of specific networks exhibited striking alignment with activities such as conversation and hand movements, revealing a multi-threaded functional network representation of concurrent naturalistic behaviors. Network structures and their relationships to behaviors were stable across days, but were altered during N3 sleep. Our results provide a new framework for understanding real-time, brain-wide neural-network dynamics.
RESUMO
INTRODUCTION: Intracranial epidermoid tumors (ETs) are rare, benign lesions that present significant challenges in neurosurgical management due to their propensity to encase vital neurovascular structures. OBJECTIVE: To evaluate the impact of clinical, demographic, and tumor-specific factors on surgical decisions (gross total resection [GTR] vs. subtotal resection [STR]) and outcomes and identify patient clusters with distinct profiles and outcomes post-resection. METHODS: We retrospectively analyzed 72 epidermoid brain tumor patients treated from 1998 to 2022, employing multivariable logistic regression for GTR vs. STR predictors and Kaplan-Meier curves for progression-free survival (PFS). K-prototype clustering classified patients based on clinical data. RESULTS: The mean age of our cohort was 39.8±20.1 years. 13.9% of patients had a recurrence, with a median PFS of 108 months (IQR 57-206). Seizures significantly predicted GTR (p<0.05), whereas adherence to critical structures reduced GTR likelihood (p<0.05). Initial surgeries more often achieved GTR, correlating with longer PFS (p<0.0001) and reduced recurrence (p<0.01). History of previous ET surgery was predictive of increased recurrent tumor size (p<0.05) and reduced overall PFS (p<0.05). Clustering analysis revealed three clusters with significant differences in recurrence rates (p<0.0001), long term neurological deficits (p<0.05), PFS greater than 10 years (p<0.0001), and significant differences in median PFS between clusters 1 and 3 (p<0.0001) as well as 2 and three (p<0.01). CONCLUSION: This study emphasizes the importance of tailored surgical strategies in managing intracranial ETs, advocating for GTR to optimize long-term outcomes where possible. Future prospective studies are essential to further refine treatment approaches, enhancing survival for ET patients.
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BACKGROUND: Cellular senescence can have positive and negative effects on the body, including aiding in damage repair and facilitating tumor growth. Adamantinomatous craniopharyngioma (ACP), the most common pediatric sellar/suprasellar brain tumor, poses significant treatment challenges. Recent studies suggest that senescent cells in ACP tumors may contribute to tumor growth and invasion by releasing a senesecence-associated secretory phenotype. However, a detailed analysis of these characteristics has yet to be completed. METHODS: We analyzed primary tissue samples from ACP patients using single-cell, single-nuclei, and spatial RNA sequencing. We performed various analyses, including gene expression clustering, inferred senescence cells from gene expression, and conducted cytokine signaling inference. We utilized LASSO to select essential gene expression pathways associated with senescence. Finally, we validated our findings through immunostaining. RESULTS: We observed significant diversity in gene expression and tissue structure. Key factors such as NFKB, RELA, and SP1 are essential in regulating gene expression, while senescence markers are present throughout the tissue. SPP1 is the most significant cytokine signaling network among ACP cells, while the Wnt signaling pathway predominantly occurs between epithelial and glial cells. Our research has identified links between senescence-associated features and pathways, such as PI3K/Akt/mTOR, MYC, FZD, and Hedgehog, with increased P53 expression associated with senescence in these cells. CONCLUSIONS: A complex interplay between cellular senescence, cytokine signaling, and gene expression pathways underlies ACP development. Further research is crucial to understand how these elements interact to create novel therapeutic approaches for patients with ACP.
Assuntos
Senescência Celular , Craniofaringioma , Aprendizado de Máquina , Neoplasias Hipofisárias , Humanos , Craniofaringioma/metabolismo , Craniofaringioma/patologia , Craniofaringioma/genética , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/metabolismo , Neoplasias Hipofisárias/genética , Biomarcadores Tumorais/metabolismo , Biomarcadores Tumorais/genética , Fenótipo , Regulação Neoplásica da Expressão Gênica , Criança , Masculino , FemininoRESUMO
BACKGROUND: Congenital basal meningoceles and encephaloceles are rare pathologies that may present in isolation or with characteristic-associated clinical features. Rarely, children with congenital midline defects may present with massive encephaloceles secondary to anterior cranial fossa agenesis. Traditionally, transcranial approaches with frontal craniotomies were used to reduce the herniated contents and repair the skull base defect. However, high rates of morbidity and mortality associated with craniotomies have favored the development and adoption of less-invasive techniques. OBJECTIVE: To present a novel technique for combined endoscopic endonasal and transpalatal repair of a giant basal meningocele through an extensive sphenoethmoidal skull base defect. METHODS: A representative case of congenital anterior cranial fossa agenesis with a giant meningocele was selected. Clinical and radiological presentations were reviewed, and the intraoperative surgical technique was documented and recorded. RESULTS: A surgical video highlighting each surgical step was included to complement the description of the technique. The surgical outcome from the selected case is also presented. CONCLUSION: This report describes a combined endoscopic endonasal and transpalatal approach to repair an extensive anterior skull base defect with herniation of intracranial content. This technique capitalizes on the advantages of each approach to address this complex pathology.
Assuntos
Fossa Craniana Anterior , Meningocele , Criança , Humanos , Fossa Craniana Anterior/cirurgia , Encefalocele/diagnóstico por imagem , Encefalocele/cirurgia , Meningocele/diagnóstico por imagem , Meningocele/cirurgia , Meningocele/patologia , Base do Crânio/diagnóstico por imagem , Base do Crânio/cirurgia , Endoscopia/métodosRESUMO
Abnormal signaling through the platelet-derived growth factor receptor (PDGFR) has been proposed as a possible mechanism of spinal cord glioma initiation and progression. However, the extent of PDGFR expression in human spinal cord gliomas remains unknown. In this study we perform immunohistochemical analysis of PDGFRα expression in a series of 33 primary intramedullary spinal cord gliomas of different types and grades. PDGFRα was seen to be expressed in a significant subset of these tumors across all major glioma types including ependymoma, oligodendroglioma, pilocytic astrocytoma, astrocytoma, and glioblastoma. These results support the hypothesis that growth factor signaling through the PDGFR may be important for the development of at least a subset of human spinal cord gliomas. Further studies investigating the prognostic significance of PDGFR expression as well as the role of PDGF signaling on the development of intramedullary spinal cord gliomas are warranted.