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Surgical indications for arteriovenous malformations (AVMs) arising at the region of the basal ganglia are questionable and the majority of cases are considered inoperable. Albeit not free from risks of morbidity and mortality, the treatment of choice is usually radiosurgery for small lesions, and embolization plus radiosurgery for larger lesions. Nevertheless, some lesions may be amenable for surgical resection due to their favorable location. Eighteen cases of such AVMs were selected for a direct approach in our series. Seventeen cases were classified as Spetzler and Martin [22] grade III, and one case was a grade V. Ten patients were males and eight females. The mean age was 28.3 years (ranged from 2 to 43 years). Sixteen patients had had previous hemorrhagic events prior to hospital admission. Eleven patients had pre-operative well stablished neurological deficits, and seven patients although symptomatic had a normal neurological exam at admission. Fifteen patients had their AVMs completely resected. Among the eleven patients with previous neurological deficts nine had no change in their pre-operative condition and two experienced postoperative neurological worsening. In the long-term follow-up six patients had a complete recovery and five recovered only partially. Among the seven patients whose neurological examination was normal pre-operatively five remained unchanged, one had a transient motor deficit, and one died due to a thalamic venous infarction and massive bleeding into the thalamus. The anatomical knowledge and the precise localization of the arteriovenous malformation through the aid of neuroimage studies has provided the means to classify these AVMs and plan operative strategies for some small selected cases with relatively low morbidity. [ABSTRACT FROM AUTHOR]

Microsurgery of multiple aneurysms is still a controversial subject. In order to avoid the risk of rebleeding and the consequent increase in morbidity in such cases all aneurysms or at least as many aneurysms as possible should be treated in the first operative procedure. To reach that goal aneurysms located on the contralateral side should also be considered for clipping during the first operation. Between 1984 and 1994 a series of 51 patients harboring multiple aneurysms of which 55 aneurysms were located on the contralateral side of the craniotomy were operated at our institution. No mortality or morbidity could be directly ascribed to the aneurysm that was clipped contralaterally. Based on that series we have described the anatomical features, technical aspects and surgical difficulties of approaching bilateral aneurysms through the same craniotomy. [ABSTRACT FROM AUTHOR]

A pretemporal approach to the interpeduncular and petroclival regions is described. Through a frontotemporal craniotomy based very low in the middle fossa the temporal lobe is completely exposed. The Sylvian, carotid, chiasmatic, and lamina terminalis cisterns are widely opened. The arachnoid fibers between the uncus and the frontal lobe, as well as those binding the temporal lobe to the tentorial edge and to the oculomotor nerve are also separated. The bridging veins from the temporal pole to the spheno-parietal sinus are usually coagulated and sacrificed allowing for posterior displacement of the temporal lobe. The approach combines the advantages of both the classical pterional and subtemporal approaches providing unhindered exposure of the anterior portion of the tentorial incisura in dealing with vascular and tumoural lesions arising at the sellar, parasellar, and interpeduncular regions, and at the superior aspect of the petroclival region. [ABSTRACT FROM AUTHOR]

Alkalinization of the matrix side of the mitochondrial inner membrane by pH shifts from 6.8 to 8.3 caused a reversible increase in current of 3.2 +/- 0.2 pA (mean +/- SE, n = 21) at +/- 40 mV measured using patch-clamp techniques. The current increase was reversed in a graded fashion by the addition of Mg2+ as well as a reduction in pH. Detection of single-channel events was done at 0.5, 1 and 2 M KCl. The single-channel amplitude in 0.15 M KCl corresponds to approximately 15 pS. Reversal potentials derived from whole patch currents indicated that the inner mitochondrial membrane was primarily cation selective at pH 6.8 with a PK/PCl = 32 (n = 6). Treatment with alkaline pH (8.3) increased the current and anion permeability (PK/PCl = 16, n = 6). The membrane becomes completely cation selective when low concentrations (12 microM) of the drug propranolol are added. The amphiphilic drugs amiodarone (4 microM), propranolol (70 microM) and quinine (0.6 mM) blocked almost all of the current. The pH-dependent current was also inhibited by tributyltin. These results are consistent with the presence of two pathways in the inner mitochondrial membrane. One is cation selective and generally open and the other is anion selective and induced by alkaline pH. The alkaline pH-activated channel likely corresponds to the inner membrane anion channel postulated by others from suspension studies. [ABSTRACT FROM AUTHOR]

Intact giant mitochondria isolated from the liver of mice fed a diet containing cuprizone were studied using patch microelectrodes. The current-voltage curves were nonlinear, suggesting the presence of voltage-sensitive channels. In the negative range of voltage, the channels appear to close with increasing magnitude of the voltage. The dependence of the conductance on voltage is similar to that of the outer membrane channels (VDAC) studied in planar bilayers. Occasionally, over a narrow range of positive potentials, the conductance also decreases as in the bilayer studies. However, more frequently the conductance increases sharply in a completely reversible manner at potentials greater than 10 to 20 mV. The increase in conductance with voltage may be interpreted as a major rearrangement of membrane components. Qualitatively comparable results were obtained using fused outer membranes isolated from Neurospora mitochondria. The behavior of VDAC is affected by treatment with succinic anhydride or the polyanion, polymethacrylate, maleate, styrene (1:2:3). We have found similar effects in the negative range of potentials in patches from giant mitochondria treated in the same fashion. [ABSTRACT FROM AUTHOR]

Background: Central nervous system (CNS) tumors are the second most frequent type of neoplasm in childhood and adolescence, after leukemia. Despite the incorporation of molecular classification and improvement of protocols combining chemotherapy, surgery, and radiotherapy, CNS tumors are still the most lethal neoplasm in this age group. Mass spectrometry imaging (MSI) is a powerful tool to map the distribution of molecular species in tissue sections. Among MSI techniques, desorption electrospray ionization (DESI-MSI) has been demonstrated to enable reliable agreement with the pathological evaluation of different adult cancer types, along with an acceptable time scale for intraoperative use. Methods: In the present work, we aimed to investigate the chemical profile obtained by DESI-MSI as an intraoperative surgical management tool by profiling 162 pediatric brain biopsies and reporting the results according to the histopathology and molecular profile of the tumors. Results: The 2D chemical images obtained by DESI-MSI allowed us to distinguish tumor-transformed tissue from non-tumor tissue with an accuracy of 96.8% in the training set and 94.3% in the validation set after statistical modeling of our data using Lasso. In addition, high-grade and low-grade tumors also displayed a distinct chemical profile when analyzed by DESI-MSI. We also provided evidence that the chemical profile of brain tumors obtained by DESI-MSI correlates with methylation-based molecular classes and specific immunophenotypes found in brain biopsies. Conclusions: The results presented herein support the incorporation of DESI-MSI analysis as an intraoperative assistive tool in prospective clinical trials for pediatric brain tumors management in the near future.

Objective: We aim to report the epidemiology, surgical outcomes, and survival rates of pediatric patients with posterior fossa tumors in a large single-center case series., Methods: A retrospective analysis was conducted on pediatric patients who underwent surgical treatment for posterior fossa tumors between January 2011 and January 2019., Results: A total of 135 pediatric patients, with an average age of 7.5 years at diagnosis and a mean follow-up of 35.7 months, were included in the study. Most tumors were located within the midline, with ventriculomegaly observed in 71.4% of the patients. Pilocytic astrocytomas encompassed the majority of tumors (34.1%), followed by medulloblastomas (27.4%) and ependymomas (11.8%). Gross total resection (GTR) was achieved in 71.8% of the patients, with a recurrence rate of 20%. Surgical complications were observed in 25.9% of the patients. GTR significantly impacted 5-year overall survival (OS) and 4-year progression-free survival (PFS) in patients with posterior fossa tumors. Patients who underwent GTR had a 5-year OS of 89.7%, compared to 72.7% for near-total resection and 70.8% for subtotal resection. The 4-year PFS for patients who underwent GTR was 82.5%, whereas it was 63.6% for patients who underwent near-total resection and 54.2% for patients who underwent subtotal resection., Conclusion: Surgical resection remains the main treatment for pediatric posterior fossa tumors, and higher resection rates are linked to better survival outcomes. Despite limited resources for molecular diagnosis, our institution has demonstrated that a specialized neurooncological center with a high surgical volume can still achieve favorable survival outcomes for these patients., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Cancer is a leading cause of death, and the fibrinolytic system shows cooperative effects that facilitate the growth of tumors and the appearance of metastases. This prospective study aimed to evaluate the fibrinolytic potential in cancer patients and its association with mortality outcomes using the fluorometric method of simultaneous thrombin and plasmin generation. The study included 323 cancer patients and 148 healthy individuals. During the 12-month follow-up, 68 patients died. Compared to the control group, cancer patients showed alterations in thrombin production consistent with a hypercoagulability profile, and an increase in plasmin generation. Mortality risk was associated with two parameters of thrombin in both univariate and multivariable analysis: maximum amplitude (Wald 11.78, p < 0.001) and area under the curve (Wald 8.0, p < 0.005), while such associations were not observed for plasmin. In conclusion, this was the first study able to demonstrate the simultaneous evaluation of thrombin and plasmin generation in newly diagnosed untreated cancer patients. Patients with cancer have been observed to exhibit a hypercoagulable profile. During the study, two parameters linked to thrombin generation, MA and AUC, were identified and found to have a potential association with mortality risk. However, no associations were found with parameters related to plasmin generation.

Embryonic tumors share few recurrent mutations, suggesting that other mechanisms, such as aberrant DNA methylation, play a prominent role in their development. The loss of imprinting (LOI) at the chromosome region 11p15 is the germline alteration behind Beckwith-Wiedemann syndrome that results in an increased risk of developing several embryonic tumors. This study analyzed the methylome, using EPIC Beadchip arrays from 99 sporadic embryonic tumors. Among these tumors, 46.5% and 14.6% presented alterations at imprinted control regions (ICRs) 1 and 2, respectively. Based on the methylation levels of ICR1 and ICR2, four clusters formed with distinct methylation patterns, mostly for medulloblastomas (ICR1 loss of methylation (LOM)), Wilms tumors, and hepatoblastomas (ICR1 gain of methylation (GOM), with or without ICR2 LOM). To validate the results, the methylation status of 29 cases was assessed with MS-MLPA, and a high level of agreement was found between both methodologies: 93% for ICR1 and 79% for ICR2. The MS-MLPA results indicate that 15 (51.7%) had ICR1 GOM and 11 (37.9%) had ICR2 LOM. To further validate our findings, the ICR1 methylation status was characterized via digital PCR (dPCR) in cell-free DNA (cfDNA) extracted from peripheral blood. At diagnosis, we detected alterations in the methylation levels of ICR1 in 62% of the cases, with an agreement of 76% between the tumor tissue (MS-MLPA) and cfDNA methods. Among the disagreements, the dPCR was able to detect ICR1 methylation level changes presented at heterogeneous levels in the tumor tissue, which were detected only in the methylome analysis. This study highlights the prevalence of 11p15 methylation status in sporadic embryonic tumors, with differences relating to methylation levels (gain or loss), location (ICR1 or ICR2), and tumor types (medulloblastomas, Wilms tumors, and hepatoblastomas).

Focal cortical dysplasia (FCD) is a brain malformation that causes medically refractory epilepsy. FCD is classified into three categories based on structural and cellular abnormalities, with FCD type II being the most common and characterized by disrupted organization of the cortex and abnormal neuronal development. In this study, we employed cell-type deconvolution and single-cell signatures to analyze bulk RNA-seq from multiple transcriptomic studies, aiming to characterize the cellular composition of brain lesions in patients with FCD IIa and IIb subtypes. Our deconvolution analyses revealed specific cellular changes in FCD IIb, including neuronal loss and an increase in reactive astrocytes (astrogliosis) when compared to FCD IIa. Astrogliosis in FCD IIb was further supported by a gene signature analysis and histologically confirmed by glial fibrillary acidic protein (GFAP) immunostaining. Overall, our findings demonstrate that FCD II subtypes exhibit differential neuronal and glial compositions, with astrogliosis emerging as a hallmark of FCD IIb. These observations, validated in independent patient cohorts and confirmed using immunohistochemistry, offer novel insights into the involvement of glial cells in FCD type II pathophysiology and may contribute to the development of targeted therapies for this condition., (© 2023. Springer Nature Limited.)

Introduction: Focal cortical dysplasia (FCD) is a common cause of pharmacoresistant epilepsy. According to the 2022 International League Against Epilepsy classification, FCD type II is characterized by dysmorphic neurons (IIa and IIb) and may be associated with balloon cells (IIb). We present a multicentric study to evaluate the transcriptomes of the gray and white matters of surgical FCD type II specimens. We aimed to contribute to pathophysiology and tissue characterization., Methods: We investigated FCD II (a and b) and control samples by performing RNA-sequencing followed by immunohistochemical validation employing digital analyses., Results: We found 342 and 399 transcripts differentially expressed in the gray matter of IIa and IIb lesions compared to controls, respectively. Cholesterol biosynthesis was among the main enriched cellular pathways in both IIa and IIb gray matter. Particularly, the genes HMGCS1, HMGCR , and SQLE were upregulated in both type II groups. We also found 12 differentially expressed genes when comparing transcriptomes of IIa and IIb lesions. Only 1 transcript ( MTRNR2L12 ) was significantly upregulated in FCD IIa. The white matter in IIa and IIb lesions showed 2 and 24 transcripts differentially expressed, respectively, compared to controls. No enriched cellular pathways were detected. GPNMB , not previously described in FCD samples, was upregulated in IIb compared to IIa and control groups. Upregulations of cholesterol biosynthesis enzymes and GPNMB genes in FCD groups were immunohistochemically validated. Such enzymes were mainly detected in both dysmorphic and normal neurons, whereas GPNMB was observed only in balloon cells., Discussion: Overall, our study contributed to identifying cortical enrichment of cholesterol biosynthesis in FCD type II, which may correspond to a neuroprotective response to seizures. Moreover, specific analyses in either the gray or the white matter revealed upregulations of MTRNR2L12 and GPNMB, which might be potential neuropathological biomarkers of a cortex chronically exposed to seizures and of balloon cells, respectively., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Assis-Mendonça, Athié, Tamanini, Souza, Zanetti, Araújo, Ghizoni, Tedeschi, Alvim, Almeida, de Souza, Coras, Yasuda, Blümcke, Vieira, Cendes, Lopes-Cendes and Rogerio.)

Most patients with pharmacoresistant mesial temporal lobe epilepsy (MTLE) have hippocampal sclerosis on the postoperative histopathological examination. Although most patients with MTLE do not refer to a family history of the disease, familial forms of MTLE have been reported. We studied surgical specimens from patients with MTLE who had epilepsy surgery for medically intractable seizures. We assessed and compared gene expression profiles of the tissue lesion found in patients with familial MTLE ( n = 3) and sporadic MTLE ( n = 5). In addition, we used data from control hippocampi obtained from a public database ( n = 7). We obtained expression profiles using the Human Genome U133 Plus 2.0 (Affymetrix) microarray platform. Overall, the molecular profile identified in familial MTLE differed from that in sporadic MTLE. In the tissue of patients with familial MTLE, we found an over-representation of the biological pathways related to protein response, mRNA processing, and synaptic plasticity and function. In sporadic MTLE, the gene expression profile suggests that the inflammatory response is highly activated. In addition, we found enrichment of gene sets involved in inflammatory cytokines and mediators and chemokine receptor pathways in both groups. However, in sporadic MTLE, we also found enrichment of epidermal growth factor signaling, prostaglandin synthesis and regulation, and microglia pathogen phagocytosis pathways. Furthermore, based on the gene expression signatures, we identified different potential compounds to treat patients with familial and sporadic MTLE. To our knowledge, this is the first study assessing the mRNA profile in surgical tissue obtained from patients with familial MTLE and comparing it with sporadic MTLE. Our results clearly show that, despite phenotypic similarities, both forms of MTLE present distinct molecular signatures, thus suggesting different underlying molecular mechanisms that may require distinct therapeutic approaches.

Major histocompatibility complex class I (MHC-I) has been implicated in several types of neuroplasticity phenomena. Interferon beta-1b (IFN-β) increases MHC-I expression by motoneurons after sciatic nerve crush in mice, improving axonal growth and functional recovery. Additionally, IFN-β induces glial hypertrophy associated with upregulation of glial fibrillary acidic protein (GFAP) and MHC-I in murine astrocytes in vitro. As knowledge about MHC-I and its role in synaptic plasticity in human astrocytes (HAs) is scarce, we investigated these aspects in mature HAs obtained from the neocortex of patients undergoing surgery due to hippocampal sclerosis. Cells were exposed to media in the absence (0 IU/ml) or presence of IFN-β for 5 days (500 IU/ml). Beta-2 microglobulin (β2m), a component of the MHC-I, GFAP and vimentin proteins, was quantified by flow cytometry (FC) and increased by 100%, 60% and 46%, respectively, after IFN-β exposure. We also performed qRT-PCR gene expression analyses for β2m, GFAP, vimentin, and pro- and anti-inflammatory cytokines. Our data showed that IFN-β-treated astrocytes displayed β2m and GFAP gene upregulation. Additionally, they presented a proinflammatory profile with increase in the IL-6 and IL-1β genes and a tendency to upregulate TNF-α. Moreover, we evaluated the effect of HAs conditioned medium (CM) on the formation/maintenance of neurites/synapses by the PC12 lineage. Synaptophysin protein expression was quantified by FC. The CM of IFN-β-activated astrocytes was not harmful to PC12 neurites, and there was no change in synaptophysin protein expression. Therefore, IFN-β activated HAs by increasing GFAP, vimentin and MHC-I protein expression. Like MHC-I modulation and astrocyte activation may be protective after peripheral nerve damage and in some neurodegenerative conditions, this study opens perspectives on the pathophysiological roles of astroglial MHC-I in the human CNS., Competing Interests: Declaration of competing interest The authors have no relevant financial or nonfinancial interests to disclose., (Copyright © 2022 International Society of Differentiation. Published by Elsevier B.V. All rights reserved.)

Focal cortical dysplasia is a highly epileptogenic cortical malformation with few treatment options. Here, we generated human cortical organoids from patients with focal cortical dysplasia type II. Using this human model, we mimicked some focal cortical dysplasia hallmarks, such as impaired cell proliferation, the presence of dysmorphic neurons and balloon cells, and neuronal network hyperexcitability. Furthermore, we observed alterations in the adherens junctions zonula occludens-1 and partitioning defective 3, reduced polarization of the actin cytoskeleton, and fewer synaptic puncta. Focal cortical dysplasia cortical organoids showed downregulation of the small GTPase RHOA, a finding that was confirmed in brain tissue resected from these patients. Functionally, both spontaneous and optogenetically-evoked electrical activity revealed hyperexcitability and enhanced network connectivity in focal cortical dysplasia organoids. Taken together, our findings suggest a ventricular zone instability in tissue cohesion of neuroepithelial cells, leading to a maturational arrest of progenitors or newborn neurons, which may predispose to cellular and functional immaturity and compromise the formation of neural networks in focal cortical dysplasia., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Objectives: We compared the proteomic signatures of the hippocampal lesion induced in three different animal models of mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE+HS): the systemic pilocarpine model (PILO), the intracerebroventricular kainic acid model (KA), and the perforant pathway stimulation model (PPS)., Methods: We used shotgun proteomics to analyze the proteomes and find enriched biological pathways of the dorsal and ventral dentate gyrus (DG) isolated from the hippocampi of the three animal models. We also compared the proteomes obtained in the animal models to that from the DG of patients with pharmacoresistant MTLE+HS., Results: We found that each animal model presents specific profiles of proteomic changes. The PILO model showed responses predominantly related to neuronal excitatory imbalance. The KA model revealed alterations mainly in synaptic activity. The PPS model displayed abnormalities in metabolism and oxidative stress. We also identified common biological pathways enriched in all three models, such as inflammation and immune response, which were also observed in tissue from patients. However, none of the models could recapitulate the profile of molecular changes observed in tissue from patients., Significance: Our results indicate that each model has its own set of biological responses leading to epilepsy. Thus, it seems that only using a combination of the three models may one replicate more closely the mechanisms underlying MTLE+HS as seen in patients., (© 2022 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate., Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation., Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental)., Results: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01)., Conclusions: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

Hippocampal sclerosis (HS) is a common cause of pharmacoresistant focal epilepsy. Here, we (1) performed a histological approach to the anterior temporal pole of patients with HS to evaluate cortical and white matter (WM) cell populations, alteration of myelin integrity and markers of neuronal activity, and (2) correlated microscopic data with magnetic resonance imaging (MRI) findings. Our aim was to contribute with the understanding of neuroimaging and pathophysiological mechanisms of temporal lobe epilepsy (TLE) associated with HS. We examined MRIs and surgical specimens from the anterior temporal pole from TLE-HS patients ( n = 9) and compared them with 10 autopsy controls. MRIs from healthy volunteers ( n = 13) were used as neuroimaging controls. Histological techniques were performed to assess oligodendrocytes, heterotopic neurons, cellular proliferative index, and myeloarchitecture integrity of the WM, as well as markers of acute (c-fos) and chronic (ΔFosB) activities of neocortical neurons. Microscopic data were compared with neuroimaging findings, including T2-weighted/FLAIR MRI temporopolar blurring and values of fractional anisotropy (FA) from diffusion-weighed imaging (DWI). We found a significant increase in WM oligodendrocyte number, both in hematoxylin and eosin, and in Olig2-stained sections. The frequencies of oligodendrocytes in perivascular spaces and around heterotopic neurons were significantly higher in patients with TLE-HS compared with controls. The percentage of 2',3'-cyclic-nucleotide 3'-phosphodiesterase (CNPase; a marker of myeloarchitecture integrity) immunopositive area in the WM was significantly higher in TLE-HS, as well as the numbers of c-fos- and ΔFosB-immunostained neocortical neurons. Additionally, we demonstrated a decrease in axonal bundle integrity on neuroimaging, with a significant reduction in the FA in the anterior temporal pole. No differences were detected between individuals with and without temporopolar blurring on visual MRI analysis, considering the number of oligodendroglial cells and percentage of WM CNPase-positive areas. Also, there was no relationship between T2 relaxometry and oligodendrocyte count. In conclusion, our histopathological data support the following: (1) the hypothesis that repetitive neocortical neuronal activity could induce changes in the WM cellular constitution and myelin remodeling in the anterior temporal pole from patients with TLE-HS, (2) that oligodendroglial hyperplasia is not related to temporal blurring or T2 signal intensity on MRI, and (3) that reduced FA is a marker of increase in Olig2-immunopositive cells in superficial temporopolar WM from patients with TLE-HS., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Zaidan, Cardoso, Campos, Silva, Coelho, Silveira, Amorim, Alvim, Tedeschi, Yasuda, Ghizoni, Cendes and Rogerio.)

Objective: We aimed to better characterize the magnetic resonance imaging (MRI) findings of mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE), a rare clinicopathological entity associated with pharmacoresistance recently described in patients with frontal lobe epilepsy., Methods: We studied 12 patients who underwent epilepsy surgery and whose surgical specimens showed histopathological findings of MOGHE, characterized by preserved cortical lamination, blurred gray-white matter interface due to increased number of oligodendrocytes, and heterotopic neurons in the white matter. The age at MRI evaluation ranged from 11 to 58 years, except for one 4.5-year-old patient., Results: Following a detailed MRI analysis using an in-house protocol, we found abnormalities in all cases. The lesion was circumscribed in the frontal lobe in six (50%) and in the temporal lobe in three (25%) patients. In the remaining three patients (25%), the lesion was multilobar (frontotemporal and temporoparieto-occipital). Cortical thickening was mild in all patients, except in the 4.5-year-old patient, who had pronounced cortical thickening and white matter blurring. We also identified cortical/subcortical hyperintense T2/fluid-attenuated inversion recovery signal associated with gray/white matter blurring in all but one patient. When present, cleft cortical dimple, and deep sulci aided in localizing the lesion. Overall, the MRI findings were like those in focal cortical dysplasia (FCD) Type IIa. Surgical outcome was excellent in five patients (Engel Class I in 25% and II in 17%). The remaining seven patients (58%) had worthwhile seizure reduction (Engle Class III). Incomplete lesion resection was significantly associated with worse outcomes., Significance: MRI findings associated with MOGHE are similar to those described in FCD Type IIa. Although more frequent in the frontal lobe, MOGHE also occurred in the temporal lobe or involved multiple lobes. Multilobar or extensive MOGHE MRI lesions are associated with less favorable surgical outcomes. Because this is a rare condition, multicenter studies are necessary to characterize MOGHE further., (© 2021 International League Against Epilepsy.)

Objective: This study's objective was to compare the transinsular (TI-AH), transuncus (TU-AH), and temporopolar (TP-AH) amygdalohippocampectomy approaches regarding seizure control, temporal stem (TS) damage, and neurocognitive decline., Methods: We included 114 consecutive patients with unilateral hippocampal sclerosis (HS) who underwent TI-AH, TU-AH, or TP-AH between 2002 and 2017. We evaluated seizure control using Engel classification. We used diffusion tensor imaging and postoperative Humphrey perimetry to assess the damage of the TS. We also performed pre- and postoperative memory performance and intelligence quotient (IQ)., Results: There were no significant differences in the proportion of patients free of disabling seizures (Engel IA+IB) among the three surgical approaches in the survival analysis. However, more patients were free of disabling seizures (Engel IA+IB) at 2 years of postsurgical follow-up with TP-AH (69.5%) and TI-AH (76.7%) as compared to the TU-AH (43.5%) approach (p = .03). The number of fibers of the inferior fronto-occipital fasciculus postoperatively was reduced in the TI-AH group compared with the TU-AH and TP-AH groups (p = .001). The rate of visual field defects was significantly higher with TI-AH (14/19, 74%) in comparison to the TU-AH (5/15, 33%) and TP-AH (13/40, 32.5%) approaches (p = .008). Finally, there was a significant postoperative decline in verbal memory in left-sided surgeries (p = .019) and delayed recall for both sides (p < .001) regardless of the surgical approach. However, TP-AH was the only group that showed a significant improvement in visual memory (p < .001) and IQ (p < .001) for both right- and left-sided surgeries., Significance: The TP-AH group had better short-term seizure control than TU-AH, a lower rate of visual field defects than TI-AH, and improved visual memory and IQ compared to the other groups. Our findings suggest that TP-AH is a better surgical approach for temporal lobe epilepsy with HS than TI-AH and TU-AH., (© 2021 International League Against Epilepsy.)

Background: Despite the various treatment protocols available, survival evaluation is a fundamental criterion for the definition of surgical management; there are still many inconsistencies in the literature on this topic, especially in terms of the value of surgery and its morbidity in patients with very short survival., Objective: The objective was to analyze the association of clinical, oncological, and surgical factors in the survival of patients undergoing spinal surgery for spinal metastases (SM)., Materials and Methods: A retrospective cohort of forty patients who were surgically treated at our institution for SM between 2010 and 2018 were included in the study. We applied the prognostic scales of Tomita and Tokuhashi in each patient and evaluated the systemic status using Karnofsky Performance Scale (KPS) and Eastern Cooperative Oncology Group Performance Scale. Survival rate in months was estimated using the Kaplan-Meier curve, with death considered as primary outcome and, for the evaluation of the association between the variables, the Chi-square test, Fisher's exact test, or Fisher-Freeman-Halton test was applied for better survival. The level of statistical significance was considered as 5% ( P ≤ 0.05)., Results: The mean survival was 8.4 months. Patients with KPS <70 had a mean survival of 6.36 months, while those with KPS >70 had a mean survival of 14.48 months ( P = 0.04). The mean survival of patients classified as ECOG 2 was 7.05 months (95% confidence interval [CI]: 3.4-10.7), and that of patients classified as ECOG 3 and 4 was 1.24 months (95% CI: 0.8-1.59). The mean survival rate among the patients with unresectable metastases in other organs was 6.3 months (95% CI: 3.9-8.9), while the survival rate of those who did not have metastases was 13.8 months (95% CI: 10.0-17.68; P = 0.022)., Conclusion: Survival was associated with the preoperative functional status defined by the KPS and ECOG scales and with the presence of nonresectable visceral metastases., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Craniovertebral Junction and Spine.)

Study Design: Retrospective cohort., Objective: Evaluate the epidemiology of surgical patients with spinal metastases, identify the complications, and evaluate their neurological prognoses., Summary of Background Data: The development of new oncological treatments and screening tests have increased the survival of oncologic patients, and consequently, the incidence of metastatic lesions of the spine., Methods: Retrospective cohort of 40 patients surgically treated at the Hospital de Clínicas of UNICAMP for spinal metastases from January 2010 to September 2018, after diagnosis of symptomatic spinal cord compression and/or mechanical instability of the spine. Retrospectively analyzed patient charts applied the SINS score to evaluate the presence of mechanical instability. Neurological function was classified based on the Frankel index preoperative and postoperatively. To evaluate the association between variables, the Chi-square test, Fisher exact test, or Fisher-Freeman-Halton test was applied. For evaluating the improvement of neurological status between the Frankel scores before and after surgery, the McNemar test was applied for categorical and qualitative variables. In both the tests, variables with values of P > 0.05 were considered., Results: Pain as the reason for the first visit presented an odds ratio (OR) = 2.44 (95% [CI]: 1.14-5.2) for instrumentation need (P = 0.024). A higher SINS score corresponded to the indication for instrumentation surgery due to the instability of the spine (P = 0.004). Within 30 days postoperative, five patients (11.1%) had complications. There was a statistically significant neurological improvement in patients who underwent surgery (P = 0.002)., Conclusion: Pain as the first symptom was related to mechanical instability of the spine and surgical instrumentation. Patients treated with surgery presented improvement of the neurological function in the postoperative period., Level of Evidence: 3.

Objective: The objective of this study was to evaluate the efficacy and safety of a modified surgical approach for the treatment of temporal lobe epilepsy secondary to hippocampal sclerosis (HS). This modified approach, called temporopolar amygdalohippocampectomy (TP-AH), includes a transsylvian resection of the temporal pole and subsequent amygdalohippocampectomy utilizing the limen insula as an anatomical landmark., Methods: A total of 61 patients who were diagnosed with HS and underwent TP-AH between 2013 and 2017 were enrolled. Patients performed pre- and postoperative diffusion tensor imaging and were classified according to Engel's scale for seizure control. To evaluate the functional preservation of the temporal stem white-matter fiber tracts, the authors analyzed postoperative Humphrey perimetries and pre- and postoperative neurocognitive performance (Rey Auditory Verbal Learning Test [RAVLT], Weschler Memory Scale-Revised [WMS-R], intelligence quotient [IQ], Boston Naming Test [BNT], and semantic and phonemic fluency). Demographic data and surgical complications were also recorded and described., Results: After a median follow-up of 36 ± 16 months, 46 patients (75.4%) achieved Engel class I, of whom 37 (60.6%) were Engel class IA. No significant changes in either the inferior frontooccipital fasciculus and optic radiation tractography were observed postoperatively for both left- and right-side surgeries. Reliable perimetry was obtained in 40 patients (65.6%), of whom 27 (67.5%) did not present any visual field defects (VFDs) attributable to surgery, while 12 patients (30%) presented with quadrant VFD, and 1 patient (2.5%) presented with hemifield VFD. Despite a significant decline in verbal memory (p = 0.007 for WMS-R, p = 0.02 for RAVLT recognition), there were significant improvements in both IQ (p < 0.001) and visual memory (p = 0.007). Semantic and phonemic fluency, and scores on the BNT, did not change postoperatively., Conclusions: TP-AH provided seizure control similar to historical temporal lobe approaches, with a tendency to preserve the temporal stem and a satisfactory incidence of VFD. Despite a significant decline in verbal memory, there were significant improvements in both IQ and visual memory, along with preservation of executive function. This approach can be considered a natural evolution of the selective transsylvian approach.

Objective: Mesial temporal lobe epilepsy (MTLE) is the most common type of focal epilepsy in adolescents and adults, and in 65% of cases, it is related to hippocampal sclerosis (HS). Selective surgical approaches to the treatment of MTLE have as their main goal resection of the amygdala and hippocampus with minimal damage to the neocortex, temporal stem, and optic radiations (ORs). The object of this study was to evaluate late postoperative imaging findings on the temporal lobe from a structural point of view., Methods: The authors conducted a retrospective evaluation of all patients with refractory MTLE who had undergone transsylvian selective amygdalohippocampectomy (SAH) in the period from 2002 to 2015. A surgical group was compared to a control group (i.e., adults with refractory MTLE with an indication for surgical treatment of epilepsy but who did not undergo the surgical procedure). The inferior frontooccipital fasciculus (IFOF), uncinate fasciculus (UF), and ORs were evaluated on diffusion tensor imaging analysis. The temporal pole neocortex was evaluated using T2 relaxometry., Results: For the IFOF and UF, there was a decrease in anisotropy, voxels, and fibers in the surgical group compared with those in the control group (p < 0.001). An increase in relaxometry time in the surgical group compared to that in the control group (p < 0.001) was documented, suggesting gliosis and neuronal loss in the temporal pole., Conclusions: SAH techniques do not seem to totally preserve the temporal stem or even spare the neocortex of the temporal pole. Therefore, although the transsylvian approaches have been considered to be anatomically selective, there is evidence that the temporal pole neocortex suffers structural damage and potentially functional damage with these approaches.

Purpose: This study assessed whether optic radiations (OR) microstructure after temporal lobe epilepsy (TLE) surgery correlated with visual field defects (VFD)., Methods: Patients were subjected to diffusion tensor imaging (DTI) tractography of the OR and Humphrey perimetry after TLE surgery. We used Spearman's test to verify correlations between tractographic parameters and perimetry mean deviation. Tractographic variables were compared between patients with VFD or intact perimetry. Multiple logistic regression was applied between DTI and perimetry values. DTI sensitivity and specificity were assessed with a receiver operating characteristic (ROC) curve to evaluate VFD., Results: Thirty-nine patients had reliable perimetry and OR tractography. There was a significant correlation between (1) fractional anisotropy (FA) and both total (rho = 0.569, p = 0.0002) and quadrant (rho = 0.453, p = 0.0037) mean deviation and (2) radial diffusivity and total mean deviation (rho = - 0.350, p = 0.0286). There was no other significant correlation. Patients with VFD showed a significantly lower FA compared with patients with normal perimetry (p = 0.0055), and a 0.01 reduction in FA was associated with a 44% increase in presenting VFD after surgery (confidence interval, CI = 1.10-1.88; p = 0.0082). Using a FA of 0.457, DTI tractography showed a specificity of 95.2% and a sensitivity of 50% to detect VFD after surgery (area under the curve = 0.7619, CI = 0.6020-0.9218)., Conclusion: The postoperative OR microstructure correlated with visual loss after epilepsy surgery. DTI postoperative OR tractography may be helpful in evaluating VFD.

Objective: Many repair techniques have been proposed to treat large myelomeningocele (MMC), and although effective in many cases, some of these techniques can be complex and time consuming, with complications such as cerebrospinal fluid (CSF) leakage, flap loss, tip necrosis, and wound dehiscence. The purpose of this study was to analyze cases of large skin defects and the methods applied and to report the outcomes of the keystone design perforator island flap (KDPIF) technique for large MMC closure., Methods: The authors performed a retrospective review of all neonatal patients who had undergone KDPIF for MMC closure in the period from 2013 to 2018. All patients had a diagnosis of lumbosacral MMC based on obstetric ultrasound. The neurosurgeons and plastic surgeons had selected the cases after concluding that primary closure would be unlikely. The design of the flap is based on the randomly located vascular perforators, creating two identical opposing flaps to fashion a double keystone flap. During wound closure, V-Y advancement of each end of the double flap in the longitudinal axis creates redundancy in the central portion of the flap and reduces the horizontal tension. After discharge, both the neurosurgery and plastic surgery teams followed up all patients, tracking the results with photography., Results: No skin flap dehiscence or necrosis, infection, or CSF leakage was detected, proving the reliability of the flap. One of the patients required further surgery for the large skin defects after insufficient intrauterine closure of the MMC and successfully underwent KDPIF treatment. Another patient (14.3%) had severe neonatal sepsis, which ultimately led to death. A ventriculoperitoneal shunt was required after the skin defect repair in 5 (83.3%) of the 6 surviving patients. Exceptional aesthetic results were achieved for all patients during the follow-up., Conclusions: The KDPIF technique is based on well-known vascular perforators of the intercostal, lumbar, and gluteal regions. Wound tension is widely distributed by the flap and, as a consequence, relevant tissue bulk, reliable vascularity, and important geometrical versatility are provided. In addition, most of the muscles and fascia are preserved, which is another advantage in terms of minimizing secondary morbidity to local tissue rearrangement. The use of KDPIF closure was successfully shown to be a viable alternative for more complex MMCs that present with large skin defects.

Angiogenesis, induced by the vascular endothelial growth factor A through its ligation to the vascular endothelial growth receptor 2, has been described as a crucial point in high-grade glioma development. The aim of this study was to evaluate the influence of VEGFA -2578C/A, -2489C/T, -1154G/A, -634G/C, and -460C/T, and KDR -604T/C, -271G/A, +1192G/A, and +1719A/T single-nucleotide polymorphisms on risk and clinicopathological aspects of high-grade glioma. This case-control study enrolled 205 high-grade glioma patients and 205 controls. Individuals with VEGFA -2578 CC or CA, VEGFA -1154 GG, VEGFA -634 GC or CC, and VEGFA -460 CT or TT genotypes were under 2.56, 1.53, 1.54, and 1.84 increased risks of high-grade glioma, compared to others, respectively. And 1.61, 2.66, 2.52, 2.53, and 2.02 increased risks of high-grade glioma were seen in individuals with VEGFA -2578 CC plus VEGFA -1154 GG, VEGFA -2578 CC or CA plus VEGFA -634 GC or CC, VEGFA -2578 CC or CA plus VEGFA -460 CT or TT, VEGFA -1154 GG or GA plus VEGFA -634 GC or CC, and VEGFA 634 GC or CC plus VEGFA -460 CT or TT combined genotypes, respectively, when compared to others. The "CAGT" haplotype of KDR single-nucleotide polymorphisms was more common in patients with grade IV than in those with grade III tumors, and individuals carrying this haplotype were at 1.76 increased risk of developing grade IV tumors than others. We present, for the first time, preliminary evidence that VEGFA -2578C/A and VEGFA -1154G/A single-nucleotide polymorphisms increases high-grade glioma risk, and "CAGT" haplotype of the KDR gene alters high-grade glioma aggressiveness and risk of grade IV tumors in Brazil.

Objective: To ascertain the safety, effectiveness and reproducibility of screening potential neurosurgical patients by means of smartphones., Methods: This is a retrospective and multicentric study. Data were collected from the medical records of patients subjected to real emergency neurosurgical evaluations and compared with assessments by neurosurgeons using smartphones to determine the feasibility of identifying changes in cranial computed tomography scans, potentially serious conditions of patients, and the need for transfer to reference centers., Results: We analyzed 232 cases. The main diagnosis was traumatic brain injury, with 119 cases (51.3%). Of this, 105 (45.3%) patients were discharged immediately after the assessment. The telemedicine evaluators presented 95.69% accuracy in the identification of changes in computed tomography scans, with 0.858 concordance. Accuracy in the identification of severity was 95.26%, with 0.858 concordance. As for procedure, the concordance among evaluators was 0.672, increasing to 100% in cases that required surgical treatment., Conclusion: Our study indicated that the use of telemedicine for screening patients with acute neurological disorders was safe, effective and reproducible. Implementation of the method shows a promising potential to improve the patient's outcome by reducing unnecessary transfers and decreasing the time elapsed until a specialist can be consulted.

Objective: To present the surgical results of patients who underwent axis screw instrumentation, discussing surgical nuances and complications of the techniques used., Methods: Retrospective case-series evaluation of patients who underwent spinal surgery with axis instrumentation using screws., Results: Sixty-five patients were included in this study. The most common cause of mechanical instability was spinal cord trauma involving the axis (36 patients - 55.4%), followed by congenital craniocervical malformation (12 patients - 18.5%). Thirty-seven (57%) patients required concomitant C1 fusion. Bilateral axis fixation was performed in almost all cases. Twenty-three patients (35.4%) underwent bilateral laminar screws fixation; pars screws were used in twenty-two patients (33.8%), and pedicular screws were used isolated in only three patients (4.6%). In fourteen patients (21.5%), we performed a hybrid construction. There was no neurological worsening nor vertebral artery injury in this series., Conclusion: Axis screw instrumentation proved to be a safe and efficient method for cervical stabilization. Laminar and pars screws were the most commonly used.

Epilepsy is a common disease presenting with recurrent seizures. Hippocampal sclerosis (HS) is the commonest histopathological alteration in patients with temporal lobe epilepsy (TLE) undergoing surgery. HS physiopathogenesis is debatable. We have recently studied, by using mass spectrometry-based proteomics, an experimental model of TLE induced by electrical stimulation. Specifically, protein expressions of both the beta subunit of mitochondrial ATP synthase (ATP5B) and of membrane ATPases were found to be reduced. Here, we investigated tissue distribution of ATP5B and sodium/potassium-transporting ATPase subunit alpha-3 (NKAα3), a protein associated with neuromuscular excitability disorders, in human hippocampi resected "en bloc" for HS treatment (n = 15). We used immunohistochemistry and the stained area was digitally evaluated (increase in binary contrast of microscopic fields) in the hippocampal sectors (CA1-CA4) and dentate gyrus. All HS samples were classified as Type 1, according to the International League Against Epilepsy (ILAE) 2013 Classification (predominant cell loss in CA1 and CA4). ATP5B was significantly decreased in all sectors and dentate gyrus of HS patients compared with individuals submitted to necropsy and without history of neurological alterations (n = 10). NKAα3 expression showed no difference. Moreover, we identified a negative correlation between frequency of pre-operative seizures and number of neurons in CA1. In conclusion, our data showed similarity between changes in protein expression in a model of TLE and individuals with HS. ATP5B reduction would be at least in part due to neuronal loss. Future investigations on ATP5B activity could provide insights into the process of such cell loss.

Purpose: Understand the variables that could interfere with diagnosis and prompt treatment in CNS childhood cancer in Brazil, a developing country with continental dimensions., Methods: From 2005 to 2010, we retrospectively evaluated factors, which could represent a negative influence on the time period elapsing from the onset of symptoms until the diagnosis of the central nervous system (CNS) neoplasia in children and adolescents attended in our service., Results: Two hundred seventeen records were analyzed retrospectively. Factors of the households were evaluated, and this data was related to the time period elapsing from presentation of the first symptoms until the diagnosis of CNS neoplasia. The average time elapsed from the onset of the symptoms until seeking medical assistance was 96 days, and from medical assistance to patient referral to a reference service was 33 days. The symptoms which most contributed to a shorter delay in diagnosis were changes in gait and paresis, mother's occupation, father's education level, patient gender, and living in the state of São Paulo. Besides that, variables such as male gender, mother's education level, and lower patient age were associated with an early diagnosis time., Conclusion: There is great difficulty in performing early diagnosis of CNS tumors, partly due to parent's inability to recognize signs and symptoms, and in part due to an educational deficit among healthcare professionals. Identification of measures that can minimize these causes of delay is fundamental to increasing the chance of cure and survival of these patients.

Background: The objective was to evaluate if there is an association of the spino-pelvic relationships and the global spinal alignment with the outcome of AO type A injuries treated nonsurgically., Methods: This is a retrospective case series. Patients treated nonsurgically for AOSpine type A fractures (T1-L5) with at least 1 year follow-up identified. A standing antero-posterior and lateral 36-inch radiographs and measures of spino-pelvic relationships and sagittal alignment were obtained, as well as clinical assessment using the visual analog scale, the Short-Form 36 (SF-36) questionnaire, the Oswestry Disability Index (ODI), and labor status., Results: Twenty-two patients with 33 fractures were included (L1 was the most injured level with 18.2%). There were 17 men (77.2%) and the mean age was 47.1 years. Follow-up ranged from 12 to 60 months (mean of 27.8 months). There were 22 type A1 (66.7%), 3 type A2 (9%), 6 type A3 (18%), and 2 type A4 (6%) fractures. The ODI ranged from 4% to 58%, with a mean of 24.4%. The SF-36 physical health score ranged from 23 to 82.25 (mean 49.59), and the mental health score ranged from 14.75 to 94.25 (mean 63.28). No association was identified between the spino-pelvic measurements, global alignment, and patient-reported outcomes., Conclusions: Type A fractures had a clinically relevant amount of long-term disability even when surgical treatment is not required. Spino-pelvic relationships and final global spinal alignment did not associate with outcome measurements., Competing Interests: Disclosures and COI: This paper was supported by FAPESP – Fundação de Amparo a Pesquisa de São Paulo – process 2015/09848-8. No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this manuscript. The authors have no financial interest in the subject of this article. The manuscript submitted does not contain information about medical device(s).

Introduction: Congenital disorders of the craniovertebral junction (CVJ) include a wide range of conditions, such as Chiari malformation (CM), basilar Invagination (BI), and atlantoaxial dislocation (AAD). The objective of this paper is to critically review the literature related to the management of congenital CVJ disorders focusing on: the significant developments in the past (from anterior open ventral decompression to modern CVJ realignment); the diagnosis of CVJ instability; the role of atlantoaxial fixation without posterior fossa decompression in patients with tonsillar herniation and no evidence of CVJ instability; use of C1-2 inter-articular spacers and use of C1-2 interarticular spacers with intra-operative manipulations to correct BI with AAD along with its deformity., Materials and Methods: We performed a review of articles showing up on PubMed database without time restriction. Articles were included according to the purpose of our review and selected by two authors (AFJ and PSC)., Results: CVJ instability may be inferred when there is atlantoaxial abnormal motion seen on dynamic studies, facet joint subluxation or severe symptomatic CVJ kyphosis; routine fixation of patients with CM without clear CCJ instability, while based on an interesting hypothesis, still requires further studies; use of C1-2 inter-articular spacers for re-establishing CVJ alignment is probably the most effective surgery for posterior CVJ realignment and reduction of AAD and BI, potentially avoiding the need for an open or endoscopic anterior odontoidectomy (AO); current development of deformity correcting surgeries and the measurement of joint indices to plan surgery have provided new strategies for treatment., Conclusion: We present a critical review of important new concepts involved in the surgical treatment of CVJ congenital disease., Competing Interests: There are no conflicts of interest

Introduction: Ankylosing spondylitis (AS) is an idiopathic seronegative spondyloartropathy that involves mainly the axial skeleton and the sacroiliac joints. AS promotes biomechanical changes in the spine that predispose to fractures, spinal deformity and spondylodiscitis. The aim of this article is to report the clinical and laboratorial characteristics of patients with AS who underwent spinal surgery at our Institution., Methods: Retrospective review of medical charts of patients who had AS and underwent spinal interventions., Results: Nine patients were found and eight were included in the present study. There were three men and six women and the patients' mean age was 57 years old. All patients had pain at the involved spinal level and one patient had tetraparesis due to cervical myelopathy. Acute-phase proteins were positive in six patients (75%), and HLA-B27 was found in two patients (25%). Four patients had the radiological diagnosis of spondylodiscitis (50%) and underwent a spinal disc biopsy. They were all characterized as having aseptic spondylodiscitis. Three patients were free of pain with analgesics in their last follow-up and one patient had only partial solution of his pain. Three additional patients had spinal fractures surgically treated (37.5%) and one patient was operated because of a cervical kyphotic deformity (12.5%). There were no deaths or surgical complications in this series., Conclusions: the majority of our clinical and laboratories findings were discrepant with the medical literature. These differences may be secondary to regional characteristics or by the fact that our population included only those patients who underwent spinal surgery.

Objective: To compare surgical outcome in mesial temporal lobe epilepsy (MTLE) patients with unilateral hippocampal sclerosis (MTLE-HS) who had or did not have preoperative video-electroencephalographic monitoring (VEEG)., Methods: A prospective study was undertaken with 166 consecutive pharmacoresistant unilateral MTLE-HS patients. All patients were investigated with detailed seizure semiology, serial routine outpatient EEG, magnetic resonance imaging, neuropsychological evaluation, and if necessary, other examinations. Postoperative follow-up ranged between 2 and 16 years. Patients were divided into: (1) patients operated on based on routine outpatient EEG information, with >80% of EEGs with unilateral interictal epileptiform discharges (IEDs) ipsilateral to HS or ictal events (n = 71); and (2) patients submitted to preoperative VEEG (n = 95). To avoid the bias generated by ictal recordings, we performed a subanalysis of: (1) patients without preoperatively ictal recordings (n = 80) and (2) patients with ictal recordings in VEEG or routine outpatient EEG (n = 86)., Results: Groups were similar regarding gender, age at surgery, seizure onset, preoperative seizure frequency, and duration of follow-up. Overall, 136/166 (81.92%) were classified as Engel I seizure outcome, with no difference between groups; 76.84% and 88.73% of patients with and without VEEG, respectively, had Engel I postoperative seizure outcome (P = .77). The time lag until surgery was shorter in the group without VEEG (80 vs 38 months; P = .01). Considering ictal recordings, 76.74% of patients with seizures recorded and 87.50% without ictal recordings had Engel I outcome (P = .11)., Significance: We performed the first prospective study in a tertiary epilepsy center comparing surgical outcomes in unilateral MTLE-HS patients investigated preoperatively with and without VEEG. Based on the surgical outcome, VEEG is not imperative in patients with unilateral MTLE-HS who have compatible semiology and clearly ipsilateralized IEDs evaluated by a multidisciplinary and experienced epilepsy group., (Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.)

Background: Craniosynostosis is a complex disease once it involves deep anatomic perception, and a minor mistake during surgery can be fatal. The objective of this report is to present novel 3-dimensional-printed polyamide craniosynostosis models that can improve the understanding and treatment complex pathologies., Methods: The software InVesalius was used for segmentation of the anatomy image (from 3 patients between 6 and 9 months old). Afterward, the file was transferred to a 3-dimensional printing system and, with the use of an infrared laser, slices of powder PA 2200 were consecutively added to build a polyamide model of cranial bone., Results: The 3 craniosynostosis models allowed fronto-orbital advancement, Pi procedure, and posterior distraction in the operating room environment. All aspects of the craniofacial anatomy could be shown on the models, as well as the most common craniosynostosis pathologic variations (sphenoid wing elevation, shallow orbits, jugular foramen stenosis). Another advantage of our model is its low cost, about 100 U.S. dollars or even less when several models are produced., Conclusions: Simulation is becoming an essential part of medical education for surgical training and for improving surgical safety with adequate planning. This new polyamide craniosynostosis model allowed the surgeons to have realistic tactile feedback on manipulating a child's bone and permitted execution of the main procedures for anatomic correction. It is a low-cost model. Therefore our model is an excellent option for training purposes and is potentially a new important tool to improve the quality of the management of patients with craniosynostosis., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Chiari malformation (CM) is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI). Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation) is posterior fossa decompression, with or without duraplasty. The authors describe in details and in a stepwise fashion the surgical approach of patients with CM as performed at the State University of Campinas, emphasizing technical nuances for minimizing the risks of the procedure and potentially improving patient outcome.

Aims: To assess the validity of an online method to quantitatively evaluate cerebral hypometabolism in patients with pharmacoresistant focal epilepsy as a complement to the visual analysis of the 18 F-FDG positron emission tomography (PET)/CT exam., Methods: A total of 39 patients with pharmacoresistant epilepsy and probable focal cortical dysplasia [22 patients with frontal lobe epilepsy (FLE) and 17 with temporal lobe epilepsy (TLE)] underwent a presurgical evaluation including EEG, video-EEG, MRI, and 18 F-FDG PET/CT. We conducted the automated quantification of their 18 F-FDG PET/CT data and compared the results with those of the visual-PET analysis conducted by experienced nuclear medicine physicians. For each patient group, we calculated Cohen's Kappa coefficient for the visual and quantitative analyses, as well as each method's sensitivity, specificity, and positive and negative predictive values., Results: For the TLE group, both the visual and quantitative analyses showed high agreement. Thus, although the quantitative analysis could be used as a complement, the visual analysis on its own was consistent and precise. For the FLE group, on the other hand, the visual analysis categorized almost half of the cases as normal, revealing very low agreement. For those patients, the quantitative analysis proved critical to identify the focal hypometabolism characteristic of the epileptogenic zone. Our results suggest that the quantitative analysis of 18 F-FDG PET/CT data is critical for patients with extratemporal epilepsies, and especially those with subtle MRI findings. Furthermore, it can easily be used during the routine clinical evaluation of 18 F-FDG PET/CT exams., Significance: Our results show that quantification of 18 F-FDG PET is an informative complementary method that can be added to the routine visual evaluation of patients with subtle lesions, particularly those in the frontal lobes.

Introduction:: Spinal tumors are rare in the pediatric population, presenting many specific peculiarities when compared to adults. We have performed a broad narrative review to describe the most common spinal tumors in children, discussing their main characteristics and management options., Method:: The authors have performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives., Results:: Multimodality radiological studies (plain films, 3D computed tomography scan and magnetic resonance imaging) are necessary for proper evaluation and differential diagnosis of spinal tumors in children. In selected cases nuclear medicine imaging is used to improve the chances of a more accurate diagnosis. As a general rule, a fine needle biopsy is recommended after radiological evaluation to confirm the tumor's histology. Primary bone tumors can be divided into benign bone tumors, mostly represented by vertebral hemangiomas, osteoid osteomas, osteoblastomas, aneurismal bone cysts, and eosinophilic granulomas, and malign or aggressive tumors, such as Ewing's or osteogenic sarcomas. Secondary bone tumors (spinal metastases) comprise different tumor histologies, and treatment is mainly based on tumor's radiosensitivity. The characteristics and treatment options of the main spinal tumors are discussed in details., Conclusion:: Spinal tumors in children are rare lesions that demand a thorough understanding of their main characteristics for their proper management. Understanding the nuances of spinal tumors in children is of paramount importance for improving outcomes and chances of cure.

Background: Various reports have described the transuncus (TU) approach as a selective route to the amygdala and hippocampus, but this approach has not yet been submitted to solid postoperative imaging analysis. The objective of this study was to evaluate the anatomy, surgical technique, postoperative imaging analysis, and outcome in a series of patients with temporal lobe epilepsy who underwent selective amigdalohippocampectomy via a TU approach., Methods: This was a prospective study of 25 consecutive patients who underwent selective amigdalohippocampectomy through a TU approach. The temporal stem and temporal pole were evaluated through different modalities of 3-Tesla magnetic resonance imaging, including tractography of optic radiation (OR), uncinate fascicle, and inferior fronto-occipital fascicle. Visual field analysis was performed with automated perimetry., Results: The mean age was 40 ± 8.21 years, and mean follow-up was 26.44 + 12.58 months. Postoperatively, 21 patients (84%) were classified as Engel I (good seizure control). Diffusion tensor imaging (DTI) data showed that 78.2% of patients had some structural damage to the temporal stem and fibers of the uncinate fascicle were identified postoperatively in only 3 patients (13.04%). The inferior fronto-occipital fascicle was identified in 18 patients (78.3%); however, subsequent DTI analysis of the remaining fibers showed them to be damaged. Integrity of the OR did not differ between these 2 groups., Conclusions: A TU approach is a feasible and efficient approach to selective amigdalohippocampectomy for surgical treatment of temporal lobe epilepsy. Postoperative DTI analysis suggests that a TU approach results in more injury to the temporal stem and its associated white matter fiber tracts than expected by previous anatomic studies; however, it was efficient in preserving OR., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aim: To evaluate the role of dynamic computed tomography (CT) scan imaging in diagnosing craniovertebral junction (CVJ) instability in patients with congenital CVJ malformations., Methods: Patients with symptomatic congenital CVJ malformations who underwent posterior fossa decompression and had a preoperative dynamic CT scan in flexion and extended position were included in this study. Measurements of the following craniometrical parameters were taken in flexed and extended neck position: Atlanto-dental interval (ADI), distance of the odontoid tip to the Chamberlain's line, and the clivus-canal angle (CCA). Assessment of the facet joints congruence was also performed in both positions. Comparison of the values obtained in flexion and extension were compared using a paired Student's t -test., Results: A total of ten patients with a mean age of 37.9 years were included. In flexion imaging, the mean ADI was 1.76 mm, the mean CCA was 125.4° and the mean distance of the odontoid tip to the Chamberlain's line was + 9.62 mm. In extension, the mean ADI was 1.46 mm ( P = 0.29), the mean CCA was 142.2° ( P < 0.01) and the mean distance of the odontoid tip to the Chamberlain's line was + 7.11 mm ( P < 0.05). Four patients (40%) had facetary subluxation demonstrated in dynamic imaging, two of them with mobile subluxation (both underwent CVJ fixation). The other two patients with a fixed subluxation were not initially fixed. One patient with atlantoaxial assimilation and C23 fusion without initial facet subluxation developed a latter CVJ instability diagnosed with a dynamic CT scan. Patients with basilar invagination had a lower CCA variation compared to the whole group., Conclusion: Craniometrical parameters, as well as the visualization of the facets location, may change significantly according to the neck position. Dynamic imaging can provide additional useful information to the diagnosis of CVJ instability. Future studies addressing the relationship between craniometrical changes and neck position are necessary., Competing Interests: Conflict-of-interest statement: The authors declare no conflicts of interest regarding this manuscript.

Objective: To evaluate the safety and reliability of the new AO Classification, a recent classification system for Thoraco-Lumbar Spine Trauma (TLST)., Design: Retrospective study., Methods: We applied the new AO system in patients with TLST treated according to the TLICS. Two researchers classified injuries independently. Eight weeks later, the classification was repeated for intra and inter-observer agreement evaluation. To evaluate safety, we correlated the treatment performed based on the TLICS with the newer AO classification obtained., Results: Fifty-four patients were included in this study, with a mean follow-up of 363.8 days. Twenty-three neurologically intact patients were initially treated conservatively. Their mean TLICS was 1.78 (1-4 points). Four patients underwent late surgery. Thirty-one patients were treated surgically. Their average TLICS was 7.22 points (4-10 points). Agreements in the four independent evaluations according to AO groups and subgroups were of 64.8% (35/54) and 55.5% (30/54) respectively. Kappa index for groups A, B and C was 0.75, 0.7 and 0.85 respectively. Kappa index for subgroups ranged from 0.16 to 0.85. Regarding safety, thirty (57.6%) patients with total subgroups agreement were analyzed. All patients with fracture in groups B and C underwent surgical treatment and patients in group A received surgery according to neurological status or failure of conservative treatment., Conclusion: The newer AO spine classification demonstrated good reliability at the level of groups. Subgroups demonstrated worse and varying reliability. Although the safety analysis was limited due to the low level of total concordance among all evaluations, patients from group A can be treated conservatively or surgically, whereas those from groups B and C are treated surgically.