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OBJECTIVE Positron emission tomography (PET) imaging has assumed an essential role in the presurgical evaluation of epileptogenic foci in drug-resistant epilepsy by identifying the hypometabolic cerebral cortex. The authors herein designed a pilot study to test a novel technique of PET asymmetry after anatomical symmetrization coregistered to MRI (PASCOM), utilizing interhemispheric metabolic asymmetry on interictal fluorine 18–labeled fluorodeoxyglucose (FDG)-PET to better localize the epileptogenic zone. METHODS The authors analyzed interictal FDG-PET scans from 23 patients with drug-resistant epilepsy, mean (± SD) age 20.9 ± 13.1 years old, who had an Engel class I postsurgical outcome while followed up for > 12 months. T1-weighted and FLAIR MRI were used to create a patient-specific, structurally symmetrical template. The asymmetry index (AI) image was computed to detect the cerebral region of hypometabolism using different z-score threshold criteria to optimize sensitivity and specificity. The detected regions were compared with the resection cavity on postoperative MRI using predefined anatomical labels. PASCOM was compared with the visual analysis of FDG-PET by a nuclear medicine consultant blinded to other clinical data (VIS) and visual analysis during multidisciplinary team discussion (MDT). The efficacy of each technique was compared based on a performance score (S), sensitivity, specificity, and correct lateralization of epileptogenicity. RESULTS The mean S was maximum (1.30 ± 1.23) for AI images when thresholded at z > 4 and retaining the cluster of more than 100 voxels containing the peak AI value (Z4C) with 73.03% sensitivity and 96.43% specificity. The mean S was minimum for VIS (0.27 ± 0.31). The mean sensitivity was maximum for MDT (85.04%) and minimum for Z5C (AI images thresholded at z > 5 and clustered; 59.47%), whereas the mean specificity was maximum for Z5C (97.77%) and minimum for VIS (64.60%). Z3C (AI images thresholded at z > 3 and clustered) and Z4C were able to correctly identify the side of epileptogenicity in all the patients. CONCLUSIONS The PASCOM technique with a Z4C threshold had a maximum performance score with good sensitivity and specificity in localizing and lateralizing the epileptogenic zone. The described technique outperformed the conventional visual analysis of FDG-PET and hence warrants further prospective verification.

The key challenge in epilepsy surgery is precise localization and removal of the epileptogenic zone (EZ) from the brain. Localization of the epileptogenic network by visual analysis of intracranial EEG is extremely difficult. In this retrospective study, we used interictal connectivity and graph theory analysis on intracranial EEG to better delineate the epileptogenic zone. Patients who underwent surgery for drug-refractory mesial temporal and neocortical epilepsy were included. Computational measures, such as h2 nonlinear correlation and mutual information, were used to estimate the interdependency of intracranial EEGs. We observed that the Out-Degree, Out-Strength, and Betweenness centrality (graph properties) were the best predictors of EZ. From the results, we also found that graph properties with a normalized value above 0.75 were found to be a useful measure to localize the EZ with a sensitivity of 87.88 and a specificity of 87.13. Our results also validate that frequently occurring types of interictal fast discharges (IFD) with connectivity measures and graph properties can better localize the EZ. We foresee graph theory analysis of interictal intracranial EEG data can help precise localization of EZ for cortical resection as well as in minimally invasive radiofrequency ablation of epileptogenic hubs. Further, prospective validation is required for clinical use.

BackgroundNanoparticle siRNA-conjugates are promising clinical therapeutics as indicated by recent US-FDA approval. In glioma stem cells (GSC), multiple stemness associated genes were found aberrant. We report intracranially injectable, multi-gene-targeted siRNA nanoparticle gel (NPG) for the combinatorial silencing of 3 aberrant genes, thus inhibiting the tumorogenic potential of GSCs.MethodsNPG loaded with siRNAs targeted against FAK, NOTCH-1, and SOX-2 were prepared by the self-assembly of siRNAs with protamine–hyaluronic acid combination. Electron microscopy, DLS, and agarose gel electrophoresis were used for the physicochemical characterization. Cell transfection and gene-silencing efficiency were studied using human mesenchymal stem cells and rat C6 glioma-derived GSCs. Neurosphere inhibition was tested in vitro using GSCs derived from C6 cell line and glioma patient samples. Patient-derived xenograft model and orthotopic rat glioma model were used to test the effect of NPG on in vivo tumorigenicity.ResultsThe siRNA nanoparticles with an average size ~ 250 nm and ~ 95% loading efficiency showed cellular uptake in ~95.5% GSCs. Simultaneous gene silencing of FAK, NOTCH-1, and SOX-2 led to the inhibition of neurosphere formation by GSCs, whereas normal stem cells remained unaffected and retained neuronal differentiation capability. GBM PDX models manifested significant impairment in the tumorigenic potential of NPG treated GSCs. Intracranial injection of NPG inhibited tumor growth in orthotopic rat brain tumor model.ConclusionIntracranially injectable n-siRNA NPG targeted to multiple stem-cell signaling impairs glioma initiation capabilities of GSCs and inhibited tumor growth in vivo.

OBJECTIVE The purpose of this study was to evaluate the clinical outcome in patients with medically refractory epilepsy who had undergone resective or ablative surgery for suspected insulo-opercular epileptogenic foci. METHODS The prospectively maintained database of patients undergoing epilepsy surgery was reviewed, and all patients who underwent insulo-opercular surgery for medically refractory epilepsy with a minimum of 12 months of postoperative follow-up were identified, excluding those who had insulo-opercular resection in combination with temporal lobectomy. The presurgical electroclinicoradiological data, stereo-EEG (SEEG) findings, resection/ablation patterns, surgical pathology, postoperative seizure outcome, and neurological complications were analyzed. RESULTS Of 407 patients undergoing epilepsy surgery in a 5-year period at the Amrita Advanced Centre for Epilepsy, 24 patients (5.9%) who underwent exclusive insulo-opercular interventions were included in the study. Eleven (46%) underwent surgery on the right side, 12 (50%) on the left side, and the operation was bilateral in 1 (4%). The mean age at surgery was 24.5 ± 12.75 years. Onset of seizures occurred on average at 10.6 ± 9.7 years of life. Characteristic auras were identified in 66% and predominant seizure type was hypermotor (15.4%), automotor (15.4%), hypomotor (11.5%), or a mixed pattern. Seventy-five percent of the seizures recorded on scalp video-EEG occurred during sleep. The 3T MRI results were normal in 12 patients (50%). Direct single-stage surgery was undertaken in 5 patients, and SEEG followed by intervention in 19. Eighteen patients (75%) underwent exclusive resective surgery, 4 (16.7%) underwent exclusive volumetric radiofrequency ablation, and 2 (8.3%) underwent staged radiofrequency ablation and resective surgery. Immediate postoperative neurological deficits occurred in 10/24 (42%), which persisted beyond 12 postoperative months in 3 (12.5%). With a mean follow-up of 25.9 ± 14.6 months, 18 patients (75%) had Engel class I outcome, 3 (12.5%) had Engel class II, and 3 (12.5%) had Engel class III or IV. There was no statistically significant difference in outcomes between MRI-positive versus MRI-negative cases. CONCLUSIONS Surgery for medically refractory epilepsy in insulo-opercular foci is less common and remains a challenge to epilepsy surgery centers. Localization is aided significantly by a careful study of auras and semiology followed by EEG and imaging. The requirement for SEEG is generally high. Satisfactory rates of seizure freedom were achievable independent of the MRI lesional/nonlesional status. Morbidity is higher for insulo-opercular epilepsy surgery compared to other focal epilepsies; hence, the practice and development of minimally invasive strategies for this subgroup of patients undergoing epilepsy surgery is perhaps most important.

As an initial step to develop guidelines for epilepsy monitoring units (EMUs) appropriate for developing countries, we inquired the existing practices in EMUs in India.After checking for the content and face validity as well for clarity, we sent a 52-item online non-anonymized questionnaire to all the 52 EMUs in India.The questionnaire was completed by 51 of the 52 EMUs (98% response rate). The majority of the EMUs are located in major cities and 51% are located in non-governmental corporate hospitals. There are total of 122 prolonged video-EEG monitoring (PVEM) beds in India and 70% EMUs have ≤2 beds. Approximately two-thirds of the EMUs have defined protocols for pre-procedure consent and risk assessment, management of seizure clusters and status epilepticus, continuous observation of patients, and peri‑ictal testing. Only one-third of the EMUs have protocols for management of post-ictal psychosis, anti-suffocation pillows, and protected environment within bathrooms. The waiting period for PVEM is more (49.9 ± 101 vs. 4.9 ± 10.9 days; p = 0.04) and mean cost for 3-day PVEM is less (INR 8311 ± 9021 vs. 30,371 ± 17,563; p0.0001) in public as compared to private hospitals. There was a negative correlation between cost of PVEM and the waiting period (r=-0.386; p = 0.01). Safety practices are similar in public and private hospitals.Although practices in EMUs in India vary widely, they are comparable to those in developed countries. India has severe shortage of EMUs and long waiting lists for affordable PVEM.

BACKGROUND The reevaluation and management of seizure relapse following resective surgery in magnetic resonance imaging (MRI)-negative pharmacoresistant epilepsy remains a significant challenge. OBSERVATIONS A 25-year-old right-handed male with medically refractory epilepsy presented with nonlocalizing electroencephalography (EEG) and MRI. Stereo-EEG (SEEG) implantation based on semiology and positron emission tomography imaging revealed a left frontal opercular focus with rapid bilateral insular ictal synchrony. The initial epileptogenic zone was resected and pathologically proven to be type 2A focal cortical dysplasia (FCD). Seizure relapse after 9 months was eventually reinvestigated, and repeat SEEG revealed a secondary epileptogenic focus in the contralateral insula. A novel technique of volumetric stereotactic radiofrequency ablation (vRFA) was utilized for the right insular focus, following which, the patient remains seizure-free for 20 months. He suffered a transient bilateral opercular syndrome following the second intervention that eventually resolved. LESSONS The authors present clinical evidence to suggest epileptogenic nodes distant from the primary focus as a mechanism for seizure relapse following FCD surgery and the importance of bilateral insular SEEG coverage. The authors also describe a novel technique of minimally invasive vRFA that allows ablation of a larger volume of cerebral cortex when compared to conventional bedside SEEG electrode thermocoagulation.

We report a young female with medically refractory multiple daily seizures since childhood with semiology suggestive of an epileptic nystagmus. She had a large multilobar parieto-temporo-occipital dysplasia and became seizure-free after parieto-temporo-occipital disconnection with preserved visual functions.

Background: Central nervous system (CNS) hemangioblastoma (HB) is the most common tumor in the von Hippel Lindau (VHL) disorder, the hereditary tumor syndrome caused by the biallelic mutations of the VHL gene. The disrupted VHL and Elongin protein interaction on hypoxia-inducible factor-1α (HIF-1α) induces a set of hypoxia-inducible genes, resulting in an unchecked endothelial cell proliferation that then leads to hemangioblastoma formation. However, recent studies have shown that disruptive germline mutations of VHL need not result in hemangioblastoma, though it can cause other manifestations of the VHL syndrome. Similarly, sporadic hemangioblastoma can occur rarely without a somatic biallelic VHL mutation. The VHL protein was earlier found to be associated with the deposition of matrix fibronectin (FN) protein in the renal extracellular matrix. Methods: The present study was designed to investigate the deposition of the matrix FN protein in VHL-associated hemangioblastoma. Results: Seven HB tumor samples from the VHL syndrome had lower expressions of tissue FN compared to the control cerebellum samples or the control blood vessel sample. On comparing the VHL and FN protein expressions in a timed endothelial tube assay, the VHL protein expression was absent during the initial phase of tube formation but started expressing after 6 h. The levels of matrix form of FN gradually increased along with the VHL expression during the maturation of tube formation. Tube formation was found to be enhanced with extraneously added soluble FN and inhibited by matrix FN. Similarly, tube formation was inhibited by a modified tripeptide (RGD) inhibitor of integrin (-αVβ3), namely, Cyclo-Ala-Arg-Gly-Asp-3-aminomethylbenzoyl. Conclusions: Our study implicates that the extracellular deposition and matrix formation of FN is important for vascular endothelial proliferation, and that its absence has roles in the development of hemangioblastoma in the VHL syndrome.

The management of ventriculoperitoneal (VP) shunt failure is a common problem in neurosurgical practice. On occasion, extraperitoneal sites for CSF diversion are required when shunting to the peritoneal cavity has failed after multiple attempts. The authors report a novel minimally invasive procedure allowing cannulation of the ureter for the purpose of ventriculo-ureteral (VU) shunting. Sixteen years prior to presentation, this 46-year-old woman had contracted tuberculous meningitis and had chronic hydrocephalus, with multiple distal shunt failures in recent months. A percutaneous nephrostomy was used to pass the distal catheter based on intraoperative retrograde pyelography. Following successful placement of the VU shunt, the patient's hydrocephalus stabilized and she returned to her regular functional status. The only long-term complication noted within 36 months of follow-up was a transient episode of electrolyte disturbance and dehydration associated with a diarrheal illness that responded to adequate hydration and salt supplementation. By its minimally invasive nature, this approach offers a reasonable extraperitoneal alternative after multiple distal shunt catheter failures have occurred.

The neurofibromatoses, which include neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and schwannomatosis, are a group of syndromes characterized by tumor growth in the nervous system. The RASopathies are a group of syndromes caused by germline mutations in genes that encode components of the RAS/mitogen-activated protein kinase (MAPK) pathway. The RASopathies include NF1, Noonan syndrome, Noonan syndrome with multiple lentigines, Costello syndrome, cardio-facio-cutaneous syndrome, Legius syndrome, capillary malformation arterio-venous malformation syndrome, and SYNGAP1 autism. Due to their common underlying pathogenetic etiology, all these syndromes have significant phenotypic overlap of which one common feature include a predisposition to tumors, which may be benign or malignant. Together as a group, they represent one of the most common multiple congenital anomaly syndromes estimating to affect approximately one in 1000 individuals worldwide. The subcontinent of India represents one of the largest populations in the world, yet remains underserved from an aspect of clinical genetics services. In an effort to bridge this gap, the First International Conference on RASopathies and Neurofibromatoses in Asia: Identification and Advances of New Therapeutics was held in Kochi, Kerala, India. These proceedings chronicle this timely and topical international symposium directed at discussing the best practices and therapies for individuals with neurofibromatoses and RASopathies. Children's Tumor Foundation; Fonds De La Recherche Scientifique ‐ FNRS, Grant/Award Numbers: T002614, T024719F; Manchester NIHR Biomedical Research Centre, Grant/Award Number: IS‐BRC‐1215‐20007; NIH/NIAMS, Grant/Award Number: R01AR062165; Axis Health Biomedicals and the Doctor's Academy; Schiller India; MedGenome; Wales Gene Park; University of Wales Trinity Saint David; Cardiff University; AstraZeneca

35 yr old steroid dependent lady with Pulmonary TB underwent debridement of epidural abscess & posterior stabilization for paraparesis. With histopathology and cultures showing Aspergillus fumigatus, voricanozole was started. By the fourth week, she developed persistent fever, and altered mental status. Brain MRI and CSF study including multiplex PCR evaluation confirmed cerebral aspergillosis. Voricanozole was changed to intravenous lipid complex Amphotericin B to achieve sustained clinical and radiological response after six months of therapy.

Neurofibromatosis type 2 (NF-2) is associated with mainly three types of recurrent benign tumors restricted to the central nervous system: schwannoma, meningioma and ependymoma. The absence of the protein NF2/Merlin causes an uninterrupted cell proliferation cascade originating from an abnormal interaction between an extracellular mucopolysaccharide, hyaluronan (HA), and schwann cell surface CD44 receptor, which has been identified as one of the central causative factors for schwannoma. Most tumors in NF-2 have a predilection to originate from either arachnoid cap cells or schwann cells of the cisternal portion of nerve rootlets that share a continuous exposure to cerebrospinal fluid (CSF). We hypothesize that the CSF HA may play a role in tumorigenesis in NF-2. In a prospective analysis over a period of one year, the levels of medium to low molecular weight HA (LMW HA) was estimated in the CSF of three subjects with central schwannomas and compared against that of age-sex matched controls, using Cetyltrimethylammonium bromide coupled turbidimetric assay and found to be seventeen-fold higher in the schwannoma subjects compared to the controls. HA was observed to be actively secreted by cultured schwannoma cells isolated from tumor tissues commensurate with their proliferation rate. On cell viability index analysis to compare the cell proliferation of astrocytoma cells with LMW HA vs. oligomeric HA (OHA), we found a decrease in cell proliferation of up to 30% with OHA. The study provides initial evidence that CSF HA may have a central role in the tumorigenesis of schwannoma in NF-2.

Subaxial spine injuries during infancy are uncommon. We present the case of an infant referred to our brachial plexus clinic with proximal weakness of both arms noted immediately following a complicated breech delivery. Nerve conduction studies were consistent with bilateral proximal upper plexus injury; however, radiological evaluation revealed a C4–C5 subluxation and MRI features of bilateral C5 root avulsions. He was immobilized in a cervical-body orthosis and underwent open reduction and posterior C4-C5-C6 fixation at 10 weeks age using luque rods, sublaminar wires, and rib graft. The spine was immobilized in a custom molded cervicothoracic brace for a total of 3 months, and a home exercise program prescribed. Follow-up radiographic evaluation showed good posterior bony fusion by 2 months but persistent bilateral proximal upper limb weakness with reduced compound motor action potential (CMAP) amplitudes of the axillary and musculocutaneous nerves. Bilateral supraclavicular exploration and C5 root neurotization using the ipsilateral C7 nerve roots were performed at 7 months of age. Voluntary biceps activity followed by voluntary external rotation with gravity eliminated was noted within the next 2–3 months. He progressed to develop near normal motor control of bilateral upper limbs within 1 year. We wish to report this rare entity and our favorable outcome using a strategy of early spinal stabilization and neurotization repair to restore function following proximal nerve root injury.

The general prevalence of the familial multi-organ tumor disorder, von Hippel-Lindau syndrome (VHL), was estimated to be 1 in 25-40,000 in western studies two decades back. Few studies were done in Indian sub-continent, amidst a surge in clinical reports on VHL specific manifestations. The syndrome is correlated with mutations of the gene VHL (located in Chr 3p25.3). We aimed to conduct a prospective case series describing phenotypic and genotypic characteristics in Indian population. The VHL-specific clinical and radiological features were collected from patients and family members. Genotypic changes such as deletion/duplication or point mutation in the VHL locus were identified using sequencing and MLPA. Thirty-one subjects, from fifteen families with diagnosed VHL, were included in the study. Multicystic pancreas was found in 71% (22/31), CNS hemangioblastoma in 68% (21/31), renal cell carcinoma and retinal angiomas in 23% (7/31) each, pheochromocytoma in 9.7% (3/31) of the population and endolymphatic sac tumor in one subject. Four families (9 subjects) had full length deletion of VHL, three families (4 subjects) had a deletion of exon 3, eight families (18 subjects) had different exonic, splice-site and intronic point mutations and one subject had a de novo in-frame indel in exon 1. Multicystic pancreas and CNS hemangioblastomas were the most common manifestations in our population. The phenotypic expression patterns in terms of tumorigenesis, tissue tropism and penetrance in comparison to the genotypic features were found to be different from previous correlative studies.

Background Robotic automation and haptic guidance have multiple applications in neurosurgery. Objective To define the spectrum of cranial procedures potentially benefiting from robotic assistance in a university hospital neurosurgical practice setting. Methods Procedures utilizing robotic assistance during a 24-mo period were retrospectively analyzed and classified as stereotactic or endoscopic based on the mode utilized in the ROSA system (Zimmer Biomet, Warsaw, Indiana). Machine log file data were retrospectively analyzed to compare registration accuracy using 3 different methods: (1) facial laser scanning, (2) bone fiduciary, or (3) skin fiduciary. Results Two hundred seven cranial neurosurgical procedures utilizing robotic assistance were performed in a 24-mo period. One hundred forty-five procedures utilizing the stereotactic mode included 33% stereotactic biopsy, 31% Stereo-EEG electrode insertion, 20% cranial navigation, 7% stereotactic catheter placement, 6% craniofacial stereotactic wire placement, 2% deep brain stimulation lead placement, and 1% stereotactic radiofrequency ablation. Sixty-two procedures utilizing the haptic endoscope guidance mode consisted of 48% transnasal endoscopic, 29% ventriculoscopic, and 23% endoport tubular access. Statistically significant differences in registration accuracies were observed with 0.521 ± 0.135 mm (n = 132) for facial laser scanning, 1.026 ± 0.398 mm for bone fiduciary (n = 22), and 1.750 ± 0.967 mm for skin fiduciary (n = 30; ANOVA, P Conclusion The combination of accurate, automated stereotaxy with image and haptic guidance can be applied to a wide range of cranial neurosurgical procedures. The facial laser scanning method offered the best registration accuracy for the ROSA system based on our retrospective analysis.

BACKGROUND AND IMPORTANCE The lateral suboccipital approach for microvascular decompression (MVD) of the trigeminal nerve has become a standard-of-care over the past several decades. Syndromic cranial base settling, a rare but known cause for trigeminal neuralgia (TN), poses significant dilemmas in clinical management. In such cases, distorted anatomy may render surgery via the suboccipital approach difficult or even impossible. CLINICAL PRESENTATION A 34-yr-old male with osteogenesis imperfecta and severe basilar invagination suffered from TN that was refractory to medication and stereotactic radiosurgery. MVD of the fifth nerve root was performed via a pterional trans-sylvian approach after 3-dimensional trajectory modeling suggested the feasibility of this approach. He experienced complete resolution of TN and remains free of both pain and medications after 18 mo of follow-up. CONCLUSION This microsurgical trajectory, previously undescribed for TN, provides good visualization of the trigeminal root entry zone, thereby offering an excellent surgical option for severe TN in the setting of abnormal posterior fossa anatomy.

In selected patients with drug-resistant focal epilepsies (DRFE), who otherwise are likely to be excluded from epilepsy surgery (ES) because of the absence of a magnetic resonance imaging (MRI)-demonstrable lesion or discordant anatomo-electro-clinical (AEC) data, magnetoencephalography (MEG) may help to generate an AEC hypothesis and stereo-electroencephalography (SEEG) may help to verify the hypothesis and proceed with ES. The sensitivity of MEG is much better in localizing the spiking zone in relation to lateral temporal and extratemporal cortical regions compared to the mesial temporal structures. MEG has a dominant role in the presurgical evaluation of patients with MRI-negative DRFEs, insular epilepsies, and recurrent seizures after failed epilepsy surgeries, and in guiding placement of invasive electrodes. Moreover, postoperative seizure freedom is better if MEG spike source localized cortical region is included in the resection. When compared to subdural grid electrode recording, SEEG is less invasive and safer. Those who are otherwise destined to suffer from uncontrolled seizures and their consequences, SEEG guided ES is a worthwhile and a cost-effective option. Depending on the substrate pathology, there is80-90% chance of undergoing ES and 60-80% chance of becoming seizure-free following SEEG. Recent noninvasive techniques aimed at better structural imaging, delineating brain connectivity and recording specific intracerebral EEG patterns such as high frequency oscillations might decrease the need for SEEG; but more importantly, make SEEG exploration more goal-directed and hypothesis-driven.

Introduction The patent expiration of imatinib mesylate (Gleevec; Novartis) on February 1, 2016, has brought the focus back on generic versions of the drug, and an opportunity to provide a safe and cost-effective alternative. The objective of our study was to determine the molecular and cytogenetic responses, survival endpoints (event-free survival, failure-free survival, transformation-free survival, overall survival), and safety of innovator and generic brands of imatinib. Materials and Methods In this retrospective analysis, data from 1812 patients with chronic myeloid leukemia treated with frontline imatinib mesylate (innovator/generic) at a single institution between 2008 and 2014 is included. Of these, 445 were excluded owing to inadequate data and follow-up, and a further 156 were excluded as they were in either the accelerated phase or blast crisis at diagnosis. Thus, data from 1067 patients who were treated with Gleevec (Novartis), and 144 patients with Veenat (NATCO) were available for analysis, and included in the study. Results There was no significant difference in event-free survival ( P = .05), failure-free survival ( P = .07), transformation-free survival ( P = .12), or overall survival ( P = .24) between the 2 groups. The frequency of reported nonhematologic adverse events and hematologic adverse events was comparable between the study groups. Conclusion The findings of the present study showed comparable efficacy and safety of the generic and innovator versions of imatinib in the treatment of patients with chronic myeloid leukemia.

Introduction: The Philadelphia chromosome (Ph) is the most common cytogenetic abnormality associated with adult acute lymphoblastic leukemia (ALL) occurring in 20% to 40% of patients. It is also detected in 2% to 5% of children with ALL. Historically, patients with Ph-positive ALL carried a dismal prognosis, with poor response to most chemotherapy combinations, short remission durations, and long-term disease-free survival rates of 10% to 20%. The advent of tyrosine kinase inhibitors (TKIs) has revolutionized therapy of Ph-positive ALL. Materials and Methods: This retrospective and descriptive single center study was carried out based on data retrieved of 508 patients treated for ALL from 2007 to 2014. Of these thirty patients were Ph-positive ALL and were available for analysis, and these patients were included in the study. Ph-positive ALL was defined as ALL carrying the t(9;22) translocation on standard karyotype and/or fluorescent in situ hybridization analysis and/or positivity for BCR-ABL fusion transcript detection by real-time quantitative polymerase chain reaction (RQ-PCR) analysis. Patients were treated with combination chemotherapy and oral TKIs and responses were classified as either CR defined by the absence of circulating blasts and

The authors present the case of an 11-year-old boy with an intraventricular chordoid meningioma, which is a rare presentation of prolonged fever of unknown origin due to a rare tumor in a rare location. The fever resolved after excision of the lesion. Subsequent imaging revealed recurrence at 1 year. After a repeat excision and fractionated radiotherapy, the patient has remained disease free 5 years after the first surgery. Very few cases of intraventricular chordoid meningioma have been reported to date. The pathological features and clinical course are described. A review of the literature describing management options for this tumor type, recently found to have a higher recurrence rate, is described herein.

Background: Hysterectomy is one of the common gynaecological major surgeries performed worldwide. In spite of technological advancement with laparoscopic and robotic hysterectomy conventional hysterectomy through vaginal route of nonprolapse uterus popularly known as, Non-Descent Vaginal Hysterectomy (NDVH) remains a justifiable cost effective, cosmetically appealing option especially in resource-crunched developing country. NDVH in post caesarean scarred uterus too a technically challenged procedure requiring skills and expertise.Assessment of technical feasibility and safety of non-descent vaginal hysterectomy in women with previous caesarean section scar were studied.Methods: The study was a prospective observational study of 72 patients with LSCS scar requiring hysterectomy for benign conditions were selected based on the inclusion and exclusion criteria carried out from June 2012 to May 2017. Operating time, blood loss, surgical techniques, intra/postoperative challenges, conversion to laparotomy or laparoscopic assistance and length of hospital stay were recorded for each case. Patients were followed up till 03 months of surgery.Results: Vaginal hysterectomy was successful in all cases. Morcellation, bisection or myomectomy, were done in 86% cases. Two patients had bladder injury, which was repaired vaginally, two cases required support of laparoscopy. No patients needed blood transfusion. None of the patients were converted to laparotomy.Conclusions: Vaginal hysterectomy is a safe and effective procedure for benign non-prolapsed uteri in women with previous caesarean section scar when uterine size is less than 14 weeks. Standby operating laparoscopy provides added advantages to surgeon in doubtful or difficult cases to avoid conversion laparotomy.

Supplemental Digital Content is available in the text, Asymptomatic myocardial injury following noncardiac surgery (MINS) is an independent predictor of 30-day mortality and may go unrecognized based on standard diagnostic definition for myocardial infarction (MI). Given lack of published research on MINS in India, our study aims to determine incidence of MINS in patients undergoing noncardiac surgery at our tertiary care hospital, and evaluate the clinical characteristics including 30-day outcome. The prospective observational study included patients >65 years or >45 years with either hypertension (HTN), diabetes mellitus (DM), coronary artery disease (CAD), cerebrovascular accident (CVA), or peripheral arterial disease undergoing noncardiac surgery. MINS was peak troponin level of ≥0.03 ng/dL at 12-hour or 24-hour postoperative. All patients were followed for 30 days postoperatively. Predictors of MINS and mortality were analyzed using multivariate logistic regression. Patients categorized based on peak troponin cut-off values determined by receiver operating characteristic curve were analyzed by Kaplan–Meir test to compare the survival of patients between the groups. Among 1075 patients screened during 34-month period, the incidence of MINS was 17.5% (188/1075). Patients with DM, CAD, or who underwent peripheral nerve block anaesthesia were 1.5 (P 1 ng/mL (P = .01) were significantly associated with mortality. A peak troponin cut-off of >0.152 ng/mL predicted mortality among MINS patients at 72% sensitivity and 58% specificity. Lack of antithrombotic therapy following MINS was independent predictor of mortality (P

We report a seven-year-old girl who presented with a sudden tumor apoplexy due to a parasagittal hemangiopericytoma. Following tumor excision, the child was noted to have bilateral optic nerve dysfunction and progressive papilledema, despite rapid overall neurological improvement. Based on the clinical features, we feel that this case represents an unusual form of visual deterioration related to impaired CSF absorption somehow precipitated by the acute tumour apoplexy. This unusual case of blindness responded significantly to CSF shunting. Several reports exist describing raised intracranial pressure with papilledema caused by nonthrombotic sinus occlusion due to tumors in proximity to the posterior superior sagittal sinus, torcular herophili and the jugular outlet. Communicating hydrocephalus, pseudotumor syndrome or intracranial venous sinus obstruction should be considered when otherwise inexplicable visual loss coexists with optic nerve dysfunction and papilledema. We emphasize the importance of a thorough search for the cause of visual loss.

Object Middle cerebral artery infarction often occurs at a younger age than other strokes and is associated with significant rates of mortality and morbidity. After a period of pessimism regarding decompressive hemicraniectomy in the management of acute stroke, the method has reemerged in the past decade. The present study was undertaken to assess the immediate and long-term outcome of this intervention and to help better define the selection criteria for surgery. Methods The authors conducted a nonrandomized prospective study using decompressive hemicraniectomy with duraplasty in patients at various stages of clinical deterioration due to a space-occupying middle cerebral artery infarct. Patients were assessed at 6 and 12 months postinfarction by using functional scales. Subjective reconsideration was assessed using a questionnaire. Twenty-six patients were included in the study. The mean age was 48.4 ± 11.2 years, and the mean preoperative Glasgow Coma Scale score was 9.9 ± 3.2. The median time from ictus to surgery was 54 hours (range 13–288 hours). The rate of survival at 1 year postsurgery was 73%. Among survivors, 33.3% were independent (Barthel Index [BI] > 95) and 55.6% were partially dependent (BI 60–95) at 1 year postsurgery, with 72% attaining the ability to walk independently by 1 year postsurgery. No patient remained in a vegetative state. The 1-year BI score was inversely related to patient age (r = − 0.47, p = 0.048). Conclusions Survival after decompressive hemicraniectomy was better than previously reported using medical management alone. A vegetative state was avoided and functional independence was possible, especially in younger patients. Increasing age was a statistically significant predictor of disability and long-term functional dependence.

Objectives We present a case series of patients who underwent perirolandic resection for medically refractory focal epilepsy due to focal cortical dysplasia (FCD). Our aim was to specifically evaluate the outcome of a surgical strategy intended for seizure freedom while preserving primary motor cortex function. Materials and methods Thirteen patients undergoing perirolandic resection for pharmacoresistant focal epilepsy between 2010 and 2015 who demonstrated histological evidence of FCD were selected from a prospectively maintained database. Presurgical evaluation included video EEG telemetry and 3T MRI brain for all patients. Eight patients underwent interictal FDG PET scan. Intracranial EEG monitoring was done for 8 patients – six by conventional subdural grid and depth electrodes and two by Stereo EEG. Additional techniques included extraoperative cortical stimulation mapping, intraoperative electrocorticography (ECoG), intraoperative motor cortex mapping and awake surgery in various combinations. In all cases (lesional and nonlesional), resection was intentionally limited for anatomic preservation of the primary motor cortex. Results Amongst the thirteen patients with age ranging 14–44 years (mean 26.8 ± 9.2) 62% of them had daily seizures. MRI abnormalities were identified in 8 patients (62%), PET showed concordant findings in 7 patients (88%). When utilized, the mean duration of intracranial EEG recordings was 8.0 ± 7.2 days (range 2–23 days). All patients underwent a primary motor cortex-sparing resection of the suspected epileptogenic cortex. The mean postoperative follow up period was 23 months (range 7.5–62 months). Twelve out of 13 (92%) were seizure free (Engel 1) outcome at the last follow-up assessment; one patient had Engel 2a outcome at 28 months. Six patients (46%) had immediate new focal neurological deficits, however all six patients had recovered completely within three months. Conclusion The surgical strategy of a primary motor cortex-sparing resective surgery for perirolandic FCD is associated with an excellent early seizure-freedom rate and no permanent neurological deficits. Since the ultimate goal of resective epilepsy surgery is seizure freedom with simultaneous functional preservation, similar long term outcome studies should ultimately guide the resection strategy.

✓ The authors report an intrinsic brainstem lesion that was diagnosed initially as a pontine cavernoma, which finally proved to be a choroid plexus papilloma. Choroid plexus papillomas are rare tumors of the central nervous system and are usually intraventricular in location. The occurrence of this tumor in an intraparenchymal location is extremely rare, and its occurrence within the brainstem is previously unreported. The authors also report a trial of chemotherapy with lomustine in the management of the residual tumor.

e21507 Background: Data on Ewing’s sarcoma from the developing world is limited. Despite uniformity in diagnosis and treatment protocols outcomes have differed between centers. Methods: We retrospe...

e21005 Background: About 25% of adult patients on Imatinib for chronic myeloid leukemia (CML) did not take the drug as prescribed, and ended up taking less than 90% of their doses. This had an adve...

Neurofibromatosis type 1 (NF-1) is a heterogeneous autosomal dominant disease with an incidence ranging from 1 in 2500 to 1 in 3000. Rare intrathoracic vascular disorders resulting in massive spontaneous hemothorax with fatal consequences may occur in these patients, so also are various types of skeletal dysplasia which may result in dramatic presentations, posing management challenges to the attending physicians. We report the case of a 43-year-old woman with NF-1 who developed spontaneous massive hemothorax and was discovered to have a concurrent giant intrathoracic meningocele and thoracic kyphoscoliosis with severe vertebral dysplastic changes. Surgical treatment via a right thoracotomy with primary repair of the meningocele and spinal fusion with fibula graft resulted in good outcome. This case represents an extreme manifestation of this otherwise indolent disease in clinical practice.

Seizures that cause loss of consciousness (LOC) can be classified as epileptic or nonepileptic based on evaluation of ictal semiology and analysis of changes in EEG events, recorded with continuous scalp EEG and video monitoring. We report 3 patients who had hippocampal electrographic seizures documented with intracranial EEG recording with no accompanying scalp EEG change immediately preceding psychogenic unresponsiveness. Each patient also had complex partial seizures (CPS) originating in the hippocampus. Some individuals can have complex interactions of epileptic and nonepileptic seizures.

A seven-month-old infant presented with a generalized seizure. The radiological evaluation revealed a large arteriovenous fistula in the left sylvian region. The fistula connected the left middle cerebral artery and the vein of Trolard. A giant varix was present at the venous end. The child underwent craniotomy, complete disconnection of the fistula and excision of the varix. Except for recurrent seizures, which were eventually controlled on anticonvulsants, the child's neurological development has been good on long-term follow-up of three years. Pial arteriovenous fistulae are rare intracranial vascular malformations. Though significant operative risks exist, they can be successfully managed surgically with good long-term prognosis.

Introduction Intracranial arachnoid cysts are considered to be congenital malformations with a predilection for the temporal fossa. They are often asymptomatic but can sometimes be symptomatic due to enlargement or hemorrhage. There are multiple case reports of arachnoid cysts becoming symptomatic with hemorrhagic complications following head trauma. In such cases, the bleeding is often confined to the side ipsilateral to the arachnoid cyst. Occurrence of contralateral subdural hematomas in patients with temporal fossa arachnoid cysts has rarely been observed and is reported less frequently in the medical literature. Case presentation We report two cases of people (a 23-year-old man and a 41-year-old man) with temporal fossa arachnoid cysts complicated by a subdural hematoma following head injury. Both patients developed a subdural hematoma contralateral to the side of a temporal fossa arachnoid cyst. It is likely that lack of adequate intracranial cushioning in the presence of an intracranial arachnoid cyst may result in injury not only to ipsilateral but also to contralateral bridging veins, following head trauma. Conclusion It is important to identify and report such rare complications with intracranial arachnoid cysts, so that asymptomatic patients with an intracranial arachnoid cyst can be counseled about such possibilities following head trauma.