Background and Aims: Many studies have conclusively proven that meditative techniques derived from the Indian systems of philosophy, meditation and ritual classified as “Tantra” can bring about sustained changes in the structure and function of the nervous system of practitioners. The aim of this study is to provide neuroscientists a framework through which to interpret Tantra, and thereby provide a foundation upon which future interdisciplinary study can be built. Methods: We juxtapose Tantric concepts such as the subtle body, nadis and mantras with relevant neuroscientific findings. Our premise is that through sustained internalization of attention, Tantric practitioners were able to identify and document subtle changes in their field of awareness, which usually do not cross the threshold to come into our perception. Results: The descriptions left by Tantric philosophers are often detailed and empirical, but they are about subjective phenomena, rather than external objects. They also focus on individual experiences, rather than the group-level analyses favored by modern medical science. Conclusion: Systematic exploration of Tantric texts can be of tremendous value in expanding our understanding of human beings' experiential reality, by enabling us to build bridges between first-person and third-person approaches to the nervous system. This may open up new avenues for cognitive enhancement and treating neurological diseases.

Objective: Aneurysm rupture is often a fatal complication of giant intradural aneurysm (GIA) treatments. The purpose of this study was to review aneurysm rupture in GIA treatment. Materials and Methods: We performed a systematic review on aneurysm rupture related to GIA treatment. For each reported case, we collected the following information: aneurysm location, size and rupture status, the mode of treatment, timing of the hemorrhage, anatomical, and hemodynamic factors. Results: We identified 56 aneurysm ruptures related to treatment in 38 published studies. Of the nine intraoperative ruptures, eight occurred during endovascular procedures and one in surgical treatment. Of the 47 delayed ruptures, 72.3% occurred within 2 weeks. The prognosis of intraoperative and delayed ruptures was poor, with 83.9% experiencing death. Of these aneurysms, 75% were initially unruptured. Of the delayed ruptured aneurysms, 21.3% had prior surgical treatment, 74.4% had prior endovascular treatment, and 4.3% had prior combined surgical and endovascular treatments. Vertebrobasilar artery (VBA) location was significantly associated with aneurysm rupture after treatment, occurring at 57.2%. Flow diverter (FD) treatment seemed to elevate the delayed rupture proportion of giant paraclinoid internal carotid artery (ICA) aneurysms from 22.0% to 42.9%. FD treatment did not lower the rupture risk of giant VBA aneurysms and the corresponding death rate. Conclusion: Intraoperative and delayed aneurysm ruptures were the most challenging in endovascular treatment of GIAs. Giant VBA aneurysm had the highest rupture risk after treatment. FD seemed to elevate the delayed rupture proportion of giant paraclinoid aneurysms.

As the population is aging, clinicians are coming across more patients with atrial fibrillation and venous thromboembolism requiring anticoagulation to prevent stroke and systemic embolisms. Due to a high prevalence and unfavorable consequences, managing thromboembolic diseases have become areas of clinical concern. Traditional anticoagulants like heparin, low molecular weight heparin and warfarin have been used for the prevention and treatment of venous and arterial thromboses. But, issues of bleeding, parenteral route of administration, or the need for frequent monitoring due to variability in response respectively limit their use. The article gives an overview of coagulation along with existing therapy available for anticoagulation and to present an update on utility and recent advances of new oral anticoagulants (NOACs) beginning from their nomenclature, advantages, disadvantages, precautions and contraindications compared with those of vitamin K antagonists (VKAs) based on a large number of recent studies and clinical trials.

Inherited neuromuscular diseases are a heterogeneous group of rare diseases for which the low general awareness leads to frequent misdiagnosis. Advances in DNA sequencing technologies are changing this situation, and it is apparent that these diseases are not as rare as previously thought. Knowledge of the pathogenic variants in patients is helping in research efforts to develop new therapies. Here we present a review of current knowledge in GNE myopathy, a rare neuromuscular disorder caused by mutations in the GNE gene that catalyzes the biosynthesis of sialic acid. The most common initial symptom is foot drop caused by anterior tibialis muscle weakness. There is a progressive wasting of distal skeletal muscles in the lower and upper extremities as well. The quadriceps is relatively spared, which is a distinguishing feature of this disease. The characteristic histological features include autophagic rimmed vacuoles with inclusion bodies. GNE variant analysis of Indian patients has revealed a founder mutation (p.Val727Met) common within the normal Indian populations, especially in the state of Gujurat. We discuss therapeutic options, including metabolite supplementation, pharmacological chaperones, and gene therapy. Initiatives that bring together patients, researchers, and physicians are necessary to improve knowledge and treatment for these rare disorders.

Addiction is a major cause of mortality and morbidity. Apart from psychotropic substances, alcohol and nicotine remain the common addictive materials responsible for the majority of deaths. Conventional conservative therapies are beneficial to certain populations, but the majority may require interventional treatments such as deep brain stimulation (DBS) in view of increasing mortality from drug abuse in recent years. We present a brief review on a novel neuromodulation target of the nucleus accumbens (NA) and its promising role in the management of addiction. The three stages of the addiction cycle are known to be mediated by dopaminergic pathways located in the mesolimbic dopamine system with connections to dorsal striatum, extended amygdala, cingulate gyrus, orbitofrontal cortex, prefrontal cortex, and ventral tegmental area. Recent advanced neuroimaging in humans and several animal studies demonstrated NA to be a vital anatomical area modulating this network. DBS of NA in animals reduced addictive behavior to alcohol, cocaine, and other narcotics significantly. The accidental observation that DBS of NA for psychiatric illnesses induced relief from addiction to alcohol and smoking has encouraged further research of late. Bilateral NA ablative surgery had shown nonrelapse in more than 50% of cases. Small series of patients have benefited so far from DBS of NA, but larger numbers are required to provide evidence-based treatment. The modulation of dopaminergic pathways through DBS of NA as a valid treatment for addiction is substantiated extensively by animal studies and also in a few clinical studies. However, this needs to be validated by a well-structured, multicenter controlled study in a large group of patients suffering from substance abuse.

Objective: Several studies have indicated that decompressive craniectomy (DC) for traumatic brain injury (TBI) is lifesaving. However, there is lack of level 1 evidence to define the role of DC in TBI. We performed a meta-analysis of all the randomized controlled trials (RCTs) published so far on the role of DC in adult patients with TBI. Materials and Methods: A systematic literature search was performed for articles published until September of 2016 for RCTs of DC in adult patients with TBI. The primary end-point was mortality at six-months. We also evaluated the overall adverse outcomes at six months. Assessment of risk of bias of the RCTs was also performed. Results: Three trials evaluating adult population satisfied the eligibility criteria. Pooled analysis involved 285 and 288 patients in DC group and control groups respectively. Patients undergoing DC for TBI had a lower mortality association of nearly 50 percent. However, patients surviving DC were more likely to have a poor neurological outcome compared to patients undergoing medical management. Conclusion: Based on the available RCTs on DC in TBI, the results of our meta-analysis show that there is a mortality benefit of performing a DC over the best medical management in adult patients. Furthermore, surviving following DC, a greater incidence of a poor neurological outcome is noted. In the event of small number of high-quality RCTs, our results must be interpreted with caution.

Background: It has been acknowledged that delayed cerebral ischemia (DCI) can be diagnosed by computed tomography perfusion (CTP) when it occurs following aneurysmal subarachnoid hemorrhage (aSAH); however, the clinical role of CTP in the prediction of DCI remains unclear. We performed a meta-analysis to investigate the role of CTP in the identification of patients at risk for DCI during the acute phase (<4 days) after aSAH. Materials and Methods: Relevant articles were systematically searched for analysis on PubMed, EMBASE, and Cochrane databases. The best CTP parameter or the definition of abnormal CTP scan result were collected, and the data with the greatest overall predictive value for DCI was extracted to assess the strength of association between a positive CTP result and an impending DCI. In addition, pooled estimates of sensitivity and specificity were determined. Results: Three relevant articles involving 128 patients were included in the analysis wherein DCI developed in 48 patients (37.5%). The pooled odds ratio was 32.15 (95% CI, 9.92–104.21), suggesting that the patients with a positive CTP test in the acute phase after aSAH were approximately 32 times as likely to develop DCI compared with those without aSAH. The pooled sensitivity and specificity of CTP for detecting impending DCI after aSAH was 65% (95% CI: 0.49–0.78) and 91% (95% CI: 0.83–0.96). Conclusions: CTP can detect abnormal brain perfusion before the occurrence of DCI. This may allow close monitoring and preemptive therapy for improvement in the prognosis in patients with aSAH.

Background: Minimally invasive surgical techniques for cerebral aneurysms have been developed. Aims: To compare the efficacy and safety of modified mini-pterional (mMPT) and standard pterional (PT) craniotomies for ruptured anterior circulation aneurysms. Materials and Methods: A total of 45 patients with ruptured anterior circulation aneurysms underwent surgical clipping; for 21 patients PT was used and for 24 patients mMPT was used. Initial clinical demographics and outcomes were retrospectively compared. A systemic inflammatory response syndrome (SIRS) score was derived by summing the number of variables meeting standard criteria for SIRS. Results: The two groups were comparable with initial clinical demographics. Total operative time was significantly shorter in the mMPT (166.6 minutes, P = 0.001) compared with the PT (235 minutes). The rate of permanent operative morbidity were similar in both groups (P = 0.92). The mean SIRS score at 24 hours after the completion of the operation was significantly lower for patients in the mMPT group (0.96, P = 0.01) as compared to the patients in the PT group (1.81). The rate of postoperative symptomatic vasospasm was significantly lower in patients operated through the mMPT (8.3%, P = 0.03) than the PT (38.1%). Good outcome at discharge was more frequently seen in the mMPT (91.7%) than in the PT (70%), but this difference was not statistically significant (P = 0.11). Conclusion: The mMPT craniotomy is a safe and less invasive approach for ruptured anterior circulation aneurysms, leading to a significant lower rate of postoperative symptomatic vasospasm and a marginally significant improvement in clinical outcomes.

Background: Aneurysms arising from the proximal segment (A1) of the anterior cerebral artery (ACA) are relatively rare. Because of their small size, abnormal location in relation to the parent artery and the risk of damage to the surrounding perforators, their surgical management is a big challenge. We present our experience with 7 patients of A1 segment aneurysms. Settings and Design: Tertiary care referral center. Materials and Methods: Seven patients who were diagnosed with A1 aneurysms between 2009 and 2017 were included. Preoperative evaluation included Non-Contrast Computed Tomography (NCCT) head and angiography (Digital Subtraction Angiography with/without CT-Angiography). The clinicoradiological condition of the patients was graded as per World Federation of Neurological Surgeons (WFNS), Fisher and Hunt and Hess (H and H) Grading systems. A retrospective review of clinical features, radiological descriptions, surgical treatment, and outcomes was done. Results: All patients underwent microneurosurgical clipping. All aneurysms were saccular, ranging in size from 4 to 14 mm and neck size varied from 2 to10 mm. Most aneurysms 5 (71.4%) had a posterior direction. Anatomical variations were noticed in 3 (42.8%) patients. Posteroinferiorly directed aneurysms were difficult to clip. As per Glasgow Outcome scale (GOS), 6 (85.7%) patients had a good outcome, whereas 1 (14.2%) had poor outcome. As per the modified Rankin Scale (mRS) too, 6 (85.7%) had a favorable outcome. There were no deaths. Conclusion: A1 aneurysms are frequently associated with vascular anomalies and generally rupture when small. A1 aneurysms with a superior and anterior direction are relatively easy to clip whereas those directed postero-inferiorly are difficult. Close association with critical perforators also compounds the situation. Due to the rarity of A1 aneurysms, large series are few in literature.

Aim: To retrospectively evaluate the outcome of patients with intracranial meningeal hemangiopericytomas (MHPCs) and to analyze various factors for recurrence and survival in these patients. Materials and Methods: We retrospectively reviewed the clinical data of 39 patients undergoing microsurgical resection for MHPCs at our institute from 2009 to 2015. Results: Gross total excision (GTE) was achieved in 27 (69.2%) patients, whereas 12 (30.8%) underwent subtotal excision (STE). A total of 25 patients received radiotherapy (RT) (Conventional RT-15; GKT-10), 21 patients had a low grade tumor, while 18 had an anaplastic variant. Twenty patients (51.3%) developed recurrences and the average recurrence-free survival (RFS) was 56 months (range: 12–180 months). Eight patients (20.5%) died during the study period. The average overall survival (OS) was 77.2 months (range: 36–192 months). Two patients (5.1%) developed systemic metastases during follow-up. Patient age was not found to affect RFS or OS. GTE was associated with prolonged RFS and OS but the impact was not statistically significant (P-values = 0.160 and 0.414, respectively). Low tumor grade was associated with statistically significant longer RFS as well as OS (P-values = 0.049 and 0.013, respectively). Addition of adjuvant RT was associated with statistically significant prolongation of RFS (P value = 0.016); however, it was not associated with statistically significant OS benefits (P-value = 0.758). Conclusions: Our study suggests that a greater extent of excision, lower tumor grade, and addition of adjuvant RT have a positive impact on both RFS and OS; however, low grade and adjuvant RT were the only factors associated with statistically significant prolongation of RFS and only tumor grade was associated with statistically significant OS benefits.

Objectives: Microsurgery of vestibular schwannoma (VS) after radiosurgery is not common. In this study, we analyze nerve functional outcomes and technical challenges associated with the microsurgery following radiosurgery of VS. Materials and Methods: Sixteen patients underwent microsurgery at an average of 37 months (range 3-96 months) after radiosurgery. Indications of surgery included tumour progression or clinical worsening. Twelve of them had never undergone microsurgery before radiosurgery, and three had partial excision before radiosurgery. Maximum tumour diameter of all patients ranged from 23 to 51 mm, of which four were giant tumours. Operative findings and post-microsurgery clinical course, imaging and outcome were analysed. Results: The tumours found at surgery were firmer, with an avascular core but increased vascularity near the surface. There were thickened arachnoid and often adhesions with surrounding structures making total excision difficult. Near total excision was employed in such cases. Eleven patients underwent gross total/near total resection. No major morbidity or death was reported. Post-surgery, eight patients had grade I/II HB facial function, four had grades III and three had grades IV/V. Of the last three, two had the same deficit preoperatively. At the last follow-up (average 50 months), all patients had a stable facial function. None of the patients have undergone any further radiosurgery/microsurgery since the last intervention. Conclusion: For the few patients who require microsurgery after radiosurgery, excellent tumour resection with an acceptable outcome can be achieved with proper techniques and neuromonitoring. Leaving tiny residue attached to critical structures is the key to an optimal outcome.

Background: The need to study prognosis after incidence of acute ischemic stroke (AIS) has fueled researchers to identify predictors apart from neurological, functional, or disability measures. The purpose of this study was to test and validate a newly developed clinico-biomarker assessment module in AIS and also to investigate the role of serum vascular endothelial growth factor (VEGF) after AIS. Materials and Methods: A randomized controlled study with sample size of 250 patients suffering from AIS within 2 weeks of the index event were conducted and followed up for a period of three months. Age, gender, stroke subtype, previous stroke history, dysarthria, stroke localization, wakeup strokes, and Glasgow Coma Scale (GCS) were dichotomized as present or absent using the National Institute of Health Stroke Scale (NIHSS) which consists of four subcategories. The additional serum VEGF was scored between 1 and 4 (0–200 = 1, 200–300 = 2, 300–400 = 3, and 400–500 = 4). All these were summed under a clinical biomarker (CB) module with highest score of 30. Results: The mean VEGF in 125 patients was 378.4 + 98.9 pg/ml, indicating a moderately high increase with a score of 3 on CB module. Multiple regression analysis revealed that the CB model was fit to predict prognosis and severity [R² = 0.86, F (23.4, 6);P = 0.001], with NIHSS subscore, prestroke status, and VEGF being very strong predictors. When only the clinical module was tested on all 250 patients, it was found that the NIHSS subscore, time to stroke onset and prestroke functional status were the most common [R² = 0.79; F (45,9);P = 0.005]. Conclusion: This study demonstrates that VEGF is highly upregulated in AIS with severe disability as compared to healthy controls. This biomarker is a strong predictor of severity and functionality when combined with clinical variables three months post the ishemic event.

Background: A number of pharmacological agents have been tried to circumvent the problem of delayed cerebral ischemia (DCI) with ozagrel sodium being one such agent aimed at the prevention of DCI. Ozagrel is an inhibitor of thromboxane synthetase. It has anti-platelet aggregation action and it dilates vessels. Ozagrel was not available outside Japan till recently. It is available now in India and we had the opportunity to use it among patients with aneurysmal subarachnoid hemorrhage (SAH). Aims: To analyse the results of ozagrel administration for patients with aneurysmal SAH. Settings and Design: Tertiary care neurosurgical center. Materials and Methods: Retrospective analysis of the outcomes of patients who received ozagrel after microsurgical cllipping of aneurysm and comparison with a control grpup who received treatment as usual. Statistical Analysis: The t-test (two-tailed), Chi-square test, and Mann–Whitney U-test asymptomatic significance (two-tailed), were used respectively for continuous, categorical, and ordinal variables. The significance was determined at P = 0.05 level. Results: A total of 106 patients underwent surgical clipping of their ruptured intracranial aneurysms over a period of 22 months. Forty two (39.6%) patients received ozagrel, and 62 (60.4%) received the standard treatment. Ozagrel was started at a median of one [interquartile range (IQR) 0.75] day after the surgery, and was given for a median of five (IQR 5) days after the surgery. There was no difference in age, postictal days, World Federation Neurosurgical Society grade, Fisher grade, and the size of ruptured aneurysm in patients who received ozagrel compared to the patients who did not receive ozagrel. Of the 42 patients who received ozagrel, 30 patients (71.4%) had preoperative angiographic vasospasm which improved after the administration of ozagrel. Fifteen (35.5%) patients who received ozagrel developed delayed cerebral ischemia compared to only 11 (17.2%) patients who did not receive ozagrel. Thirty-six (85.7%) patients who received ozagrel had favorable outcome at discharge compared to 52 (81.3%) patients who did not receive ozagrel. No adverse event was observed with ozagrel therapy. At 3-month follow-up, 37 patients (88.1%) who received ozagrel had favorable outcomes against 53 patients (82.8%) who did not receive ozagrel. Conclusion: Ozagrel may be a useful drug in the armamentarium to treat vasospasm after aneurysmal SAH. A future multicenter large cohort study may validate the findings of our study.

Background: Radiation-induced brain edema (RIBE) is a serious complication of radiation therapy. It may result in dramatic clinico-radiological deterioration. At present, there are no definite guidelines for management of the complication. Corticosteroids are the usual first line of treatment, which frequently fails to provide long-term efficacy in view of its adverse complication profile. Bevacizumab has been reported to show improvement in cases of steroid-resistant radiation injury. The objective of this study is to evaluate the role of Bevacizumab in post-radiosurgery RIBE. Material and Methods: Since 2012, 189 out of 1241 patients who underwent radiosurgery at our institution developed post-radiosurgery RIBE, 17 of which did not respond to high-dose corticosteroids. We systematically reviewed these 17 patients of various intracranial pathologies with clinic-radiological evidence of RIBE following gamma knife radiosurgery (GKRS). All patients received protocol-based Bevacizumab therapy. The peer-reviewed literature was evaluated. Results: 82 percent of the patients showed improvement after starting Bevacizumab. The majority began to improve after the third cycle started improvement after the third cycle of Bevacizumab. Clinical improvement preceded radiological improvement by an average of eight weeks. The first dose was 5 mg/kg followed by 7.5–10 mg/kg at with two-week intervals. Bevacizumab needs to be administered for an average of seven cycles (range 5–27, median 7) for best response. Steroid therapy could be tapered in most patients by the first follow-up. One patient did not respond to Bevacizumab and needed surgical decompression for palliative care. One noncompliant patient died due to radiation injury. Conclusion: Bevacizumab is a effective and safe for treatment of RIBE after GKRS. A protocol-based dose schedule in addition to frequent clinical and radiological evaluations are required. Bevacizumab should be considered as an early treatment option for RIBE.

Background: Most of the neurointerventional procedures use various anticoagulants, antiplatelets, and fibrinolytic drugs, and it can predispose arterial access site complications. Safe and efficient closure arteriotomy site has extreme importance in reducing the morbidity. Percutaneous vascular closure devices (VCDs) enable us to close the arteriotomy sites. In this prospective study, we evaluated the factors affecting the safety and efficacy of Perclose ProGlide VCD in neurovascular interventions. Materials and Methods: In this prospective study, we have evaluated the safety and efficacy of 327 Perclose ProGlide devices deployed in 217 patients who underwent various neurointerventions in our institute from October 2014 to October 2016. Time to achieve hemostasis (TAH) was calculated for various groups and the statistical significance of mean values between groups was estimated. Results: Out of the 327 Perclose ProGlide deployed, complication rate was 0.91% and the mean TAH was 77.14 s. Assessment of TAH mean value showed statistically significant prolongation of TAH in obesity and those with larger arterial sheaths. Age, sex, post-heparin ACT, peri-procedure medications, and type of diseases had no significant role in increasing TAH. The evaluation also showed the presence of a learning curve in using this device. Conclusion: Perclose ProGlide VCD is safe and effective in closing the arteriotomy after neurointerventions. Obesity and larger arterial sheaths are independent factors prolonging the TAH. Learning curve associated with this device mildly increases the hemostatic time and device-related complications.

Background: Metabolic patterns on brain F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) can predict the decline in amnestic mild cognitive impairment (aMCI) to Alzheimer's disease dementia (AD) or other dementias. Objective: This study was undertaken to evaluate the diagnostic accuracy of baseline F-18 FDG-PET in aMCI for predicting conversion to AD or other dementias on follow-up. Patients and Methods: A total of 87 patients with aMCI were enrolled in the study. Each patient underwent a detailed clinical and neuropsychological examination and FDG-PET at baseline. Each PET scan was visually classified based on predefined dementia patterns. Automated analysis of FDG PET was performed using Cortex ID (GE Healthcare). The mean follow-up duration was 30.4 ± 9.3 months (range: 18–48 months). Diagnosis of dementia at follow-up (obtained using clinical diagnostic criteria) constituted the reference standard, and all the included aMCI patients were divided into two groups: the aMCI converters (MCI-C) and MCI nonconverters (MCI-NC). Diagnostic accuracy of FDG PET was calculated using this reference standard. Results: There were 23 MCI-C and 64 MCI-NC. Of the 23 MCI-C, 19 were diagnosed as probable AD, 1 as frontotemporal demetia (FTD), and 3 as vascular dementia (VD). Of the 64 MCI-NC, 9 had subjective improvement in cognition, and 55 remained stable. The conversion rate for all types of dementia in our series was 26.4% (23/87) and for Alzheimer's type dementia was 21.8% (19/87). The of PET-based visual interpretation was 91.9%. Sensitivity, specificity, positive predictive value, and negative predictive value for FDG-PET-based prediction of dementia conversion were 86.9% [confidence interval (CI) 66.4%–97.2%)], 93.7% (CI 84.7%–98.2%), 83.3% (CI 65.6%–92.9%), and 95.2% (CI 87.4%–98.9%), respectively. Kappa for agreement between visual and Cortex ID was 0.94 indicating excellent agreement. In the three aMCI patients progressing to VD, no specific abnormality in metabolic pattern was noted; however, there was marked cortical atrophy on computed tomography. Conclusion: FDG-PET-based visual and cortex ID classification has a good accuracy in predicting progression to dementia including AD in the prodromal aMCI phase. Absence of typical metabolic patterns on FDG-PET can play an important exclusionary role for progression to dementia. Vascular cognitive impairment with cerebral atrophy needs further studies to confirm and uncover potential mechanisms.

Background and Aims: Dorsal root entry zone (DREZ) lesioning is a widely-used procedure for neuropathic pain which is refractory to other modes of treatment. However, all current techniques depend on thermal or radiofrequency (RF) lesioning of the DREZ. The authors describe a new technique in which mechanical lesioning of DREZ using microscissors. Methods: The authors describe their technique of only using straight microscissors for the whole procedure of DREZotomy. No cautery is used except for hemostasis. Results: Our technique is a continuing evolution of the original DREZotomy described by Nashold and Sindou, and appears more atraumatic and simpler. Conclusion: Microscissor DREZotomy appears to be the most atraumatic way of carrying out DREZ lesioning and overcomes the disadvantages of other methods like thermal and RF lesioning.

Surgical management of an intracranial aneurysm is mainly clipping. Various types of clips have been designed for complex, giant, and other aneurysms. Recently, the Yasargil-fenestrated T-bar clip has been introduced for the management of such complicated aneurysms. The literature regarding its efficacy, limitations, and method of application in complicated aneurysms is limited. We used the fenestrated T-bar clip in four patients of intracranial aneurysms – middle cerebral artery cortical branch aneurysm, anterior communicating artery partially thrombosed aneurysm, internal carotid artery paraclinoid simple aneurysm, and giant aneurysm. All aneurysms were successfully clipped with no residue or branch occlusion. The recovery of all patients was satisfactory. The fenestrated T-bar clip effectively reconstructed the parent artery and occluded the aneurysm in all cases. The application of this clip may be advantageous over the clip application in some uniquely challenging cases.

Intramedullary spinal cord tumors are rare neoplasms and harbour full spectrum of primary neoplasms as seen within the intracranial compartment. They include tumors with diverse pathologies, arising in both adults and children and pose considerable diagnostic challenge. The differentials at this site include wide ranging pathologies from benign, circumscribed pilocytic to diffuse astrocytoma, myxopapillary or tanycytic ependymoma and malignant diffuse midline glioma. Rare instances of glioneuronal tumors, pleomorphic xanthoastrocytoma have also been described at this location. H3K27M mutant diffuse midline high grade glioma is the new entry to this list in 2016 updated WHO classification. We describe the morphologic features of a diagnostically challenging intramedullary spinal cord tumor masquerading as a high grade lesion due its cellular composition and discuss its differentials. The report also emphasizes the role of already established and recently introduced immunohistochemical markers and other ancillary techniques as useful adjuncts in the diagnosis.

Pericallosal lipomas (PCLs) are rare tumors of the central nervous system. They may be associated with some parenchymal and vascular anomalies of brain. Magnetic resonance imaging is the modality of choice to assess the extent of the PCLs and possible concomitant malformations such as callosal agenesis/disgenesis. Computerized tomography angiography may be indicated to evaluate the vasculature of the lesion. We report here a case of PCL with rare features including asymptomatic callosal agenesis, bilateral choroid plexus lipomas and abnormal vasculature.

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome of certain mesenchymal tumors which secrete fibroblast growth factor-23 (FGF-23) responsible for causing features of hypophosphatemia and osteomalacia in these patients. Most of them involve the appendicular skeleton and occasionally the craniofacial regions. Involvement of spine is exceedingly rare. Through this paper, the authors present a rare case of a 71-year-old male with TIO due to a lesion in the cervical spine (right C2 lamina) which was proven to be a phosphaturic mesenchymal tumor-mixed connective tissue type on histopathology. This is the fifth reported case of TIO localized to the cervical spine. The patient underwent a hemilaminectomy and gross total resection of the tumor following which he made a gradual but steady recovery and does not have any recurrence 24 months after surgery. The authors not only provide a comprehensive literature review of all 18 spinal cases reported till date but also discuss the management of these patients in light of the published literature.

Neurodegeneration with brain iron accumulation (NBIA), previously called Hallervorden Spatz disease, is a group of disorders which share the hallmark of iron deposition in the brain. They are collectively characterized by extrapyramidal movement disorders, particularly those of parkinsonism, dystonia, cognitive regression, neuropsychiatric abnormalities, pyramidal features, optic atrophy, and retinal abnormalities. There is aberrant brain iron metabolism, with large amounts of iron deposited in the globus pallidus and the substantia nigra pars reticulata. NBIA displays a marked genetic heterogeneity, and 10 genes have been associated with different NBIA subtypes at present. We present a 12-year-old boy with a one and a half-year history of a slow, progressive gait disturbance. An MRI of his brain revealed T2, FLAIR bilateral symmetrical hypointensities in globus pallidus and substantia nigra s/o NBIA. His genetic analysis revealed a novel homozygous missense variation in exon 2 of the C19orf12 gene (chr19:30199203; A>C) that results in the amino acid substitution of valine for phenylalanine at codon 51 (p.F51V; ENST00000392278). This is consistent with the MPAN (mitochondrial membrane protein-associated neurodegeneration) subtype.

X-linked myopathy with excessive autophagy (XMEA) is a rare, slowly progressive muscle disease characterized by membrane-bound sarcoplasmic vacuoles distinct from other forms of myopathies with vacuoles. We report this rare condition in a 5-year-old boy with proximal muscle weakness and morphological evidence of autophagic vacuoles.

Intracranial myoepithelial tumors are extremely rare with <10 cases reported outside the sellar region. The authors describe a case of a 43-year-old male patient who presented with headache, numbness in the face, and a dumbbell-shaped lesion in the Meckel's cave clinically and radiologically suggestive of a Schwannoma. The histopathological and immunohistochemical evaluation led to a diagnosis of myoepithelioma. A review of literature reveals that this is only the ninth case of intracranial myoepithelial tumor reported, fifth benign case, and the first to be reported in the Meckel's cave region.

Dorsolumbar intradural extramedullary ependymoma is a rare entity. Spinal metastases in patients with intracranial ependymoma are well described, but it is extremely rare for a spinal ependymoma to metastasize to brain. We describe a case of aggressive dorsolumbar intradural extramedullary ependymoma mimicking arachnoid cyst radiologically, which developed intracranial metastasis.

Acute encephalitis syndrome (AES) is a clinical condition that occurs due to infectious and noninfectious agents- however, viruses are considered to be the dominant pathogen. agents- however, viruses are considered to be the dominant pathogen. In this study, suspected AES cases were enrolled and tested for viral etiology through serology and polymerase chain reaction (PCR)/reverse transcriptase PCR from August 2012–July 2013. During this period, 820 cases were investigated and 96 cases were diagnosed to have a viral etiology whereas 20 patients had IgM antibodies for measles in serum and HSV-1 DNA in cerebrospinal fluid. All 20 of the patients were children below 14 years of age. The median hospital stay was 15 days (IQR: 14.2-17 days) and median GCS score was 7(IQR: 6–8) and were significantly different with patients with co-infections when comapred with patients having HSV-1 infection only. It may be suspected that the measles infection may have a role in the pathogenesis and thus an impact on the prognosis of the AES when present with HSV-1.