Background: The COVID-19 infection outbreak has aroused increasing attention and affected thousands of people nationwide. The long incubation period, high infectious rate, varied manifestation, and absence of effective treatment make it difficult to manage the disease transmission. Objective: The intended goals are to encourage efficient management of neurological and neurosurgical patients, resource utilization, and protecting the healthcare provider during the COVID-19 epidemic. Herein, we present a consensus statement from various centers in India Methodology: In addition to the literature review, recommendations were included from neurologists and neurosurgeons from various centers in India. Results: Every patient presenting for treatment should be treated as a potential asymptomatic infected case. Patients should be categorized based upon the priority as acute (require immediate treatment/surgery within 24 h), sub-acute (requiring treatment within a maximum of 7–10 days), or chronic (requiring treatment within a month). Non-essential elective surgeries and outpatient clinics should be avoided after informing the patient(s). There is a high risk of aerosol dispersion during intubation and certain neurosurgical procedures particularly those involving drills and endoscopes. These procedures should be performed wearing full personal protective equipment. The workflow of the operating rooms should also be modified significantly. Minor modifications in personal and professional lifestyles and routine training to use the PPE will ensure efficient management of resources. Conclusion: These recommendations could be used to mitigate the risks and reduce exposure to other patients, public, and healthcare staff.

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The actual investigation of the body of a patient by the clinician in search for the signs of the disease beginning with the primary vital signs and continues with the careful and attentive observation of the patient. This article reviews the key findings in the physical examination of patients with ischemic stroke that have the potential to indicate the etiology of the infarct and to help to choose the use of ancillary tests. Through a systematic search of articles published in English related to the physical examination of patients with stroke, we identified key findings in the vital signs and classic components of the physical exam (appearance of the patient, auscultation, and eye examination) that have shown clinical significance when determining ischemic stroke etiology. We further suggest that the prompt identification of such findings can translate into better use of diagnostic tools and selection of ancillary confirmatory tests, thus, reducing the time to etiology based treatment and secondary prevention of ischemic stroke. in this manuscript, we aim to show that even though nowadays the clinical skills tend to be overlooked due to the overreliance on technology, the physical exam continues to be a valuable tool in the clinician armamentarium when facing the challenge of a patient with ischemic stroke.

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Background: The current study was planned at a tertiary centre in northern India to develop and validate a Diagnostic and Statistical Manual-5 (DSM-5)-based diagnostic tool and design a severity score for attention deficit hyperactivity disorder (ADHD) in children aged 6–18 years. An existing DSM-IV-based tool, INDT (International Clinical Epidemiology Network [INCLEN] diagnostic tool) for ADHD has been modified and named All India Institute of Medical Sciences (AIIMS)-modified INDT ADHD tool. Method: The first phase was development of the tool and the second phase was validation of the same against the gold standard of diagnosis by the DSM-5. A severity score was developed for ADHD in concordance with the Conners rating scale. Results: The tool was validated in 66 children with a sensitivity and specificity of 100 per cent and 90 per cent, respectively. A cut-off score of 12 was decided for labelling severity of ADHD, which corresponded to 63 in the Conners rating scale. Conclusion: This diagnostic tool for ADHD based on DSM-5 has acceptable psychometric properties. The severity score will be useful for prognostication, monitoring treatment response, and designing intervention trials.

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Background: The treatment of the syrinx prior to correction of the scoliosis in syringomyelia-associated scoliosis (SMS) patients remains controversial. The aim of this study is to evaluate the role of the syrinx size in the management of SMS patients. Materials and Methods: This was a retrospective study of 36 SMS patients. They were divided into 2 groups: Group A (26 with a small syrinx, syrinx(S)/spinal cord(C) ratio ≤0.7) and Group B (10 with a large syrinx, S/C ratio >0.7). Patients with a large syrinx accepted prophylactic neurosurgery prior to scoliosis surgery. They were evaluated at baseline, 1-week and last follow-up after correction surgery for changes in curve correction, global coronal balance, thoracic kyphosis (TK), sagittal vertical axis (SVA), and Scoliosis Research Society (SRS)-22 scores. Results: The syrinx size of patients in Group A was significantly smaller than that of in Group B. The syrinx size was significantly decreased after prophylactic neurosurgery in Group B. The radiographic parameters of scoliosis at baseline, 1-week and last follow-up after scoliosis surgery were comparable between two groups. No abnormal signal was detected during the process of neuromonitoring in both groups. Pre- and postoperative SRS-22 scores were similar between two groups. Conclusions: Prophylactic neurosurgery may be beneficial for decreasing the risk of correction surgery in SMS patients with large syrinx (S/C ratio >0.7). After the intervention of syrinx prior to scoliosis correction, SMS patients with large syrinx could obtain similar clinical and radiographic outcomes of treatment with pedicle-screw-based spinal instrumentation and fusion compared to the patients with small syrinx.

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Gamma knife for gliomas is a relatively obscure treatment modality with few reports and small series available on the same. An extensive search of English Language literature yields no comprehensive reviews of the same. We here, attempt to review the available literature on gamma knife for all types of gliomas: Low grade, High grade, recurrent, and also for pediatric populations. We used keywords such as “Gamma Knife Glioma,” “Stereotactic Radiosurgery Glioma,” “Gamma Knife,” “Adjuvant therapy Glioma” “Recurrent Glioma” on PubMed search engine, and articles were selected with respect to their use of gamma Knife for Gliomas and outcome for the same. These were then analyzed and salient findings were elucidated. This was combined with National Comprehensive Cancer Network guidelines for the same and also included our own initial experience with these tumors. Gamma-knife improved long term survival and quality of life in patients with low grade gliomas. In pediatric low grade gliomas, it may be considered as a treatment modality with a marginal dose of 12–14 Gy, especially in eloquent structures such as brain stem glioma, anterior optic pathway hypothalamic glioma. However, in newly diagnosed high-grade glioma gamma knife radiosurgery (GKRS) is not recommended because of a lack of definitive evidence in tumor control and quality of life. GKRS may find its role in palliative care of recurrent gliomas irrespective of type and grade. Inspite of growing experience with GKRS for gliomas, there is no Level I evidence in support of GKRS, hence better designed randomized controlled trials with long term outcomes are warranted. Although this modality is not a “one size fits all' therapy, it has its moments when chosen correctly and applied wisely. Gliomas being the most common tumors operated in any neurosurgical setting, knowledge about this modality and its application is essential and useful.

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Abstract: Background and Aims: External ventricular drainage (EVD) is one of the commonest procedures in neurosurgical practice to manage acute hydrocephalus. We evaluated the infectious and non-infectious complications associated with a modified technique for EVD using an Ommaya reservoir. Methods: Ommaya reservoir was placed in all patients who required EVD placement for CSF drainage. CSF drainage was achieved using a needle placed in a non-coring fashion percutaneously into the Ommaya reservoir to achieve CSF drainage externally. CSF was monitored for signs of infection regularly using CSF biochemistry and cultures. CSF infection was defined by a positive culture or a secondary infection in patients with already infected CSF. Results: 59 patients required continuous CSF drainage during the study period from January 2014 to June 2017. 46 (77.96%) patients had non-infected CSF at time of starting drainage and 13 (22.03%) patients required external CSF drainage for primarily infected CSF. The study period had a total of 793 CSF drainage days (Range 3-64 days) with an average of 13.4 days per patient. The cumulative rate of new infection was 5.08%. No ventricular catheter blockage or dislodgement was seen in any of the patients. Conclusions: External ventricular drainage through an Ommaya chamber is a safe and effective method and can be used to reduce the catheter related complications like infection, catheter blockage and dislodgement.

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Background: The diagnosis of isolated cortical vein thrombosis (ICVT) involving superficial middle cerebral vein (SMCV) remains challenging even in the present era of modern MRI protocols. Objective: The purpose of this study is to review the clinical and radiological characteristics of SMCV thrombosis in our hospital. Methods: Chart review of cases of SMCV thrombosis admitted in a tertiary care university hospital in South India during a 1-year period from September 2015 to August 2016. Results: Five SMCV thrombosis patients were identified and presented with focal seizures. Neuroimaging showed edema (with or without hemorrhage) of cortex and white matter of inferior frontal gyrus, temporal pole, superior temporal gyrus, insular cortex, and external capsule. The thrombosis of SMCV was demonstrated by Spin echo T1-weighted, GRE-weighted axial, and postcontrast T1-weighted images in coronal and sagittal planes, with a slice thickness of <3 mm. Four received anticoagulation and all improved rapidly and completely. Conclusion: SMCV thrombosis should be considered in patients having recent onset seizures in appropriate setting based on MRI evidence of parenchymal edema and/or hemorrhage in the perisylvian region along with evidence of thrombosed vein in that region. Appropriate imaging sequences help in confirmation of diagnosis.

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Background and Aims: DNA methylation and demethylation play a crucial role in the regulation of gene expression, though their interplay during pathogenesis of hippocampal scelerosis (HS) remains elusive. The present study was designed to investigate the DNA methylation regulated changes in expression of HS patients. Methods: We performed integrative analysis of genome-wide CpG-DNA methylation profiling and RNA sequencing to profile global changes in promoter methylation and gene expression in HS patients. Real time PCR was performed to validate the findings of methylation and RNA sequencing. Results: A total of 16040 sites showed altered DNA methylation in all the CpG islands. Of these, 3185 sites were in the promoter regions, of which 66 genes showed an inverse correlation between methylation and expression. These genes are largely related to pathways predicted to participate in axon guidance by semaphorins, MAPK, ionotropic glutamate receptor pathway, notch signaling, regulatory activities related to TFAP2A and immune response, with the most distinct ones included TFAP2A, NRP1, SEMA3B, CACNG2, MAP3K11, and ADAM17. Conclusion: We performed integrated analysis of genomic methylation signature and differential gene expression patterns of hippocampal tissues resected from patients with HS for the first time. Collectively, our findings implicate DNA methylation as a critical regulator of the pathogenic mechanisms of epileptogenesis associated with HS.

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Background: Delayed hyponatremia is a serious complication seen after pituitary surgery. We document the incidence, presentation, outcome and risk factors for this condition. Materials and Methods: This was a retrospective study involving 222 patients operated for nonfunctioning pituitary macroadenomas between 2007-2016. Delayed hyponatremia was defined as serum sodium <135 mmol/L, occurring after the third post-operative day. Hyponatremia was categorized as mild (134-130 mmol/L), moderate (129-125 mmol/L) and severe (<125 mmol/L). All patients received intravenous (0.9%) saline, intravenous hydrocortisone and 12g oral salt over 24 hours. Patients with severe hyponatremia were given 3% saline. Results: Fifty eight patients (26%) developed delayed hyponatremia; thirty (13.5%) had severe hyponatremia. Delayed hyponatremia usually (43.1%) occurred on the seventh post-operative day (range, 3-15 days). Most patients (81%) remained asymptomatic; 11 patients developed vomiting (5), seizures (3), lethargy (1), fever (1) and paralytic ileus (1). One patient developed status epilepticus. Patients who manifest symptoms had lower sodium levels as compared to those who did not have symptoms (mean 117.7 mmol/L vs. 123 mmol/L; P < 0.01). Male gender (P = 0.002) and intra-operative CSF leak (P = 0.003) were risk factors for developing delayed hyponatremia. Other factors like, age, pre-operative cortisol levels, extent of resection and post-operative diabetes insipidus did not correlate with the occurrence of delayed hyponatremia. Patients who maintained their mean serum sodium levels >138 mmol/L (day 1–day 3) were unlikely to develop delayed hyponatremia (sensitivity, 55.2% and specificity, 83.9%), positive predictive value, 63.2% [confidence interval (CI) 48, 76.7%] and negative predictive value, 78.8% (CI 70.6, 85.5%). In most patients (57%) hyponatremia was corrected within 48 hours (h). Conclusions: We recommend routine serum sodium testing on the seventh post-operative day for all patients undergoing pituitary surgery. Most patients remain asymptomatic and unless they are detected early they can go on to develop serious complications.

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Background and Purpose: The purpose of this study is to evaluate posterior cerebral artery (PCA) aneurysms along with the efficacy, safety, procedural, and clinical outcome of the endovascular management of these aneurysms. We studied different techniques of endovascular treatment such as selective aneurysmal coiling, parent artery occlusion, and stent-assisted coiling in PCA aneurysms. Methods: From 2010 to 2017, 11 patients (8 females, 3 males) harboring a PCA aneurysm were treated via an endovascular approach. Seven of eleven aneurysms were saccular in nature; four were fusiform shaped. All aneurysms were treated using detachable coils either by selective obliteration of the aneurysm sac or by parent artery occlusion. In one patient, stent-assisted coiling of PCA aneurysm was done, and in one patient, flowdivertor along with few coils used to treat the aneurysm. Results: Five of the eleven aneurysms were successfully treated with preservation of the parent artery, and the other six were treated with aneurysm coiling along with parent vessel occlusion. Of the six where parent vessel occlusion was done, one developed transient hemiparesis which recovered on follow-up and none developed significant disabling vision abnormality. No mortality was noted. Conclusion: Aneurysms of the PCA are rare compared with other locations in the intracranial circulation. These aneurysms can effectively be treated by permanent occlusion of the parent artery even in this era of flowdivertors - however, in these cases, thorough knowledge of PCA segmental anatomy is crucial in order to select the site of occlusion and to avoid major neurological deficits.

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Background: Neurology services in rural and semi-urban part of India are very limited, due to poor infrastructure, resources, and manpower. Tele-neurology consultations at a non-urban setup can be considered as an alternative and innovative approach and have been quite successful in developed countries. Therefore, an initiative to bridge this health gap through Tele-Medicine has been taken by the Government of India. Aim: To study the sociodemographic and clinical profiles of patients who have received collaborative Tele-Neurology consultations from the Tele-Medicine Centre, National Institute of Mental Health and Neurosciences, Bengaluru. Methodology: We reviewed case files of such patients between December 2010 and March 2017. A total 189 collaborative tele-neurology outpatient consultations were provided through the Tele-Medicine Centre, located at a tertiary hospital-based research centre in southern India. Results: The mean age of the patients was 39.6 (±19) years and 65.6% were aged between 19 to 60 years; 50.8% were male. The most common diagnosis was a seizure disorder in 17.5%, followed by cerebrovascular accident/stroke in 14.8%. Interestingly, 87.3% were found to benefit from tele-neurology consultations using interventions such as a change of medications in 30.1%, referral to a specialist for review in 15.8%, and further evaluation of illness and inpatient care for 7.93%. Conclusion: This study has demonstrated the successful implementation of outpatient-based collaborative tele neurology consultation in Karnataka.

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The manifestations of CNS syphilis are unfamiliar to a differential of patients with dementia to many physicians today as of the relative rarity of this condition. This is a classical case report of a patient with syphilis and dementia in a 55-year-old female. General paresis of insane is a progressive disease of the brain leading to mental and physical worsening. It is important to consider tertiary syphilis in the differential diagnosis of dementia. Conventional presentations of neurosyphilis such as tabes dorsalis and general paresis of insane are read in textbooks only and rarely encountered in clinical practice in the 21^st century.

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Introduction: Pituitary adenomas comprise approximately 10% of all intracranial tumors. Initially, subtle changes occur in the field of vision, which are difficult to assess clinically. It has been seen that following surgery of pituitary macroadenoma, total recovery of normal vision occurs in 35% of the patients, improvement of vision occurs in 60%, and in the rest there is no change in vision. Retinal nerve fiber layer thickness (RNFLT) undergoes retrograde degeneration following compression of optic apparatus by pituitary tumor. We planned a study to evaluate RNFLT before and after pituitary adenoma surgery and its correlation with visual acuity. Material and Methods: Twenty patients (40 eyes) with diagnosed pituitary adenoma were included in the study. Preoperative visual acuity, fundus and RNFL thickness were calculated using spectral-domain OCT Optovue, Heidelberg Engineering, Heidelberg, Germany (RT 100 version 5.1), and postoperative measurement was done after 1 and 3 months. Four-quadrant mean of RNFLT was calculated. Results were tabulated and analyzed. Statistical Analysis: Results of the study were analyzed using IBM SPSS Statistics version 19.0. Results: There was no significant change in RNFLT after pituitary adenoma surgery, and it was found that patients with RNFLT within normal range preoperatively showed improvement in visual acuity after pituitary surgery. On the other hand, patients who had thinned-out RNFLT preoperatively showed no improvement in visual acuity. It was also found that once optic disc pallor sets due to chronic compression, then chances of its reversion to normal depend on its grading: only mild pallor disc has some chance to revert to normal, whereas moderate and severe pallor do not revert to normal. Conclusion: RNFLT and optic disc can be used as prognostic factors for evaluation of visual outcome in pituitary adenoma surgery.

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Background: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. Materials and Methods: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. Results: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12–52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. Conclusion: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

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Introduction: For the past two decades, diffusion tensor imaging (DTI)-derived metrics allowed the characterization of Alzheimer's disease (AzD). Previous studies reported only a few parameters (most commonly fractional anisotropy, mean diffusivity, and axial and radial diffusivities measured at selected regions). We aimed to assess the diagnostic performance of 11 DTI-derived tensor metrics by using a global approach. Materials and Methods: A prospective study performed in 34 subjects: 12 healthy elders, 11 mild cognitive impairment (MCI) patients, and 11 patients with AzD. Postprocessing of DTI magnetic resonance imaging allowed the calculation of 11 tensor metrics. Anisotropies included fractional (FA), and relative (RA). Diffusivities considered simple isotropic diffusion (p), simple anisotropic diffusion (q), mean diffusivity (MD), radial diffusivity (RD), and axial diffusivity (AD). Tensors included the diffusion tensor total magnitude (L); and the linear (Cl), planar (Cp), and spherical tensors (Cs). We performed a multivariate discriminant analysis and diagnostic tests assessment. Results: RD was the only variable selected to assemble a predictive model: Wilks' λ = 0.581, χ² (2) = 14.673, P = 0.001. The model's overall accuracy was 64.5%, with areas under the curve of 0.81, 0.73 and 0.66 to diagnose AzD, MCI, and healthy brains, respectively. Conclusions: Global DTI-derived RD alone can discriminate between healthy elders, MCI, and AzD patients. Although this study proves evidence of a potential biomarker, it does not provide clinical guidance yet. Additional studies comparing DTI metrics might determine their usefulness to monitor disease progression, measure outcome in drug trials, and even perform the screening of pre-AzD subjects.

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The posterior quadratic epilepsy (PQE) is a form of a multilobar epilepsy, involving the temporal-parietal and occipital lobes. Basically, epilepsies with localized networks to the posterior temporal, posterior parietal, and occipital lobes can benefit from this type of surgery. Gliosis due to perinatal insult and cortical dysplasis and angiomas in Sturge Weber syndrome involving the PQ have often been cited in the literature as the etiology for PQE. However, before considering surgery, it is important to localize the epileptogenic focus through a complete pre operative work up involving; EEG (Electro-Encephalo-Graphy), video EEG, single photon emission computed tomography (SPECT), positron emission tomography (PET), and magneto encephalography (MEG). Historically, these pathologies were dealt with multi-lobar resections, which were associated with high morbidity and mortality, owing to blood loss, especially in young children, hydrocephalus, and hemosiderosis. Based on the theory of networks involved in epileptogenesis, the concept of disconnection in epilepsy surgery was introduced. Delalande and colleagues, described the technique of hemispheric disconnection (functional hemispherectomy) for pathologies like: hemimegalencephaly, rasmussens encephalitis involving the entire hemisphere. The technique has evolved with time, moving towards minimally invasive endoscopic vertical hemispherotomy, described by Chandra and colleagues.^[1],[2] The posterior quadrant disconnection (PQD) evolved as a tailored disconnection on similar lines as hemispherotomy, for managing refractory epilepsy arising from the posterior quadrant.^[3] The technique and principles involved in the PQD surgery are similar to the those of peri-insular hemispherotomy and has been described in the literature by few authors.^{[3],[4],[5],[6]} The technique of performing PQD will be described here in a step-wise fashion with illustrations supplemented by a surgical video.

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Background: Neurofilament proteins as biomarkers of axonal degeneration have the potential to improve our capacity to predict and monitor neurological outcome in experimental autoimmune encephalitis (EAE), a model of multiple sclerosis (MS). We urgently need more accurate early predictive markers to direct the clinician when to provide neuroprotective therapy. Aims: To highlight the possible roles of neurofilament proteins in physiological and pathophysiological processes in the MS. Materials and Methods: Fifty female Wistar rats with MOG35–55 peptide induced EAE were randomly divided into two parts: control group and EAE group. All of them were along with expanded disability status scale (EDSS). The mice were sacrificed on day 0, 1, 3, 7, 14, and 28 after the first immunization. Supernatant and pellet were separated at the same time. The degradation rates of NF in the brain nerve and spinal cord of each rat were measured by Western Blotting. Statistical Analysis: The data were expressed as mean ± SD. Statistical analysis was performed with one-way analysis of variance (ANOVA), followed by LSD's post-hoc tests, which was provided by SPSS 23.0 statistical software. Results and Conclusions: Neurofilament light protein may be more useful as a measure of ongoing neurodegenerative activity in EAE, which would make this protein a potential candidate for use as a surrogate marker for assessment of treatments aimed at reducing axonal injury. Future studies are warranted to support or refute the value neurofilament in clinical practice.

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Optic neuritis is an inflammatory demyelinating disorder of the central nervous system. Anti-NMDA receptor (NMDAR) antibodies encephalitis is also mediated by the inappropriate immunological response. We report a girl who was readmitted because of unilateral eye pain and vision impairment in the remission period of anti-NMDAR encephalitis. Based on the limited available literature on the treatment of optic neuritis associated with anti-NMDA receptor antibody, she was treated with a combination of corticosteroids and intravenous immunoglobulin with clinical improvement. Optic neuritis in the remission phase of anti-N-methyl-D-aspartate receptor encephalitis remains relatively uncommon. It is important to document and follow these patients to understand the pathogenesis of optic neuritis in the setting of anti-NMDAR encephalitis and formulate effective therapeutic strategies.

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The incidence and prevalence of Parkinson's (PD) are increasing rapidly in developing countries. PD is difficult to diagnose based on clinical assessment. Presently, magnetic resonance imaging (MRI) methods such as R2* and Quantitative Susceptibility Mapping (QSM) were found to be useful in diagnosing the PD based on the iron deposition in different regions of the brain. The objective of this review was to evaluate the efficacy of QSM over R2* in assessment of PD. A comprehensive literature search was made on PubMed-Medline, CINAHL, Science Direct, Scopus, Web of Science, and the Cochrane library databases for original research articles published between 2000 and 2018. Original articles that reported the efficacy of QSM and R2* in assessment of PD were included. A total of 327 studies were identified in the literature search. However, only ten studies were eligible for analysis. Of the ten studies, five studies compared the accuracy of QSM over R2* in measuring the iron deposition in different regions of brain in PD. Our review found that QSM has better accuracy in identifying iron deposition in PD patients compared to R2*. However, there is discrepancy in the results between MRI Imaging methods and Postmortem studies. Additional longitudinal research studies are needed to provide a strong evidence base for the use of MRI imaging methods such as R2*and QSM in accurately measuring iron deposition in different regions of brain and serve as biomarkers in PD.

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Introduction: Autologous Stem Cell Transplant (ASCT) provides long periods of progression-free-survival in multiple sclerosis (MS). This is an observational study to demonstrate the safety of ASCT in MS patients at a transplant center in North India using a lymphoablative regimen. Materials and Methods: MS patients > 18 years referred by a neurologist or who came of their own volition were evaluated. Kurtzke Expanded Disability Status Scale (EDSS) score was calculated and those with a score of >7 were excluded. Informed written consent was taken. Mobilization was done with G-CSF with prednisolone to prevent disease flare-up. A minimum of 2 × 10⁶ CD34 cells/kg was collected. Conditioning regimen consisted of rabbit ATG and cyclophosphamide. Rituximab 375 mg/m² was given to prevent EBV reactivation and disease relapse. Antibiotic prophylaxis was given with levofloxacin, fluconazole, and valacyclovir. Any persistent change in EDSS scores ≥0.5 was considered significant. Results: Twenty patients were included. Seven patients had positive urine cultures prior to transplant and were treated before starting any chemotherapy. Majority patients were women (13/20). All patients developed febrile neutropenia, which was managed as per department policy. There was no mortality. Subjective symptoms improved in all patients. EDSS score improved in 6/19 patients (5/6 with RRMS) with no disease progression in any patient at a median follow-up duration of 242 days. Conclusion: ASCT can be done safely for patients with relatively high EDSS scores with additional precautions for screening for infections. RRMS patients with the active disease show most improvement. SPMS patients may not show significant improvement in the short term.

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Context: A well-established cell line of hemangioblastomas (HBs) is still lacking. Aim: This study aims to explore a stable way to establish primary cell lines of HB stromal cells and investigate the morphological and molecular features of these cells. Patients and Methods: Specimens of HBs from 13 patients were collected for establishment of primary cell lines of stromal cells. The details on cell culture were described, and the characterizations of cultured cells were conducted by morphological observation, immunocytochemical staining of inhibin-α, brachyury, CD133, CD34, GFAP, CD31, NeuN, CD45, Oligo2, and transmission electron microscopy. Results: Eleven cases were successfully cultured with a success rate of 84.6%. The cultured cells survived for 10 generations with an estimated doubling time of 77.2 ± 5.89 h. Light microscopy revealed that these cells showed vigorous growth status and presented as polygons or trigons with significant heterogeneity. The immunocytochemical staining showed that inhibin-α, brachyury, CD133, and CD34 were expressed in all the cultured cells, whereas the expression of GFAP, CD31, NeuN, CD45, and Oligo2 was all negative. Transmission electron microscopy confirmed that the cultured cells were stromal cells with typical lipid droplets. The phenomenon of lysosomal autophagy was commonly observed without apoptotic cells in late stage. Conclusion: Appropriate selection of tumor specimens, short duration of devascularization, ideal digestion time, and nutritious medium are critical points for establishment of primary cell line of HB stromal cells. Stromal cells from both von Hippel–Lindau disease-related HBs and sporadic HBs might originate from embryologically arrested hemangioblasts.

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Background: This prospective study analyzes the factors responsible for pre and postoperative persistent tinnitus following vestibular schwannoma (VS) surgery and discusses the possible etiopathogenetic mechanisms. Materials and Methods: Sixty-seven consecutive patients with unilateral VS operated via the retrosigmoid–suboccipital approach were included in the study. The Cochlear nerve, often unidentifiable from the tumor capsule, was resected during the surgery. Tinnitus Handicap Inventory (THI) score assessed the severity of pre and postoperative tinnitus. Result: Twenty-eight (41%) patients had preoperative tinnitus. Out of those 28 patients, 24(85%) had significantly improvement in postoperative THI score. In 15 of the 24 patients, tinnitus subsided completely. In 3 of the 28 (10%) patients, THI scores were unaltered, and in 1 of the 28 (3.5%) patients, THI scores worsened. In 39 (58.2%) patients without preoperative tinnitus, 4 (10%) developed a new-onset postoperative tinnitus. Patients with severe sensory neural hearing loss (SNHL) had significantly higher incidence of postoperative persistent tinnitus (PPT) (P = 0.00) compared to those with mild-to-moderate SNHL. Patients with profound SNHL, however, had a much lower incidence of PPT (P = 0.007; odds ratio = 0. 0.077; 95% CI: 0.009–0.637). Large (P = 0.07) and giant schwannomas (P = 0.03) VS had an increased risk of PPT. Patients with PPT further analyzed with brain stem auditory evoked response (BAER) showed normal contralateral waveform. Conclusion: Assessment of tinnitus is mandatory during the management of VS as there are high chances (nearly 46%) of PPT. Preoperative tinnitus, linked to the degree of SNHL (higher incidence in severe SNHL compared to mild-to-moderate/profound SNHL), is dependent on an intact cochlear nerve functioning. However, PPT is dependent on other mechanisms (brain stem/ipsilateral cochlear nuclei compression, and cortical reorganization) as it persists despite cochlear nerve resection.

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Introduction: Cerebral amyloid angiopathy (CAA) is a major cause of intracerebral hemorrhage (ICH) and cognitive decline in the elderly. Since it is rarely reported from the developing world, we looked into the clinical profile and neuroimaging associations of CAA. Materials and Methods: Ours was a retrospective case series of subjects diagnosed with probable/possible CAA between January 2006 and December 2015 as per Boston criteria. Clinical profile and neuroimaging were reviewed for markers of CAA. Details of any recurrent clinical events and functional status were collected from follow-up records. Results: We had 28 subjects in the series with men outnumbering women, and the mean age was 70.17 ± 8.85 years (55–87 years). At the initial presentation, ICH was most frequent—10/28 (35.7%) patients, followed by transient neurological events (TNE = 25%) and cognitive disturbances (21.4%). Less than half of the patients received a diagnosis of CAA at the initial presentation itself. In total, 68% of our patients had cognitive dysfunction at admission. In our series, 12 had seizures and 9 had a history of TNE. The majority of our patients had vascular risk factors also. Leukoaraiosis showed an association with cognitive dysfunction (P = 0.044). Superficial siderosis and subarachnoid hemorrhage (SAH) showed a positive association with seizures and TNE, respectively. However, ICH showed no association with risk factors or imaging markers of CAA. Conclusions: CAA patients, with a high prevalence of vascular risk factors mostly presented with ICH. The presence of SAH and superficial siderosis on MRI was associated with presentation as TNE and seizures, respectively.

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Background: Although it is well known that patients with Parkinson's disease (PD) have low bone mineral density (BMD) and serum vitamin D level, there are no studies evaluating their relationship with the stage and clinical features of the PD. Objective: The purpose of this study was to evaluate the relationship between BMD and serum vitamin D level and stage or clinical features of the PD. Materials and Methods: One hundred twenty-four patients with PD recruited from Movement Disorders Outpatient Clinic and age- and sex-matched 116 healthy controls were included in the study. BMD and serum vitamin D level of all participants were measured. After patients had been divided into four groups according to Hoehn and Yahr (H and Y) staging, a total of 5 groups with controls, BMD (lumbar and femoral) and serum vitamin D level were compared between groups. The relationship between the clinical features of the PD [disease duration, medication history, Unified Parkinson's Disease Rating Scale (UPDRS) part II and III, and subscores of UPDRS part III] and BMD or vitamin D was investigated. Results: Lumbar and femoral BMD values and serum vitamin D level were significantly lower in patients with PD compared to controls. Low BMD and low serum vitamin D level were identified in the early stages of the disease (H and Y stage 1 and 1.5) and were marked by the progress of the stage of the disease. There was a negative relationship between the clinical features of the PD and both BMD and serum vitamin D level. Conclusion: All patients with PD should be screened for developing osteoporosis and for sufficient vitamin D level in the early stages of the disease. Preventive methods for bone quality should be taken into consideration at the onset of PD.

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In order to make a correct diagnosis of idiopathic Parkinson's disease (PD), it is essential to exclude atypical parkinsonian features, such as early dementia, fall, and autonomic dysfunction. Rheumatoid arthritis (RA), which is a systemic inflammatory disorder, although most patients present in a polyarticular manner. Still some may also present with extra-articular involvement including skin, lung, heart, and the central or peripheral nervous systems. A possible pathogenetic link between RA and PD are proposed. However, the coexistence of RA and progressive supranuclear palsy (PSP) is rarely reported. Here, we report a parkinsonian patient with a newly diagnosed flare-up RA presenting with early falls, postural instability and supra-nuclear gaze palsy, which suggestive of clinically probable PSP. Furthermore, the parkinsonian features respond to anti-rheumatic agents, but not levodopa. Finally, the patient looks like a clinical possible PD. In summary, Parkinsonian patient with newly diagnosed flare-up RA can present with clinically probable PSP. Unbearably painful limb contracture is a clue of the coexistence of RA. Both typical and atypical parkinsonian features respond dramatically to anti-rheumatic medication, but not levodopa.

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Werner syndrome (WS), also known as adult progeria, is extremely rare, with about 1300 known cases in the world, with over 1000 of these in Japan. It occurs due to loss of function mutations in the WRN gene located on chromosome 8p12. WS is characterized by premature aging and increased risk of neoplasms, with meningiomas being the commonest intracranial tumor. We report the case of a 39-year-old male patient, who presented with occasional numbness in right arm for three weeks. The patient had developed signs and symptoms of premature aging which started in his adolescence. MRI brain done was suggestive of left frontal convexity extra-axial lesion, suggestive of meningioma. Genetic analysis performed has identified an autosomal recessive, apparently homozygous c.3383+3A>G mutation, a mutation not previously reported. As per the existing literature, this is the index case of meningioma in Werner syndrome from India. A new mutation has been identified.

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Introduction: Neurological diseases can be due to direct diseases of the central nervous system (CNS) or peripheral nervous system (PNS) or be a bystander syndrome of systemic diseases. Treatment options depend on the cause. Toxic, metabolic and nutritional, and immune-mediated consequences of clinically occult neoplasms produce a spectrum of neurological diseases, recognition of which has therapeutic and prognostic importance. Patients and Methods: Children, as well as adults who presented to the authors in the last 5 years with neurological diseases and later their diseases could be diagnosed or attributed to neoplasms which were occult, were included for the study. Observation: 28 patients were seen by the authors in the last 5 years with neurological manifestation and hidden tumor. Maximum incidence was in the age of above 60 years followed by the age group of 21–40 years. The commonest neurological presentation was muscle and nerve in adults and seizure in children. Discussion: Short duration, rapid progression, severe weight loss, and poor response to treatment given for nontumor associated neurological syndrome are the red flags which point to the diagnosis. Conclusion: Seizures and psychosis formed the commonest features in children, muscle and nerve in adults. Short duration, rapid progression, and resistance to treatment are the markers for possible underlying neoplasm.

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Objectives: Microvessel constriction plays an important role in delayed cerebral ischemia after aneurismal subarachnoid hemorrhage (SAH). This constriction has been demonstrated in both animal model and clinical operation. The present study examined the time-related membrane potential (Em) alteration of arterioles isolated from SAH model rats and the correlation between the potential alteration of arterioles and the diameter of basilar artery. Materials and Methods: Sprague–Dawley rats (n = 90), weighing 300 g to 350 g, were divided into t control, sham, and SAH groups. In the SAH group, blood was injected into the prechiasmatic cistern of the rats. The Em of arterioles and basilar artery diameter was measured using whole-cell clamp recordings and pressure myograph, respectively, 1, 3, 5, 7, and 14 days after SAH. The correlation was evaluated using Pearson correlation coefficients. Results: The Em of arterioles in the SAH group depolarized on days 3, 5, and 7, and peaked on day 7. The diameters significantly decreased on days 1, 3, 5, 7, and 14, and the smallest diameter was observed on day 7. A significant correlation between potential alteration of arterioles and diameter of basilar artery was found. Conclusions: Similar to the artery, arteriole constriction is also involved in the pathophysiological events of delayed cerebral ischemia.

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Aim: We aimed to determine the safety and effectiveness of endovascular treatment for cerebellar arteriovenous malformations (AVMs). Materials and Methods: Between January 2006 and January 2016, 75 patients with cerebellar AVMs underwent endovascular treatment at our department. The clinical and angiographic features, post-procedure complications, occlusion rate, and follow-up outcomes (modified Rankin Scale, mRS) of all the patients were retrospectively reviewed and collected. Multivariable logistic analysis was used to calculate potential risk factors for predicting poor outcomes (mRS ≥3). Results: Of the 75 patients, 61 (81.3%) presented with initial hemorrhage, and 44 (58.7%) presented with 63 cerebral aneurysms. Immediate digital subtraction angiography (DSA) after the procedure showed complete occlusion of the cerebral aneurysms in all the patients, and total occlusion of the AVM nidus in 32/75 (42.7%) patients, 99–90% occlusion in 31/75 (41.3%) patients, and <90% occlusion in 12/75 (16.0%) patients. Favorable functional outcome (mRS <3) was achieved in 61 (81.3%) patients. After adjusting for other factors, multivariate logistic analysis showed that increasing patient age (OR, 1.086; 95% CI, 1.098–1.182), the size of AVM (OR, 9.072; 95% CI, 1.164–20.703), and eloquent location (OR, 9.209; 95% CI, 1.557–35.481) were significantly independent predictors of poor outcome. Conclusions : Endovascular treatment of cerebellar AVMs is safe and feasible. The high rate of associated cerebral aneurysms could explain the tendency of initial hemorrhage in cerebellar AVMs; targeted embolization of coexisting cerebral aneurysms should be the first priority. Increasing patient age, eloquent AVM location, and the size of AVM are independent predictors of poor outcome after endovascular treatment of cerebellar AVMs.

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Background: Thoracic spine has complex pedicle anatomy with a narrow canal diameter which makes pedicle screw insertion challenging. Fennell et al. have described a simple freehand technique of thoracic pedicle screw placement. We have tested the accuracy of Fennell technique using computed tomography-based (CT-based) simulation model with pedicle screw simulator (PSS). Methods: Normal CT thoracic spine obtained from CT thorax data of five patients were used in the 3D slicer environment using PSS for simulation. Entry points and axial trajectory as described by Fennell et al. and a sagittal trajectory parallel to the superior endplate were used for simulating the freehand technique using EA (entry angle) mode in the PSS. An ideal trajectory through the midsection of the pedicle from the same entry point and a sagittal trajectory parallel to the superior endplate were simulated using the ET (Entry Target) mode. Angle predicted by the software for an ideal axial trajectory was compared with the Fennell technique and this angle difference was noted at all the levels. Presence of pedicle breach was noted while simulating the Fennell technique. Results: A total of 240 thoracic pedicle screw insertions were simulated, 120 screws by each technique. A sagittal trajectory parallel to the superior endplate caused no pedicle breach in the cranial-caudal direction at any level. No medial or lateral breach was noted while using an axial trajectory of 30° at T1-T2 and 20° from T3-T10. A 20° axial trajectory at T11 and T12 resulted in a breach of the medial cortex and the ideal mean axial angles at T11 and T12 were 2.8° and 6.5°, respectively. Conclusions: Fennell technique was effectively simulated using PSS. A uniform entry point and sagittal trajectory parallel to the superior endplate serves as a useful guide for freehand insertion of thoracic pedicle screws. At T11 and 12, ideal axial trajectories are less than 10°.

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Objective: In India, few centers are using 1.5 Tesla intraoperative MRI systems. We are using a 3 Tesla iMRI system. We share our initial experience of 3T iMRI in neurosurgical procedures with evaluation of its utility and pitfalls. Methods: A prospective observational study conducted between August 2017 to July 2018 at Yashoda Hospital, Secunderabad. All patients undergoing iMRI guided resection of intracranial SOL were included. Results: First 100 patients with various intracranial SOLs were included. The mean time required in shifting and image acquisition was 85.6 minutes in first 20 cases which was reduced to 37.4 minutes in next the next cases. Primary GTR was achieved in 44% cases, and residues were detected in 56%, secondary GTR was achieved in 37% cases, and surgery was discontinued in 19%. Maximum residues were detected in intraaxial sols and pituitary macroadenomas. No major iMRI associated complications were seen, minor issues involving transportation and minor contact burns were seen in 4 cases, insignificant anesthetic procedure related complications in 19 cases. Conclusion: As per our experience iMRI is an excellent tool to guide and improve the extent of safe resection by 37% in brain tumor surgeries. Good image quality, less time for image acquisition was observed advantages of 3T system. iMRI success depends on multidepartment coordinated teamwork and multiple iterations of the process to smoothen the workflow.

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Intracranial Rosai-Dorfman Destombes (RDD) disease is a rare entity. Lesions can lead to cranial nerve palsies and visual loss, especially in suprasellar location. Resection is considered to be definitive treatment; however, complete excision is difficult to achieve in view of the close proximity of critical structures. Radiotherapy (RT) is sometimes used for refractory or progressive disease for local tumor control and amelioration of symptoms. We report two patients with suprasellar RDD's with progressive symptoms treated with conformal RT after subtotal excision. These patients were treated with high precision conformal techniques to a dose of 45 Gy with significant and durable improvement in vision.

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Objective: This study aimed to explore metabolic features in the peritumoral tissue of different-rank brain astrocytic tumors using multivoxel proton magnetic resonance spectroscopy (¹H-MRS) and to estimate its application value in the MRS quantitative ratio of brain astrocytic tumors. Materials and Methods: A total of 82 patients with brain astrocytic tumors, confirmed by postoperative pathological evaluation, were divided into low-grade astrocytic tumors [World Health Organization (WHO) grade I–II; 32 cases] and high-grade astrocytic tumors (WHO grade III–IV; 50 cases). The semi-quantitative and relative quantitative metabolite ratios of the parenchyma area, peritumoral tissue area, and normal area were measured. The P value was set as <0.05. Results: The relative quantitative ratios of choline (Cho)/creatine (Cr) and Cho/N-acetyl aspartate (NAA) significantly differed in the peritumoral tissues of the high-grade astrocytic tumors and low-grade astrocytic tumors (P < 0.05), but NAA/Cr showed no significant difference. Conclusions: The changes in the metabolite ratio of the peritumoral tissue area in brain astrocytic tumors reflect the biological behavior of different tumors. They have a significant clinical value in classifying brain astrocytic tumors and indicating the scope of invasion.

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Objective: To investigate the dynamic change in the serum Tau protein early after acute traumatic brain injury (TBI) and its association with neurological outcome and cognitive function. Subjects and Methods: Around 229 patients with acute TBI and 30 healthy subjects were evaluated for the serum levels of Tau protein on 1, 3, 5, 7, and 14 days after TBI. The relationships of the serum levels of Tau protein and initial GCS and GOS at 6 months post-injury were also analyzed. Further, 95 TBI patients were assessed with their cognitive function with Montreal cognitive assessment (MoCA) score. Results: Serum Tau was significantly higher in patients with TBI at 1, 3, 5, 7, and 14 days. The serum Tau at each point was significantly lower respectively in the patients with mild TBI than that in medium and severe TBI. The serum Tau was significantly lower in patients with good outcome compared to the poor outcome group. The early serum Tau was negatively correlated with both GCS and GOS. In the TBI group, 39 (41%) out of 95 patients developed cognitive dysfunction assessed by MoCA. Tau protein at day 1, 3, and 5 after TBI was significantly correlated with cognitive dysfunction at 6 months after TBI. Conclusions: Acute Tau associations with neurological outcomes and cognition may implicate white matter damage and neuronal degeneration. Serum Tau may be used as a reliable biological marker for early diagnosis and cognitive recovery following TBI.

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Multiple sclerosis is a major cause of neurological disability, especially in young adults. There have been several case reports of an increased risk of cancer after long-term treatment for multiple sclerosis. Fingolimod is an immunomodulating agent used in the treatment of relapsing-remitting multiple sclerosis. The side effects commonly associated with fingolimod are cardiac side effects, macular edema, and elevated liver enzyme levels. Increased risks of infection and cancer have also been reported. High grade glioma is an aggressive primary brain tumor. There has been one case report of high grade glioma during fingolimod treatment. Here, we report the case of a 58-year-old woman diagnosed with glioblastoma multiforme after one year of fingolimod treatment for multiple sclerosis.

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Background: This study was performed to examine the efficacy of endoscopic transsphenoidal surgery (ETS) for Cushing's disease at a single institute and to review past reports. Material and Methods: We studied eight consecutive patients who underwent ETS for Cushing's disease. The radiological evaluation comprised a detailed examination of preoperative magnetic resonance images (MRIs), including inferior petrosal sinus sampling, for cases with normal renal function. Remission was evaluated at least three months after surgery and was defined by the presence of hypocortisolemia that required steroid replacement therapy or eucortisolemia with suppression to <1.8 μg/dL after 1mg of dexamethasone. Results: In all cases preoperative MRI was abnormal and included two macroadenomas (25 %). Pathological confirmation of an adenoma was possible in all patients. The mean follow-up period was 5.6 (2–7) years. Remission was confirmed in 75.0% of the cases and was higher in rate for microadenoma (100%) than for macroadenoma (50%). Postoperatively , no cerebrospinal fluid rhinorrhea occurred, but new endocrine deficits were noted in 25% of patients. Conclusion: Based on this study, ETS enhanced the intrasellar identification of adenomatous tissue, which led to low remission and complication rates that were comparable with those of traditional microsurgery for Cushing's disease.

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Aim: To evaluate the safety and efficacy of adenosine-induced transient asystole (AITA) during surgery for basilar artery aneurysms. Materials and Methods: All the patients with basilar artery aneurysms operated using AITA at our institute during two years period (August 2013–July 2015) were included in this study. Results: Adenosine was used in 11 patients with 13 basilar artery aneurysms. Seven of these aneurysms were basilar bifurcation aneurysms, four were basilar–superior cerebellar artery junction aneurysms, and two were distal basilar trunk aneurysms. The indications for AITA were narrow corridor for placement of temporary clip in 11 aneurysms, intraop rupture in 1 aneurysm, and circumferential dissection of a large aneurysm in 1. The mean dose of adenosine used for inducing asystole was 19.4 mg (range: 15–30 mg) and the mean total dose of adenosine used was 40.6 mg (range: 18–90 mg). A mean of 2 (range: 1–5) AITAs were required during surgical treatment of these aneurysms. The mean duration of a systole was 27 s (range: 9–76 s). There were no complications related to AITA in these patients except for transient rebound hypertension in one patient. Check angiogram revealed complete obliteration of 11 aneurysms and small residual neck in 2 aneurysms. Modified Rankin Scale at three months of follow-up was 0 in seven patients, 1 in two patients, 4 in one patient, and 6 in one patient. Conclusion: AITA during surgical management of basilar artery aneurysms is a safe and effective technique and has an important role during surgery for these aneurysms.

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Introduction: Craniosynostosis (CS) syndrome is an autosomal dominant condition (ADC) classically combining with CS and nonsyndromic CS (NSCS) including digital anomalies of the hands and feet. The majority of cases caused by a heterozygous mutation (HM) in the third immunoglobulin-like domain (IgIII) of fibroblast growth factor receptor (FGFR) 2 mutations outside this region of the protein. Material and Methods: We tried to find out the spectrum of genes involved in CS syndrome caused by the heterozygous missense mutation, the IgII and IgIII of FGFR2. FGFR3, FGFR4, TWIST, and MSX genes were performed and verified through the Indian population with CS children. Results: We find out that at conserved linker region (LR), the changes occurred among the larger families. Independent genetic origins, but phenotypic similarities add to the evidence supporting the theory of selfish spermatogonial selective advantage for this rare gain-of-function FGFR2 mutation. Polygenic novel mutation in both syndromic and nonsyndromic cases of CS promotes the translational research and holds a great promise to reproduce the molecular-based therapy and treatment as well. In this article, we summarized that genes involved in CS as evidence-based approach for characterization, identification, gene expression, and tissue engineering. We also described other related genes and proteins for the CS involvement and improvement of the diseases progression. Conclusion: HM again repeated the old story for both groups of syndromic CS and NSCS of Asian Indian children. Here, for the first time, we clearly reported that IgIII of FGFR2 mutations outside this region of the protein and tyrosine kinase (TK1 and TK2) responsible for both in molecular and cellular level for CS. It adds an evidence for future molecular targeting therapy to repair CS.

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Aim: To summarize our experience gathered during the use of different intraoperative electrophysiological modalities in children. Materials and Methods: We analyzed the data collected from 96 pediatric neurosurgical interventions. During the operations, we used a combination of intraoperative electrophysiological examinations tailored to the actual pathologies. The modalities included cortical and white matter mapping, cranial nerve and cranial nerve nucleus stimulation, motor evoked potential (MEP), somatosensory evoked potential (SSEP), peripheral nerve stimulation, bulbocavernosus reflex, and a special setup for selective dorsal rhizotomy. Results: The success ratio of the different modalities varied between 25% and 100%. All the applied methods could be used in children. Conclusion: Although the application of certain intraoperative techniques could be limited due to the ongoing developmental and maturation processes in childhood, we can not exclude the possibility of successful recording in any modality. Thus, we recommend to apply all the available methods in children bearing in mind that the success ratio might be lower than that in the adult population.

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