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Coverpage
September-October 2017
Volume 65 | Issue 5
Page Nos. 943-1205

Online since Wednesday, September 6, 2017

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NI FEATURE - COMMENTARY: THE FIRST IMPRESSION  

The cover page Highly accessed article p. 943

DOI:10.4103/neuroindia.NI_724_17  PMID:28879871
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NI FEATURE - COMMENTARY: TIMELESS REVERBERATIONS Top

Memorable clinical experiences, all in one year at Queen Square, London, United Kingdom (1968-1969): A tribute to Neurology legends Highly accessed article p. 944
K Srinivasan
DOI:10.4103/neuroindia.NI_736_17  PMID:28879872
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NI FEATURE: JOURNEY THROUGH THE EONS Top

Dr. Harry M. Zimmerman (1901 – 1995): Neuropathologist who autopsied Dr. Harvey Cushing, and his interactions with Indian colleagues p. 948
Sunil K Pandya, SK Shankar
DOI:10.4103/neuroindia.NI_730_17  PMID:28879873
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NI FEATURE: THE EDITORIAL DEBATE I-- PROS AND CONS Top

Diffusion tensor imaging in spinal pathology: A robust investigative tool in clinical practice p. 964
Rishi M Kanna, S Rajasekaran
DOI:10.4103/neuroindia.NI_735_17  PMID:28879874
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Spinal cord diffusion tensor imaging: Developing a research tool for clinical use p. 966
Aditya Vedantam
DOI:10.4103/neuroindia.NI_740_17  PMID:28879875
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NI FEATURE: THE EDITORIAL DEBATE II-- PROS AND CONS Top

Muscular dystrophy: The long road ahead p. 968
Satish V Khadilkar
DOI:10.4103/neuroindia.NI_737_17  PMID:28879876
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Muscular dystrophies: An Indian scenario p. 969
Atchayaram Nalini, Kiran Polavarapu, Veeramani Preethish-Kumar
DOI:10.4103/neuroindia.NI_733_17  PMID:28879877
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NI FEATURE: THE EDITORIAL DEBATE III-- PROS AND CONS Top

Guillian-Barre syndrome and variants: Antiganglioside antibodies p. 971
J M K Murthy
DOI:10.4103/neuroindia.NI_738_17  PMID:28879878
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Enigmas in immunobiology of Guillain-Barré syndrome: Ganglioside antibodies and beyond! p. 973
Madhu Nagappa, Monojit Debnath, Arun B Taly
DOI:10.4103/neuroindia.NI_728_17  PMID:28879879
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Antiganglioside antibody: A hope or hype? p. 975
Jayantee Kalita, Usha K Misra
DOI:10.4103/neuroindia.NI_706_17  PMID:28879880
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NI FEATURE: THE EDITORIAL DEBATE IV-- PROS AND CONS Top

Can arterial spin labelling really replace dynamic susceptibility contrast perfusion techniques for assessing brain tumours in clinical practice? p. 977
Markand D Patel, Vijay Sawlani
DOI:10.4103/neuroindia.NI_727_17  PMID:28879881
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Arterial spin labelling: A novel promising perfusion technique – Can it be a replacement to dynamic susceptibility contrast MRI ? p. 979
Sona A Pungavkar, Rama Yanamandala
DOI:10.4103/neuroindia.NI_732_17  PMID:28879882
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REVIEW ARTICLES Top

The role of diffusion tensor imaging in spinal pathology: A review p. 982
Dan C Li, James G Malcolm, Rima S Rindler, Griffin R Baum, Avinash Rao, Shekar N Khurpad, Faiz U Ahmad
DOI:10.4103/neuroindia.NI_198_17  PMID:28879883
Diffusion tensor imaging (DTI) allows for noninvasive, in vivo visualization of white matter fiber tracts in the central nervous system by measuring the diffusion of water molecules. It provides both quantitative and qualitative (i.e., tractography) means to describe a region-of-interest. While protocols for the use of DTI are better established in the brain, the efficacy and potential applications of DTI in spinal cord pathology are less understood. In this review, we examine the current literature regarding the use of DTI in the spinal cord pathology, and in particular its diagnostic and prognostic value in traumatic injury, spinal tumors, cervical myelopathies, amyotrophic lateral sclerosis, and multiple sclerosis. Although structural magnetic resonance imaging (MRI) has long been the gold standard for noninvasive imaging of soft tissues, DTI provides additional tissue characteristics not found in the conventional MRI. We place emphasis on the unique characteristics of DTI, its potential value as an adjunct imaging modality, and its impact on clinical practice.
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Global muscular dystrophy research: A 25-year bibliometric perspective Highly accessed article p. 993
Shri Ram
DOI:10.4103/neuroindia.NI_1241_16  PMID:28879884
Muscular dystrophy is a genetic disorder leading to progressive weakness of muscles caused due to dysfunction in or lack of protein in muscle cells. The prevalence of muscular dystrophy has been observed globally and is becoming a critical area of study for better health services. The purpose of the study is to analyze the research strength of muscular dystrophy using bibliographic literature. A quantitative literature analysis was carried out on muscular dystrophy from 1991 to 2015 for assessing the global research trends. This literature-based study was conducted using the documents retrieved from the Science Citation Index using the keywords: Duchenne Muscular Dystrophy (DMD), Becker Muscular Dystrophy (BMD), Congenital Muscular Dystrophy (CMD), Myotonic Dystrophy, Emery-Dreifuss Muscular Dystrophy, Facioscapulohumeral Muscular Dystrophy, Oculopharyngeal Muscular Dystrophy, and Limb-Girdle Muscular Dystrophy. Analysis was done for annual productivity of publication, authorship, collaboration, country performance, citation frequency, characteristics of most cited document, journal productivity, etc.
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ORIGINAL ARTICLES Top

Anti-ganglioside antibodies profile in Guillain-Barré syndrome: Correlation with clinical features, electrophysiological pattern, and outcome p. 1001
G Sivaram Naik, A Kanikannan Meena, B Ashok Kumar Reddy, Rukmini K Mridula, Shaik A Jabeen, Rupam Borgohain
DOI:10.4103/neuroindia.NI_1226_15  PMID:28879885
Background: Guillain-Barré syndrome (GBS) and its subtypes are associated with distinct anti-ganglioside antibodies. Hence, we aimed to determine the frequency of anti-ganglioside antibodies and its correlation with clinical features, electrophysiological patterns, and outcome in patients with GBS. Material and Methods: The data regarding clinical features, electrophysiological patterns, and outcome at 6 months were collected and analyzed from the case records of patients diagnosed with GBS during 2008–2013 at a tertiary care hospital in south India. Results: A total of 204 patients with GBS were studied, and 73 patients (mean age: 37.6 ± 17.5 years) who underwent anti-ganglioside antibody testing were analyzed. Male-to-female ratio was 2.5:1. IgG anti-ganglioside antibodies were positive in 41/73 patients. The most common IgG anti-ganglioside antibody observed in the acute demyelinating variant was anti-GT1b (n = 13; 17.8%), and, those in the acute axonal variant were anti-GM1, anti-GM2, anti-GD1b, and anti-GT1b antibodies (n = 9;12.3% each). Three patients died and 5 patients were unable to walk independently at the end of 6 months. Conclusions: The frequency of anti-ganglioside antibodies in our cohort with GBS was 56%, with IgG anti-GT1b antibody being the most common. The anti-ganglioside antibodies were significantly positive in acute motor axonal neuropathy (AMAN) subtype of GBS. The presence of anti-ganglioside antibodies was not found to be of significant use in predicting the outcome. Although it was observed that the absence, and not the presence, of anti-ganglioside antibodies was associated with antecedent infection, dysautonomia, and requirement of ventilator support, the overall disease severity was not antibody dependant.
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A study on the clinical outcomes and management of meningitis at a tertiary care centre p. 1006
Aparna Yerramilli, Priya Mangapati, Subhashini Prabhakar, Harish Sirimulla, Shravani Vanam, Yashaswini Voora
DOI:10.4103/neuroindia.NI_785_15  PMID:28879886
Background: Meningitis is a life threatening central nervous system infection that is prevalent worldwide. Many studies have been conducted on bacterial meningitis, but the information is inadequate on the other types of meningitis, especially prevalent in India. Regional information regarding trends, in terms of etiology and microbiological susceptibility, are essential for timely and adequate management of meningitis. Aims: The present study was undertaken to evaluate the changing epidemiology of meningitis by reviewing the causative agents, the available laboratory methods for making the etiological diagnosis, the risk factors and the clinical outcomes, and the management of meningitis in our patient population. Materials and Methods: It is an observational study conducted at a tertiary care centre. A structured data form was designed to collect the information from the medical records and charts of medical investigations of the patients. Details regarding etiological agents, prevalence of clinical features and management were recorded and interpreted. Results: A total of 147 patients suspected to be having meningitis were included. The common types of meningitis in our study were aseptic (39%), tuberculous (28%), pyogenic (28%), fungal (3%) and others (2%). The classical triad of headache with fever, neck stiffness, and altered mental sensorium was seen in 26% patients, and 83% had at least 2 out of these 4 symptoms. The incidence of seizures was found to be 63% in the presence of aseptic meningitis. Significant clinical improvement was seen in 89% of the patients suffering from aseptic meningitis. Conclusions: Aseptic meningitis was found to be the predominant type among all different varieties of meningitis. An increased incidence of meningitis was seen in patients with diabetes mellitus. The incidence of seizures was high in viral/aseptic meningitis. The empirical treatment given in most of the cases was ceftriaxone. Isolation of the culpable organisms was possible in a very few cases due to the usage of empirical antibiotics prior to the performance of the lumbar puncture for the diagnostic analysis of the cerebrospinal fluid.
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Characteristics of nerve conduction studies in carpal tunnel syndrome p. 1013
Parag P Moon, Dilip Maheshwari, Vijay Sardana, Bharat Bhushan, Sankalp Mohan
DOI:10.4103/neuroindia.NI_628_16  PMID:28879887
Background: Numerous nerve conduction tests are used for the electrodiagnosis of carpal tunnel syndrome (CTS), with a wide range of sensitivity and specificity reported for each test in clinical studies. Aims: The purpose of this study was to compare the diagnostic accuracy of various nerve conduction tests and determine the properties of the most accurate test. Settings and Design: A prospective observational case control study. Patients and Methods: Eighty patients with clinically confirmed CTS and 80 asymptomatic healthy controls were included in the study. All patients underwent the routine hematological investigations as per the protocol. All cases and controls were subjected to various nerve conduction study protocols for CTS. Results were analyzed statistically. Statistical Analysis Used: The two-tailed Student's t-test was used for the comparative statistical analysis. The sensitivity of each test was calculated as (the number of hands with an abnormal study result/the number of CTS hands) × 100. Comparison between percentages was performed by the McNemar test. Results: The mean age was 38.19 ± 10.13 years and the female:male ratio was 1.5:1. The mean duration of disease was 0.89 ± 0.61 years. Hypothyroidism was present in 21 (26.25%) patients, whereas 13 (16.25%) and 4 (5%) patients had diabetes mellitus and rheumatoid arthritis, respectively. The median nerve motor latency was 4.73 ± 0.83 ms while the sensory latency was 3.44 ± 0.56 ms. The median nerve orthodomic sensory latency was found to be 2.57 ± 0.31 ms. The conduction velocity across the palm and wrist was 41.37 ± 0.67 ms. The sensitivity was the highest in the inching method (86.25%) and lowest for the conventional median motor and sensory latencies (56.25% and 45%, respectively). Conclusions: Addition of a single test of median and ulnar sensory latency, the median and radial sensory latency or the inching method, in routine protocol will improve the sensitivity for the diagnosis of CTS in all patients.
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COMMENTARY Top

Carpal tunnel syndrome with equivocal electrophysiological findings: Additional testing may improve diagnostic sensitivity p. 1017
Suraj A Muley
DOI:10.4103/neuroindia.NI_818_17  PMID:28879888
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ORIGINAL ARTICLES Top

Predictors of quality of life among adolescents with epilepsy in the state of Andhra Pradesh p. 1019
M Nagarathnam, B Shalini, V Vijayalakshmi, B Vengamma, S A A Latheef
DOI:10.4103/neuroindia.NI_1251_15  PMID:28879889
Objectives: Assessment of quality of life (QOL) reveals the impact of diseases and factors responsible for the impairment of quality of life. The purpose of this study was to evaluate the QOL among adolescents with epilepsy (AWE) in the state of Andhra Pradesh. Materials and Methods: One hundred and fifty AWE aged 13–19 years were evaluated for QOL using the Telugu version of the Quality of Life in Epilepsy Inventory for Adolescents (QOLIE) AD-48 and the data were analyzed to predict the factors responsible for determining the QOL. Results: The mean age of AWE was 15.86 ± 2.14 years. The age at onset of seizures among AWE was 9.28 ± 4.90 years. Generalized (45%) and partial seizures (34%) were the predominant types of seizures. The majority of AWE (77%) were taking anti epileptic medication for 1–8 years, were on monotherapy (55%), and were seizure free for the last 1 year (56%). The mean total QOL score in AWE was 72 ± 15. The high school-educated, seizure-free, and monotherapy-taking AWEs showed a significantly higher mean total QOL when compared to the primary school- educated, seizure-frequent, and polytherapy-taking AWEs (P < 0.01). Education (standardized beta [] = 0.163 P < 0.05), seizure frequency (−0.603; P < 0.01), and poly therapy (−0.08; P < 0.01) were significant predictors of QOL in AWE. Conclusions: The results of the study suggest that in addition to seizure control, encouraging monotherapy and enhancing the education level may improve the QOL in AWE.
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Evaluation of hand function in healthy individuals and patients undergoing uninstrumented central corpectomy for cervical spondylotic myelopathy using nine-hole peg test p. 1025
Sanil John, Ranjith K Moorthy, Tunny Sebastian, Vedantam Rajshekhar
DOI:10.4103/neuroindia.NI_12_17  PMID:28879890
Objectives: To evaluate the hand function in healthy individuals and in patients with cervical spondylotic myelopathy (CSM) undergoing central corpectomy using the nine-hole peg test (NHPT). Materials and Methods: The NHPT was performed in healthy adults and in patients with CSM; overall, five trials were performed in the right and left hand separately. The preoperative and follow up NHPT score was compared to the normal and correlated with Nurick and modified Japanese Orthopedic Association (mJOA) scales. Results: The NHPT score was significantly less in adult healthy female compared to adult healthy male subjects (difference, 0.71 s, P < 0.002). The distribution of the NHPT scores in normal adults followed the normal binomial distribution. The time taken to perform the NHPT with the right hand was significantly lower than the time taken to perform the NHPT with the left hand in both the sexes (P < 0.001). Thirty-six of the 47 patients with CSM (76.6%) had a prolonged preoperative NHPT score. There was a strong negative correlation between the preoperative NHPT score and the preoperative upper limb component of the modified-Japanese Orthopedic Association (UlmJOA) score. No significant change was detected in the NHPT score at one week postoperatively. On follow-up at six months or more (n = 21), the NHPT score normalized in five (35.7%) of the 14 patients in whom it was prolonged preoperatively. The NHPT score remained the same as the preoperative status in the other 16 patients, 7 of whom had a normal score preoperatively. The change in the NHPT score at follow-up did not correlate with the change in the UlmJOA score. Conclusions: Normative data among the Indian population suggest that female subjects have significantly lower scores than the male ones, and that there is a difference between the two sides that needs to be considered while reporting the NHPT scores in disease. The NHPT scores were prolonged preoperatively in CSM and showed a correlation with the UlmJOA score, and there was no significant change noted at one week follow-up. While the NHPT score is a good quantitative test to evaluate hand function in patients with CSM and could detect subtle hand dysfunction preoperatively, it has a limited role, when used alone, to detect changes in hand function postoperatively.
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COMMENTARY Top

Restoration of hand function: A critical parameter during neurorehabilitation, and the confounding factors influencing its evaluation p. 1031
K Sridhar, ES Krishnamoorthy
DOI:10.4103/neuroindia.NI_729_17  PMID:28879891
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Nine-hole peg test for evaluation of hand function: The advantages and shortcomings p. 1033
Luis Carelli Teixeira da Silva
DOI:10.4103/neuroindia.NI_739_17  PMID:28879892
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ORIGINAL ARTICLE Top

A novel swine model of subarachnoid hemorrhage-induced cerebral vasospasm p. 1035
Zion Zibly, Lory Fein, Mayur Sharma, Yaron Assaf, Anton Wohl, Sagi Harnof
DOI:10.4103/neuroindia.NI_357_16  PMID:28879893
Objective: One of the most serious complications following subarachnoid hemorrhage (SAH) is delayed cerebral ischemia (DCI) secondary to symptomatic vasospasm. An animal model mimicking post SAH vasospasm is essential for enabling the translation of newer technologies from the conceptual phase to animal studies, and eventually to clinical trials. Various animal models of DCI following SAH have been reported, with canine models being the most common. Due to the similarity of the swine cardiovascular system and its dimensions to the human's system, the main objective of this study was to establish a consistent and quantitatively representative model of SAH-induced vasospasm in swine. Materials and Methods: Twelve female swines (57 ± 3 kg) were injected twice (with a 2-day interval between injections) with autologous blood into the subarachnoid space at the level of C2-3 vertebrae. Different volumes were injected to identify clinical and radiological changes. The effect of volume variations on hematoma size and vasospasm intensity in the circle of Willis arteries were studied 7–14 days after the first injection using ascending pharyngeal angiographic measurements of vessel diameter. Neurological outcome using a modified scoring table based on clinical parameters (e.g., appetite, behavior, walking, posture, and eye movement) was recorded. Results: Our results demonstrate that between volume combinations, intrathecal injection of 12 ml followed by 15 ml, with a 2-day interval in between, resulted in the most extensive angiographically-assessed vasospasm 12 ± 2 days following the first injection. The degree of vasospasm in the intracranial internal carotid artery was 22% and 16% for the left and right sides, respectively. Vasoconstriction of the anterior cerebral artery was 34% and 27% for the left and right sides, respectively. The vasoconstriction was not associated with either overt clinical signs or clinical outcome, which is indicative of an ischemic event. Conclusions: The relative scarcity of swine models for SAH-induced vasospasm motivated us to develop and quantify a straightforward protocol for producing consistent mild-to-moderate vasospasm following SAH. As swine is commonly used in translational cardiovascular research, we believe that this study constitutes an important phase in the study of SAH and in developing pharmacological agents and medical devices for interventions.
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COMMENTARY Top

Animal models for cerebral vasospasm: Where do we stand? p. 1043
Dhananjaya I Bhat
DOI:10.4103/neuroindia.NI_731_17  PMID:28879894
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ORIGINAL ARTICLES Top

Perfusion MR imaging of enhancing brain tumors: Comparison of arterial spin labeling technique with dynamic susceptibility contrast technique p. 1046
Neetu Soni, Devender Pal S Dhanota, Sunil Kumar, Awadhesh K Jaiswal, Arun K Srivastava
DOI:10.4103/neuroindia.NI_871_16  PMID:28879895
Objective: Arterial spin labeling (ASL) magnetic resonance (MR) perfusion is a noninvasive and repeatable method for quantitatively measuring cerebral blood flow (CBF). This study aims to compare measurements of ASL-derived CBF with dynamic susceptibility contrast (DSC) MRI in the assessment of enhancing brain tumors (primary and metastatic), with an aim to use ASL as an alternative to DSC. Materials and Methods: Thirty patients with newly diagnosed brain tumors (16 meningiomas, 6 gliomas, 3 metastases, 2 cerebellopontine angle schwannoma, 1 central neurocytoma, and 2 low-grade gliomas) were examined using a 3T MR scanner. Values of CBF, regional cerebral blood flow (rCBF), and regional cerebral blood volume (rCBV) were determined in the tumor (T) as well as in the contralateral normal gray matter (GM) and white matter (WM). Tumor-to-GM or WM CBF, rCBF, and rCBV ratios were calculated to estimate normalized perfusion values (i.e., ASL normalized tumor blood flow [nTBF], DSC nTBF, and DSC normalized tumor blood volume [nTBV]) from the ASL and DSC techniques. ASL and DSC MRI derived perfusion parameters were compared using paired t-test and correlated using Pearson correlation coefficient. Results: Mean values for ASL nTBF and DSC nTBF using contralateral GM as the reference point were 2.98 ± 1.67and 2.91 ± 1.43, respectively. A very strong correlation coefficient was found between ASL nTBF and DSC nTBF with contralateral GM as the reference region (r = 0.903; R2= 0.813). Mean DSC nTBF and DSC nTBV also showed strong correlation (r = 0.83; R2= 0.701). Conclusion: Our study results suggested that measurement of CBF from ASL possesses the potential for a noninvasive assessment of blood flow in intracranial tumors as an alternate to DSC MRI, in those patients requiring multiple follow-up imaging and in patients with impaired renal functions.
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A comparison of the recovery profiles of desflurane and isoflurane anesthesia in patients undergoing elective supratentorial craniotomy: A randomized controlled trial p. 1053
Aparanjit P Paul, Aditya Vedantam, Grace Korula, Ari G Chacko
DOI:10.4103/neuroindia.NI_1136_15  PMID:28879896
Context: Few studies have compared recovery profiles of desflurane and isoflurane for patients undergoing elective supratentorial craniotomy. It is not known if the choice of inhalational agent can affect the duration of transient postoperative neurological deficits in these patients. Aims: To compare the effect of desflurane and isoflurane on time-to-emergence and time-to-recovery of transient postoperative neurological deficits in patients undergoing supratentorial craniotomy. Settings and Design: Prospective, double-blinded, randomized controlled trial at a tertiary care hospital. Methods and Materials: We randomly assigned 60 patients to receive either desflurane or isoflurane during elective supratentorial craniotomy for intra-axial mass lesions. Time-to-emergence and time-to-recovery of transient postoperative neurological deficits were recorded and compared. Statistical Analysis Used: Parametric variables were compared by the Student's t test. Baseline data was compared using Pearson's chi square test, Fisher's exact test and two proportion Z test. Results: There was a 35.7%, 31.4% and 34.5% reduction in median times to eye opening, obeying commands and orientation in the desflurane group (n=27) as compared to the isoflurane group (n=28). Five patients were enrolled but not included for analysis-Twelve patients sustained transient neurological deficits after surgery (desflurane, n=3; isoflurane, n=9). No significant difference in the time-to-recovery of transient postoperative neurological deficits was observed. Conclusions: Desflurane significantly reduced emergence times, and was able to facilitate an early neurological examination for patients. Additional studies are required to establish the impact of inhalational agents on transient postoperative neurological deficits.
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COMMENTARY Top

The art of waking up a neurosurgical patient: It is time to hone our skills p. 1059
Hemant Bhagat, Ankur Luthra
DOI:10.4103/neuroindia.NI_674_17  PMID:28879897
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CASE REPORTS Top

Progressive multifocal leukoencephalopathy in a 44-year old male with idiopathic CD4+ T-lymphocytopenia treated with mirtazapine and mefloquine p. 1061
Aruna Nambirajan, Vaishali Suri, Vijay Kataria, Mehar C Sharma, Vinay Goyal
DOI:10.4103/neuroindia.NI_535_16  PMID:28879898
Progressive multifocal leukoencephalopathy (PML) is an opportunistic viral infection of the central nervous system caused by the reactivation of John Cunningham virus (JCV) in immunocompromised patients, most commonly in human immunodeficiency virus (HIV) infection, and less commonly in those receiving various immunosuppressive regimens. Prognosis of untreated PML is grave and the mainstay of treatment is the reversal of immunosuppression, usually by institution of antiretroviral drugs in HIV patients and cessation of immunosuppressive therapies in others. PML is increasingly being reported in those with minimal or occult immunosuppression. A small fraction of these patients meet the criteria for idiopathic CD4+ T-lymphocytopenia (ICL) after exclusion of all secondary causes of lymphocytopenia, including HIV. A 44-year-old previously healthy male presented with clinical and radiological features suggestive of PML. Cerebrospinal fluid samples were repeatedly negative for JCV. Immunohistochemistry on brain biopsy eventually confirmed PML. Despite extensive work-up, the only abnormality detected was an unexplained and persistently low absolute CD4+ T-lymphocyte count. Based on the limited available literature on the treatment of non-HIV PML, he was treated with a combination of mirtazapine and mefloquine with clinical improvement. Non-HIV PML remains relatively uncommon, and PML as a presenting feature of ICL is rare. It is important to document and follow these patients to be able to assess the relative risks associated with various causes and formulate effective therapeutic strategies.
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Hyperekplexia: A forgotten diagnosis clinched by next-generation sequencing p. 1065
Meenakshi Lallar, Anukool Srivastava, Shubha R Phadke
DOI:10.4103/neuroindia.NI_851_16  PMID:28879899
Hyperekplexia is a rare early neonatal onset, potentially treatable, neurological disorder, characterized by a triad of immediate neonatal-onset stiffness, an exaggerated startle reflex in response to tactile or auditory stimuli followed by short periodical generalized stiffness. It is a monogenic genetically heterogeneous condition which can be potentially life threatening due to apneic episodes and is usually misdiagnosed as seizures. Here, we report two female siblings with hyperekplexia who were being treated by multiple antiepileptic medications for seizure-like episodes. Hyperekplexia was diagnosed by next-generation sequencing, which has emerged as a powerful diagnostic tool over the last few years.
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Simultaneous odontoid excision with bilateral posterior C1-2 distraction and stabilization utilizing bilateral posterolateral corridors and a single posterior midline incision p. 1068
Arun K Srivastava, Sanjay Behari, Jayesh Sardhara, Kuntal Kanti Das
DOI:10.4103/neuroindia.NI_819_17  PMID:28879900
A simultaneous odontoid decompression and bilateral posterior atlanto-axial facetal distraction, C1-2 joint spacer/bone graft placement and stabilization may be performed utilizing the ‘posterior-only’ approach. This procedure may be performed utilizing a single posterior midline incision, a bilateral posterior approach to the C1-2 facet joints and a bilateral posterolateral approach to the odontoid process and C2 body. It may be carried out in situations where a C1-2 non-reduction/partial reduction using a ‘posterior alone’ procedure is anticipated due to the complex bony/soft tissue configuration anterior at the thecal sac existing at the cervicomedullary junction. In the four cases described in this report, the procedure led to a successful circumferential decompression at the level of foramen magnum along with posterior C1-2 facetal distraction and stabilization in various complex craniovertebral junction anomalies (atlantoaxial dislocation [AAD] and/or a high basilar invagination [BI] associated with a significantly retroverted dens, along with a rotatory component, due to grossly asymmetrical facet joints). This technique may also be utilized in those diseases that result in an anterior osteoligamentous mass at the CVJ associated with C1-2 instability.
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NI FEATURE: PATHOLOGY PANORAMA - ORIGINAL ARTICLE Top

BRAF gene alterations and enhanced mammalian target of rapamycin signaling in gangliogliomas p. 1076
Aanchal Kakkar, Atreye Majumdar, Pankaj Pathak, Anupam Kumar, Kalpana Kumari, Manjari Tripathi, Mehar C Sharma, Vaishali Suri, Vivek Tandon, Sarat P Chandra, Chitra Sarkar
DOI:10.4103/neuroindia.NI_207_17  PMID:28879901
Background: Gangliogliomas (GGs) are slow-growing glioneuronal tumors seen in children and young adults. They are associated with intractable epilepsy, and have recently been found to harbor BRAF (B- rapidly accelerated fibrosarcoma) gene mutations. However, the mammalian target of rapamycin (mTOR) signaling pathway, downstream of BRAF, has not been evaluated extensively in GGs. Materials and Methods: GG cases were retrieved, clinical data obtained, and histopathological features reviewed. Sequencing for BRAF V600E mutation, analysis of BRAF copy number by quantitative real-time polymerase chain reaction, and immunohistochemistry for mTOR pathway markers p-S6 and p-4EBP1 were performed. Results: Sixty-four cases of GG were identified (0.9% of central nervous system tumors). Of these, 28 had sufficient tumor tissue for further evaluation. Mixed glial and neuronal morphology was the commonest (64%) type. Focal cortical dysplasia was identified in the adjacent cortex (6 cases). BRAF V600E mutation was identified in 30% of GGs; BRAF copy number gain was observed in 50% of them. p-S6 and p-4EBP1 immunopositivity was seen in 57% cases each. Thus, mTOR pathway activation was seen in 81% cases, and was independent of BRAF alterations. 87% patients had Engel grade I outcome, while 13% had Engel grade II outcome. Both the Engel grade II cases analyzed showed BRAF V600E mutation. Conclusion: BRAF V600E mutation is frequent in GGs, as is BRAF gain; the former may serve as a target for personalized therapy in patients with residual tumors, necessitating its assessment in routine pathology reporting of these tumors. Evidence of mTOR pathway activation highlights similarities in the pathogenetic mechanisms underlying GG and focal cortical dysplasia, and suggests that mTOR inhibitors may be of utility in GG patients with persistent seizures after surgery.
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NI FEATURE: THE QUEST - COMMENTARY Top

Review of tremor in Parkinson's disease and atypical parkinsonian disorders p. 1083
Pooja Mailankody, M Netravathi, Pramod Kumar Pal
DOI:10.4103/neuroindia.NI_880_16  PMID:28879902
Rest tremor (RT), a cardinal feature of Parkinson's disease (PD) is often accompanied by other types of tremor such as action tremor, which includes postural tremor, kinetic tremor, re-emergent tremor (ReT), and orthostatic tremor (OT). Literature on other tremors of PD, especially ReT and OT, is scarce. Tremor can be present in any of the atypical parkinsonian disorders such as progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. RT can even be the presenting symptom of these disorders. The objective of this review is to provide a comprehensive review of lesser known tremors in PD and to critically look at the prevalence of tremor in atypical Parkinsonian disorders.
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Management strategies in tremors: A bird's eye view p. 1091
Milind Sankhe
DOI:10.4103/neuroindia.NI_749_17  PMID:28879903
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NI FEATURE: CENTS (CONCEPTS, ERGONOMICS, NUANCES, THERBLIGS, SHORTCOMINGS) - COMMENTARY Top

A neurosurgical perspective to approaches to the orbit: A cadaveric study p. 1094
S Balaji Pai, MN Nagarjun
DOI:10.4103/neuroindia.NI_726_17  PMID:28879904
Neurosurgeons are getting increasingly involved in surgery for orbital tumours. The various approaches to the orbit can be challenging. The authors have demonstrated on cadavers the various approaches to the orbit which are required to be mastered by the neurosurgeon. The superior approaches which include the transcranial, supraorbital and the hybrid approaches are the most commonly utilized surgical approaches by the neurosurgeon for excision of orbital pathologies. The lateral orbitotomy is an elegant approach for excision of the tumours lateral to the optic Nerve and lacrimal gland tumours. The authors also demonstrate the medial approach through a medial orbitotomy which is a relatively unfamiliar approach to neurosurgeons. The importance of the various landmarks in each approach is emphasised along with the methods to minimize complications. It is imperative that neurosurgeons are intimately familiar with the microsurgical anatomy of the orbit and the various approaches to it.
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Endonasal endoscopic approach to orbit p. 1102
Awadhesh Kumar Jaiswal
DOI:10.4103/neuroindia.NI_723_17  PMID:28879905
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NI FEATURE: CITADELS SCULPTING FUTURE - COMMENTARY Top

Neurosurgery at Kasturba Medical College: Past, present and future p. 1105
Girish Menon, Rajesh Nair, IK Laskhman, Vinod Kumar, G Lakshmi Prasad
DOI:10.4103/neuroindia.NI_564_17  PMID:28879906
Kasturba Medical College (KMC), Manipal, is the oldest and one of the most reputed medical institutes in coastal Karnataka, catering to a population of over 4 million, spanning a stretch of over 350 kms along the southwest coast of India from Mangalore to Goa. The Department of Neurosurgery at KMC, established in 1968, continues to be the leading and most preferred referral center providing high quality neurosurgical services in this region. The article provides an insight into the origin of the department, its infancy and teething troubles, its continuous growth and landmark achievements over the years. This brief review also highlights the current area of focus and describes the plans for its future development.
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NI FEATURE: THE FOURTH DIMENSION - COMMENTARY Top

A summary of some of the recently published, seminal papers in neuroscience p. 1112
K Sridhar, Mazda K Turel, Manjul Tripathi, Ravi Yadav, PR Srijithesh, Aastha Takkar, Sahil Mehta, Kuntal K Das, Anant Mehrotra, Chirag K Ahuja
DOI:10.4103/neuroindia.NI_734_17  PMID:28879907
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NI FEATURE: FACING ADVERSITY…TOMORROW IS ANOTHER DAY! - LETTERS TO EDITOR Top

Multiple paraneoplastic antibodies (anti-SOX1, anti-Hu, and anti-Amphiphysin) detected in a patient with limbic encephalitis and small cell lung cancer p. 1127
Thiago Gonçalves Fukuda, Mateus Santana do Rosário, Renan Carvalho Castello Branco, Jamile Seixas Fukuda, Ricardo Antenor de Souza e Souza, Jamary Oliveira-Filho, Pedro Antonio Pereira de Jesus
DOI:10.4103/neuroindia.NI_1256_15  PMID:28879908
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Acute spinal cord infarction after aortobifemoral bypass p. 1128
Sami Kouki, Emna Labben, Nejmeddine B Abdallah
DOI:10.4103/neuroindia.NI_745_16  PMID:28879909
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MRI findings in a fetus with a tight cord around the neck p. 1130
Haree S Meganathan, R Rajeshwaran, S Bhuvana
DOI:10.4103/neuroindia.NI_671_16  PMID:28879910
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Neurosarcoidosis-induced longitudinal extensive transverse myelitis p. 1131
Sushilkumar S Gupta, Shyam Shankar, Evan Stein, Sina Khasani
DOI:10.4103/neuroindia.NI_505_16  PMID:28879911
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A case of clear cell ependymoma with bizarre aggressiveness: Are we underestimating its ferocity? p. 1134
Kirti Gupta, Manjul Tripathi, Nalini Gupta, Sachin Ranganath, Pravin Salunke
DOI:10.4103/neuroindia.NI_378_16  PMID:28879912
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LETTERS TO EDITOR Top

Acute hemicerebellitis in children: A report and literature review p. 1137
Riad M Elsayed, Hala E Sayyah
DOI:10.4103/neuroindia.NI_1289_15  PMID:28879913
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Palatal tremor secondary to a dolichoectatic basilar artery p. 1138
Sanjay Pandey, Arun Koul, Priyanka Tater, Neelav Sarma
DOI:10.4103/neuroindia.NI_983_16  PMID:28879914
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Novel c.775_781dup,p(Thr261fs) mutation in SPG 7 gene in a patient with hereditary spastic paraparesis p. 1141
Shakya Bhattacharjee, Victoria Stinton, Diane Cairns, Brian Murray
DOI:10.4103/neuroindia.NI_96_17  PMID:28879915
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Meningitis, polyradiculopathy, and optic nerve involvement in neurobrucellosis: A rare clinical presentation p. 1142
Waseem Dar, Muzamil Latief, Imtiyaz Dar, Najeebullah Sofi
DOI:10.4103/neuroindia.NI_127_16  PMID:28879916
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Anarthria in a patient with bilateral striopallidodentate calcinosis p. 1144
Shoba Sreenath Meera, N Shivashankar, Ravi Yadav, Maya Dattatraya Bhat
DOI:10.4103/neuroindia.NI_130_17  PMID:28879917
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Magnetic resonance imaging findings in heat stroke-related encephalopathy p. 1146
Ravi K Jakkani, Vijay K Agarwal, Suryanarayana Anasuri, Sriharish Vankayalapati, Rahul Koduri, Sandeep Satyanarayan
DOI:10.4103/neuroindia.NI_740_16  PMID:28879918
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Faciobrachial dystonic spells: Presenting feature of autoimmune encephalopathy p. 1149
Divyanshu Dubey, Abdulradha Alqallaf, Worthy Warnack, Puneet Gupta, Jay Harvey, Steven Vernino
DOI:10.4103/neuroindia.NI_452_16  PMID:28879919
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Stiff-person syndrome after thymectomy in myasthenia gravis mimicking a post-thymectomy myasthenic crisis p. 1152
Hye Lim Lee, Ju-Hong Min, Jin Myoung Seok, Eun Bin Cho, Sang-soo Lee, Hye Jin Cho, Byoung Joon Kim
DOI:10.4103/neuroindia.NI_493_16  PMID:28879920
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Spinocerebellar ataxia type 2 associated with amyotrophic lateral sclerosis p. 1153
Rajesh K Singh, Kamlesh K Sonkar, Sanjeev Bhoi, Jayantee Kalita, Ushakant Misra
DOI:10.4103/neuroindia.NI_910_16  PMID:28879921
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Commentary: Amyotrophic lateral sclerosis: Ongoing search for prognostic biomarkers of longevity p. 1155
Sanjay Pandey, Neelav Sarma
DOI:10.4103/neuroindia.NI_7_17  PMID:28879922
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"Round the Houses” sign and apraxia of eyelid opening in a 62-year old man p. 1157
Shakya Bhattacharjee, Kher Lik Ng
DOI:10.4103/neuroindia.NI_46_17  PMID:28879924
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Unusual presentation and temporal events in a patient with cavernous carotid aneurysm p. 1157
Swati D Chinchure, Varun Kataria, Om Prakash Lekhara, Rahul Awashi, Ankit Mathur, Rajneesh Kachhara
DOI:10.4103/neuroindia.NI_118_17  PMID:28879923
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Granular cell tumor at an unusual site masquerading as a neurofibroma p. 1159
Irneet Mundi, Ashis Pathak, Ashru K Banerjee, Rahat Brar
DOI:10.4103/neuroindia.NI_67_17  PMID:28879925
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Thyrotropic pituitary adenoma with plurihormonal immunoreactivity p. 1162
Tarang K Vora, Sudish Karunakaran
DOI:10.4103/neuroindia.NI_577_16  PMID:28879926
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Hemorrhagic colloid cyst p. 1164
Girish Menon, Lakshman I Kongwad, Rajesh P Nair, Vinod Kumar
DOI:10.4103/neuroindia.NI_909_16  PMID:28879927
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Primary pituitary abscess in preadolescence mimicking a sellar mass p. 1167
Hesam Akbarian-Tefaghi, Kanika Sharma, Piyush Kalakoti, Aileen Cangiano-Heath, Devi Prasad Patra, Papireddy Bollam, Anil Nanda, Bharat Guthikonda
DOI:10.4103/neuroindia.NI_997_16  PMID:28879928
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Intracranial inflammatory pseudotumor presenting as an en plaque mass p. 1169
Siddharth Gautam, Vengalathur G Ramesh, Kavindapadi V Karthikeyan, Murugan Krishnakumar
DOI:10.4103/neuroindia.NI_395_17  PMID:28879929
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Management of a case of neglected atlantoaxial rotatory dislocation p. 1170
Atul Goel, Sonal Jain, Abhidha Shah
DOI:10.4103/neuroindia.NI_397_17  PMID:28879930
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Dural-based giant cavernous hemangioma mimicking a meningioma: Lessons learnt p. 1173
Pavan K Pelluru, Alugolu Rajesh, Megha S Uppin
DOI:10.4103/neuroindia.NI_1151_15  PMID:28879931
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Rosette-forming glioneuronal tumor of the fourth ventricle p. 1176
Rajashree Pradhan, Sajeeb Mondal, Subrata Pal, Sharmistha Chatterjee, Arindam Banerjee, Debosmita Bhattacharyya
DOI:10.4103/neuroindia.NI_862_16  PMID:28879932
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Subgaleoatrial shunt: Further progress in the management of iatrogenic cranial pseudomeningoceles p. 1178
Ananth P Abraham, Edmond Jonathan Gandham, Krishna Prabhu, Ari G Chacko
DOI:10.4103/neuroindia.NI_881_16  PMID:28879933
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Primary epithelioid hemangioendothelioma of the dorsal spine: A review p. 1180
Joe M Das, Rony Louis, BS Sunilkumar, Anil Kumar Peethambaran
DOI:10.4103/neuroindia.NI_896_16  PMID:28879934
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Spontaneous resolution of syringomyelia in Chiari I malformation: A review of literature p. 1187
Pradeep Kumar Jain, SV Sreeharsha, Suresh Dugani
DOI:10.4103/neuroindia.NI_899_16  PMID:28879935
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NEUROIMAGES Top

Gestational Wernicke's encephalopathy p. 1190
Vidhi S Shah, Maheshkumar Shanmugam, Akkayasamy Kowsalya, KG Srinivasan, Prathibha Siddappa, Vellaiappan Neethiarasu, Anand K Annamalai
DOI:10.4103/neuroindia.NI_35_17  PMID:28879936
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Canavan disease with typical brain MRI and MRS findings p. 1191
Anil V Israni, Anirban Mandal
DOI:10.4103/neuroindia.NI_92_17  PMID:28879937
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Isoniazid toxicity presenting as bilateral dentate hyperintensities p. 1193
Aarthi Deepesh, Hima S Pendharkar
DOI:10.4103/neuroindia.NI_249_17  PMID:28879938
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A report of third case of Susac syndrome from India p. 1194
Kamble J Harsha, Jeevan S Nair
DOI:10.4103/neuroindia.NI_543_16  PMID:28879939
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Isolated globus pallidi hypointensities in type 2 GM1 gangliosidoses p. 1195
Mahesh Kamate, Nishant Mittal
DOI:10.4103/neuroindia.NI_926_16  PMID:28879940
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A rare cause of neck pain: Holocord intramedullary lipoma p. 1196
Sami Kouki, Emna Labben, Nejmeddine B Abdallah
DOI:10.4103/neuroindia.NI_749_16  PMID:28879941
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Incidental finding of an accessory middle cerebral artery in a patient with multiple aneurysms p. 1198
Manish Garg, Shaam Bodeliwala, Vikas Kumar, Anita Jagetia
DOI:10.4103/neuroindia.NI_280_17  PMID:28879942
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BOOK REVIEWS Top

Epilepsy and law in India p. 1201
Vikas Dhiman
DOI:10.4103/neuroindia.NI_329_17  
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Admissions. A life in brain surgery p. 1202
Sunil Pandya
DOI:10.4103/neuroindia.NI_725_17  
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Practical Neurosurgery: Analysis of clinical cases p. 1205
VG Ramesh
DOI:10.4103/neuroindia.NI_748_17  
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Online since 20th March '04
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