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.

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.

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.

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.

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.

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 [Sβ] = 0.163 P < 0.05), seizure frequency (Sβ−0.603; P < 0.01), and poly therapy (Sβ−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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.