Background: Attention-deficit/hyperactivity disorder (ADHD) is a neuro-developmental disease commonly seen in children and it is diagnosed via extensive interview procedures, behavioral studies, third-party observations, and comprehensive personal history. ADHD causes regional atrophy in brain regions and alters the pattern of functional brain connectivity networks. Automated/computerized methods based on magnetic resonance imaging (MRI) can replace subjective methods for the identification of ADHD. Objectives: The aim of this study was to analyze various machine-learning algorithms for ADHD by feeding in vital input features extracted from functional brain connectivity and different existing methods and to review factors crucial for the diagnosis of ADHD. Methods: This paper is a concise review of machine learning methods for the diagnosis of ADHD from MRI. Techniques for feature extraction, dimensionality reduction/feature selection, and classification, employed in the computerized techniques for the diagnosis of ADHD from MRI and the accuracy of classification offered by the individual methods, are focussed on the review. Conclusions: Machine learning algorithms with features of functional brain connectivity networks as input, with hierarchical sparse feature elimination, exhibits the highest accuracy. Augmentation of the behavioral features does not contribute much to increased accuracy. The level of accuracy offered by the frameworks meant for the computer-aided diagnosis of ADHD, available in the literature, does not justify their feasibility in clinical practice. Computerized methods that exploit highly specific biomarkers of ADHD like brain iron concentration in Globus Pallidus, Putamen, Caudate nucleus, and thalamus as features are not available.

Background: Lamotrigine (LMT) is a phenyltriazine derivative that was originally described as an antiepileptic drug. Objective: This literature review aims to evaluate the clinical epidemiological profile, pathological mechanisms, and management of lamotrigine-associated movement disorders. Methods: Relevant reports in six databases were identified and assessed by two reviewers without language restriction. Reports that the individuals only developed tremor or ataxia after LMT use were not included. Results: In total 48 reports of 108 cases from 19 countries were assessed. The movement disorders associated with LMT found were 29 tics, 21 dyskinesias, 14 myoclonus, 13 parkinsonism, 10 dystonia, and 1 stuttering. The not clearly defined cases included 10 akathisia, 4 myoclonus, 4 cerebellar syndromes, 1 hypertonia, 1 dyskinesia, and an unknown number of dystonia cases. The mean reported age was 33.34 years (range: 1.574 years). The male was the predominant sex and the most common LMT indication was epilepsy. The mean LMT-dose at the movement disorder onset was 228 mg. The time from LMT start to the onset of movement disorder was within 6 months in 81%. The time from LMT withdrawal to complete recovery was within 1 month in 83%. The most common management was LMT withdrawal. Conclusions: In the literature, the majority of the cases did not give a clear picture of the individual, and the times of movement disorder onset and recovery are not described. We believe that before withdrawal LMT, a dose adjustment based on the benefits and adverse events with careful evaluation case-by-case can be done.

Background: Multiple sclerosis is a chronic demyelinating disorder with a myriad of imaging and clinical features that overlap with number of other neurological conditions. Incorrect diagnosis poses a significant risk to patients, it may lead to delays in management, increased morbidity, and also adds to the financial cost. Objective: The aim of this study was to highlight strategies for the efficient differentiation of multiple sclerosis from other diseases which may masquerade as MS clinico-radiologically. Material and Methods: A systematic literature review was conducted through online databases including PubMed and Medline. Relevant publications on radiological aspects of multiple sclerosis, white matter diseases and mimickers of Multiple sclerosis were included in the analysis. Results: Common mimickers of MS include small vessel disease, acute disseminated encephalomyelitis, neuromyelitis optica, anti-MOG encephalomyelitis, vasculitis, and CADASIL. Contrast-enhanced MRI study performed using MS protocol on high strength MRI system evaluated following a structured protocol along with clinical correlation is effective in differentiating MS from its mimickers. Conclusions: Contrast-enhanced MRI performed on a high strength scanner using MS protocol with structured protocol for evaluation along, with a better collaboration between radiologists and clinicians may help in minimizing errors in diagnosis of multiple sclerosis.

This review article highlights the necessity and importance of peer review of articles submitted to journals. Publication in a peer reviewed, good impact indexed journal is an indirect endorsement of the article contents. The editor and the readership depend on the integrity and total involvement of reviewers. What is expected of the reviewer and the authors is discussed. Reviewers need to be sensitive to authors' reactions. Authors should also respond, not react or be hypersensitive.

Background and Introduction: Intraoperative ultrasound imaging can be a multi-dimensional tool with various applications, including localization, resection control, and biopsy of brain tumors. Objective: We demonstrate a frameless biopsy technique by using real-time 2D ultrasound (RT-2DUS) for localizing and guiding the biopsy of cranial tumors. Technique: A 60-year-old male presented with progressive right hemiparesis, memory, and behavioral disturbances. MRI brain showed a multifocal lesion in the left temporal, parietal, and occipital lobes with extension into the splenium. He underwent left parietal mini-craniotomy with frameless RT-2DUS-guided biopsy. Result: Postoperative US scan showed a small biopsy site hematoma. Histopathology confirmed it to be IDH-negative primary glioblastoma. For multifocal glioma, the patient underwent adjuvant WBRT with concurrent and adjuvant temozolomide. Conclusion: RT-2DUS improves the sampling accuracy of the tumor by detecting the solid component of the lesion intraoperatively. In addition, the detection of operative site hematoma can be picked up immediately, avoiding post-procedure imaging.

Background and Introduction: Dural arteriovenous fistulas (dAVFs) are a rare pathology with a clinical presentation related to their anatomical location. Craniocervical junction (CCJ) dAVFs are challenging to treat given the delicate structures that surround the CCJ. Endovascular treatment has evolved significantly in the past decade, but open microsurgery remains an invaluable tool for this pathology. Objective: To demonstrate the step-by-step elements of the far lateral approach for microsurgical ligation of CCJ dAVF. Surgical Technique: A retroauricular incision is created, extending down the neck, and the suboccipital triangle muscles are dissected, exposing the posterior arch of C1. The vertebral artery (VA), as well as its entrance point in the dura, is also dissected and exposed. Next, a C1 hemilaminectomy is performed, followed by a suboccipital craniectomy and drilling of the posteromedial portion of the condyle. The dura is opened behind the VA entrance in the dura, and the intradural VA is exposed. Once the fistula is identified, a temporary clip is placed on the draining vein. Indocyanine green video angiography is used to confirm that there is no further connection; the clip is then removed and the fistula obliterated. The dura is closed in a watertight fashion with a fat bolster to prevent a pseudomeningocele. Results: Postoperative angiogram showed complete resolution of the pathology. The patient was discharged neurologically intact on postoperative day 4. Conclusions: Microsurgical obliteration of CCJ dAVFs can be achieved safely and efficiently through a far lateral approach.

Background and Introduction: A dermoid is an inclusion cyst. Its presence in the posterior third ventricle is highly infrequent. It usually compresses rather than infiltrates the posterior third ventricle's anatomical structures due to a well-defined capsule. Hence, the surgical anatomy in these tumors is less distorted. The approach to the posterior third ventricular tumors depends upon their relation to the galenic venous complex. Objective: This video abstract presents a case of the posterior third ventricular dermoid operated by gravity-assisted retraction less occipito transtentorial combined with the trans-splenial approach. Surgical Technique: A 36-year-old gentleman presented with a headache without any neurological deficits; the magnetic resonance imaging (MRI) revealed a well-defined heterogeneous lesion in the posterior third ventricular region. It was mainly toward the right side, just beneath the splenium, pushing the galenic venous system downward. This made the occipital transtentorial approach favorable as it required the least vessel handling. A right parieto-occipital craniotomy was performed. The patient was placed in a lateral semi-prone position with the head slightly rotated toward the right side with a slight neck extension. This allowed the right occipital lobe to fall away from the Falco-tentorial junction. With sharp dissection, an inter-Rosenthal corridor was made. But as the tumor was higher up in the posterior third ventricle, it was modified to another trans-splenial corridor. Near-total excision was achieved with a thin capsule left attached to the vein of Galen. The capsule was thick, filled with a cheesy white material, and a calcified sebaceous lump within. The postoperative scan showed no residual tumor. Results: The patient had improvement in the headache. There were no field cuts. The histopathology was suggestive of a dermoid cyst. Conclusions: Dermoid cysts of the posterior third ventricular region are rare, and judicious surgical decisions result in better outcomes.

Background: Insular glioma was considered a nonsurgical entity due to resection-associated morbidities. The advancement in neurosurgical techniques and adjuncts used in the last two decades made the resection of insular gliomas simpler for neurosurgeons with the maximum extent of resection and acceptable morbidity rates. The complex anatomy of this region remains a challenge for neurosurgeons and requires expertise. The key factors to achieve complete resection in the insular region are the thorough knowledge of surgical anatomy and meticulous microsurgical techniques. Intraoperative adjuncts such as image guidance along with cortical and subcortical mapping assist in excellent outcomes. Objective: In this study, we describe the operative technique and application of trans-Sylvian approach to do the compartmental dissection done by the senior author, along with challenges faced, with the hope to highlight the efficacy of the approach to achieve maximal resection of this entity without compromising patient's safety. Materials and Methods: A 32-year-old right-handed gentleman presented with episodes of excessive salivation, tingling sensation on the right side of the body along with nausea that lasted for 15–20 s for the last 8 months and one episode of speech arrest without any loss of consciousness and motor or sensory deficit. CEMRI of the brain was suggestive of left insular glioma. The patient underwent left pterional craniotomy, and gross total resection of the tumor was done using compartmental dissection. Results: At follow-up after 1 month, the patient is seizure-free without any speech difficulty and motor or sensory deficit. Conclusion: Compartmental dissection of insular glioma is a safe and efficacious technique to achieve gross total resection of the tumor in this complex region without morbidity.

Background: Back pain and radicular pain due to disc degeneration are probably the most common problems encountered in neurosurgical practice. The experience and results of stem cell therapy in animal disc degeneration model will help us while doing clinical trials. Objective: To study the effect of bone marrow-derived mesenchymal stem cells in an established mouse disc degeneration model. Methods: An easily reproducible mouse coccygeal (Co) 4-5 disc degenerated model by CT-guided percutaneous needle injury was established. The mesenchymal stem cells (MSCs) were cultured from mouse bone marrow and validated. By an established technique, 24 mice disc degenerative models were generated and divided equally into 3 groups (test, placebo, and control). The test group received MSCs with fibrin glue scaffold and placebo group received only scaffold after 6 weeks of degeneration. The control group did not receive any injection. The effects of MSCs were analyzed 8 weeks post injection. Results: The test group showed a significant change in disc height index (%) in micro CT, whereas in the placebo and control groups, there was no change. The Safranin O staining showed an increase in glycosaminoglycan content and the polarized imaging of picrosirius red staining showed restoration of the collagen fibers in annulus fibrosus, which was statistically significant. Conclusion: Intradiscal MSC injection restored disc height and promoted regeneration in the discs at the end of 8 weeks. MSC's niche depends on the microenvironment of the host tissue. These findings will be helpful for clinical trials.

Background: Intraventricular tumors pose a surgical challenge because of the difficulty in reaching their deep location through safe corridors and their adherence or proximity to vital neurovascular structures. Although microneurosurgery is the mainstay of surgical management, neuroendoscopy aided by adjuncts, namely, navigation and ultrasonic aspirators, has made a great contribution to improving surgical results. Objective: This article reviews the experience of a neurosurgical unit with endoscopic procedures for intraventricular tumors. The current indications, benefits, and complications of neuroendoscopy are described. Materials and Methods: This is a retrospective, observational study of lateral and third ventricular tumors tackled either purely with an endoscope or with its assistance over 19 years in a single unit at Bombay Hospital Institute of Medical Sciences, Mumbai. Results: Of a total of 247 operated patients with intraventricular tumors, 85 cases operated using an endoscope were included. The majority of the patients had a tumor in the third ventricle (n = 62), whereas 23 patients had tumor in the lateral ventricle. The most common pathologies were colloid cyst and arachnoid cyst (n = 18). An endoscope was used for microsurgical assisted excision of tumors in 31 cases, biopsy in 24, cyst fenestration in 23, and pure endoscopic excision in seven cases. Conclusion: Microsurgery remains the gold standard for the removal of giant, vascular intraventricular tumors. However, endoscopic fenestration or excision of cysts and biopsy have become better alternatives in many cases. Endoscope-assisted microsurgery affords safety and helps in achieving a more complete excision.

Background: Delirium after surgery is a spectrum of clinical syndrome constituting emergence delirium (ED) and/or postoperative delirium (POD). Objectives: The primary objective of this study was to evaluate the incidence of ED and POD in patients after neurosurgical procedures. The secondary objectives were to examine the relationship between ED and POD and identify perioperative risk factors of ED and POD. Materials and Methods: This is a prospective cohort study conducted at the National Institute of Mental Health and Neurosciences. After obtaining the ethics committee approval, consecutive adult patients scheduled for elective neurosurgical procedures from February 2018 to November 2018 were included. We excluded children, patients with preoperative Glasgow Coma score <15, and patients with preoperative delirium. ED was assessed using Riker's Sedation-Agitation Score and POD was assessed using Confusion Assessment Method. Data collection included patient demographics, details of anesthetics and analgesics, and neurosurgical details. Results: The incidence of ED and POD was 41% (N = 82/200) and 20% (N = 40/200), respectively. The occurrence of ED and POD coexisting as a continuous spectrum was 15%. Patients undergoing spine surgeries were found to have 44% less risk of ED than after cranial surgeries (P = 0.032). Presence of ED was associated with 1.8 times higher risk of POD (P < 0.001) and male gender was associated with 2.5 times higher risk of POD (P = 0.005). Conclusions: Incidences of ED and POD are higher after neurosurgery as compared with that reported in nonneurosurgical population previously.

Background and Purpose: The aim of the research was to compare the efficiency of multiphase and single-phase computed tomography (CT) angiography in assessing the leptomeningeal collaterals and in predicting the long-term clinical outcome as well as the risk of hemorrhagic transformation. Materials and Methods: A prospective study was conducted from October 2016 to May 2018 in consecutive patients who presented within 8 hours of the onset of acute anterior circulation ischemic strokes, with NIHSS (National Institutes of Health Stroke Scale) scores ≥5. They underwent triple-phase cerebral CT angiography, and the collaterals were assessed separately using both single-phase and multiphase techniques. The ability of the collaterals to predict the 24-hour CT ASPECTS (Alberta Stroke Program Early CT score), risk of cerebral hemorrhagic transformation, and 90-day clinical outcome was assessed. Results: Fifty-six patients, which included 42 with an involvement of the middle cerebral artery and 14 with mixed occlusions, were assessed. In the multivariate logistic analysis, multiphase CT angiography collateral grading is one of the independent predictors of favorable outcomes. Area under the curve (AUC) was 0.853 (95% confidence interval [CI; 0.73, 0.97]) for multiphase CT collateral scoring in predicting the long-term functional independence, whereas single-phase CT (sCT) scoring displayed an AUC value of only 0.609 (95% CI [0.43, 0.78]). Eighty-two percent of the patients with good multiphase CT collaterals were functionally independent. CT ASPECTS at 24 hours was significantly better in patients with a good multiphase CT collateral score than in those with a good single-phase score. None of the patients with good collaterals in multiphase CT angiography had a significant hemorrhagic transformation. Good multiphase CT collaterals demonstrated 78% sensitivity, 81% specificity, and 80% accuracy in predicting the functional outcome. Conclusion: Multiphase CT collateral system was superior to single-phase angiography in predicting the long-term functional outcomes. Prediction of the hemorrhagic transformation risk was also observed to be better for multiphase CT.

Background: Tuberculum sellae (TS) meningiomas are benign lesions that produce neurological deficits through a mass effect on vital neurovascular structures. The treatment of choice is surgical removal with the ultimate goal to improve vision and achieve total tumor removal. Objective: We analyzed clinical features, tumor characteristics, and surgical approaches in 62 consecutive patients with TS meningiomas to identify factors influencing the postoperative outcome. Methods: The authors reviewed the medical records of all patients of TS meningiomas who underwent surgery at our institute between 2005 and 2018. Pre- and postoperative clinical data, including the operative findings, of these patients were analyzed. Results: Sixty-two patients who underwent surgery for removal of TSM, transcranial (TCA) in 38, and endoscopic endonasal (EEA) in 24, were identified. Gross total resection was performed in 84% and 87.5% patients in the TCA and EEA groups, respectively. A significantly higher rate of visual improvement was observed in the EEA group as compared to TCA (96% versus 79%). Visual worsening after surgery was greater with TCA than EEA (21% versus 4%). Fourteen (37%) patients in the TCA group experienced different postoperative complications, and CSF leak was the main complication in the EEA group. Conclusions: TCA and EEA are both associated with higher rates of GTR and visual improvement without compromising the safety of the procedure. An optimal outcome, however, depends on the careful selection of cases based on the tumor location and morphology, and the surgeon's familiarity with the surgical approach.

Background: miRNAs are crucial regulators of angiogenesis, but there have been no detailed studies on the role of miR-191-5p in cerebral infarct angiogenesis. Here, we investigated the role of miR-191-5p in regulating cerebral infarction angiogenesis. Material and Methods: Mice were injected intracerebroventricularly with antagomir negative control (NC-antagomir), miR-191-5p antagomir, or pcDNA-BDNF 2 h before middle cerebral artery occlusion (MCAO), followed by neurobehavioral score and foot-fault test. The cerebral infarct volume was performed by TTC staining. The microvessel density was detected by FITC-dextran. RT-qPCR was used to detect the levels of miR-191-5p and its target gene BDNF. Western blotting was applied to detect the protein levels of BDNF. The luciferase reporter assay verified that miR-191-5p targeted BDNF. Results: We found an increased level of miR-191-5p in the brain tissue of mice to MCAO. Down-regulation of miR-191-5p reduced the infarct volume and ameliorated neurological deficits in MCAO mice. Further investigation showed that miR-191-5p directly targeted BDNF and that the protective effect of miR-191-5p inhibition in angiogenesis was achieved by regulating BDNF. Conclusions: Our results indicated that miR-191-5p disturbed the angiogenesis in the mouse models of cerebral infarction by inhibiting BDNF.

Background: Skull base chordoma (SBC) is relatively rare and data on its clinical outcome after surgical resection and adjuvant radiotherapy are still limited. Objective: Analyzing the clinical postoperative outcome of SBC patients and defining prognostic factors regarding current treatment modalities. Methods and Material: In this study, 41 SBC patients from 2001 to 2017 were retrospectively analyzed in this single-center study. Results: The most common clinical symptoms were headache (63%) and problems concerning vision (54%) like diplopia. The follow-up controls took place from 1 to 192 months. The mean survival time for the patients was 123.37 months (95% CI 90.89–155.86). The 5- and 10-year survival rates were 73.3 and 49%, respectively. Regarding the Karnofsky-Performance Scale (KPS), Cox regression showed a significant relationship between the survival rates in the overall study population and pre-surgery KPS (P = 0.004). This was further supported with a positive significant correlation between the pre-surgery KPS and the KPS at the last follow-up (P = 0.039). Conclusion: Statistical analysis showed that repeat surgical resection and radiotherapy could be prognostic factors. Furthermore, we were able to show that mortality decreased by 4.5% with each 10 points increase of pre-surgery KPS. This could be a major prognostic factor when deciding treatment modalities. Nevertheless, further standardized clinical studies with a larger patient population should be carried out to extrapolate prognostic factors and improve treatment modalities.

Background: High-grade gliomas have limited time of survival despite aggressive treatment. Patients experience a decline in their physical and mental capacities, affecting their quality of life (QoL), and require proper therapeutic strategies. Objective: To assess the QoL of malignant glioma patients before and after the treatment in a longitudinal study of six months. Methods and Material: Forty-nine patients who were pathologically diagnosed with glioblastoma and anaplastic glioma according to WHO 2016 were included in this prospective study. The assessment of quality of life was done using the European Organization for Research and Treatment of Cancer (EORTC) quality of life (QoL) questionnaire core-30 prior to surgery, 1 and 5 months after the operation. Results: The decline in Karnofsky scores of the patients was statistically significant. Among the symptom scales, fatigue was more prominent after surgery while pain was noticeable during chemotherapy which was correlated with increased age. The mean overall QoL scores showed a clinically significant decline during the postoperative period. The functional scores demonstrated a significant decline in between all periods. Sex was significantly correlated with preoperative emotional and physical functioning. The patients with right-sided lesions had higher mean scores for social and cognitive functioning. Conclusions: Low KPS, older age, and female gender may affect cancer symptoms and physical and social activities in malignant glioma patients. Cognitive functions as well as social and occupational roles gradually decline during the first six months of treatments. Overall QoL of high-grade glioma patients deteriorates especially after radiotherapy and during the first months of chemotherapy.

Background: Acute ischemic stroke (AIS) leads to neuronal and endothelial damage that activate the release of proinflammatory mediators such as lipoprotein-associated phospholipase A2 (Lp-PLA2), which lead to the development of brain edema injury. Most of statins produce differential effects on Lp-PLA2 activity and mass with a comparable reduction in low-density lipoprotein (LDL) serum levels. Aims: The aim of this study is to evaluate the differential effect of different statins on the mass of level of Lp-PLA2 in patients with AIS. Methods: A total of 69 patients with AIS aged 40–70 years compared with matched 39 healthy controls were involved in this case-control study. The AIS patients were divided according to the statins therapy into 39 patients on statins therapy (statins on), and 30 patients were not on the statins therapy (statins off). Anthropometric variables including weight, height, body mass index (BMI), and blood pressure profile were estimated. Besides, biochemical variables including lipid profile[total cholesterol (TC), triglyceride (TG), LDL, very low-density lipoprotein (VLDL), high-density lipoprotein (HDL)], Lp-PLA2 mass levels, high-sensitive C-reactive protein (Hs-CRP) were estimated. Results: Patients with AIS had high Lp-PLA2 mass levels (P < 0.01) that positively correlated with high Hs-CRP, blood pressure, BMI, TC, TG, VLDL, LDL, and negatively correlated with HDL as compared with healthy controls. As well, statins on patients had lower Lp-PLA2 mass levels (9.82 ± 3.19 IU/mL) compared with statins off patients (16.55 ± 4.72 IU/mL), (P = 0.0001). Regarding the gender differences in the Lp-PLA2 mass level, it was higher in men patients with AIS compared with comparable females (P = 0.03). Conclusions: Lp-PLA2 mass level was higher in patients with AIS and linked with underlying poor cardio-metabolic disorders. Therefore, the Lp-PLA2 mass level is observed to be a surrogate biomarker of AIS mainly in patients with poor cardio-metabolic disorders. Statin therapy improves the Lp-PLA2 mass level and the poor cardio-metabolic profile in patients with AIS.

Background: Pre-stroke anti-platelet (PAP) therapy can potentially influence the severity and outcome after ischemic stroke. Methods: We analyzed data from the prospective multicenter Indo–US collaborative stroke project for the impact of PAP therapy. Outcome measures included the admission National Institute of Health Stroke Scale (NIHSS) score, 3-month modified Rankin scale (mRS) score, and rates of in-hospital mortality and post-ischemic intracerebral hemorrhage. Results: Among 2048 of 2066 patients (M:F = 2:1) with known pre-stroke medication status, 336 (16.3%) were on PAP therapy. As compared to the non-PAP group, the PAP group had significantly higher mean age (62.2 vs 57.4 years, P < 0.001) and significantly more men, vascular risk factors, cerebral microbleeds (12.8% vs 6.2%, P = 0.001) and intravenous thrombolysis treatment (17% vs. 10.6%, P = 0.001). Cardioembolic strokes were significantly more in the PAP group (P < 0.001), but not large artery atherosclerosis. No significant differences were observed in the median NIHSS score (9 vs. 10, P = 0.274), 3-month mRS (score 0-2,51.4% vs. 49.0%, P = 0.428), in-hospital mortality (8.6% vs. 7.8%, P = 0.592), or symptomatic post ischemic intracerebral haemorrhage (12.2% vs. 10.6%, P = 0.382). The PAP group had more stroke recurrence (6.6% vs. 2.9%, P = 0.002) which was not significant (P = 0.065) after multivariate regression analysis adjusting for age, sex and vascular risk factors. PAP therapy was not an independent predictor of initial stroke severity or stroke outcome. Conclusion: PAP therapy has no significant effect on initial stroke severity, rates of post-ischemic hemorrhage with or without thrombolysis, in-hospital mortality, stroke recurrence, and 3-month outcome after ischemic stroke.

Background: Osteomalacic myopathy secondary to vitamin-D deficiency is an under-recognized cause of muscle weakness in children and adolescents. Aim: To describe a cohort of children and adolescents with osteomalacic myopathy. Settings and Design: Pediatric neurology unit of a tertiary care hospital. Methods and Material: Charts of children and adolescents with osteomalacic myopathy were retrospectively reviewed for demographics, clinical presentation, laboratory investigations, and treatment response. Diagnosis of vitamin-D deficiency was made on the basis of a combination of clinical, biochemical, and radiographic findings. Response to treatment with vitamin-D confirmed vitamin-D deficiency as the cause of myopathic symptoms. Results: Twenty-six children—15 girls and 11 boys aged between 20 months and 19 years—with osteomalacic myopathy were identified. Fifteen (58%) children were between 10 years and 19 years of age. Twenty-one (81%) children presented with myopathic symptoms of progressive walking difficulty, with eventual loss of ambulation in six. Four children came to attention through hypocalcemic seizures. One nonambulatory child with cerebral palsy presented with loss of previously attained ability to roll over and sit. All children had proximal muscle weakness on examination. Fifteen (58%) children had clinical signs of rickets. All the children who underwent biochemical (n = 24) and radiographic (n = 16) investigations had results consistent with vitamin-D deficiency. Only in one child, the diagnosis of osteomalacic myopathy was made on the basis of clinical findings. Response to vitamin D was uniformly good. Conclusions: Vitamin-D deficiency should be considered in the differential diagnosis of proximal myopathy in children and adolescents.

Context: Strokes that remain without a definite cause even after an extensive workup, termed cryptogenic strokes, constitute up to 30–40% of ischemic strokes (ISs) in the young. Some of them can have a genetic basis. However, the well-established genetic causes account for only a small percentage of these cases. Aim: To evaluate the association of cryptogenic young IS with 16 candidate gene polymorphisms. Settings and Design: A case-control study with cryptogenic young IS patients (South and North Indians; n = 105) and age, sex, and ethnicity-matched controls (n = 215). Subjects and Methods: Genotyping was carried out by PCR-RFLP method using DNA extracted from the blood. Statistical Analysis Used: Association of the genotypes with the disease was studied using Chi-square test. Results: MTHFR rs1801133 and KNG1 rs710446 showed significant statistical association with cryptogenic young IS (P = 0.0261 and 0.0157, respectively) in the Indian population. Significant association of KNG1 rs710446 (P 0.0036) and FXII rs1801020 (P 0.0376) with cryptogenic young stroke in South Indian males, SERPINC1 rs2227589 in South Indian female patients (P = 0.0374), and CYP4V2 rs13146272 in North Indian males (P = 0.0293) was observed. Conclusions: Our study indicates that in the Indian population MTHFR rs1801133, KNG rs710446, FXII rs1801020, SERPINC1 rs2227589, CYP4V2 rs13146272, and FXIII V34L may be significant risk factors for cryptogenic IS in the young. In addition, ethnicity and gender play a significant role. Further studies with larger sample size are required to completely establish these polymorphisms as risk factors for cryptogenic IS in young Indians.

Purpose: The aim of this research was to investigate the relationship between remodeling patterns and degree of enhancement in patients with atherosclerotic middle cerebral artery (MCA) stenosis using high-resolution magnetic resonance imaging (HR-MRI). Materials and Methods: From August 2015 to May 2016, 38 consecutive patients with unilateral MCA stenosis on time-of-flight (TOF) MR angiography were prospectively enrolled. The routine MR scan and cross-sectional images of the stenotic MCA vessel wall on HR-MRI were performed in all patients. Among them, 17 patients displayed positive remodeling (PR) and the other 21 patients displayed negative remodeling or non-remodeling (non-PR). The patients displaying hyperintense on diffusion-weighted imaging (DWI) in the territory of ipsilateral stenotic MCA were considered to have had acute stroke. Subsequently, the differences in the degree of enhancement and the number of acute stroke patients between the PR group and the non-PR group were compared. The Spearman rank correlation analysis of the enhancement degree (ED) and the remodeling index (RI) was calculated. Then, receiver operating curve (ROC) was used to evaluate diagnostic efficiency of RI and ED for acute infarction. Results: The PR group had more obvious enhancement plaques than the non-PR group (10 versus 3, P = 0.006). The PR group also had a larger number of acute stroke patients than the non-PR group (15 versus 4, P = 0.000). The spear-man rank correlation analysis showed that the degree of enhancement had a weak positive correlation with the remodeling index (r = 0.379, P = 0.019). The area under the curve (AUC) of RI and ED was higher than that of RI (0.924: 0.842). Conclusion: The PR, obvious enhancement predicted vulnerable plaques that were more prone to causing acute stroke. RI and ED had valuable diagnostic efficiency for acute infarction.

Background: In brain injury patients, positive end-expiratory pressure (PEEP) may potentially increase the intracranial pressure (ICP). ICP can be noninvasively assessed from the sonographic measurement of the optic nerve sheath diameter (ONSD). Herein, we aim to evaluate the association between PEEP and ICP via measuring ONSD in traumatic brain injury (TBI) patients. Methods: TBI patients with age ≥18 years, severe brain injury (GCS 8 or less), receiving mechanical ventilation, initial PEEP ≤4 mmHg and no history of severe cardiopulmonary disease were included in this study. Patients with intracranial hypertension (defined as ICP >20 mmHg) and already receiving PEEP >15 cm H₂O at enrollment were excluded from the study. ONSD measurement was performed when hemodynamic parameters were most stable. Variables included central venous pressure, invasive blood pressure, heart rate, saturation, and ventilator parameters. Results: The ONSD and ICP did not increase significantly while PEEP increased from 0–5 cm and 5–10 cm H₂O. However, ONSD and ICP significantly increased when PEEP increased from 10–15 cm H₂O. There was no significant difference noted while comparing measurements of optic nerve sheath (ONS) diameter in both eyes at all PEEP values in cases as well as control patients. Mean arterial pressure (MAP) decreased with an increase in PEEP value. Highly significant decrease occurred in MAP change from PEEP 10–15 in cases (P < 0.001) and control (P < 0.001). Conclusions: The PEEP up to 10 cm H₂O can be safely applied in patients with TBI. In addition, the increment of PEEP might further increase the oxygenation, at the cost of ICP accentuation.

Background: Only a few studies have investigated the risk factors for the prognosis of progressive cerebral infarction (PCI) and the relationship between PCI and cerebral infarction (CI) recurrence. Objective: The objective of this study is to analyze the risk factors for PCI and PCI prognosis and evaluate the relationship between PCI and CI recurrence, mortality, short-term outcomes. Methods: The retrospective study included 221 CI patients. PCI and non-PCI patients were divided into the observation (91) and control (130) groups, respectively. Patients' clinical data, including diabetes history, laboratory blood indices, National Institutes of Health Stroke Scale (NIHSS) scores at admission, and presence of carotid and intracranial artery stenoses, were retrospectively analyzed. Instances of CI recurrence, adverse short-term outcomes, and death within 1 year postanalysis were recorded. Results: Diabetes, homocysteine, NIHSS score at admission, fibrinogen, and intracranial artery stenosis were associated with PCI. Age, NIHSS score at admission, and pneumonia were associated with PCI prognosis. By 12-month follow-up, the mortality and adverse outcome rate in the observation group were significantly increased than those of the control group. There was no diference in CI recurrence rates between the groups. Adverse outcomes were associated with PCI, age, and homocysteine. Conclusions: PCI risk factors included diabetes, homocysteine, NIHSS score at admission, fibrinogen, and intracranial artery stenosis. The independent risk factors for PCI prognosis included age, high NIHSS score at admission, and pneumonia. PCI did not affect CI recurrence but may affect adverse short-term outcomes.

Background: Postoperative peritumoral brain edema (PTBE) is the progressively exacerbating cerebral edema following meningiomas resection. Objective: The study aims to identify the predictive factors of postoperative PTBE. Materials and Methods: A retrospective study was conducted on the 117 cases of patients who underwent meningioma. The histopathological features of the tumors were re-assessed according to WHO 2016 classification. Clinical and pathohistological features were analyzed. Results: Thirteen patients (11.1%) were diagnosed having postoperative PTBE. Preoperative seizure (odds ratio [OR] = 6.125, P = 0.039) and histological prominent nucleoli (OR = 3.943, P = 0.039) were the independent risk factors for postoperative PTBE. Meningiomas with a parietal localization were more likely to develop postoperative PTBE (OR = 3.576, P = 0.054). Brain invasion and large tumor volume did not increase complication rate. Preoperative edema index was significantly higher in brain invasive meningiomas (3.0 ± 2.2 versus 1.8 ± 1.7, P = 0.001). Patients having moderate preoperative PTBE were prone to the complication (21.4% versus 7.9%, P = 0.100). Conclusions: Preoperative seizure were the predictive factors for postoperative PTBE. Careful venous protection during the operation may be helpful, especially for tumors locating in the parietal lobe. Prominent nucleoli observed in postoperative pathology should warrant surgeons' attention. Comprehensive perioperative management is essential for these patients.

Objectives: To study the brain mechanism and behavioral performance of cognitive functional differences between children with primary nocturnal enuresis (PNE) of different genders by resting-state functional magnetic resonance imaging (rs-fMRI) and attention behavior research. Methods: All the children participating the test were 5–11 years old including 32 PNE children (15 boys and 17 girls) and 35 normal children (16 boys and 19 girls). They respectively went through MRI scan and the data were analyzed by the method of amplitude of low frequency fluctuation (ALFF). Then continuous performance test (CPT) was done and the correct number and reaction time were recorded. The between-group variance was analyzed by two sample t-test. Results: 1. fMRI: There were no obviously different brain regions of ALFF between normal boys and girls. The brain regions which had obvious differences of ALFF between PNE boys and girls were right middle frontal gyrus, left inferior parietal lobule and right posterior cerbellar lobe. And the group of PNE boys were higher than PNE girls on all the three regions (P < 0.05). 2. CPT: The reaction time of PNE and normal boys was both lower than those of girls, but there were no obvious differences on the correct number. Conclusion: This study found that girls with PNE performed more obvious damages on attention, executive function and working memory than boys which might be related to the fact that girls were more likely to suffer from confidence impairment due to enuresis leading to cognitive dysfunctions. The method of ALFF based on resting-state fMRI provided a new approach for studying the cognitive characteristics of PNE children.

Background: The prevalence of mild cognitive impairment (MCI), herein China, was without involving the suburban communities, where the awareness of MCI still remains extremely weak. Objective: The objective of this study is to investigate the prevalence of MCI in the Chinese residents aged ≥65 in the suburban communities of Shanghai, China, and study the awareness of MCI in terms of its symptom, prevention, and intervention. Methods: A total of 925 suburban community residents aged ≥65 were evaluated with a series of clinical examinations and scale questionnaire, and 600 participated in a five-dimension questionnaire survey pertaining to the awareness of MCI. Results: The prevalence of MCI was up to 29.8% and of dementia was 11.1%, respectively. A difference was observed among the three groups of dementia, MCI, and normal in each dimension of age, gender, education, being widowed, and living with the next generation (P < 0.05). The degree of cognitive impairment was linearly correlated with age (P < 0.001). The prevalence of MCI was higher in the females (P < 0.001), in the group of low educational level (P < 0.001), in the widowed residents (P < 0.01), and in those who did not live with their next generations (P < 0.01). The family's concern for MCI symptoms in the elderly accounted for 60%; the awareness rate of MCI symptoms, 25.5%; the awareness rate of MCI prevention, 15.5%; and the rate of taking MCI seniors to the doctor, 32%. Conclusions: The prevalence of MCI in the suburban communities of Shanghai was high but the awareness of MCI was low.

Background: Sporadic nonlesional intractable visual-sensitive epilepsies of childhood represent a challenging subset of epilepsies in terms of management and prognostication given a propensity to evolve as epileptic encephalopathy. Objective: To study the genetic heterogeneity of drug-resistant visual sensitive epilepsy of childhood. Methods: A retrospective chart review was conducted on patients in the pediatric age group between 2016 and 2018, with drug-resistant epilepsy (DRE) and video electro encephalography (VEEG) documented reflex photosensitivity, eye-condition sensitivity. Those patients who underwent genetic testing with targeted next-generation sequencing using an epilepsy gene panel were selected. Results: During the study period, out of 96 patients who underwent genetic testing, 4 patients (4.17%) with sporadic DRE presented with clinical phenotypes ranging from myoclonic-atonic epilepsy, generalized epilepsy with eyelid myoclonia as well as febrile and unprovoked seizures, along with visual sensitivity. Video EEG documented abnormalities ranged from occipital, posterior-cortex and generalized discharges with “eyes-closed state” triggered, self-induced “smart-phone” triggered, photosensitive focal-onset and generalized myoclonic seizures. Accompanying developmental impairment was noted. These patients who were investigated with clinical exome sequencing were detected to have mutations in not only SCN1A genes (pathogenic exonic and intronic variants) but also CHD2 (pathogenic) and CACNA1H genes (a familial febrile-seizure susceptibility variant of unknown significance). Conclusions: The series highlights the complex genetics of drug-resistant visual-sensitive epilepsy of childhood. Such genotype–phenotype associations throw light on the role of ion-channel and non-ion channel genes on reflex epileptogenesis in this group of patients.

Background: In patients with Parkinson's disease (PD), the occurrence of motor and non-motor symptoms increases with disease progression. The range of neuropsychiatric symptoms (NPS) vary among individuals and can be burdensome for caregivers. Only a few studies have identified the contributing factors of NPS and caregiver burden in India. Objectives: We aimed to study the clinical profile, disability, and predictive factors of NPS in PD patients and associated caregiver's burden. Methods and Material: This was a cross-sectional observational study carried out in PD patients and their respective caregivers attending a movement disorder clinic in a tertiary care teaching hospital in Kerala. A total of 104 patients diagnosed with idiopathic PD receiving levodopa therapy and who had a primary caregiver were enrolled in the study. Structured questionnaires were administered to both patients and caregivers to collect data. Data analysis was done using an independent t-test, linear, and multiple regression analysis. Results: Among 104 patients recruited for the study, 61.5% of patients had shown at least one NPS and 40.44% showed multiple NPS. Results from the study showed that depression is the primary NPS occurring in IPD patients (55.8%) followed by irritability, anxiety, and apathy. On linear regression models, the prime determinant of NPS was the Everyday Abilities Scale for India (EASI). For caregiver burden, the main determinants were the presence of NPS, duration of caregiving, EASI, and RBDSQ score. Conclusions: NPS in PD are highly associated with and are determinants of caregiver burden. Detailed assessment and specific interventions aimed at NPS could alleviate caregiver burden.

Purpose: This study evaluated the hemodynamic effects of carotid artery stenting (CAS) on cerebral blood flow velocity (CBFV) in patients with carotid artery stenosis, before, 3 d, and 3 months after the procedure using transcranial Doppler ultrasound (TCD). Methods: The study included 36 patients with atheromatous carotid artery stenosis. Cerebral computed tomography (CT) or magnetic resonance imaging (MRI) was performed in every patient, and carotid stenosis was evaluated using duplex sonography, CT, and MRI angiography before the procedure. To obtain baseline values, the CBFV was evaluated 1 d before CAS. Follow-up TCD evaluations were performed 3 d and 3 months postoperatively. Results: The median degree of internal carotid artery (ICA) stenosis in the participants was 90% (range 50%–99%). The median CBFV at the anterior cerebral artery (ACA) was significantly lower on the ipsilateral side than on the contralateral side before stenting; however, there were no significant differences in CBFV in the ipsi and contralateral middle cerebral artery (MCA). The median CBFV in the ipsilateral MCA increased significantly 3 d after the procedure and remained higher than the basal values after 3 months. Conclusions: We observed significant increases in the median CBFV and pulsatility index (PI) in the MCA bilaterally, especially on the stented side, measured 3 d and 3 months after stenting in patients with severe ICA stenosis.

Background and Aims: Cryptogenic ischemic strokes (CIS) are treated with antiplatelets for stroke prevention in routine clinical practice. The objective of this study was to investigate whether the CHADS₂ and CHA₂DS₂-VASc scores may be used to identify the patients with CIS at higher risk of ischemic stroke despite antiplatelet therapy. Material and Methods: We calculated CHADS₂ and CHA₂DS₂-VASc scores in patients with first ever CIS; those previously managed with antiplatelets (AP group) and in those without antiplatelets (non-AP group), using the prospectively recorded data of the Istanbul Medical School Stroke Registry from 1996-2014. Results: Of the 4466 IS patients, 886 patients with first ever IS had complete data for score calculation. Seventy-five (39 women) of them were diagnosed with CIS. CHADS₂ and CHA₂DS₂-VASc scores were significantly higher in the AP group of 19 patients in comparison to the non-AP group of 56 patients (P = 0.005 and P = 0.009, respectively). ROC curve analyses showed an area under curve (AUC) of 0.705 (CI: 0.57-0.84; P = 0.008) for CHADS₂ score ≥3 and AUC of 0.699 (CI: 0.57-0.82; P = 0.01) for CHA₂DS₂-VASc score ≥4. Vascular diseases were more frequent in the AP group and these patients were older than the patients in the non-AP group (P = 0.025, P = 0.024; respectively). Conclusions: CHA₂DS₂-VASc score ≥ 4 and CHADS₂ score ≥3 may be used as a predictor of the occurrence of IS despite regular antiplatelet use and suggest an embolic source which will respond better to anticogulation. Our results support that CHADS₂ and CHA₂DS₂-VASc scores may be useful to identify subgroups among patients with CIS for individualizing diagnostic approach, planning future workup and preventive treatment.

Background: The study in consideration aimed to do morphometry of internal carotid artery (ICA) and substantiate any differences significant in relation to sex and age on digital subtraction angiography (DSA) imaging. Objectives: Our study aims to ascertain morphometric parameters of ICA through DSA evaluation and to build a normative data which is accurate enough for the neurovascular procedures to be performed. The study will also carry out correlation of morphometry of intracerebral blood vessels with sex, age, and side-related dimorphism. Material and Methods: Our study, cross-sectional in design was done on 70 patients (>20 years). Diameter of different segments of ICA were measured after imaging was done on DSA using a Philips biplane system clarity (Allura FD20/20). Statistical analysis was done. Result and Conclusion: In males, mean diameter of petrous, cavernous, supraclinoid, and choroidal segments of ICA were more when compared to females. Statistically significant differences were found in supraclinoid segment of ICA between males and females. The mean diameter of supraclinoid and choroidal segments of ICA showed a decreasing trend from younger to older age groups. The difference in the mean diameter of petrous segment of ICA with increasing age was statistically significant. The results of our study show that differences were statistically significant in supraclinoid segment of ICA in males and females. Neurosurgeons in order to be confident in tackling the various emergencies in this region need to have in-depth knowledge of morphometery of cerebral arterial circle.

Background: Neurological diseases are phenotypically and genotypically heterogeneous. Clinical exome sequencing (CES) has been shown to provide a high diagnostic yield for these disorders in the European population but remains to be demonstrated for the Indian population. Objective: The study aimed to understand the utility of clinical exome sequencing for the diagnosis of neurodevelopmental disorders. Materials and Methods: A cohort of 19 idiopathic patients with neurological phenotypes, primarily intellectual disability and developmental delay, were recruited. CES covering 4620 genes was performed on all patients. Candidate variants were validated by Sanger sequencing. Results: CES in 19 patients provided identified 21 variants across 16 genes which have been associated with different neurological disorders. Fifteen variants were reported previously and 6 variants were novel to our study. Eleven patients were diagnosed with autosomal dominant de novo variants, 7 with autosomal recessive and 1 with X-linked recessive variants. CES provided definitive diagnosis to 10 patients; hence, the diagnostic yield was 53%. Conclusion: Our study suggests that the diagnostic yield of CES in the Indian population is comparable to that reported in the European population. CES together with deep phenotyping could be a cost-effective way of diagnosing rare neurological disorders in the Indian population.

Background: Despite recommendations and guidelines, surgical antibiotic prophylaxis protocol for neurosurgical cases is not uniformly followed. Objective: To report trends in the use of prophylactic antibiotics by Indian neurosurgeons for non-trauma neurosurgical cases. Materials and Methods: An email survey consisting of 17 questions was sent in 2018 to 2,175 surgical members with a registered email in the Neurological Society of India (NSI) registry. Three reminders were sent at 3-month intervals. The results were analyzed for the number, type, and duration of antibiotic use for different surgical procedures. The differences in the antibiotic policy in different practice settings were also studied. Results: The response rate was less than 5% (103 responses). Almost all (98.1%) surgeons used prophylactic antibiotics. A single antibiotic was most used for cranial surgeries (85%) and least for spine surgeries with instrumentation (64%) (P = 0.001). One dose or 1 day of antibiotics was used by the least number of responders (29%) for spinal instrumentation surgeries and the most responders (66.7%) for spinal surgery without instrumentation (P < 0.0001). Surgeons working in corporate teaching hospitals or non-teaching hospitals were more likely to use antibiotics for longer duration than surgeons working in government/trust teaching hospitals. Conclusions: Substantial numbers of surgeons use prophylactic antibiotics for more than 1 day with a higher proportion of surgeons working in corporate teaching and non-teaching hospitals pursuing such a policy.

Background: Antiplatelet therapy (APT) is an integral part of secondary stroke prevention. Noncompliance to APT is an important factor in stroke recurrence. In this study, we have evaluated the reasons for noncompliance to APT. Objective: The aim of this study was to identify the various causes of nonadherence to APT in recurrent stroke patients. Material and Methods: The study was conducted in a tertiary care hospital in south India with a huge stroke burden. The study period was from October 2017 to September 2018. A total of 60 consecutive patients of recurrent stroke who were nonadherent to antiplatelet therapy were evaluated for various factors that prevented compliance. Results: During the 12-month study period among 604 ischemic stroke patients, 128 (21%) had recurrent strokes. Of this 128, 60 (46.8%) were due to discontinuation of APT. The main factor for nonadherence to APT was lack of awareness about the need for lifelong medication (41/60; 68.3%). 10 patients (16.7%) stopped treatment as they opted for alternative therapy and 4 (6.7%) discontinued antiplatelets due to side effects. A small proportion of the patients (3.3%) cited financial constraints and forgetfulness as the issue, while 1.7% had difficulty in finding assistance to administer medicine. 27 (45%) patients had recurrent stroke within 2-15 days of stopping APT. Conclusions: The main reason for nonadherence to antiplatelet therapy is lack of awareness about the need for lifelong antiplatelet therapy. Stroke patients should be educated about the importance of lifelong antiplatelet therapy to prevent recurrent strokes.

Background: Intramedullary schwannomas (IS) at cervicomedullary junction (CMJ) are exceedingly uncommon. There is hardly any clinicoradiological marker for preoperative diagnosis and prognostication. Case: We report a case of a 17-year-old boy with progressive spastic quadriparesis of six months duration. On radiology, there was a contrast-enhancing lesion expanding the cord extending from the medulla to C5 level. During surgery, the cord was expanded and the tumor was eccentric. Histopathology and immunohistochemistry were suggestive of schwannoma. In view of the ill-defined plane of separation from the normal neural tissue, only subtotal resection could be achieved. Conclusion: Schwannoma should be considered as a remote differential of intramedullary lesions. The extent of resection should be tailored according to the plane of dissection and intraoperative neuromonitoring guidance. Though a masquerader, schwannoma carries better prognosis than rest of the pathologies.

Mucopolysacharidosis type I is a multisystem disease and often presents with neurobehavioral problems, corneal clouding, cardiac valve involvement, hepatomegaly, coarse facies, and skeletal abnormalities. It has three subtypes – with Hurler subtype (MPS-1H) being the most severe phenotype with early neurological involvement, rapid progression and mortality, while the other two subtypes – Hurler-Scheie (MPS-1H/S) and Scheie (MPS-1S) are of intermediate and milder severity, respectively. Even though neuropsychiatric symptoms have often been reported in the pediatric age group, MPS type I presenting as a major psychiatric illness in adulthood has rarely been reported in literature. Here, we report a female presenting as bipolar affective disorder in the fourth decade of life, where neuroimaging and systemic involvement gave a clue to the diagnosis.

Chin-on- chest deformity is not uncommon sequelae of ankylosing spondylitis. Apart from difficult airway, several other considerations might include co-existing cardio-respiratory embarrassment, osteoporotic bones, and neurological perturbations. We describe the successful anesthetic management of a case of chin-on-chest deformity with no access to midline neck structures and extremely difficult airway posted for corrective spine surgery.

Migraine is characterized by severe, intermittent headache attacks with associated symptoms including nausea, vomiting, phonophobia, and photophobia. Still Triptans (selective 5-HT_1B/D agonists) are considered as the first-line therapy in acute attack of migraine. Recently two new drugs Lasmiditan and Ubrogepant were approved by United States Food and Drug Administration in acute attack of migraine with or without aura in adults. Lasmiditan is a highly selective 5-HT_1F receptor agonist which demonstrated superiority to placebo in the acute treatment of migraine in adults with moderate/severe migraine disability in two similarly designed phase-3 trials, SAMURAI and SPARTAN. Ubrogepant is a novel small molecule oral calcitonin gene-related peptide receptor antagonist. The approval was supported by two pivotal phase-3, randomized, double-blind, placebo-controlled trials (ACHIEVE I and ACHIEVE II) that evaluated the efficacy, safety, tolerability. Hopefully, these two drugs may soon be a new addition to the mounting armory of drugs against migraine and may fulfill a substantial unmet need.

This is a report of two patients who were diagnosed to have NF-1. The patients had severe dystrophic soft tissue and bone changes leading to craniovertebral junction and subaxial cervical spinal instability and deformity. Both the patients underwent atlantoaxial and subaxial cervical spinal stabilization. No bone, soft tissue or tumor resection was done for decompression. Both patients had gratifying clinical recovery. Follow-up in both the patients is more than 12 months.

Presurgical devascularization of neoplasms of the head and neck can be achieved by endovascular as well as direct percutaneous embolization techniques. We report a case of percutaneous glue embolization of an orbital meningioma, complicated by delayed acute stroke due to the distal migration of polymerized glue in the left middle cerebral artery. To the best of our knowledge, this is the first report to discuss the percutaneous embolization of orbital meningioma complicated by stroke due to intracranial glue migration.

This report describes a patient with thymomatous myasthenia gravis (MG) with aplastic anemia in pharmacological remission and COVID-19 who developed respiratory failure in the course of the disease and reviews the published literature on this topic. Analysis of the clinical characteristics of the eight patients with MG including our patient suggests two possible mechanisms for respiratory failure: myasthenic crisis (MC) or pulmonary complications of COVID-19. Patients with MC were young women in high-grade MGFA Class whereas patients with respiratory failure due to pulmonary complications of COVID-19 were elderly men in pharmacological remission or MGFA Class I. These observations suggest that COVID-19, like other infections, may precipitate MC in patients with severe grade MG before COVID-19. The only differentiating feature between the two types of failure was severity myasthenic weakness. This clinical distinction has management implications. These observations need to be validated in a larger sample.

Thoracic outlet syndrome (TOS), a rare condition, results from the compression of neurovascular structures traversing from the neck through the thoracic outlet into the axilla. It can develop from chronic repetitive activities of the upper extremities, commonly reported in athletes playing sports involving vigorous use of arms and shoulders. While symptoms of neurovascular compression can occur, stroke due to TOS in children is not commonly reported. We describe a rare case of a healthy 14-year-old boy, a competitive violinist, with acute limb ischemia from extensive occlusive thrombi involving several arteries in the right upper extremity as well as the right vertebral artery, which ultimately caused infarcts in the bilateral posterior circulation. The etiology was determined to be TOS leading to impingement of the right subclavian artery by a fused cervical rib aggravated by patient's prolonged violin practice. This case represents the first description of stroke from TOS in an adolescent violinist.

Epilepsy is a chronic neurological disorder affecting 50 million patients worldwide, in need of continuous treatment, while 30% of them are refractory to treatment. Despite multiple antiepileptic drugs are available for the treatment of epilepsy, still refractory epilepsy especially in children represents a social burden in developing countries. Low-dose naltrexone (LDN) has been proposed as an immune modulator in multiple diseases and proved benefit especially in diseases with immune dysregulation. The purpose of this study is to demonstrate the effect of LDN in the treatment of children with intractable epilepsy and discuss its potential role in epileptogenesis process.

Pregabalin, a gabapentinoid frequently prescribed for neuropathic pain, also increasingly identified as a drug for abuse. We describe a unique case of 31-year-old man presented with subacute neuro-psychiatric symptoms and a spectrum of movement disorders, suspicious of autoimmune encephalitis. Initial response to IV methylprednisolone followed by recurrence of symptoms strengthened our suspicion for autoimmune encephalitis. His autoimmune encephalitis workup was negative, however, his two MRIs showed parenchymal changes. The patient, finally, confessed to chronic pregabalin abuse. He recovered completely upon stopping pregabalin abuse and remained asymptomatic at follow-up. To the best of our knowledge, we are the first to describe parenchymal changes in MRI mimicking autoimmune encephalitis in a case of pregabalin abuse. Despite the limited number of reports of pregabalin abuse in India, it is time to consider restricting the pregabalin availability, in line with many Western countries. This is particularly relevant to India, where, one legitimate prescription can be used by many to buy medicines at multiple stores without any questions being asked by the pharmacists.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder with varied manifestations. Progressive gait freezing (PGF) is considered to be a rare and uncommon presentation of PSP. Here we present 2 patients with freezing of gait as the initial manifestation of PSP-PGF. One patient fulfilled the criteria of PSP-PGF, while the second did not. Nevertheless, according to the movement disorders society-PSP criteria, he met the threshold for possible PSP with progressive gait freezing. We emphasize a broad PSP-PGF spectrum of symptoms and sensitize to the fact that freezing of backward gait could indeed represent an unusual manifestation of atypical parkinsonism.

Cerebral venous thrombosis (CVT) is a rare form of venous thromboembolism. The presentation of symptoms is highly variable in this disease. The findings on MRI vary depending on the age of the thrombus within the vessel. We report a 53-year-old male patient with CVT who presented with a sudden-onset grand mal seizure and limb paralysis. Primary MRI presented long T1 long T2 signals, persistent high signals on DWI and ADC maps in the cortex of right frontal lobe. Contrast-enhanced MR detected a lesion with ring-like enhancement in right frontal lobe. His symptoms were significantly improved with anticoagulant therapy of Warfarin. Our findings represent the description of abnormal MRI contrast enhancing tumor-like masses. CVT should be added to the differential diagnosis of supratentorial ring-enhancing lesions.

Background: Trunk stabilization is important in providing postural control and extremity movements, and is maintained by the muscles called “core”. Objective: The aim of this report was to demonstrate the effect of core muscles contraction on upper extremity tremor. Materials and Methods: A 22-year-old Multiple Sclerosis patient with right extremity ataxia was included in this report. Scale of Assessment and Rating Ataxia (SARA), Expanded Disability Status Scale (EDSS), Accelerometric Tremorogram and Purdue Peg Board Test (PPBT) were performed. Assessments were made with and without the core muscles contraction. Results: The total score of SARA decreased from 16 to 14 due to the reduction of dysmetria and tremor scores. Tremor amplitude decreased with contraction in tremorogram. At the 1^st position, the tremor amplitude changed from -14.6 before the contraction to -18.3 after the contraction. At position 2, 6 Hz tremor was disappeared with contraction. In the PPBT, ataxic extremity performance increased from 5 to 7. Conclusion: Contraction of core muscles reduced postural tremor and improved upper extremity performance. It should be considered planning the training program of ataxic MS patients.

A 28-year-old male presented to us with new onset refractory status epilepticus. Prior to his seizures, he had a history of fever, headache and blurring of vision, for which he was admitted elsewhere. No cause was found; he was treated symptomatically and was discharged as his symptoms had abated. Post-discharge, he started having multiple seizures. Neuro-imaging suggested encephalitis. He progressed to status epilepticus and was referred to our center. We intensified the anti-epileptic medications but owing to no response, he had to be put under coma with thiopental, yet the seizures persisted. Workup for the etiology of his seizures was negative except for CSF TB-PCR (Gene Xpert) being positive and hence anti-tuberculous therapy was initiated. By this time, he developed rhabdomyolysis, and thereafter renal failure with dyselectrolytemia, and thus there was a plan to initiate dialysis. But before this could be done, he succumbed to a cardiac arrest secondary to ventricular tachyarrhythmias. We believe this to be the first reported case of new onset refractory status epilepticus secondary to tuberculosis of the central nervous system.

Ophthalmoplegic migraine (OM) also called recurrent painful ophthalmoplegic neuropathy (RPON) is not a so common disorder. It is characterized by childhood onset, ophthalmoplegia and migraine type of headache. The most common involved nerve is third cranial nerve. Involvement of fourth and sixth cranial nerve is unlikely. Adult cases are not so common. This is a case report of a man who presented with left-sided severe headache and diplopia of left eye. He had left oculomotor nerve palsy. The patient responded to treatment and recovered.

Background: Rosette-forming glioneuronal tumor (RGNT) is a rare and distinctive glioneuronal tumor. Although surgical excision is considered the standard treatment for these slow growing WHO Grade I tumors, gross-total resection is achieved in less than 50% of RGNTs due to its localisation amidst vital structures. With very few cases with long term follow-up reported, there is limited knowledge of the natural clinical course and the role of radiotherapy in inoperable RGNTs. Case Description: A previously well 26-year old male, presented with long standing headache, increasing gait instability and fainting episodes. Imaging revealed a tectal plate mass with hydrocephalous. An endoscopic third ventriculostomy and biopsy was done, revealing RGNT. He received radiotherapy with a curative intent. The patient remained neurologically stable for 4 years. Follow-up imaging done after 4 years showed decrease in tumor size. Conclusions: The current case highlights a role for radiotherapy in RGNTs occurring in surgically challenging sites.

Although gelastic seizures (GSs) with extrahypothalamic epileptogenic zones such as the frontal, temporal, or parietal lobes have been previously reported, reports of GSs arising from the occipital region are rare. Herein, we describe the seizure propagation pattern of mirthless GSs confirmed by intracranial EEG in a case of MRI-negative occipital lobe epilepsy. In this patient, EEG onset was localized to the right occipital lobe while the onset of laughter coincided with seizure propagation to the right basal temporal region. This finding suggested that the symptomatogenic area for GSs in the occipital lobe may reside in the basal temporal region, and the basal temporal region may play a role in laughing behaviors. This case demonstrated that an elaborate analysis of electroclinical features combined with imaging findings may lead to successful seizure localization.

Mitochondrial DNA depletion syndromes (MDS) are rare mitochondrial disorders with evolving broad genotype and phenotype. This is a first case report from India about MPV 17, a mitochondrial inner membrane protein gene variant mutation, presenting with neuropathy, leucoencephalopathy and subclinical hepatic dysfunction with detailed clinical and imaging description.

Background: Post-traumatic pseudoaneurysm of the middle meningeal artery is a rare entity. We report an atypical case of a delayed presentation as parenchymal hemorrhage due to a ruptured middle meningeal artery pseudoaneurysm. Case Description: A 22-year-old man with an alleged history of cranial trauma following a road traffic accident presented 10 days later with a new right temporal intraparenchymal hemorrhage. The CT revealed a differentially hypodense circumscribed structure in the anterior temporal location eccentrically in the hematoma. The cerebral angiogram depicted a pseudoaneurysm arising from the middle meningeal artery. The patient underwent craniotomy and excision of the aneurysm. On follow up, the patient was asymptomatic and had no focal neurological deficits. Conclusion: Despite its rare occurrence, meningeal artery pseudoaneurysm should be considered as a possible etiology of a post-traumatic delayed presentation as an intracerebral hematoma. Prompt diagnosis and management are warranted in view of the mortality and morbidity.

Anomalous origin of extracranial and intracranial carotid system is subject to permutations of embryonal regression of the developing primitive embryonal vasculature. We present a case of segmental agenesis of left external carotid artery from the cervical carotid circulation. The entire left external carotid artery was supplied by the vertebral artery through muscular branches via the occipital artery. This anomaly of proximal external carotid agenesis with the entire vascular tree dependent on the posterior circulation via occipital artery muscular collaterals is unique and has not been described before. We discuss this anomaly with its embryology, clinical implications, and its relationship to a Proatlantal artery.

Intracranial hypotension due to multiple spinal epidural cerebrospinal fluid (CSF) leak is rare and has multiple etiologies. These patients require epidural blood patches (EBP). We report a patient with recurrent paraplegia due to intracranial hypotension following recurrent epidural CSF leak. Cerebrospinal fluid leak was noted at D1, D10, D11, and L2 levels. Autologous epidural blood patch (EBP) at lower thoracic (3 ml) and lumbar region (4 ml) was performed. The patient developed paraplegia with sensory level at D2 for which 3 ml of EBP was done at D1 level after two months. Following EBP, the patient developed quadriplegia and root pain, which recovered in one month. The patient was free of symptoms for 18 months. Spinal epidural CSF leak should be suspected in patients with features of low pressure headache and recurrent paraplegia at multiple levels. Nuclear scintigraphy and CT myelography help in localizing the site of leak.

Lateral medullary syndrome (LMS), known as Wallenberg's syndrome and posterior inferior cerebellar artery syndrome, is a rare cause of stroke. It often results from thrombosis or emboli of the vertebral artery or posterior inferior cerebellar artery. The triad of Horner's syndrome, ipsilateral ataxia, and ipsilateral hyperalgesia clinically identify patients with LMS. We report a case of a 62-year-old diabetic, hypertensive male who presented with symptoms involving the left lateral dorsal medulla along with partial Horner's syndrome, left lateral rectus palsy, and left lower motor neuron–type facial palsy. Atypical presentation in LMS could be explained by infraction of left facial colliculus in addition to the left lateral medulla. Association of these entities with Wallenberg's syndrome is atypical features in our case, which questioned the diagnosis of a simple LMS.

Background: Laminopathies are a group of diseases caused by mutations in the LMNA gene. Congenital dystrophy of the LMN is a rare disease, with less than 100 cases described in the literature. Objectives and Materials and Methods: We present the clinical case of a patient with congenital muscular dystrophy associated with an undescribed mutation in the LMNA gene. Results: The patient presented progressive motor delay from 10 months with a physical examination consisting of global hypotonia, bilateral winged scapula, areflexia, hip and knee flexion posture, and positive Gowers. The patient developed progressive weakness with neck tone loss, functional impairment, and loss of gait at 5 years. Conclusions: To date, more than 20 mutations associated with congenital LMNA muscular dystrophy have been identified, most due to a single amino acid change (aa), few due to the gain or loss of several aa as in our patient.

Mutations in mammalian membrane-bound O-acyltransferase domain-containing (MBOAT) 7 gene are a rare cause for intellectual disability, developmental delay, autistic findings, epilepsy, truncal hypotonia with appendicular hypertonia, and below-average head sizes. Pathogenic variants in MBOAT7 gene show these nonspecific clinical features that are seen in many other neurometabolic diseases. Therefore, specific neuroimaging findings can be valuable key factors for differential diagnosis. Magnetic resonance imaging (MRI) findings of T2 hyperintensity in bilateral globus pallidi and dentate nuclei are seen in a few neurometabolic diseases with similar clinical features of developmental delay and hypotonia, as in our cases. While evaluating the patients with similar phenotypes and specific MRI findings, MBOAT7 deficiency should be kept in mind. Here, we identified two brothers who had a novel homozygous variant in MBOAT7 gene and aimed to raise awareness about this newly described disease.