Amyotrophic lateral sclerosis (ALS), characterized by the progressive loss of both upper and lower motor neurons, is a fatal neurodegenerative disorder. This disease is often accompanied by a tremendous physical and emotional burden not only for the patients, but also for their families and friends as well. There is no clinically relevant treatment available for ALS. To date, only one Food and Drug Administration (FDA)-approved drug, Riluzole, licensed 18 years ago, has been proven to marginally prolong patients' survival without improving the quality of their lives. Because of the lack of an effective drug treatment and the promising outcomes from several preclinical studies, researchers have highlighted this disease as a suitable candidate for stem cell therapy. This review article highlights the finding of key preclinical studies that present a rationale for the use of different types of stem cells for the treatment of ALS, and the most recent updates on the stem cell-based ALS clinical trials around the world.

Though the necessity of cadaver dissection is felt by the medical fraternity, and described as early as 600 BC, in India, there are no practical guidelines available in the world literature for setting up a basic cadaver dissection laboratory for neurosurgery skills training. Hands-on dissection practice on microscopic and endoscopic procedures is essential in technologically demanding modern neurosurgery training where ethical issues, cost constraints, medico-legal pitfalls, and resident duty time restrictions have resulted in lesser opportunities to learn. Collaboration of anatomy, forensic medicine, and neurosurgery is essential for development of a workflow of cadaver procurement, preservation, storage, dissection, and disposal along with setting up the guidelines for ethical and legal concerns.

Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) has been reported in many geographical regions. However, relatively few reports about CADASIL in Chinese were reported. Materials and Methods: We retrospectively collected and analyzed clinical characteristics, magnetic resonance (MRI) features and genetic data of 52 Chinese mainland CADASIL patients. Results: Mean age of onset was 42.43 years. The primary clinical manifestations included: Ischemic stroke/transient ischemic attack (62.5%), primary intracerebral hemorrhage (25%), vertigo (25%), migraine (39.58%), dementia (18.75%) and emotional disturbance (20.83%). The most frequently observed MRI abnormalities were hyperintensity in the cerebral white matter on T2-weighted images and multiple infarcts, high-signal lesions on T2 images in anterior temporal lobes and external capsule were uncommon. The highest mutation frequency was in exon regions, 4 and 3, followed by exon 11. Granular osmiophilic material (GOM) was identified in 66.67% of the cases examined with biopsy. Conclusions: Most characteristics of Chinese mainland CADASIL patients are similar to those of CADASIL patients living in other regions. However, the prevalence of primary intracerebral hemorrhage and vertigo is much higher in Chinese mainland CADASIL patients. Significant leukoaraiosis in anterior temporal poles on T2-weighted image are uncommon. Exons 3 and 4 are the mutation hotspots.

Background: Cognitive dysfunction after subarachnoid hemorrhage (SAH) has been attributable to presence of subarachnoid blood, hydrocephalus (HCP), cerebral edema, vasospasm, and temporary clipping of intracranial aneurysm. Provision of neuroprotection during temporary clipping may improve postoperative cognition in such patients. Materials and Methods: Good-grade aneurysmal SAH patients undergoing temporary clipping during surgery were allocated either to group C (control) or group P (propofol). Patients in group P received propofol in titrated doses to attain a burst suppression ratio of 75 ± 5% on bispectral index (BIS) monitor. The cognitive function as assessed by Hindi-language modification of mini-mental state examination (HMSE) score was evaluated preoperatively, 24 h after surgery, and at discharge from hospital. A score of ≤23 was indicative of cognitive dysfunction. Perioperative complications, duration of intensive care unit (ICU) and hospital stay, and outcome at discharge were noted. Results: A total of 66 patients (32 and 34 in group C and P respectively) were included in the study. 97% of the patients had anterior circulation aneurysms. At 24 h after surgery, eight and 12 patients in group C and P respectively; and at discharge, five patients in each group had cognitive dysfunction. In both groups, the trend showed a decline in cognition at 24 h followed by improvement at discharge. Glasgow outcome score in both the groups was comparable (P > 0.05). Intraoperative brain bulge, postoperative vasospasm, and cerebral infarction were found to be independent risk factors for cognitive dysfunction. Conclusions: Pharmacologic neuroprotection with propofol at the time of temporary clipping during surgery for aneurysmal SAH did not offer any advantage as far as preservation of cognition is concerned.

Background and Purpose: Posterior inferior cerebellar artery (PICA) like other intracranial arteries is prone to aneurysm formation. Aneurysms usually arise from the vertebral artery (VA) - PICA junction and the proximal segment of the PICA. The use of endovascular treatment as an alternative treatment to surgery has been increasing. We present our last 5 years experience in treating the ruptured proximal PICA aneurysms. Materials and Methods: Retrospective analysis of records of all patients with ruptured VA-PICA junction aneurysms treated at our referral center between July 2008 and July 2013 was performed. Over the last 5 years, we came across 17 patients who had aneurysms of proximal PICA or VA-PICA junction out of which 13 patients underwent endovascular treatment for ruptured saccular VA-PICA junction aneurysms and were the focus of this research. Follow-up studies ranged from 6 months to 3 years. Results: All the patients presented with an acute intracranial hemorrhage on NCCT. All the aneurysms were at VA-PICA junction with partial or complete incorporation of PICA origin in the sac. Endovascular treatment of all the 13 aneurysm was successful in the first attempt. Aneurysms were treated with balloon assisted coiling either by placing the balloon across the VA-PICA junction (n = 3) or in the vertebral artery proper (n = 9). Stent assisted coiling VA-PICA was performed in one aneurysm (n = 1). There was no intra-procedural rupture of the aneurysms. Conclusion: Endovascular therapy of ruptured proximal PICA aneurysms is possible and safe with the use of adjuvant devices and should be considered as first line treatment.

Background: In India, late presentation due to poor awareness about stroke precludes intravenous thrombolysis (IVT). Endovascular therapy can be used in these circumstances. We present our experience of endovascular treatment of acute ischemic stroke in a tertiary care center. Aim: Endovascular treatment of acute ischemic stroke in a tertiary care center in India. Settings, Design, Materials, and Methods: The study period was from 2009 till 2013. Consecutive patients with acute ischemic stroke, who either had contraindications to IVT or had failed IVT, underwent endovascular treatment. Before the publication of interventional management of stroke 3 results, we took a few patients, with large vessel occlusions, directly for endovascular treatment. After imaging, patients were considered for endovascular treatment, using either intra-arterial thrombolysis or mechanical thrombectomy, if there was a documented large vessel occlusion. Outcome was assessed at 3 months using the modified Rankin Scale. Statistical Analysis: Statistical analysis was done using the Statistical Package for the Social Sciences 17.0 software. Results: A total of 45 patients underwent endovascular treatment. The mean age at presentation was 49 years, median National Institutes of Health Stroke Scale (NIHSS) was 19 and the most common site was the middle cerebral artery (23 patients). Solitaire™ stentriever was used in 33 patients. The median pre-procedure Thrombolysis In Myocardial Infarction (TIMI) score was 0 and the median post-procedure TIMI score was 3. Nine patients underwent decompressive craniectomy. On follow-up at 3 months, the median Modified Rankin Scale (mRS) was 0. Eight patients died during 3 months following stroke. Conclusion: Endovascular treatment of acute ischemic stroke is a viable treatment option in patients who either have contraindications to IVT or who fail IVT.

Objective: To study the dynamic changes of pituitary hormones in traumatic brain injury (TBI) and to correlate the severity and neurological outcome. Patients and Methods: Dynamic changes in the pituitary hormones were evaluated in 164 patients with TBI on day-1, day-7, day-14, day-21, and day-28 post injury. Admission TBI severity and long-term outcome were assessed with Glasgow Coma Scale (GCS) score and Glasgow Outcome Scale (GOS) score. The pituitary hormonal changes were correlated with TBI severity and outcome. Results: Of the 164 patients included in the study, pituitary dysfunction was found in 84 patients and in the remaining 80 patients pituitary function was normal. Most of the pituitary hormone deficiencies observed resolved over time; however, a significant proportion of patients had pituitary dysfunction at one month post injury. The hormones associated with poor outcome included growth hormone, thyrotropic hormone, and gonadotropic hormone. Conclusion: Dynamic changes of pituitary hormones in patients with TBI may reflect the severity of injury and also determine the outcome. Deficiency of growth hormone, gonadotropic hormone, and thyrotropic hormone can adversely affect neurological outcome.

Background: We observed that in patients with traumatic brain injury (TBI) who did not improve as expected, serum levels of phenytoin were in the toxic range and that their sensorium improved with modification of the dose. This led us to study the usage of phenytoin in patients with TBI. Aims: To determine the prevalence of phenytoin toxicity in TBI patients and to study the suitability of using ideal body weight (IBW) to guide phenytoin dosing. Setting and Design: Neurotrauma unit of a tertiary care centre in India. Prospective data collection from an already established protocol of drug level monitoring. Materials and Methods: The study cohort included 100 consecutive adult patients with mild or moderate TBI who were administered phenytoin based on IBW. Trough serum phenytoin and albumin levels were measured on day 4 after administration of the loading dose and actual body weight obtained when it was possible. Statistical Analysis: Chi-square was used for comparing categorical variables, student's t-test for continuous variables and multivariate regression analysis to obtain independent risk factors. Results: Clinical toxicity was observed in 15% of patients and biochemical toxicity in 36%, with a significant association between the two (P < 0.01). Using multivariate analysis, abdominal girth ≤75 cm (P = 0.07), neck circumference ≤34 cm (P = 0.025) and IV dose proportion ≥80% (P = 0.003) were independent risk factors for biochemical toxicity. The plot between actual weight and IBW showed that toxicity occurred when IBW was higher than actual weight. Conclusion: The prevalence of biochemical phenytoin toxicity was high, with independent risk factors being a higher proportion of IV administration and overestimation of weight by IBW. Clinical suspicion of phenytoin toxicity was a good predictor of biochemical toxicity.

Surgical excision of rare, large-to-giant posterior third ventricular (PTV) meningiomas [including velum-interpositum meningiomas (VIM; postero-superior venous complex displacement; without falco-tentorial attachment) and falco-tentorial meningiomas (FTM; falco-tentorial attachment; displacing major veins antero-inferiorly)] is extremely challenging. To study the management nuances in the excision of large-to-giant PTV meningiomas. Tertiary care referral center. Four patients with large (>3 cm; n = 2) and giant (>5 cm; n = 2) meningiomas (FTM = 2; VIM = 2, mean tumor size = 4.9 cm) underwent occipital transtentorial approach (OTT) for tumor excision. One also underwent a second-stage supracerebellar infratentorial (SCIT) approach. The side of approach was determined by lateral tumor extension and venous displacement (right = 3, left = 1). Near-total removal or subtotal excision (<10% remaining) with radiotherapy was performed in 2 patients each, respectively. At follow-up (mean: 14.75 months), clinical improvement without tumor recurrence/re-growth was achieved. Extent of excision was determined by position of great vein of Galen; tumor attachment to falco-tentorium or major veins; its consistency; its lateral and inferior extent; and, presence of a good tumor-neuraxial arachnoidal plane. OTT is the preferable approach for large-to-giant meningiomas as it provides a wider corridor and better delineation of tumor-neurovascular arachnoidal interface.