Dural sinus malformation presenting with proptosis: report and review of a rare entity.

Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features.

An Eye Opener for a Blinding Disease-Orbital Infarction Syndrome.

Orbital infarction syndrome (OIS) is a disease of rare occurrence owing to the rich orbital vascular anastomotic network. We describe a case of a middle-aged female who presented with an acute left middle cerebral artery (MCA) territory infarct with left terminal internal carotid artery and MCA occlusion, underwent emergency mechanical thrombectomy, and developed painful loss of vision shortly after diagnosed as OIS based on clinical and radiological findings. The rarity and severity of OIS, especially in the setting of mechanical thrombectomy, warrant radiologists to be aware of this entity to ensure preventive measures or aid in prompt diagnosis to institute timely treatment.

High flow arteriovenous malformation with transverse-sigmoid sinus stenosis and congestive venopathy mimicking DAVF.

BACKGROUND: Arteriovenous malformations commonly present with hemorrhage, seizures, headache and other symptomatology. However, AVMs presenting as venous hypertension, owing to downstream stenosis/occlusion of venous sinuses, are very rare. This presentation is much more common in patients with dural AVFs. CASE DESCRIPTION: We present a young lady with left frontal arteriovenous malformation with bilateral transverse-sigmoid sinus stenosis, presenting with features of venous hypertension, which was treated with surgical excision of AVM. This case demonstrates a rare example of AVM with co-existing venous sinus stenosis distal to the nidus. CONCLUSIONS: High flow AVMs may co-exist with venous sinus stenosis or occlusion and lead to congestive venopathy. Treatment of AVM with surgical resection can be performed safely to relieve the hyper-dynamic venous congestion.

Is Elective Neurosurgery Justified During COVID-19 Pandemic?

BACKGROUND: As the world faces a new viral pandemic, which has spread very rapidly, initial response from most countries was to suspend nonemergent health services so that available resources can be diverted to handle the large numbers of patients with COVID-19 infection. Many societies issued guidelines to suspend or postpone nonemergent surgeries. METHODS: We reviewed the emerging evidence regarding the impact of COVID-19 infection in neurosurgery and the postponement of elective surgeries. RESULTS AND OBSERVATIONS: COVID-19 infection poses serious threat in hospitals in the form of cross-infection, hospital staff falling sick, with potential risk to overwhelm or paralyze the healthcare. In addition, we have come to realize the significant perioperative morbidity and mortality secondary to active COVID-19 infection. All these strongly favor suspension of elective neurosurgical services. However, these have to be weighed against the fallout due to prolonged postponement of neurosurgical treatment for conditions, which can progress and cause neurological deterioration. CONCLUSION: This article discusses the contemporary published literature regarding the perioperative risk of COVID-19 infection, impact of postponed surgeries, challenges, risk assessment and guidelines for elective neurosurgery at this point of time.

Alpha Internexin: A Surrogate Marker for 1p/19q Codeletion and Prognostic Marker in Anaplastic (WHO grade III) Gliomas.

BACKGROUND: The WHO 2016 classification of diffuse gliomas has incorporated molecular markers isocitrate dehydrogenase (IDH) gene mutations (IDHmut) and codeletion of chromosomal arms 1p and 19q (1p/19q codeletion) as tumor defining entities. The diagnosis of diffuse oligodendrogliomas (ODG) and anaplastic oligodendroglioma (AO) mandatorily requires the demonstration of IDH1 and/or IDH2 mutations along with 1p/19q codeletion, whereas the 1p/19q noncodeleted diffuse gliomas are labeled as astrocytomas. The current methodologies for assessing 1p/19q codeletion status are expensive and not widely available. Studies have proposed alpha internexin (INA) expression on immunohistochemistry (IHC) as a surrogate marker for 1p/19q codeletion and a good prognostic marker in gliomas. MATERIALS AND METHODS: In this study, we performed IHC for INA expression on the retrospective cohort of anaplastic gliomas (AGs) from our previously published study. RESULTS: INA positivity on IHC showed a significant positive correlation with 1p/19q codeletion (P < 0.001) with a Spearman's rank correlation coefficient (Rho) of 0.804, sensitivity of 87.5%, specificity of 93.0%, and a diagnostic odds ratio of 93:1 in AGs. Similar to the 1p/19q codeletion status, INA positivity showed a positive correlation with IDHmut (P = 0.002) and a negative correlation with α-thalassemia mental retardation X-linked protein (ATRX) loss of expression (P < 0.001). On univariate survival analysis, INA positivity was associated with significantly prolonged overall survival (OS) and recurrent free survival (RFS) in AGs (P < 0.001). Furthermore, within AO, INA positivity significantly improved RFS (P = 0.022) with OS trending towards significance (P = 0.094). CONCLUSIONS: INA expression on IHC could serve as a potential surrogate marker for 1p/19q, and highlights its prognostic value in AO and AGs.

Establishing a Training Model for Side-to-Side Anastomosis using Rat Femoral Vessels: Immediate and Delayed Patency.

INTRODUCTION: Side-to-side anastomoses are a relatively newer and more difficult technique used in neurovascular surgery for complex aneurysms/lesions. In view of the complex surgical technique and the infrequent clinical opportunities to acquire skills relevant to this surgery, laboratory training assumes great importance. The authors studied the feasibility of establishing a training model for performing side-to-side anastomosis using rat femoral vessels and compared the immediate and delayed patency rates in this animal model. MATERIALS AND METHODS: After Institutional Animal Ethics Committee clearance, 16 Sprague-Dawley rats were used in this prospective study between August 2013 and March 2015. Using the standard groin incision, the femoral vessels were dissected. After applying temporary clamps, opening of approximately 3-4 mm (at least double the diameter of the wider vessel) of length was made on the facing surfaces of both vessels. 10-0 nylon was used for anastomosis. The clamps were released, and the anastomoses patency confirmed. Leg movements following the anastomoses were studied. The animals were subsequently sacrificed to assess delayed patency. RESULTS: The immediate patency rate was 87.5% (14/16). The delayed patency rate (mean follow-up 209 days) was 53.8% (7/13). Three rats died during the follow-up period. The mean clamp duration and suturing time was 57.25 and 41.50 min in the first eight cases and 57.50 and 36.75 min in the last eight experiments, respectively. CONCLUSION: This animal model was found to be extremely useful for training in the difficult art of side-to-side anastomoses. The need of the hour is to establish well-planned training programs in centers with animal research facilities and use such models. This will promote younger colleagues and trainees to take up and perfect this difficult art. The present work could be used as a baseline study in this direction.

Lifestyle and Occupational Stress: A Potential Risk Factor for Obstructive Sleep Apnea in Nonobese Male Subjects.

PURPOSE: To record the incidence of obstructive sleep apnea (OSA) in nonobese male subjects and investigate the coexistence of lifestyle stress, sleep deprivation, and upper airway inflammation. MATERIALS AND METHODS: 552 patients were assessed during a survey of banks, government and corporate offices, recruitment agencies, and schools between January 2012 and January 2016. After applying the inclusion and exclusion criteria designed for this study, the number of patients tapered down to 120 patients who underwent diagnostic tests, viz. polysomnography, chemiluminiscence immunoassay, nephelometry, and upper airway endoscopy. This revealed the presence of OSA coexistent with elevated serum cortisol, C-reactive protein (CRP), and upper airway inflammation. RESULTS: Polysomnography showed that 57 of 120 patients suffered from OSA. Objective evaluation of these patients exposed an undercurrent of lifestyle stress in 39 patients. CRP and serum cortisol were found to be significantly high (1.60 ± 0.52 and 7.20 ± 0.76 µg/dL, respectively) in 30 patients. Endoscopy revealed 18 patients with moderate, 7 with severe, and 5 with no upper airway inflammation. CONCLUSION: The results of this study demonstrated that OSA was found to be prevalent in the cohort of nonobese male patients studied. Coexistence of lifestyle stress, sleep deprivation, and upper airway inflammation was revealed.

Training in Micro-Vascular Anastomosis Using Rat Femoral Vessels: Comparison of Immediate and Delayed Patency Rates.

AIM: Micro-vascular anastomosis is a difficult art. It can be learnt and perfected only by practice using vessels of small animals in the laboratory. The purpose of the study was to practice various anastomotic techniques using rat femoral vessels and study the patency rates. MATERIAL AND METHODS: Sixty-four Sprague-Dawley rats were used in the study. The following procedures were conducted on rat femoral vessels; Femoral artery end-to-end anastomosis- classical method, one way up method, Femoral vein end-to-end anastomosis and end-to-side anastomosis. The animals were subsequently sacrificed at a later date to assess delayed patency. RESULTS: The immediate patency rates for femoral artery end-to-end anastomosis- classical method (n=10), one way up method (n=16), femoral vein end-to-end anastomosis (n=12) and end-to-side anastomosis (n=26) were 100%, 81.3%, 58.3% and 53.8% respectively. The delayed patency rates (Mean follow-up=3.6 months) were 66.7%, 69.2%, 83.3% and 16% respectively. The clamp duration and suturing time significantly decreased and patency rates improved significantly in the latter half of the study. CONCLUSION: The clamp duration, suturing time and patency rates improved considerably with time. The need of the hour is to establish well-planned training programs in centers with Animal Research facilities. This will promote younger colleagues and trainees to take up and perfect this difficult art. The present work could be used as a baseline study in this direction.

Multimodality Management of Cavernous Sinus Hemangiomas-An Institutional Experience.

Objective Cavernous sinus hemangiomas (CSHs) are benign lesions accounting for less than 2% of the cavernous sinus tumors. They provide a formidable surgical challenge because of their vascularity and their being surrounded by critical neurovascular structures. In this study, one of the largest in available literature, we analyze our experience in the management of these unusual tumors and review the available literature. Materials This is a retrospective analysis of patients who were managed surgically (both microsurgical and Gamma knife radiosurgery [GKRS]) for CSH at our Institution from 2007 to 2015. Complete demographic, clinical-radiologic surgical records were analyzed. Follow-up data were collected from the hospital records. Results Total 23 patients were managed. Among these, 15 patients underwent microsurgery (group 1) whereas 8 underwent GKRS (group 2). Predominant clinical presentation in both the groups included headache and involvement of multiple cranial nerves. Five patients in group 1 had deteriorating vision. The volume of tumors ranged from 29 to 115 cm 3 (mean = 64.57 cm 3 ) in group 1 and from 2.1 to 11.6 cm 3 in group 2. GKRS was performed with a mean dose of 13 Gy, an average isodose line of 50% with an average coverage of 96%. In group 1, the follow-up period ranged from 6 to 62 months (mean = 29.4 months). The extraocular movement (EOM) preservation rate in our series was not favorable, as most patients presented late with large tumors and established deficits. Recurrence/residual tumor was seen in two cases. In group 2, the follow-up was 5 to 48 months. All of them showed significant reduction in size. Conclusion Both surgery and radiosurgery are highly effective in the management of CSHs. They are complementary to each other, with individual characteristics-the size and volume of the lesion-being the main factors in deciding the choice of treatment.

Impairments in Quality of Life and Cognitive Functions in Long-term Survivors of Glioblastoma.

BACKGROUND: The incidence of long-term survival in glioblastoma (GBM), i.e., >3 years, ranges from 3% to 5%. Although extensive research is performed in novel therapies for prolonging survival, there is a scarcity of research focusing on the impact of tumor and treatment on cognitive, psychological, and social status of survivors. This study is an attempt to look into this poorly addressed important issue. MATERIALS AND METHODS: Nine patients (six adults and three children) with GBM who had survived >3 years were included in the study. The quality of life (QOL) functions were assessed with the World Health Organization QOL Questionnaire BREF questionnaire. The neuropsychological assessment was done using the National Institute of Mental Health and Neurosciences neuropsychology battery for adults and children. The scores were compared with normative data. RESULTS: The physical and psychological health-related QOL of long-term GBM survivors were affected considerably due to fatigue, poor quality of sleep, inability to concentrate, presence of depression, financial burden with impaired personal and social relationships (P < 0.05). Different domains of cognitions such as motor speed (P = 0.0173), mental speed (P = 0.0022), sustained attention (P = 0.0001), long-term memory (P = 0.0431), mental flexibility (P < 0.05), and planning and executive functions (P < 0.05) were significantly impaired affecting personal, social, and professional lives. CONCLUSION: The health-related QOL and cognition are significantly impaired in GBM long-term survivors. As the incidence of long-term survival is very less, there is a need for larger multicenter studies to come up with definitive results, which in turn can help in formatting the rehabilitative and support programs for these patients.

Microsurgical treatment of distal anterior cerebral artery aneurysms: A 25 year institutional experience.

INTRODUCTION: We present a retrospective study of distal anterior cerebral artery (DACA) aneurysms operated at our institute. MATERIAL AND METHODS: From 1988 to 2013, a total of 132 DACA aneurysms were managed surgically. The retrospective data of these patients was collected and analysed. RESULTS: They comprised 6.2% of all 2137 aneurysms operated during the same period. The mean age at presentation was 48.3 years (range: 24-77 years) and the male: female ratio was 1:2. The notable symptom in 114 (86.4%) patients was headache, in 94 (71.2%) was loss of consciousness, in 25 (18.9%) was seizures, and in 17 (12.9%) was limb weakness. There were 99 (75%) patients in World Federation of Neurosurgical Societies (WFNS) grade <3 at admission. On plain computed tomography (CT) scan, intracerebral hemorrhage was seen in 49 (37.1%) and intraventricular hemorrhage in 23 (17.4%) patients. The most common site of aneurysm was the pericallosal (A3 segment) artery noted in 65 (49.2%) patients. Multiple aneurysms were observed in 16 (12.1%) cases. The mean timing of surgery from the first ictus was 17.8 days. The delay was due to a late referral, poor general condition, recurrent bleeding, and worse WFNS grade at presentation. Aneurysms were operated through the following approaches: parasaggital craniotomy in 61 (46.2%), bifrontal craniotomy in 64 (48.5%), and frontotemporal craniotomy in 7 (5.3%). Aneurysms could be successfully clipped in 93.2% of the cases. Postoperative mortality was 8 (6%). The follow-up data was available for 83 (62.9%) patients. The mean duration of follow-up was 15.1 months (1-70 months), with more than 41 (31%) patients having a follow-up of more than 6 months. A favorable outcome (good recovery or moderate disability) was seen in 71 (85.5%) cases. CONCLUSIONS: A meticulous attention to the surgical technique provides gratifying results in DACA aneurysms even in patients who present initially in a compromised clinical status.

Highest cited papers published in Neurology India: An analysis for the years 1993-2014.

CONTEXT: The highest cited papers published in a journal provide a snapshot of the clinical practice and research in that specialty and/or region. AIMS: The aim of this study was to determine the highest cited papers published in Neurology India and analyze their attributes. SETTING AND DESIGN: This study was a citation analysis of all papers published in Neurology India since online archiving commenced in 1993. METHODS: All papers published in Neurology India between the years 1993-2014 were listed. The number of times each paper had been cited up till the time of performing this study was determined by performing a Google Scholar search. Published papers were then ranked on the basis of total times cited since publication and the annual citation rate. Statistical Techniques: Simple counts and percentages were used to report most results. The mean citations received by papers in various categories were compared using the Student's t-test or a one-way analysis of variance, as appropriate. All analyses were carried out on SAS University Edition (SAS/STAT®, SAS Institute Inc, NC, USA) and graphs were generated on MS Excel 2016. RESULTS: The top papers on the total citations and annual citation rate rank lists pertained to basic neuroscience research. The highest cited paper overall had received 139 citations. About a quarter of the papers published had never been cited at all. The major themes represented were vascular diseases and infections. CONCLUSIONS: The highest cited papers reflect the diseases that are of major concern in India. Certain domains such as trauma, allied neurosciences, and basic neuroscience research were underrepresented.