Chaotic lipoma with proximal syrinx -a (not so) rare variant - review of the literature, possible embryology and management.

Chaotic lipomas are an extremely rare variant of spinal lipomas. This entity was first defined in 2009 by Pang and colleagues. Not much has been written about this variant. Its characteristic is the haphazard distribution of DREZ (Dorsal root entry zone), nerve roots and placode-lipoma interface. Thus complete/near-total excision of this lesion is quite difficult. We describe a case of chaotic spinal lipoma and elucidate the challenges faced in the management of this entity and review the literature. We performed a thorough systematic review with the keyword 'chaotic', 'Lipomyelomeningocele', 'Complex Lipomyelomeningocele', 'LMMC', 'Lumbar lipoma', 'spinal lipoma' in the google scholar and PUBMED data system for indexed literature on the above topic with no particular time frame. The studies quoted range earliest from 1970 till currently. Additional potential relevant articles were further retrieved through a manual search of references from original reports. Out of 42 studies, a total of 21 publications were selected which could have encountered a chaotic variant, but due to the term introduced only recently in 2009, may have been described differently. Studies encompassing true lipomeningomyelocele were excluded from our review. What we found out? Chaotic lipoma may not be a new entity. The scarce description in literature may be in part due to non-introduction and unclear description of this term earlier. The management of this variant is particularly challenging with basic principles remaining the same. Meticulous near-total excision and placode-lipoma construct are the major obstacles.

Heartbroken Child: A Rare Case Report of Neurogenic Pulmonary Edema and Takotsubo Cardiomyopathy following Recurrent Medulloblastoma Excision with Possible Aetio-Patho-Bio-Physiological Mechanisms.

INTRODUCTION: Takotsubo (Japanese fishing pot for trapping octopus) cardiomyopathy is a rare phenomenon of acute coronary syndrome presenting usually with the presence of transient apical ballooning of the left ventricle in the absence of obstructive coronary artery disease. It is mainly seen in women of older age secondary to emotional, physical, or psychological stress. In age less than 18 years, it is mainly seen in adolescents suffering from psychiatric disorders and substance abuse. In children, it is rarely described. CASE PRESENTATION: We present here a peculiar case of a 12-year-old child with neurogenic pulmonary oedema and takotsubo cardiomyopathy syndrome after surgery for recurrent medulloblastoma. Also, management challenges are discussed. DISCUSSION/CONCLUSION: Takotsubo cardiomyopathy is not just a classical or inverted type but indeed a spectrum. It can be seen in any case, be it a child or adult secondary to handling or injury to the ponto-medullary junction, rostral pons, or dorsolateral medulla. The density and distribution of beta-adrenergic receptors may be different in children and adults which needs further research. Prognosis is usually excellent across all ages.

An analysis of retractions in neurosurgery and allied clinical and basic science specialties.

BACKGROUND: As the volume of scientific publications increases, the rate of retraction of published papers is also likely to increase. In the present study, we report the characteristics of retracted papers from clinical neurosurgery and allied clinical and basic science specialties. METHODS: Retracted papers were identified using two separate search strategies on PubMed. Attributes of the retracted papers were collected from PubMed and the Retraction Watch database. The reasons for retraction were analyzed. The factors that correlated with time to retraction were identified. Detailed citation analysis for the retracted papers was performed. The retraction rates for neurosurgery journals were computed. RESULTS: A total of 191 retractions were identified; 55% pertained to clinical neurosurgery. The most common reasons for retraction were plagiarism, duplication, and compromised peer review. The countries associated with the highest number of retractions were China, USA, and Japan. The full text of the retraction notice was not available for 11% of the papers. A median of 50% of all citations received by the papers occurred after retraction. The factors that correlated with a longer time to retraction included basic science category, the number of collaborating departments, and the H-index of the journal. The overall rate of retractions in neurosurgery journals was 0.037%. CONCLUSIONS: The retraction notice needs to be freely available on all search engines. Plagiarism checks and reference checks prior to publication of papers (to ensure no retracted papers have been cited) must be mandatory. Mandatory data deposition would help overcome issues with data and results.

What you see and what you don't - Utility and pitfalls during fluorescence guided resections of gliomas using 5-aminolevulinic acid.

BACKGROUND: Fluorescence guided resections have been increasingly used for malignant gliomas. Despite the high reliability of the technique, there remain some practical limitations. METHODS: We retrospectively reviewed our experience with 50 consecutive cases of 5-aminolevulinic acid (ALA)-guided resections. Clinico-radiological features and intraoperative variables (pattern and type of fluorescence) were recorded. In a subset (12 cases), we performed annotated biopsies to calculate the diagnostic accuracy of the technique. We recorded and analysed the patterns of excision and residual fluorescence and correlated this with postoperative magnetic resonance imaging (MRI). RESULTS: Majority of the tumours (92%) were resectable and predominantly enhancing. Though strong fluorescence was seen in most of them, there were 2 cases with a non-enhancing tumor which showed fluorescence. Visualized strong fluorescence had a very high predictive value (100%) for detecting the pathological tissue. However, it was not always possible to resect all the fluorescing tissue. Proximity to critical neuro-vascular structures was the commonest reason for failure to achieve a gross total excision (16 cases). Additionally, there were some cases (5 of 8) where the non-fluorescing residue was resected intraoperatively with the help of ultrasound. Despite the presence of residual fluorescence, overall radiological gross total resection was achieved in 66% cases. CONCLUSIONS: ALA guided resections are very useful in malignant gliomas, even if these lesions do not enhance signi cantly. Although ALA reliably depicts the tumour intraoperatively, it may not be possible to resect all this tissue completely. Additionally, non-fluorescing tumor may be completely missed out and may require additional imaging tools. Working within the limitations of the technique and using complementary modalities (ultrasound or brain mapping) may be ideal for achieving a radical resection of malignant gliomas.

Metastatic malignant peripheral nerve sheath tumour presenting as a spontaneous extradural haematoma - A case report.

Malignant peripheral nerve sheath tumours may arise from any cranial or somatic nerve. The median survival with best therapy is 49 months. The present case reports a patient with an MPNST that exhibited an unusually indolent behaviour. Besides this, the patient developed a dural metastasis from the lesion and presented with a spontaneous extra-dural haematoma. This has not been reported hitherto in literature.

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.

Publication performance and research output of Neurology and Neurosurgery training institutes in India: A 5-year analysis.

CONTEXT: Scientific publications are a reflection of the quality of the clinical and academic work being carried out in an institute. Training in the process of research and scientific writing are important components of the residency curriculum. AIMS: The publication performance and research output of institutes training residents in neurology and neurosurgery were evaluated. SETTING AND DESIGN: Internet-based study. METHODS: This study was based on the data available on the websites of the Medical Council of India and the National Board of Examinations. The PubMed search interface was used to determine the publication output of institutes over the past 5 years (2010-2014). Google Scholar was used to determine the citation performance of each paper. The publication parameters were normalized to the number of faculty members in each institute as listed on the institutional web page. The normalized publication performance for an institute was computed by comparing the figures for that institute with the national average. RESULTS: Institutes could be ranked on several criteria. There was a high degree of clustering of output from the top 5% of the institutes. About 13% of the neurology intake and 30.9% of neurosurgery intake over the past 5 years has been into the institutes that have not published a single paper during this period. CONCLUSIONS: This evaluation of the publication performance and research output of neurology and neurosurgery training institutes would serve as a baseline data for future evaluations and comparisons. The absence of any publication and research output from several training institutes is a matter of concern.

The posterior cranial fossa: a comparative MRI-based anatomic study of linear dimensions and volumetry in a homogeneous South Indian population.

PURPOSE: The posterior fossa contains structures that are vital to life. In this study, we aimed at establishing normal linear dimensions and volume data of the posterior fossa in a homogeneous south Indian population. We also evaluated the influence of large tumors on these parameters. We evaluated the accuracy of different techniques of measuring these dimensions and compared them with literature. MATERIALS AND METHODS: Control and tumor MRIs were selected from an imaging database. Linear posterior fossa dimensions as well as volumes were measured using Image J and Fiji. The volume data were compared with similar data from literature. The effect of the presence of a tumor on posterior fossa volume was measured. RESULTS: The posterior fossa volume was higher in men than in women, irrespective of whether the volume was estimated on axial, sagittal or coronal MR images. Despite the wide variation in the techniques used, there was no significant difference between the volumes reported in literature and the volumes calculated in the current series. The presence of large tumors did not affect linear dimensions or posterior fossa volumes. Among the techniques based on linear measurements that were assessed for concordance with manual segmentation, the technique using the formula for volume of an ellipsoid had the best agreement. CONCLUSIONS: Posterior fossa volume is higher in men than in women, Posterior fossa dimensions were not affected by the presence of large tumors. Manual segmentation remains the most accurate method to measure posterior fossa volume.

Multifocal spinal malignant peripheral nerve sheath tumor in an immunocompromised individual: case report and review of literature.

PURPOSE: Primary intraosseous spinal malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. MPNST with multifocal origin has been described to occur in the extremities. Such a lesion has not been described to occur in the spine. We describe a case of multifocal spinal MPNST and to review the literature relevant to this rare entity and its management. METHODS: A 40-year-old immunodeficient patient presented with rapidly progressive paraparesis and mid back ache. RESULTS: Despite aggressive surgical decompression, he developed multiple metastases 3 months after surgery. However, he remained stable for 1 year without any adjuvant therapy. Presently, he has received palliative radiotherapy for spinal recurrence and cerebral metastasis. CONCLUSION: Multifocal spinal MPNST is a rare lesion. In this instance, the multifocality of the disease and its odd location could be attributed to the immunodeficiency state. The prolonged survival could be due to an improvement in his immune status due to HAART.

Does Reader Engagement with Neurosurgery Journal Websites Correlate with the Number of Citations Received by Articles?

BACKGROUND: Medicine has begun adapting to new information-sharing paradigms in the hyper-connected social media era. In this milieu, the role of journal websites in the dissemination of clinical and research information needs to be reevaluated. OBJECTIVE: We sought to explore whether reader engagement with neurosurgical journal websites, reflected by the number of article views and downloads, correlated with the eventual number of citations received by the articles. METHODS: The websites of all Medline indexed neurosurgical journals were screened to identify those that provided information regarding the number of abstract and full text views and downloads. Articles published in these journals between July 2010 and June 2011 were included in this analysis. Various article attributes were identified and the number of citations per article was obtained from Google Scholar. The impact factors of the selected journals for the year 2010 were obtained from the Journal Citation Reports. RESULTS: Twenty-two journals that had published 2527 articles were finally included in this analysis. The number of abstract views, full-text views, and downloads all correlated strongly with the journal impact factors in 2010 as well as the eventual citations per article. The number of article downloads independently predicted the citations per article on multivariate analysis. Neurology India had significantly higher article views and downloads but lower citations per article than the other journals. CONCLUSIONS: Readers were found to engage significantly with neurosurgical journal websites and therefore, open access to articles would lead to increased visibility of articles, resulting in higher citation rates.

Trends in intracranial meningioma surgery and outcome: a Nationwide Inpatient Sample database analysis from 2001 to 2010.

The objective of the present study was to analyze the risk of in-patient mortality, adverse outcome, practice patterns and regional variations in patients who underwent intracranial meningioma surgery in the United States between 2001 and 2010. We performed a retrospective cohort study using the Nationwide Inpatient Sample database. In-patient mortality and adverse outcome at discharge were the outcome predictors. Multivariate analyses were done to analyze the patient, hospital and physician characteristics. The annual case-volume of patients with meningioma increased from 2001 to 2010 by 40 %. The in-patient mortality rate remained the same at 1.3 % and the rate of adverse discharge disposition remained at 35 % between 2001 and 2010. Caucasian female patients in younger age group with private insurance who underwent treatment at a high case-volume center had the best outcomes. In older patients (≥70 years), the in-patient mortality rate decreased by 25 % whereas the adverse discharge disposition rate increased by 19 %. Patients treated at high case-volume centers and by high case-volume physicians had lower rates of in-patient mortality (P < 0.05) and adverse outcome at discharge (P = 0 < 0.05). There was a 54 % decrease in the number of hospitals performing one surgery/year through the decade. A 2 % relative decrease in mortality was observed in lowest volume hospitals. Though the highest increase in admission charges through the decade was seen in hospitals located in the north-east (165 % relative increase), the highest relative decrease in mortality and morbidity was observed in hospitals located in the mid-west and the south (67.6 and 22 % respectively).

Tuberculoma of the Cavernous Sinus and Meckel's Cave in a Child.

Tuberculous infection of the cavernous sinus and Meckel's cave is extremely rare. In this report, we describe a patient with tuberculoma of the cavernous sinus and Meckel's cave, extending to the petrous apex. The patient underwent microsurgical excision of the lesion and antitubercular chemotherapy resulting in a good outcome. We describe the diagnostic difficulties and review the relevant literature.

Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association.

Numerous syndromes with craniofacial, cardiac, cutaneous and vascular anomalies have been described in the literature. Here we report on a 9-year-old girl who was diagnosed as having a clavicular arteriovenous malformation with limb enlargement, an axillary port wine stain and macrocephaly with frontal bossing. She was investigated for enlarged head size; although she did not have hydrocephalus as initially suspected, she was found to have a right cerebellar lesion. This was excised and the pathology report was diagnostic of desmoplastic ganglioglioma. The posterior fossa is a very rare location for gangliogliomas. This was a desmoplastic variant that is only encountered in infants. Thus, the location and the pathology made the tumour rare. In addition, the cranio-vasculo-cutaneous lesions made this previously unreported association noteworthy.

Opportunistic Fonsecaea pedrosoi brain abscess in a patient with non-cirrhotic portal fibrosis-induced hypersplenism--a novel association.

Non-cirrhotic portal fibrosis, a common cause of splenomegaly in tropical countries, can lead to hypersplenism and pancytopenia. Hypersplenism in this setting has not been associated with opportunistic infections. We describe a patient with hypersplenism secondary to non-cirrhotic portal fibrosis who developed a Fonsecaea pedrosoi brain abscess and succumbed to the illness despite aggressive management.

Short- and long-term outcomes of moyamoya patients post-revascularization.

OBJECTIVE: The post-bypass stroke risk factors and long-term outcomes of moyamoya patients are not well documented. Therefore, the authors studied 30-day stroke risks and patients' long-term physical, functional, and social well-being. METHODS: This was a single-institution combined moyamoya disease (MMD) database interrogation and questionnaire study. From 1991 to 2014, 1250 revascularization procedures (1118 direct bypasses, 132 indirect bypasses) were performed in 769 patients. Completed questionnaires were received from and available for analysis on 391 patients, and 6-month follow-up data were available for 96.4% (741/769) of the patients. RESULTS: The patients consisted of 548 females and 221 males, with a mean age of 32 years (range 1-69 years). Three hundred fifty-eight bypasses were performed in 205 pediatric patients (73% direct bypasses), and 892 revascularizations were performed in 564 adults (96% direct bypasses). Fifty-two patients (6.8%) developed major strokes with a worsening modified Rankin Scale (mRS) score within 30 days postoperatively. The 30-day major stroke risk was 5.3% (41/769) and 2.6% (12/467) after the first and second bypasses, respectively. Logistic regression analysis revealed that older age, modified MRI (mMRI) score, and hemodynamic reserve (HDR) score are clearly associated with higher postoperative stroke risks. Over a mean follow-up of 7.3 years (range 0.5-26 years), the long-term stroke risk among 741 patients was 0.6% per patient-year; 75% of these patients had excellent outcomes (mRS score 0-1). The long-term outcome questionnaire study showed that 84% (234/277) of patients reported resolution or improvement in their preoperative headache, 83% (325/391) remained employed or in school, and 87% (303/348) were self-caring. CONCLUSIONS: In this large, single-center surgical series, most of the adult and pediatric patients had direct revascularization, with a 4.2% per-bypass-procedure (6.8% per patient) 30-day major stroke risk and a 0.6% per-patient-year long-term stroke risk. The authors identified various risk factors that are highly correlated with postoperative morbidity (age, mMRI score, and HDR score) and are involved in ongoing work to develop the predictive modeling for future patient selection and treatment.

An Analysis of Eosinophil- and Basophil-Based Indices in Patients with Glioblastoma and their Correlation with Survival.

BACKGROUND: Glioblastoma (GBM) is the most frequently diagnosed malignant brain tumor in adults. GBM is usually lethal within 24 months of diagnosis, despite aggressive multimodality treatment. Although it has been established that cancer-related inflammation is associated with worse outcomes, the role of eosinophils, basophils, atopy, and allergy in glioma biology is only gradually being delineated. In this study, we aimed to examine if eosinophil-based and basophil-based indices were altered in patients with GBM compared with healthy controls. We also aimed to study if there was any correlation between these indices and patient-related and tumor-related factors and survival. METHODS: This study was a retrospective analysis of prospectively maintained databases. Data pertaining to patient-related and tumor-related factors, hemograms, and survival data were obtained from the electronic medical records of selected patients. Correlations between eosinophil-based and basophil-based indices and these factors were studied, as was the association with overall survival. RESULTS: All the indices were altered in patients with GBM compared with normal healthy controls. The absolute eosinophil count was higher and the neutrophils/eosinophils ratio was lower in the better prognosis groups: those with better performance status; those without features of increased intracranial pressure or altered sensorium at presentation; those with ATRX-retained tumors that did not overexpress p53; and in the long-term survivors. The total lymphocyte count/basophils ratio and the absolute eosinophil count both independently predicted survival in a multivariate analysis. CONCLUSIONS: The absolute eosinophil count was consistently higher in the better prognosis groups and is likely to be incorporated into prognostic models for GBM.

Idiopathic intraventricular aneurysm presenting with intraventricular hemorrhage: case report and review of the literature.

Intraventricular hemorrhage (IVH) is a relatively commonly encountered problem in neurosurgical practice. The underlying causes could include hypertension, arteriovenous malformations (AVM), angiomas, trauma, tumors, aneurysms and moyamoya disease. Truly idiopathic intraventricular aneurysms (IVA) are rare. A high index of suspicion needs to be maintained since, with the appropriate treatment, the outcome is generally good. We report the case of a 14-year-old boy who presented with sudden onset headache and vomiting. CT angiogram pointed to the possibility of an IVA. This was confirmed by MRI with three-dimensional constructive interference in steady state (CISS-3D) sequences and digital subtraction angiography. The patient underwent microsurgical excision of the aneurysm and is doing well on follow-up. Idiopathic IVA can present with IVH at any age. MRI with CISS-3D and MR angiography would be the imaging modality of choice since it can also rule out other causes of intraventricular bleeding such as tumors and AVM. These lesions could be managed effectively by microsurgical excision/clipping. The necessity of investigating every patient who presents with IVH is debatable.

Intramedullary metastasis in a case of vermian medulloblastoma.

Medulloblastoma is one of the commonest primary CNS malignancies in children. Leptomeningeal dissemination and distant metastasis have been associated with medulloblastoma, but intramedullary metastases are very rare. CSF cytology and contrast-enhanced MRI are the main modalities used to diagnose leptomeningeal dissemination. However, intramedullary metastases are best picked up with contrast-enhanced axial sequences on MR imaging. In this report, a patient with medulloblastoma who developed intramedullary metastasis is described. The role of imaging and CSF cytology in diagnosing the spread along the CSF pathways is reviewed. Allusions are made to the possible mechanism of intramedullary metastasis in these tumors.

The Author-Level Metrics Study: An Analysis of the Traditional and Alternative Metrics of Scholarly Impact for Neurosurgical Authors.

Background and objective There is a paucity of information regarding the concordance of traditional metrics across publicly searchable databases and about the correlation between alternative and traditional metrics for neurosurgical authors. In this study, we aimed to assess the congruence between traditional metrics reported across Google Scholar (GS), Scopus (Sc), and ResearchGate (RG). We also aimed to establish the mathematical correlation between traditional metrics and alternative metrics provided by ResearchGate. Methods Author names listed on papers published in the Journal of Neurosurgery (JNS) in 2019 were collated. Traditional metrics [number of publications (NP), number of citations (NC), and author H-indices (AHi)] and alternative metrics (RG score, Research Interest score, etc. from RG and the GS i10-index) were also collected from publicly searchable author profiles. The concordance between the traditional metrics across the three databases was assessed using the intraclass correlation coefficient and Bland-Altman (BA) plots. The mathematical relation between the traditional and alternative metrics was analyzed. Results The AHi showed excellent agreement across the three databases studied. The level of agreement for NP and NC was good at lower median counts. At higher median counts, we found an increase in disagreement, especially for NP. The RG score, number of followers on RG, and Research Interest score independently predicted NC and AHi with a reasonable degree of accuracy. Conclusions A composite author-level matrix with AHi, RG score, Research Interest score, and the number of RG followers could be used to generate an "Impact Matrix" to describe the scholarly and real-world impact of a clinician's work.

Simplifying the Surgical Strategy for Excising Medial Sphenoid Wing Meningiomas: A Stepwise Approach.

Background: Medial sphenoid wing meningiomas constitute 15%-20% of all intracranial meningiomas. These lesions have a propensity to encase the vessels of the circle of Willis and the surrounding cranial nerves. Thus, radical excision is a difficult proposition. Objectives: In this paper, we analyzed our series of sphenoid wing meningiomas. We describe our surgical strategy, which was based on zone-wise dissection of the tumor. We describe the complications and outcomes of surgery. Materials and Methods: This case series is a retrospective analysis of a single surgeon series of medial sphenoid wing meningiomas operated over a 13-year period. Clinical, radiographic, and outcome variables were studied. The surgical videos were analyzed in detail. The meningioma and its extensions were divided into several zones and a zone-wise strategy for tumor excision was evolved. Results: Twenty-four patients with medial sphenoid wing meningiomas were operated. In 14 patients, Simpson grade 3 excision could be achieved; 5 patients had Simpson grade 4 and 1 patient, grade 5 excision. Four (of 24 patients, 16.7%) had vessel injuries. Conclusions: Medial sphenoid wing meningiomas are difficult lesions to excise radically. Close follow-up of residual lesions (especially if attached to the basal dura) is warranted. Additional modalities of treatment like radiosurgery may be required in case of any progression and for higher-grade lesions.