Although rare, skull vault lesions include a vast array of pathology encompassing infection, benign, and malignant bone tumors. Given the large range of potential diagnoses, it is crucial to identify imaging features to differentiate one from another, ensuring early diagnosis. Radiographs are still valuable in modern radiology but have largely been superseded by computed tomography (CT) due to its high spatial resolution. Both are especially important in developing countries where access to magnetic resonance imaging (MRI) may be limited.There are currently several publications outlining imaging appearances of skull vault lesions. However, the majority of literature is dated, with the last dedicated textbook published in 1980 (Principles of X-ray diagnosis of the skull). Despite overlapping features, a few lesions have “aunt minnie,” type classical characteristics, which we will highlight. Most vault lesions also appear as a spectrum depending on location and the exact stage of the disease. A small subset within each disease entity also has atypical features not widely discussed in the current literature. In this pictorial review, we hope to focus on radiographic and CT imaging appearances to help differentiate between various skull vault lesions.

Background: Traumatic injury to the spine can be a complex diagnostic and therapeutic entity often with devastating consequences. Outside of the isolated vertebral column injury costs; annual costs associated with spinal cord injury (SCI) are estimated to exceed $9.7 billion. Objective: To identify the 100 most-cited articles on spine trauma. Methods: The Thomson Reuters Web of Science citation indexing service was queried. The articles were sorted by times cited in descending order. Two independent reviewers reviewed the article titles and abstracts to identify the top 100 most-cited articles. Results: The top 100 articles were found to be cited between 108 (articles #99-100) and 1595 times (article #1). The most-cited basic science article was cited 340 times (#12 on the top 100 list). The oldest article on the top 100 list was from 1953 and most recent from 2012. The number of patients, when applicable, in a study ranged from 9 (article #34) to 34,069 (article #5). Top 100 articles were published in 41 different journals with a wide range of specialities and fields most commonly multidisciplinary. Basic science research encompassed 34 of the 100 articles on the list. Conclusions: We present the 100 most-cited articles in spinal trauma with emphases on important contributions from both basic science and clinical research across a wide range of authors, specialties, patient populations, and countries. Recognizing some of the most important contributions in the field of spinal trauma may provide insight and guide future work.

Progressive external ophthalmoplegia (PEO) is a slowly progressive myopathy characterized by extraocular muscles involvement, leading to frozen eyes without diplopia. The pattern of inheritance may be mitochondrial, autosomal dominant or, rarely, autosomal recessive. Sporadic forms were also reported. Muscular involvement other than extraocular muscles may occur with varying degrees of weakness, but this mostly happens many years after the disease begins. There are also scattered data about systemic signs besides ophthalmoplegia. This article aims to review non-ophthalmic findings of PEO from a clinicogenetical point of view.

The entire world including India is currently fighting the coronavirus disease 19 (COVID-19) pandemic that threatens to disrupt healthcare systems globally in terms of capacity and resources. This outbreak necessitates an urgent review of existing management guidelines for commonly encountered tumors of the brain and central nervous system (CNS). Such a review should include a reassessment of benefit-risk ratio to align with local, national, and international priorities without compromising on delivery of care in terms of safety, compassion, efficiency, and effectiveness. Towards this end, the Indian Society of Neuro-Oncology (ISNO) constituted an online expert panel with adequate representation from all major treatment modalities (neuro-surgery, radiation oncology, and pediatric/medical oncology) to formulate a “COVID-19 context” position statement to guide the care of neuro-oncology patients during the ongoing crisis. The ISNO position statement suggests graded prioritization (based on clinical presentation, type of tumor, expected prognosis, and relevance of immediate therapy) for efficient utilization of resources and provides a framework through a set of general considerations, treatment modality-based considerations, and disease-specific considerations for the guidance of healthcare professionals involved in the delivery of care and services to patients with CNS tumors. The views expressed herein represent the current consensus of key opinion leaders from within the Indian neuro-oncology community and should not be in any case considered binding medically or legally to individual physicians and/or hospitals who may formulate their guidelines based on local setup and health-environment and update them periodically based on emerging evidence through the COVID-19 pandemic.

Introduction: Severe acute respiratory syndrome, coronavirus 2 (SARS-COV 2) has inexplicably and irreversibly changed the way of neurosurgery practice. There has been a substantial reduction in neurosurgical operations during the period of lockdown. The lockdown might be the most effective measure to curtail viral transmission. Once we return to the normalization of the lifestyle, there will be a backlog of unoperated pending cases along with the possibility of further spread of the coronavirus. Methods: We reviewed the available literature and protocols for neurosurgical practice in different geographic locations. We drafted a consensus statement based on the literature and protocols suggested by the World Health Organization (WHO) and various professional societies to prevent the spread of SARS-COV2 while streamlining the neurosurgical practice. Results: The consensus statement suggests the patient triage, workflow, resource distribution, and operational efficacy for care providers at different stages of management. The priority is set at personal protection while ensuring patients' safety, timely management, and capacity building. We performed a detailed subsection analysis for the management of trauma and set up for COVID-free hospitals for simultaneous management of routine neurosurgical indications. In this time of medicolegal upheaval, special consent from the patients should be taken in view of the chances of delay in management and the added risk of corona infection. The consensus statements are applicable to neurosurgical setups of all capacities. Conclusion: Along with the glaring problem of infection, there is another threat of neurosurgery emergency building up. This wave may overwhelm the already stretched systems to the hilt. We need to flatten this curve while avoiding contagion. These measures may guide neurosurgery practitioners to effectively manage patients ensuring the safety of caregivers and care seekers both.

Background: The COVID-19 pandemic has compelled countries to impose lockdowns to curb the spread. As a result of the lockdown and need for health care services to cater to acute diseases on priority, patients with chronic illnesses such as Parkinson's disease (PD) may be facing several difficulties. Aims: This study aimed to explore the effects of prolongation of lockdown on patients with PD by evaluating possible problems faced during a lockdown and worsening of symptoms if any. Materials and Methods: One hundred patients with PD and their caregivers were contacted. Results: We observed a significant increase in problems faced due to this pandemic, specifically, the inability to access health care, and difficulty procuring medication. Patients also reported worsening of motor symptoms. Conclusions: The present findings highlight the need for health care systems to consider a plan of action for chronic neurological diseases like PD, which are worsening in the absence of regular hospital visits.

Background and Introduction: Donning and doffing of personal protective equipments (PPE) has become relevant especially during COVID-19 pandemic and neurosurgeons operating upon COVID-19 positive or suspect patients should be aware of proper technique of donning and doffing of PPE.^[1] Surgeries involving direct exposure of anterior nasal spaces/paranasal sinuses carry significantly more risk of infection and it may be prudent to use PPE while operating all such cases.^[2] Objective: In this video, we present our extensive protocol of donning and doffing of PPE which we have devised for our operating room. Technique: Donning consists of wearing of the PPE in a proper sequence so as to afford maximal protection from viral infection while conducting the surgical procedure. Various components of PPE and procedure of donning is shown followed by doffing, the sequential and safe removal of the PPE. Results: A meticulous method of donning and doffing PPE for neurosurgeons handling COVID-19 positive / suspect cases has been shown . Conclusion: Proper sequence of donning and doffing of PPE gear is of crucial importance during the COVID pandemic to prevent infection to the health care workers while handling COVID-19 positive/suspect cases and this video demonstrates the protocol we use at our institute.

Background and Introduction: Presence of multiple aneurysms, especially a combination of anterior and posterior circulation aneurysm in the same patient, is rare. Surgical clipping of both the aneurysms in the same sitting, although ideal, may be surgically challenging and requires a good preoperative planning. Objective: In this video abstract, we present a case of a ruptured middle cerebral artery (MCA) aneurysm with a simultaneous occurrence of an unruptured basilar top aneurysm. Surgical Technique: A 45-year-old female presented with modified Hunt and Hess grade II subarachnoid hemorrhage (SAH). CT scan showed diffuse SAH with a dense bleed in the left sylvian cistern and mild hydrocephalus. Angiography showed a left MCA aneurysm (34 mm size) with a bleb and also an un-ruptured basilar bifurcation/right posterior cerebral artery aneurysm (20 mm). The patient underwent a left frontotemporal craniotomy, zygomatic osteotomy, and clipping of both aneurysms. No temporary clips were applied. The extended craniotomy allowed the surgeon to reach to the base of the aneurysm. Results: The patient had an uneventful recovery. Conclusions: Simultaneous occurrence of both anterior and posterior circulation aneurysms are rare, but maybe tackled surgically with proper planning.

Background: Low grade gliomas (LGG) are most often noted with the unpredictable overall survival and progression to higher grades. Objective: In the present study, we analyze the clinicopathological features influencing the prognostic outcomes and compared the features with criteria developed by EORTC. Materials and Methods: We observed the 130 LGG clinical cases in single institute and maintained the follow-up for more than 5 years. In addition, the molecular details were confirmed with markers as IDH, 1p/19q codeletion, p53 and ATRX mutations. Results: The mean age of patients as 37.67 years and male population contributing to 70%. We observed biased incidence among the male population with dominating occurrence at frontal and parietal lobes in the brain. 40.8% patients had oligodendroglioma, 33.8% astrocytoma, 19.2% oligoastrocytoma and 2.3% gemistocytic astrocytoma pathology. Patients who were subjected to chemotherapy and radiotherapy were noted with average survival of 29 months. Oligodendroglial tumors were found with progression free survival (PFS) of 25 months, oligoastrocytoma cases with 32 months, diffuse astrocytoma cases with 23 months while the gemistocytic astrocytoma cases had 22 months. The PFS for LGG cases was 4.7 years while the overall survival was 4.9 years. Mean survival of patients with KPS score <70 and >70 was 1.5 & 4.9 years respectively. 64 patients were observed with the tumor size >5 cm. In total, 72.3% of the patients were underwent GTR, 23.3% STR and 3.8% underwent biopsy. Conclusion: Taken together, the clinical symptoms, expression of molecular markers and the prognosis details provided by our results can help for better management of LGG cases. We further propose to use following five factors to accurately describe the prognosis and tumor recurrence: 1) Age >50 years, 2) tumor size >5 cm, 3) MIB index >5%, 4) KPS score < 70 and 5) gemistocytic pathology.

Context: Endovascular therapy is currently the most common treatment approach for intracranial dural arteriovenous fistula (DAVF), followed by microsurgery. Gamma Knife radiosurgery (GKS) is usually reserved as the last modality of treatment of intracranial DAVF. Aim: To evaluate the clinical and radiological outcome of GKS in the treatment of DAVF without CVD. Subjects and Methods: This series includes patients who underwent GKS for intracranial DAVF without CVD over 10 years (Jan 2007 to Dec 2016) in All India Institute of Medical Sciences, New Delhi. Their demographic profile, clinical presentation, imaging details, GKS details, and follow-up clinical status were obtained retrospectively. Clinical follow-up, along with radiological assessment using MRI every 6 months was done after GKS. DSA was performed once MRI strongly suggested obliteration of DAVF. Patients who had a clinical follow-up of less than 1 year were excluded from the study. Results: 5 patients (4 males and 1 female) who had DAVF without CVD were included the study. The mean age was 44.8 years. All patients had complete obliteration of fistula on digital subtraction angiography (DSA) at a mean duration of 24 months post GKS. All patients had complete resolution of symptoms at the last follow-up. Conclusions: Gamma Knife surgery is the most effective and the safest treatment modality for dealing with DAVFs without CVD. Instead of reserving it as the last resort for patients with DAVF without CVD, it should be considered as the gold standard treatment for DAVFs without CVD.

Background and Purpose: Although imaging is the mainstay to differentiate ischemic stroke (IS) from intracerebral hemorrhage (ICH), these facilities are not available everywhere. The present study observed if any blood biomarker(s) could potentially help differentiate between ischemic stroke and intracerebral hemorrhage. Methods: 250 patients with acute stroke within 24 hours of onset (187 IS and 63 patients with ICH) were recruited in the present study. The blood samples were collected closest to the hospital presentation time, but within 24 hours of stroke onset. Blood was analyzed for five biomarkers [S100, glial fibrillary acidic protein (GFAP), N-methyl-D-aspartate receptor subunit antibody (NR2), interleukin 6 (IL6) and brain natriuretic peptide (BNP)] to assess discriminatory ability of each biomarker to differentiate ICH and IS. Results: S100 levels were statistically higher among patients with ICH compared with IS (8 pg/ml versus 4.2 pg/ml respectively, P = 0.003) and IL6 was higher in patients with IS compared with ICH (12.9 pg/ml vs 8.76 pg/ml, P = 0.02). The discriminatory ability to differentiate ICH from IS was better using a combination of the above two biomarkers. The overall discriminatory ability of all biomarkers were low (Area under curve for S100 65%; GFAP 56%; NR2 53%; IL6 59% and BNP 49.8%). Although the positive predictive value of each biomarker was low, the negative predictive value was higher for all biomarkers to diagnose ICH. Conclusions: S100 and IL6 are potential biomarkers for further study and validation. Newer biomarkers with higher discriminatory ability are required in the future for diagnostic use.

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.

Background: The pathway underlying ocular convergence is less clear. Convergence weakness is usually a clinical sign of dorsal midbrain pathology. An orbital tumor causing such phenomenon is unknown. We describe its occurrence in patients with proptosis due to orbital tumors. Materials and Methods: Series of six patients with axial proptosis secondary to orbital tumors that demonstrated varying forms of convergence weakness (deficiency to complete failure). Results: Three had weakness of convergence with drifting away of proptosed eyeball on attempted near vision, while the other three had failure. One of these had convergence weakness in nonproptosed eye. MRI excluded a central etiology in all. Conclusions: The article highlights an interesting phenomenon of deficient convergence in the presence of intact medial rectus function in patients with proptosis. The symmetry of orbital contents possibly plays a significant role in the process of convergence.

Aims: The study was done to review the literature about the intriguing aspects of the aneurysmal bone cyst and to describe our experience with these cases Design: Retrospective. Material and Method: We reviewed the records of all patients with primary spinal tumours whom we managed over last 8 years. We selected the patients with biopsy proven aneurysmal bone cyst (ABC) for our study Results: Four patients (two males and two females) were included. The age ranged from 15-18 years. Three of them had neurological deficits and one had only pain. All of them were operated and three required instrumentation for stabilization. Neurological deficits improved in all the cases. However we had to re- operate one of the cases for recurrence and that patient was administered adjuvant radiotherapy. Conclusions: ABC is not a tumour in real sense but due to destructive nature that are classified as tumours. The patients have an excellent outcome as the disease is benign and has very low recurrence rates if surgical excision is complete. Best treatment modality is complete excision. Aneurysmal bone cyst is one of the uncommon tumors of the spine, and many of its features continue to be unclear even today. There is ambiguity about the definition, etiopathogenesis, radiological characteristics, histopathology and treatment modalities. They are common in young age and etiology is not clear. The presentation is varied with pain being the common symptom and neurological deficit depends on extent of cord involvement. The best treatment is controversial although surgery is believed to be curative in the majority of cases. We here describe our experience with four such cases who had varied clinical presentation and outcome.

Background: The association between appendectomy and multiple sclerosis (MS) is unknown. In this study, we explored the association between appendectomy and MS and neuromyelitis optica spectrum disorder (NMOSD). Patients and Methods: MS and NMOSD patients older than 40 were identified from neurology records from hospitals in Malaysia. The diagnoses were based on the Revised McDonald (2010) and Wingerchuk (2015) criteria. Controls were sampled from Malaysia's normal population. Individuals were interviewed telephonically or face-to-face. The age inclusion criterion (over 40) differentiated high or low lifetime risk of appendicitis, as appendicitis incidence is rare after 40. Results: 49 MS, 71 NMOSD, and 880 controls met the inclusion criteria. Seventy-two individuals (9 MS, 4 NMOSD, 59 control) had undergone appendectomy. Appendectomy rates were 18.37% in the MS group (95% CI 7.5–29.2%), 5.6% in the NMOSD group (0.3%, 11%), and 6.7% among controls (5.1%, 8.4%), (MS vs NMOSD P = 0.036, MS vs controls P = 0.007). Binary regression analysis showed that MS was an independent risk factor for appendectomy (OR 2.938, 95% CI 1.302, 6.633, P = 0.009). NMOSD showed no association with appendectomy. Conclusion: MS is positively associated with appendectomy, unlike ulcerative colitis, which is negatively associated. We hypothesize that there is a commonality in the microflora in persons who have had these two illnesses.

Background: Epilepsy is a treatable and curable brain disorder. However major proportion of individuals with this disease in developing countries receives no treatment because of misunderstandings of the public. Other than that, poor adherence to ordered medication is considered the primary cause of drug therapy failure in epilepsy. Nonadherence, therefore, results directly in a rise in health care costs and compromised quality of life. Aims: To assess the adherence pattern to antiepileptic regimen, among patients with epilepsy and to identify the clinical and patient-related factors associated with the adherence pattern to antiepileptic regimen. Methods: A cross-sectional survey design was used in 100 epilepsy patients. A consecutive sampling technique was used to enrol patients who meet inclusion criteria. Structured interview from a pre-tested questionnaire and medical records review was done to collect the data. Descriptive and inferential statistics were used for the analysis of data. Descriptive statistics (mean, standard deviation, frequency and percentages) were used to describe the clinical and demographic variables of study participants. The determinants of medication adherence were analyzed using Chi-Square test. Results: Majority (71%) of patients were not adherent to antiepileptic treatment. The severity of seizure (indicated by the presence of seizure last year; P = 0.007), medication frequency (p = 0.001) and complexity of treatment (p = 0.003) were found to have a significant association with the AED adherence status. Status of adherence is significantly associated with frequency of seizure/year and positive life style (P=0.0001). Conclusion: As Medication adherence was observed to be low, services for adherence counselling and health educational interventions in the epilepsy clinics is recommended.

Background: The people living with epilepsy (PWE) have a higher prevalence of sexual dysfunction (sexual dysfunction) as compared to the general population. About 20-66% of the PWE develop sexual dysfunction. In spite of being so common, it is still an underdiagnosed co-morbidity in epilepsy patients. Purpose: To estimate the proportion of sexual dysfunction among PWE, and determine the associated demographic and clinical factors. Material and Methods: This cross-sectional study was conducted at an Epilepsy clinic of a tertiary care hospital located in South India, from March 2017 to May 2017. The PWE satisfying the inclusion criteria were given Changes in Sexual Functioning Questionnaire (CSFQ), Patient Health Questionnaire 9 (PHQ-9) and Generalized Anxiety Disorder-7 questionnaire (GAD-7). The demographic and clinical details were recorded. We estimated the proportion of sexual dysfunction, depression and anxiety and other factors associated with sexual dysfunction. Results: After screening 3225 PWE, 108 patients were recruited. Sixtyfive (60.2%) PWE had sexual dysfunction, 64 (59.3%) had depression and 63 (58.3%) had anxiety. The sexual dysfunction had a significant association with depression (P = 0.01) and anxiety (0.04). Patients receiving sodium valproate had significantly lower rates of sexual dysfunction (P = 0.007). Other factors like seizure type, seizure frequency, enzyme inducer drugs and poly-therapy were not associated with Sexual dysfunction. Conclusions: We found Sexual dysfunction in 60% of the PWE in our setting. The proportion of depression and anxiety was 59.3% and 58.3% respectively. The depression, anxiety, and low-valproate use were significantly associated with sexual dysfunction.

Background: There is an ongoing research on the etiology of multiple sclerosis (MS). It is still unclear whether nutritional status and biochemical parameters such as serum 25(OH)D, magnesium, and potassium influence the development of disease. Aims: This study aimed to make contributions to the literature in terms of the recognition of MS by comparing nutritional status and biochemical information of people with and without MS. Materials and Methods: The study was designed as a controlled descriptive study. Total of 112 individuals were included (control group, n = 56 and MS group, n = 56). Socioeconomic and demographic characteristics, nutritional status, and biochemical information were collected from the participants. A decision tree model was built to evaluate the impact of these parameters on the presence of MS. The parameters were compared using Student's t tests and Mann–Whitney U tests. Results: A decision tree model having an accuracy rate of 86.52% was constructed. Strong statistical differences were observed among the vitamin and mineral intakes of the groups. In terms of biochemical parameters, especially for serum levels of 25(OH)D and potassium, the differences were significantly different (P < 0.001). Conclusions: Constructed decision tree indicated that the main parameters differed between an MS patient and a healthy person were as follows: serum levels of 25(OH)D, magnesium, calcium, and intakes of potassium and carbohydrate. Based on the findings of this study, nutritional precautions might be taken against MS.

Background: Patients with retroviral disease are prone to opportunistic infections (OIs) of the central nervous system which cause significant mortality and morbidity. Cryptococcosis, tuberculosis, and toxoplasma are the most commonneuroinfections occurring at all stages of the disease. Objective: This study was undertaken to evaluate the clinical pattern of OIs and in-hospital mortality in patients with acquired immunodeficiency syndrome (AIDS). Materials and Methods: This retrospective analysis was conducted in a teaching hospital from 2001 to2014. Clinical data, laboratory investigations, and outcome of patients with meningoencephalitis were obtainedfrom case records. Results: The total number of patients with human immunodeficiency virus (HIV) and AIDS with neurological manifestations was 277, among whom 167 (60.3%) had meningoencephalitis. There were 131 malesand 36 femaleswith a male-to-female ratio of 3.63:1andage ranging from 16 to 67 years (39.25 ± 8.85 years). Clinical presentation was acute in 58%, subacute in 32%, and chronic in 10%. Symptoms includedheadache (85.8%), fever (65.7%), altered sensorium (37.1%), and seizures (25.1%). Cryptococcal meningitis was the most commoninfection (67) followed by tuberculosis (32), mixed meningitis (24), and toxoplasmosis (10), and 16 patients had progressive multifocal leucoencephalopathy. Pathogenic organism could not be identified in 18 patients. Fifteen patients died during hospital admission. Mortality was the highest in mixed meningitis (16.7%) followed by cryptococcal meningitis (10.4%). Opportunistic neuroinfection occurred as AIDS-defining illness in 59.3%. Prior use of highly active anti-retroviral therapy did not affect the outcome. Conclusion: Opportunistic neuroinfections are the most commonneurological manifestation in patients with AIDS, with cryptococcal meningitis being the most commonopportunistic neuroinfection occurring as AIDS-defining illness in one-third of the patients with neuro-AIDS.

Introduction: Brain retractor is an indispensable instrument in Neurosurgery craniotomy set. Gooseneck tubing is an engineering marvel and has many applications (Reading lamp, Microphone, Tablet/phone holder, etc). We present a brain retractor made with an ultra-light duty Gooseneck tubing and report its use in five craniotomies for various pathologies. Methods and Results: The traditional flexible brain retractor (Leyla) consists of three principal parts: a flxation base, a flexible arm or arms, and a blade holder. We replaced the flexible arm by an ultra-light duty Gooseneck tubing in our new retractor. The retractor proved very useful in the five cranial surgeries done and no complications were noted during its use. Conclusion: Gooseneck flexible Brain Retractor is a safe and cheaper alternative to the presently available brain retractor system. It has several advantages compared to traditional retractor. The efficacy and supremacy over traditional retractor systems need to be confirmed by further studies.

Spinal muscular atrophy (SMA) encompasses a group of disorders with loss of spinal motor neurons.The report describes the neuropathological findings including brain and spinal cord at autopsy in a five-and-half-month-old boy with suspected type 1 SMA. The anterior motor neurons, Clarke's column at all the levels of spinal cord showed neuronal loss and degeneration while neurons at all the deep grey nuclei were preserved apart from variable degree anoxic changes. Skeletal muscle biopsy revealed features of neurogenic atrophy consistent with SMA. A differential diagnosis like storage disorders was excluded using electron microscopy. No extra-neural manifestations were seen. Neuropathological features at autopsy have seldom been reported in the literature.

Neurosyphilis is a rare disease. We describe two patients of neurosyphilis with their clinical course and long-term follow-up. Our first patient was a 47-year-old male who presented with ataxia, headache, papilledema, sensorineural hearing loss, and myelopathy. Investigations revealed pachymeningitis and cervicodorsal myelitis. Cerebrospinal fluid (CSF) was reactive with positive CSF Venereal Disease Research Laboratory (VDRL) and blood Treponema pallidum hemagglutination (TPHA). His clinical, laboratory, radiological findings, and follow-up of the last 10 years are discussed with serial imaging. Case 2 was a 61-year-old male, who presented with neuropsychiatric symptoms, which resolved with treatment. We have reviewed the Indian case reports of this disease. It is easy to confuse neurosyphilis with tubercular meningitis in an Indian setting. The role of steroids in myelitic form has also been discussed. Worldwide reported cases of syphilitic myelitis are tabulated with their outcomes.

Superficial siderosis is a rare disease resulting from the deposited hemosiderin owing to repeated subarachnoid hemorrhage. It has been reported that hemosiderin deposits on the brain surface and brain parenchyma causes nerve disorder, resulting in progressive and irreversible hearing loss, cerebellar ataxia and pyramidal disorder. The brain tumor is one of the cause of superficial siderosis. A 16-year-old female present a nearby hospital with complaints of absence seizure. A magnetic resonance imaging (MRI) revealed a heterogeneously enhanced mass at the right temporal lobe. Susceptibility-weighted imaging revealed diffuse and extensive superficial siderosis on the brain surface. The tumor was gross totally removed and diagnosed as glioneuronal tumor. The patient had been well, although susceptibility-weighted imaging performed one year after the surgery showed superficial siderosis remained. Early diagnosis and prevention of bleeding sources are recommended to prevent symptom progression associated with superficial siderosis. Susceptibility-weighted imaging is considered useful for early detection of superficial siderosis.

Cerebral hyperperfusion syndrome is a well-recognised phenomenon following carotid revascularisation. It is defined as cerebral blood flow increase of more than 100% of the baseline. A similar phenomenon can occur in the eye and maybe termed as ocular hyperperfusion syndrome. We present a 65-year-old male who developed an ipsilateral red eye with visual loss following carotid artery stenting. There was a past history of recurrent right middle cereberal artery (MCA) territory embolic infarcts and recurrent trasient episodes of vision loss in the right eye. Flow reversal was noted in the ophthalmic artery on Transcranial doppler (TCD). Digital subtraction angiography (DSA) showed more than 95% stenosis in right internal carotid artery (ICA) ostium and completely occluded left ICA. Following carotid artery, stenting patient developed severe headache and right eye pain along with vision loss despite intensive blood pressure monitoring and control. NCCT head showed mild right cortical SAH and the intra-ocular pressure (IOP) in the right eye was high. It was hypopthesised that aqueous over production due to neovascularity secondary to chronic ocular ischemia, lack of outflow and sudden change in ocular hemodynamics post stenting was the pathogenic mechanism. The patient was commenced on measures to reduce aqueous production along with strict blood pressure control. Prestenting evalvation for chronic ocular ischemia with tanscranial dopplar and angiographic flow reversal in ophthalmic artery, fluorescein angiography to look for watershed zones and slit lamp for neovascularity and angle closure can help in identifing high-risk patients, particularly in patients with bilateral carotid artery disease.

A peripheral palsy of the facial nerve that results in muscle weakness on one side of the face usually manifests as Bell's palsy. Glioma in the left half of the pons and middle cerebellar peduncle is a rare cause of isolated infranuclear facial paralysis. We report a case of 12- year-old female patient who came to our hospital with isolated unilateral facial palsy but turned out to have a low grade glioma.

Subdural hematoma is a rare complication after lumbar spine surgeries. Most of the time, the reason for this complication is intracranial hypotension related to an unintended durotomy, and the main symptom is persistent orthostatic headache. The authors present the case of a-38-year-old woman who underwent lumbar discectomy. Three weeks after the surgery, she developed subdural hematoma related to dural tear during the procedure. Emergent hematoma evacuation with craniotomy and dural tear repair was performed simultaneously because of the herniation symptoms of the patient. Clinical findings, radiological assessments, and treatment modalities have been discussed in the presence of a literature review in this case report.

Scedosporium apiospermum is a filamentous fungus causing a broad spectrum of clinical diseases especially in those who are immunocompromised. The common sites involved are lungs, skin, sinuses, eyes, bones, joints, and central nervous system (CNS). CNS is involved in invasive Scedosporiosis in the form of a cerebral abscess. An antecedent event of either near-drowning or history of some trauma is present in the majority of the cases where the patients' immune response remains intact. Prognosis is generally poor since the majority of the patients have coexistent medical morbidity. Surgical drainage followed by adjuvant antifungal, i.e., voriconazole therapy offers the best possible chance for survival in these patients. This case report discusses a rare event of brain abscess caused by S. apiospermum in an immunocompetent patient without any preceding precipitating factor.

Although levetiracetam is the antiepileptic of choice in patients after hepatic transplantation and patients with hepatic dysfunction, we report a patient in whom levetiracetam was the most probable cause of hepatic dysfunction. Treatment of this hepatic dysfunction is to have a high degree of suspicion and withdraw the drug at the earliest to prevent morbidity and rarely mortality. Though rare, this is an important unwanted side effect of this highly useful medication.

Acute disseminated encephalomyelitis (ADEM) has been linked to immunizations, viral infections, and occasionally to arthropods stings. Its association with scorpion stings has not been described. A 29-year-old man, who after being stung by a scorpion presented right hemiparesis and language impairment. Brain MRI showed subcortical bihemispheric lesion characteristics of ADEM and a bigger one compatible with Baló concentric sclerosis (BCS). Patient was managed with steroids, showing complete clinical improvement. Magnetic resonance imaging (MRI) after 6 months showed cavitation of the bigger lesion with no new findings. In 2 years of follow-up, there is no clinical relapse or new lesions in MRI. Although cases of ADEM have been reported due to venom of chilopoda and hymenoptera, the scorpion sting can be considered as another new cause; this case being even more relevant due to present a Baló-like lesion in MRI.

Lyme disease is a multi-organ infectious disease caused by the spirochete “Borrelia burgdorferi,” and transmitted by the “Ixodes” tick. Early disseminated Lyme disease can have varied central nervous system manifestations ranging from meningitis to radiculopathy and cranial neuropathy. If not suspected, misdiagnosis or delayed diagnosis can prove to be fatal. Erythema migrans is the most common clinical presentation, thereby, making dermatological examination extremely crucial in early diagnosis and treatment.

Cutis laxa is a set of genetically heterogeneous conditions with phenotypes ranging from progeria-like appearance, corneal clouding, clenched fingers with marked retardation of growth both pre and postnatal growth to very mild phenotypes with skin laxity becoming evident in 2^nd or 3^rd decade. A child who presents with predominant motor delay is written off with a clinical diagnosis of rickets in the absence of any clinical sign of lax skin. Here, we report a 2-year-old child who presented with motor delay and joint hyperlaxity. Mutation analysis demonstrated a heterozygous mutationc.G1867A in the exon 15 of ALDH18A1 gene known to cause autosomal dominant cutis laxa.

Despite being common, polyneuropathy remains a diagnostic challenge for most clinicians. Mononeuritis multiplex (MM) refers to involvement of several or many peripheral nerves at the same or different points in time by a disease process. This report describes a case of an atypical presentation of Hansen's disease (HD) as mononeuritis multiplex in the left lower limb with corresponding radiographic, electrodiagnostic, and histopathological data that confirmed pure neuritic leprosy (PNL). We reiterate that although the incidence of PNL is exceedingly low characterized by nerve involvement without the characteristic cutaneous stigmata, leprosy is still the commonest cause of MM in the Indian sub-continent.This report underscores the crucial need for a heightened multi-disciplinary awareness of this “forgotten and uncommon” presentation of PNL. It is imperative that the treating physician should also understand the various neurological presentations, both mimics and chameleons, of this treatable disease to prevent permanent neuropathic injury and disability.

Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal and rapidly progressive form of dementia caused by the spread of a prion protein within the brain. Its real incidence is unknown since its definitive diagnosis requires histopathological analysis of brain specimens. However, novel tests that detect prion proteins in cerebrospinal fluid samples, such as the real-time quaking-induced conversion (RT-QuIC) technique, now allow the pre-mortem diagnosis of sCJD. Here, we report the first case of sCJD confirmed by RT-QuIC in Latin America, providing evidence of its diagnostic performance and clinical correlation.

Congenital portosystemic shunt (CPSS) is a rare disorder characterized by a diversion of Porto-mesenteric blood into systemic veins. Type I is an end to side fistula between the portal vein and the inferior vena cava. Type II is a side to side fistula between the main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. Clinical presentation of these patients varies. Treatment and management are guided by the type of malformation and clinical presentation. Herein, we present a case of CPSS Type IIb with neurological symptoms, treated with endovascular occlusion with 6-year follow-up without remission.