Annals of Indian Academy of Neurology
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   Table of Contents - Current issue
July-August 2020
Volume 23 | Issue 4
Page Nos. 425-577

Online since Friday, August 21, 2020

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Nonmotor symptoms in dystonia: Pathophysiological implications p. 425
Anumeha Mishra, Sanjay Pandey
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Bridging the gap in epilepsy treatment in a resource-limited setting p. 427
Ravish R Keni, Sanjeev V Thomas
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Non-motor symptoms in cervical dystonia: A concept in evolution p. 428
Daniele Urso, Valentina Leta, K Ray Chaudhuri
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Conduct of virtual neurology DM final examination during COVID-19 pandemic p. 429
Roopa Rajan, Divya M Radhakrishnan, Achal K Srivastava, Venugopalan Y Vishnu, Anu Gupta, Ahamadulla Shariff, MV Padma Srivastava
Medical training programs are witnessing immense disruptions worldwide due to the ongoing COVID-19 pandemic. Keeping in mind the trainees' future prospects, it is important to provide continuity of teaching and timely certification assessments. Overcoming the obstacles to routine functioning presented by SARS-CoV-2 spread, we recently conducted the DM Neurology exit examination in a hybrid virtual format. We created a curated case repository with history and clinical examination findings followed by structured questions that could be built upon for case discussions. The external examiners assessed the candidates virtually through a video conferencing platform. The end results were well accepted by all key stake holders. The concerns, logistics and experience of conducting the DM Neurology exit exam in a virtual format are summarized here.
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Neurology and COVID-19: Acting now. Preparing for future p. 433
Annu Aggarwal, Tanu Singhal, Mohit Bhatt
COVID-19 has a wide-ranging and multimodal neurological impact. First, several neurological symptoms and complications are commonly observed in patients with COVID-19. Second, medications and vaccinations used to counter the disease can have secondary neurological effects. Third, patients with pre-existing neurological disorders bear an increased health-risk due to COVID-19. And finally, the pandemic has disrupted the delivery of neurological and vaccination services, and associated educational and research programs. In this article we review the various channels through which the pandemic is known or projected to effect individual patients or the practice of neurology. We also provide recommendations to manage its immediate effects and prepare for the longer-term fall-out.
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Spinal tuberculosis treatment: An enduring bone of contention p. 441
Divyani Garg, Vinay Goyal
Spinal tuberculosis is the most common form of extrapulmonary tuberculosis. It is of great importance to neurologists because of the potentially devastating complication of paraplegia, which may set in during active disease or the healed phase. Due to the deep-seated nature of the disease, definitive diagnosis is often challenging. There is no clear consensus on the appropriate duration of therapy for spinal tuberculosis, with various guidelines recommending treatment from as short as 6 months to up to 18 months. In this article, we present a critical appraisal of the evidence on the same. In our opinion, the duration of antitubercular therapy needs to be individualized and the decision to terminate therapy should be multifactorial (clinical, radiological, pathological/microbiological where possible) rather than being enmeshed within any particular guideline.
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Non-motor symptoms in cervical dystonia: A review p. 449
Somdattaa Ray, Pramod Kumar Pal, Ravi Yadav
Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive movements, postures, or both. Dystonic movements are typically patterned, associated with twisting of body parts, and may have tremulousness. Dystonia is usually initiated or worsened by voluntary action and associated with overflow muscle activation. Cervical dystonia (CD) is the most prevalent form of dystonia. CD is a condition characterized by cranial muscle overactivity leading to abnormal intermittent or continuous posturing of the head. Non-motor symptoms are comorbidity of dystonia, which significantly hampers the quality of life among these patients. The symptoms can be as a result of the dystonia itself. However, studies have highlighted the involvement of cortical-striatal-thalamocortical circuits in primary dystonia that could be the pathophysiological basis for the non-motor symptoms. The non-motor symptoms that are commonly associated with dystonia are anxiety, depression, restless leg syndrome, excessive daytime sleepiness, cognitive disturbances, and poor sleep. This review attempts to summarize the literature on non-motor symptoms in patients with CD.
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What is new in idiopathic inflammatory myopathies: Mechanisms and therapies p. 458
Satish V Khadilkar, Megha C Dhamne
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders that cause muscle weakness and also have extramuscular manifestations involving various organ systems; namely the lung, skin, heart, and joints. Previously classified broadly as dermatomyositis (DM) and polymyositis now the spectrum of the disease has evolved into more clinical subtypes. There are now five clinicoserological subtypes recognized worldwide DM, antisynthetase syndrome (AS), overlap myositis (OM), immune mediated necrotizing myopathy (IMNM), and inclusion body myositis. Each of these subtypes has a unique phenotype and specific antibodies associated. With the evolving treatment options from the use of immunosuppressive medications to the use of targeted therapy with biologic agents, and further understanding of the pathogenesis of inflammatory myositis, we may have more effective treatment options. We discuss in this review, various myositis-associated antibodies associated with each clinicoserological subtype of IIM and their role. We also describe the evolving therapies and the evidence for the newer biologic therapies in the treatment of IIMs.
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Neurological complications with COVID-19: A contemporaneous review p. 468
Boby Varkey Maramattom, Siddharth Bhattacharjee
The COVID-19 pandemic is raging across the world, affecting 212 Countries and Territories around the world. It has infected more than 3.7 million people with a mortality rate of around 7%. Although the causative virus, the SARS-CoV-2 is primarily a respiratory pathogen, recent observational studies have documented a high rate of neurological complications associated with COVID-19. We searched PubMed databases from December 01, 2019 to June 9, 2020 for articles published on “COVID 19” OR “coronavirus” with targeted search words. We also search preprint servers for neurological complications of COVID-19. Neurological manifestations are seen in around 36%–45% of patients with COVID-19 and can involve almost every part of the central nervous system (CNS) from the hemispheres, cranial or peripheral nerves, spinal cord, and muscle. The mechanisms vary from direct viral invasion of the CNS, to a dysregulated host immune response to molecular mimicry to multiorgan dysfunction. In many patients, neurological manifestations preceded other systemic features or the diagnosis of COVID-19. Sick patients with COVID-19 will require ICU care and many patients may present first to the neurocritical care ICU and receive a diagnosis of COVID-19 later. Hence, it is important for all healthcare personnel to be aware of the myriad neurological manifestations of this infection, so as to initiate appropriate infection control practices and refine investigation and treatment protocols.
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Clinical profile and outcome of patients with cerebral venous thrombosis secondary to bacterial infections p. 477
Manna S Jacob, Karthik Gunasekaran, Angel T Miraclin, Mohammad Sadiq, C Vignesh Kumar, Ajoy Oommen, Maria Koshy, Ajay Kumar Mishra, Ramya Iyadurai
Background: Cerebral venous thrombosis (CVT) secondary to infectious aetiology has become rare in the antibiotic era, but is still encountered in clinical practice occasionally. In this study, we describe the clinical profile, diagnosis, and management of patients with CVT secondary to an infectious aetiology. Methods: This retrospective study included all adult patients over 15 years (1 January 2002 to 1 January 2017). Adult patients with a diagnosis of infective CVT secondary to bacterial infections were included in the study. Results: Totally, 22 patients were identified with CVT complicating bacterial infections. The focus of infection in 12 (54.54%) patients was pyogenic meningitis, 9 (40.9%) patients had a parameningeal focus and one patient developed CVT secondary to bacterial sepsis from a remote focus. Fever was the most common symptom seen in 77.3% followed by headache and depressed sensorium in 72.7% and 63.6%, respectively. The most common organism in the meningitis group was Streptococcus species, and in the parameningeal group was Staphylococcus aureus. At presentation MRI identified CVT in all 7 patients as compared to CT brain with contrast in 2/3 (66.6%). Transverse sinus was the most commonly involved sinus in meningitis. All patients were treated with appropriate antibiotics and anticoagulation was used in 50% of the patients. The in hospital, mortaility was 9%. Conclusion: Septic CVT, though rare can be a complication of bacterial meningitis and facial infections. Clinical symptoms that suggest a co-existing CVT should be identified and diagnosed at the earliest. The mainstay of treatment is antibiotics; the role of anticoagulation is controversial.
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Neurological symptoms as initial manifestation of Covid-19 – An observational study p. 482
Rishu Garg, Rahul Jain, Ajoy Sodani, Dinesh Chouksey, Ravi Dosi, Sunil Athale, Nitisha Goyal, Pankaj Rathi, Hashash Singh, Kapil Telang
Background: Respiratory system involvement and fever are considered as a cardinal manifestation of Covid-19 infection for the screening of case detection. We (India) are into the fourth month of Covid-19 and cases are still rising, this could mean that fever and respiratory symptoms may not be the only initial symptoms. Therefore, we intend to investigate whether neurological symptoms can precede the cardinal symptoms. Methods: Totally, 391 Covid-19 RTPCR positive hospitalized patients were enrolled. All included subjects were presented with a questionnaire pertaining to systemic symptoms. For analysis of the chronology of symptoms, the study population was sub-grouped according to onset of their systemic involvement e.g., (1) Fever (2) Respiratory symptoms (3) Neurological symptoms (4) Gastrointestinal symptoms. Results: New-onset neurological symptoms were found in 106 (27.1%) out of 391 patients irrespective of their chronology to the onset of other symptoms. Of these 106 patients, altered taste (33.1%), altered smell (24.5%), and headache (22.6%) were the most common neurological symptoms. However, 38 (9.7%) subjects recognized neurological symptoms, as the initial manifestation of their illness. Mean duration of neurological symptoms before the onset of respiratory symptoms or fever was 2 ± 1.57 days. Conclusion: New-onset headache, altered taste, and smell were the most common neurological symptoms. In the context of the current pandemic, a high index of suspicion should be kept in patients presenting with these symptoms even in the absence of fever and respiratory symptoms. To the best of our knowledge, this is the first study from India comparing chronology of neurological symptoms with cardinal symptoms.
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Depression and quality of life after cerebral venous sinus thrombosis Highly accessed article p. 487
Aralikatte O Saroja, Ninad N Thorat, Karkal R Naik
Introduction: Cerebral venous sinus thrombosis (CVST) is an important cause of stroke in young and has a favorable outcome. Long-term sequelae of CVST include motor disability, cognitive impairment, depression, anxiety, fatigue, impaired employment and poor quality of life. Objective: To evaluate depression and quality of life after CVST. Methods: Patients who completed at least 1 year after discharge were recruited for this cross-sectional observational study from our CVST cohort. Quality of life was assessed using Stroke-Adapted Sickness Impact Profile (SA-SIP 30) and depression using Hamilton Depression scale (HAM-D). Results: A total of 100 patients (60 men and 40 women) were included in the study. Their age ranged from 14 to 60 years (34.97 ± 10.06). The interval from discharge to assessment of quality of life was 2.2 ± 1.6 years. In all, 98% of patients had good modified Rankin score at follow-up. SA-SIP 30 did not reveal any functional disability for physical functioning. Seven had impairment for psychosocial domain despite having good modified Rankin scores. Thirty patients had depression. Patients with higher mRS at discharge had increased presence of depression. Quality-of-life scores did not correlate with presence of seizure, headache, infarction and sinuses involved. Conclusion: This is the first Indian study demonstrating depression in patients with CVST and use of SA-SIP to assess quality of life in them. Occurrence of depression in CVST is as high as in arterial strokes.
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Interaction between methylenetetrahydrofolate reductase (MTHFR) gene polymorphisms and environment with susceptibility to ischemic stroke in Chinese population p. 491
Xing-Zhen Zheng, Xiao-Lin Bian, Zhe-Hong Sun, Hai-Dong Wang
Aims: To investigate the association of several single-nucleotide polymorphisms (SNPs) within methylenetetrahydrofolate reductase (MTHFR) gene, and additional gene–environment interaction with ischemic stroke (IS) risk. Methods: Testing for Hardy–Weinberg equilibrium in controls was conducted using SNPstats (online software: Generalized multifactor dimensionality reduction (GMDR) was used to screen the best interaction combination among four SNPs within MTHFR gene and smoking or alcohol drinking. Results: The frequency of the rs4846049-A allele was 28.6% in IS patients and 19.1% in normal controls, in addition, the frequency of the rs3737967-T allele was 27.9% in IS patients and 20.3% in normal controls, which was also indicating a statistically significant difference. The rs4846049-A and rs3737967-T were associated with an increased risk of IS risk; adjusted odds ratios (ORs) (95% confidence interval [CI]) were 1.76 (1.28–2.13) and 1.51 (1.13–1.97), respectively. GMDR model found significant gene–alcohol drinking interaction combination, but no significant gene–tobacco smoking interaction combinations. In order to obtain the odds ratios and 95% CI for the joint effects of gene–alcohol drinking on IS, we conducted stratified analysis for interaction effect using logistic regression. We found that alcohol drinkers with rs4846049-CA/AA genotype also have the highest IS risk, compared with never drinkers with rs4846049-CC genotype, OR (95% CI) = 3.12 (1.83–4.45), after adjustment for age, smoke, and smoking status. Conclusions: The rs4846049-A and rs3737967-T, gene–environment interaction between rs1764391 and rs918592, gene–environment interaction between rs4846049 and alcohol drinking were all associated with increased IS risk.
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Plasma biomarkers as potential predictors of functional dependence in daily life activities after ischemic stroke: A single center study Highly accessed article p. 496
Adina Hutanu, Mihaela Iancu, Smaranda Maier, Rodica Balasa, Minodora Dobreanu
Objective: Despite advances made in the treatment of ischemic stroke, it still remains one of the leading causes of mortality and disability worldwide. The main objective of this study was to identify from a panel of 10 inflammatory markers and chemokines those biomarkers that have a potential predictive role in the evolution of disability and functional dependence in daily activities after an ischemic stroke. Methods: The study included 116 patients with ischemic stroke and 40 healthy volunteers matched for gender and age. Stroke severity was assessed by the National Institute of Health Stroke Scale (NIHSS) on admission and during hospitalization and functional mobility in daily activities by Barthel index (BI). Multiplex panel with 10 biomarkers [brain-derived neurotrophic factor (BDNF), platelet-derived growth factor (PDGF)-AA, PDGF-AB/BB, neural cell adhesion molecule (NCAM), cathepsin D, soluble vascular cell adhesion molecule (sVCAM), soluble intercellular cell adhesion molecule (sICAM), myeloperoxidase (MPO), regulated on activation, normal T cell expressed and secreted (RANTES), plasminogen activator inhibitor (PAI)-1] was analyzed on days 1 and 5 after admission using the xMAP technology. Results: Plasma concentrations of RANTES and NCAM were significantly lower in patients with ischemic stroke compared with healthy controls, while MPO and sICAM were significantly higher in patients versus controls. Plasma concentrations of sICAM, sVCAM, and RANTES significantly decreased during the analyzed period. For the first-day measurement, the bivariate analysis revealed the association of NIHSS on admission with sVCAM, and on discharge negative association with PDGF-AA, PDGR-AB/BB, BDNF, and RANTES. Plasma levels of PDGF-AA, PDGF-AB/BB, BDNF, and RANTES were found to be significantly lower in patients with BI ≤ 80, on day 5 after disease onset. PDGF-AA, PDGF-AB/BB, and BDNF were univariate and multivariate predictors for functional dependence in daily life activity (BI ≤ 80), having a protective effect (odds ratio < 1). Conclusion: Plasma levels of BDNF, PDGF-AA, and PDGF-AB/BB are independent predictors for functional dependency in daily life activities and may be useful prognostic markers in the evaluation of ischemic stroke patients.
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Association of APOE gene polymorphism with stroke patients from rural Eastern India p. 504
Hilal Ahmad Ganaie, Arindam Biswas, Annada Prasad Bhattacharya, Sandip Pal, Jharna Ray, Shymal Kumar Das
Context: Studies from the different ethnic regions of the world have reported variable results on association of APOE gene polymorphism in stroke. Aim: The aim of this study is to find out the possible association of APOE polymorphism in stroke patients in ethnic Bengali population. Settings and Design: A prospective case–control study was undertaken in the Department of Neurology, Burdwan Medical College, Burdwan, West Bengal, India, over a period of 3 years. Methods: We collected 10 ml venous blood samples from 148 clinically and radiologically diagnosed acute stroke patients (80 of ischemic stroke and 68 of intracerebral hemorrhage) and consecutive 108 ethnic age- and sex-matched controls, in ethylenediaminetetraacetic acid vials after informed written consent. Genomic DNA was prepared at S.N. Pradhan Centre of Neurosciences, University of Calcutta, Kolkata, India. Exotic single-nucleotide polymorphisms (rs429358, rs 7412) were analyzed by polymerase chain reaction-restriction fragment length polymorphism for genotype of APOE. Results: The frequencies of different APOE allele among 80 ischemic stroke patients were 5.6% (n = 9) for E2, 75.68% (n = 121) for E3, and 18.7% (n = 30) for E4. The E3 allele is significantly over-represented (P = 0.004) in controls compared to the patients (88% in controls vs 75.6% ischemic stroke patients and 80% hemorrhagic patients). A significantly high frequency of APOE4 allele was observed in ischemic (18.7%) and hemorrhagic patients (11%) compared to controls (8%). The E4 allele plays a major risk for developing ischemic stroke [odds ratio (OR) = 2.744; 95% confidence interval (CI): 1.43–5.10] and E3 plays a protective role for hemorrhagic stroke (OR = 0.53; 95% CI: 0.29–0.96), while E4 allele plays a nonsignificant (P = 0.31) increase in trend in hemorrhage stroke (OR = 1.4). Conclusions: There is significant association of APOE gene polymorphism in stroke patients of ethnic Bengali population. The E4 allele increases significant risk for development of ischemic strokes, and it also plays nonsignificant increase in trend in hemorrhagic strokes.
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The impact of the Coronavirus Disease-2019 pandemic on the psychological status and quality of life of myasthenia gravis patients p. 510
Aleksandar Stojanov, Jelena Stojanov, Vuk Milosevic, Marina Malobabic, Goran Stanojevic, Milos Stevic, Gordana Djordjevic
Objectives: The coronavirus disease 2019 (COVID-19) is the largest pandemic of our times. We wanted to investigate the impact of COVID-19 pandemic on the psychological status, quality of life (QoL) and quality of sleep (QoS) of myasthenia gravis (MG) patients. Methods: Data on the epidemiological and clinical characteristics of MG were collected. We used a self-designed questionnaire (consisting of 12 questions), a revised 15-item Myasthenia Gravis Quality of Life Questionnaire (MGQOL15r), a 36-item health survey of the Medical Outcomes Study Short Form (SF36), Pittsburgh sleep quality index (PSQI), Hamilton scales for the assessment of anxiety (HAM-A), and depression (HAMD) were used. We reassessed patients who were tested three years ago using the same questionnaires. Results: The study included 64 MG patients. We noticed a statistically significant difference between the results obtained three years ago and the results from April 2020 in PSQI scores (P < 0.01). MGQOL15r, SF36, and PSQI scores correlate with severe clinical manifestation, high scores on HAM-A and HAM-D (P < 0.01). Higher scores on HAM-D and fear that MG symptoms will be worse if the patient gets an upper respiratory infection were independent predictors of the lower SF36 scores. Regarding MGQOL15r-independent predictors of the higher score were higher scores on HAM-D. Conclusions: There is a significant impact of the COVID-19 epidemic on the psychological status and especially on the quality of sleep of MG patients. Healthcare organizations need to provide professional therapeutic advice and psychosocial support for this population of patients during the pandemic.
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Anemia as a predictor of functional disability in the early stage of ischemic stroke in a South Asian Population p. 515
Thashi Chang, Praveen Weeratunga, Thamal Vithanage, Piumi Wijewickrama, Sithara Kularathne, Sachie Fernando, Carukshi Arambepola
Background: Reduced hemoglobin concentration has an adverse impact on the ischemic penumbra in patients with ischemic stroke as it causes reduced oxygen delivery to neuronal tissue and predisposes to infarct expansion. There is a paucity of data on the impact of anemia on early functional outcomes. Aims: To determine the association of anemia on early functional outcomes in a cohort of patients with ischemic stroke. Methods: This prospective study was conducted among 190 participants with acute ischemic stroke presenting to the National Hospital of Sri Lanka. Data were collected on socio-demographic determinants, clinical presentation, co-morbidities, subtype of stroke, and stroke severity (NIHSS score). Early functional outcomes were assessed by the Modified-Rankin-Score (mRS) and Barthel index (BI) within 48 h of the onset. Anemia was defined as Hb <13 g/dl in males and <12 g/dl in females. Results: The mean age of the population was 62.4 years (SD = 11.8). Most participants (75.8%) were males. Anemia was noted in 56.4% of the total study population (59.0% males; 56.5% females) with a mean Hb of 11.7 g/dl. A total of 20% of patients had moderate to severe stroke severity as defined by an NIHSS of ≥16. Functional status assessment revealed that 67.9% had mRS <3 and 85.8% had BI <75. Furthermore, 85.8% had a composite MRS <3 and/or BI 75. Univariate analysis revealed that anemia was significantly associated with “moderate-severe” functional disability. On logistic regression analyses, this retained significance when the functional disability was assessed by mRS >3 (adjusted OR = 2.36; 95% CI = 1.1–5.1). Receiver operator characteristics (ROC) curves indicated a Hb% of 10.65 g/dl as the cut-off that would predict stroke-related disability assessed by mRS >3 [sensitivity = 86.7%; specificity = 34.2%; and AUC = 0.659 (P < 0.0001)]. Conclusions: Anemia is an independent determinant of poor functional disability in early acute ischemic stroke.
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Cerebral venous thrombosis, seasonal trends, and climatic influence: A region-specificstudy p. 522
Sanjith Aaron, Jeyaseelan Lakshmanan, Thambu D Sudarsanam, Kenneth Benjamin, Jothilakshmi Durairaj, Vivek Mathew, Ajith Sivadasan, AT Prabhakar, Shyam Kumar N. Keshava, Pavitra R Mannam, Prabhu Kirubakaran, Jayaprakash Muliyil, Mathew Alexander
Background and Purpose: Studies looking at seasonal variation on cerebral venous thrombosis (CVT) are few with conflicting conclusions. In this region-specific study, we looked for climatic influence and seasonal trends on the incidence of CVT. Methods: Imaging proven adult CVT cases treated over a period of 18 years from a specific geographical location with similar seasons and climatic conditions were studied. Metrological parameters prepared using 30 years of data was used. Quantum geographical information system (QGIS software) and SPSS v 22 were used for patient plotting and analysis. Results: Total of 970 cases were studied. The incidence was significantly higher in summer 411 (42.3%) compared with autumn 317 (32.7%) and winter 242 (25.05); P = 0.038. This trend was consistent across all the 18 years in time series analysis. Mean age was 33.5 years (range 18–88 years). A significant majority 673 (69.4%) were below 40 years of age; P = 0.012. Females constituted 394 (40.6%) of cases. Postpartum CVT cases constituted 237 (30%). Interaction analysis showed younger age (<40 years) were more vulnerable for CVT in summer; P = 0.009. There was no seasonal influence on postpartum CVT. Apart for a weak positive correlation between rain fall (r = 0.18, P < 0.01); humidity and cloud cover was not influencing the incidence. Conclusions: Higher ambient temperatures were consistently associated with higher incidence of CVT. This is the largest region-specific study on CVT in the world. These results may be applicable to other regions with similar climatic conditions.
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Clinical spectrum and outcome of neurosarcoidosis: A retrospective cohort study from a teaching hospital in India p. 528
Arun Mathai Mani, AT Prabhakar, Pavithra Mannam, Rohit Ninan Benjamin, Atif Iqbal Ahmed Shaikh, Donna Mathew, Pankaj Singh, Aditya Nair, PT Alexander, Asish Vijayaraghavan, Ajith Sivadasan, Sunithi Mani, Vivek Mathew, Sanjith Aaron, Mathew Alexander
Context: Neurosarcoidosis (NS) is a chronic disease with a diverse clinical spectrum, therapeutic response, and outcome. There is scarce literature from our country regarding the same. Aims: The aim of this study was to evaluate the clinical spectrum, therapeutic responses, and outcomes of NS in an Indian cohort. Settings and Design: In a cross-sectional study, we included all patients with NS treated at a quaternary care teaching hospital in India from January 2007 to October 2019. Subjects and Methods: Patients older than 18 years of age fulfilling the diagnostic criteria for NS from the Neurosarcoidosis Consortium Consensus Group were included in the study. The therapeutic response and the degree of disability at last follow-up were assessed. Results: We identified 48 patients, among them 3 were categorized as having definite NS, 30 probable NS, and 15 possible NS. Cranial neuropathy was the most common presentation (47.9%), followed by myelopathy (25%). Systemic involvement was identified in 95.83% and mediastinal lymph nodes were the most common site. Clinical improvement was seen in 65.8% and disease stabilized in 28.9%, while 5.26% deteriorated. Fifty percent recovered without any residual disability, while 26.3% had minor and 23.7% had major residual sequelae. Conclusions: NS is a diverse illness, with a heterogeneous spectrum of clinical presentation, treatment response, and outcome. Cranial neuropathy is the most common presenting feature and has a good prognosis while myelopathy has an unfavorable prognosis. Meningeal and brain parenchymal disease is difficult to diagnose accurately unless systemic involvement is present. The diagnosis of NS should be clinically suspected in the appropriate clinical setting, the presence of systemic involvement should be investigated, and histologic confirmation should be attempted.
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High-pressure normocephalus-raised intracranial pressure with false localizing signs Highly accessed article p. 536
Arunmozhimaran Elavarasi, Deepa Dash, Pankaj Kumar Singh, Manjari Tripathi
Normal-sized ventricles and absence of papilledema do not rule out shunt failure and raised intracranial pressure (ICP). Raised ICP can present with false localizing signs which may be cranial nerve palsies or extensive polyradiculopathy. Our patient with a history of ventriculoperitoneal (VP) shunt presented with rapidly progressive vision loss without papilledema, as well as multiple cranial nerve palsies and radiculopathy. Imaging did not reveal hydrocephalus, however, cerebrospinal fluid (CSF) manometry revealed high CSF opening pressure. After lumbar thecoperitoneal shunting, vision did not improve, but the rest of cranial nerve palsies and radiculopathy improved. In a patient in whom VP shunt is in situ, headache and vomiting should prompt evaluation for raised ICP though there is no ventriculomegaly of papilledema. Vision can be saved if raised ICP is suspected, CSF opening pressure measured at presentation and prompt surgery is performed.
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Synaptic nuclear envelope Protein 1 (SYNE 1) ataxia with amyotrophic lateral sclerosis-like presentation: A novel synaptic nuclear envelope Protein 1 (SYNE 1) gene deletion mutation from India Highly accessed article p. 539
Swaleha Nurulla Nadaf, Rahul T Chakor, Kaumil V Kothari, Ashraf U Mannan
A 24-year-old female presented with wasting and weakness of both hands and fasciculations over the chin since 12 years, followed by imbalance while walking and speech changes since 10 years. Her 12-year-old sister also had a similar clinical presentation. There were fasciculations over the chin, tongue, hands, back, thighs with wasting and weakness in tongue, and C7, C8, T1 segments in both upper limbs along with bipyramidal signs. There was limb and gait ataxia. Magnetic resonance imaging brain showed pancerebellar atrophy, and electromyography was suggestive of anterior horn cell involvement in bulbar, cervical, thoracic, and lumbar segments. Next-generation sequencing identified a novel likely pathogenic deletion mutation: chr6:152527389_152527399del, c.22711_22721del, and p.Ala7571ArgfsTer4 in exon 125 of synaptic nuclear envelope protein 1 (SYNE1) gene. This mutation leads to frameshift and premature termination of the protein 'Nesprin 1'. Amyotrophic lateral sclerosis-like presentation followed by cerebellar ataxia have been described with SYNE1 ataxia. This unique phenotype and novel deletion mutation of SYNE1 gene is the first case reported from India.
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Toxic leukoencephalopathy due to inhalational heroin abuse Highly accessed article p. 542
Samir Kashyap, Gohar Majeed, Ira Bowen, Yancey Beamer, Dan Miulli
Heroin-induced spongiform leukoencephalopathy (HSLE) is a rare condition that is strongly associated with heroin vapor inhalation which has become a popular method among heroin addicts because it poses a less immediate danger to the user and makes the drug much easier to use. We present a case of a 22-year-old male who presented with dysarthria and cerebellar symptoms starting, after 3 months of heroin inhalation. Diagnosis was confirmed to be HSLE after extensive diagnostic testing. HSLE is a rare complication of which the pathogenesis is poorly understood. Clinical history and characteristic findings on magnetic resonance imaging (diffuse, symmetric T2-hyperintensity, and diffusion restriction in frontal, parietal, occipital lobs, basal ganglia, and superior cerebellum) are diagnostic; however, care should be taken to exclude other etiologies. Treatment is primarily supportive; however, there is anecdotal evidence that coenzyme Q10 may be of benefit. The growing number of victims of the opioid crisis requires that physicians be aware of and counsel patients on the devastating neurological complications that can occur with abuse of these drugs.
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Persistent craniopharyngeal canal: A rare cause for recurrent meningitis in pediatric population Highly accessed article p. 545
Lokesh Lingappa, Ramesh Konanki, Ravi Varma, Nikit Shah, Subodh Raju, Sukumar Sura, Leenatha Reddy, Sirisha Rani
We present the case of a 5-year-old girl who had six episodes of meningitis. She also had panhypopituitarism and was found to have a persistent craniopharyngeal canal (CPC) as the cause of her recurrent meningitis. Role of neuroradiology and a high index of suspicion by the clinical team are highlighted here. Persistent CPC is a rare cause of recurrent meningitis. We discuss the approach to the child with recurrent meningitis.
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Wernekinck syndrome (Image/Video in Neurology) p. 549
S Deepak Amalnath, Satarla Narendra, DK S. Subrahmanyam
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Myosonography: An easy and painless technique to detect tongue fasciculations p. 550
Y Muralidhar Reddy, ESS Kiran, Shyam K Jaiswal, Lalitha Pidaparthi, JMK Murthy
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“Pool sign” in cerebral metastatic adenocarcinoma p. 551
Ajith Cherian, KP Divya, Pranab K Prabhakaran, Jithu Jose, Poornima Narayanan, Bejoy Thomas
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Why does large venous pouch thrombose after cerebral arteriovenous shunts embolization? p. 553
Jidian Sun, Xianli Lv
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Posterior reversible encephalopathy syndrome in thrombotic thrombocytopenic purpura p. 555
Vivek Bhat, Sanjukta S Rao, Chaitanya Balakrishnan
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Cocaine-induced vascular changes on intracranial vessel wall imaging: Vasculitis or vasculopathy? p. 557
Neetu Soni, Thomas H Locke, Edgar A Samaniego, Girish Bathla
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CARASIL; The backache, baldness, brain attack syndrome: The Indian scenario p. 559
Sagar Badachi, Saji K John, Mithun Sekhar, Thomas Mathew
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Vertebral pseudo-occlusion due to acute basilar artery thrombosis p. 561
Álvaro Lambea-Gil, Herbert Tejada-Meza, Diego Rodríguez-Gascón, Beatriz Domínguez-Lagranja
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Young male with paraparesis and vision loss- A rare presentation of Eales' Disease p. 563
Tanushree Chawla, Anu Gupta, Kamakshi Dhamija, Debashish Chowdhury
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Isolated cortical vein thrombus presenting with subdural hematoma p. 566
Rithvik Ramesh, Philo Hazeena, TP Javed, Shankar Venkatasubramanian
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“Calcified clot march” after intravenous thrombolysis p. 568
Vivek Agarwal, Neha Choudhary, Sameer Vyas, Ajay Kumar, Manoj Goyal
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CADASIL and cavernomas: A common mechanism p. 570
Radhakrishna Pedapati, Venugopalan Y Vishnu, Mamta B Singh, Vinay Goyal, Ajay Garg, Padma Srivastava
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Stent-assisted coiling of brain aneurysms under conscious sedation and simultaneous coronary heart disease stenting: A new concept p. 572
Jianing Xiong, Lei Wang, Jian Tian, Yingqiang Li, Cuiling Gong, Xianli Lv
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Neuropsychiatric symptoms and caregiver burden in Parkinson's Disease: Mitigating the lack of awareness! p. 575
Devavrat Nene, Ravi Yadav
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Parkinson's Disease in India: From clinic to bench p. 577
Louis C S. Tan, Abhijeet K Kohat
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