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Annals of Indian Academy of Neurology
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   Table of Contents - Current issue
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July-August 2021
Volume 24 | Issue 4
Page Nos. 467-643

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EDITORIAL  

Inspired by Prof Ashok Panagaria, 'Man in Merc' Highly accessed article p. 467
Vinay Goyal, Chanchal Goyal
DOI:10.4103/aian.aian_786_21  
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EDITORIAL COMMENTARY Top

Evaluation of multiple system atrophy subtypes with FDG-PET Highly accessed article p. 468
Kurt Jellinger
DOI:10.4103/aian.AIAN_54_21  
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Commentary on END-IT score p. 469
Pradeep Pankajakshan Nair
DOI:10.4103/aian.aian_237_21  
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AIAN REVIEW Top

Emerging role of the ketogenic dietary therapies beyond epilepsy in child neurology p. 470
Dipti Kapoor, Divyani Garg, Suvasini Sharma
DOI:10.4103/aian.AIAN_20_21  
Ketogenic dietary therapies (KDTs) have been in use for refractory paediatric epilepsy for a century now. Over time, KDTs themselves have undergone various modifications to improve tolerability and clinical feasibility, including the Modified Atkins diet (MAD), medium chain triglyceride (MCT) diet and the low glycaemic index treatment (LGIT). Animal and observational studies indicate numerous benefits of KDTs in paediatric neurological conditions apart from their evident benefits in childhood intractable epilepsy, including neurodevelopmental disorders such as autism spectrum disorder, rarer neurogenetic conditions such as Rett syndrome, Fragile X syndrome and Kabuki syndrome, neurodegenerative conditions such as Pelizaeus-Merzbacher disease, and other conditions such as stroke and migraine. A large proportion of the evidence is derived from individual case reports, case series and some small clinical trials, emphasising the vast scope for research in this avenue. The term 'neuroketotherapeutics' has been coined recently to encompass the rapid strides in this field. In the 100th year of its use for paediatric epilepsy, this review covers the role of the KDTs in non-epilepsy neurological conditions among children.
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Critical appraisal of a machine learning paper: A guide for the neurologist p. 481
Pulikottil W Vinny, Rahul Garg, MV Padma Srivastava, Vivek Lal, Venugoapalan Y Vishnu
DOI:10.4103/aian.AIAN_1120_20  
Machine learning (ML), a form of artificial intelligence (AI), is being increasingly employed in neurology. Reported performance metrics often match or exceed the efficiency of average clinicians. The neurologist is easily baffled by the underlying concepts and terminologies associated with ML studies. The superlative performance metrics of ML algorithms often hide the opaque nature of its inner workings. Questions regarding ML model's interpretability and reproducibility of its results in real-world scenarios, need emphasis. Given an abundance of time and information, the expert clinician should be able to deliver comparable predictions to ML models, a useful benchmark while evaluating its performance. Predictive performance metrics of ML models should not be confused with causal inference between its input and output. ML and clinical gestalt should compete in a randomized controlled trial before they can complement each other for screening, triaging, providing second opinions and modifying treatment.
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ORIGINAL ARTICLES Top

Comprehensive functional evaluation of the spectrum of multi-system atrophy with 18F-FDG PET/CT and 99mTc TRODAT-1 SPECT: 5 Year's experience from a tertiary care center p. 490
Nikhil Seniaray, Ritu Verma, Rajeev Ranjan, Ethel Belho, Harsh Mahajan
DOI:10.4103/aian.AIAN_1222_20  
Aim: To elucidate the patterns of characteristic hypometabolism on 18F- fluorodeoxyglucose( 18F-FDG) positron emission tomography/computed tomography (PET/CT) in multisystem atrophy (MSA) and their correlation with the patterns of uptake on dopamine transporter imaging with 99mTc TRODAT-1 SPECT. Material and Methods: A retrospective analysis of 67 patients with clinically diagnosed MSA was performed. All the subjects underwent 99mTc TRODAT-1 SPECT and 18F-FDG PET/CT scanning on two separate days. The 99mTc-TRODAT-1 scans were analyzed visually for asymmetry and rostro-caudal gradient. The FDG uptake patterns were recorded, and areas of hypometabolism that were two standard deviations from the mean were considered abnormal. Results: All the subjects had an abnormal pattern of FDG uptake on PET scan, both on a visual inspection and semiquantitative analysis. In MSA-P subjects (n = 29), diffuse predominant hypometabolism of the globus pallidus-putamen complex was noted, with relative sparing of the caudate nuclei. In MSA-C subjects (n = 25), characteristic hypometabolism was noted in the cerebellum and brainstem. In mixed subtypes (n = 13), variable involvement of the basal ganglia, cerebellum, and brainstem was noted with frontoparietal hypometabolism. A statistically significant difference between MSA-P and MSA-C for gradient reduction and asymmetry with gradient reduction was observed. Conclusion: Dopamine transporter imaging with 99mTc TRODAT-1 SPECT not only helps in confirmation of parkinsonian disorders but also demonstrates varying patterns of distribution in different subtypes of MSA. Characteristic patterns of hypometabolism in 18F-FDG PET may help in the differentiation of the subtypes of MSA in the presence of clinically overlapping symptoms.
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Role of interictal arterial spin labeling magnetic resonance perfusion in mesial temporal lobe epilepsy p. 495
Ashima Mittal, Devinder Pal Singh Dhanota, Kavita Saggar, Gagandeep Singh, Archana Ahluwalia
DOI:10.4103/aian.AIAN_1274_20  
Context: Electrophysiological and hemodynamic data can be integrated to accurately identify the generators of abnormal electrical activity in drug-resistant focal epilepsy. Arterial Spin Labeling (ASL), a magnetic resonance imaging (MRI) technique for quantitative noninvasive measurement of cerebral blood flow (CBF), can provide a direct measure of variations in cerebral perfusion associated with the epileptogenic zone. Aims: 1. To evaluate usefulness of ASL for detecting interictal temporal hypoperfusion to localize the epileptogenic zone in patients of drug resistant mesial temporal lobe epilepsy (MTLE). 2. Correlation of localization of epileptogenic zone on ASL MR perfusion with structural MRI and EEG. Methods and Materials: 30 patients with MTLE and10 age and gender matched normal controls were studied. All patients underwent ictal video EEG monitoring non-invasively, MR imaging with epilepsy protocol and pseudocontinuous ASL (PCASL) perfusion study. Relative CBF (rCBF) values in bilateral mesial temporal lobes were measured utilizing quantitative analysis of perfusion images. A perfusion asymmetry index (AI) was calculated for each region. Results: In patients, ipsilateral mesial temporal rCBF was significantly decreased compared with contralateral mesial temporal rCBF (p = 0.021). Mesial temporal blood flow was more asymmetric in patients than in normal control participants (p = 0.000). Clear perfusion asymmetry on PCASL-MRI was identified despite normal structural-MRI in 5 cases, agreeing with EEG laterality. Conclusions: Pseudo-continuous ASL offers a promising approach to detect interictal hypoperfusion in TLE and as a clinical alternative to SPECT and PET due to non-invasiveness and easy accessibility. Incorporation of ASL into routine pre-surgical evaluation protocols can help to localize epileptogenic zone in surgical candidates.
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Medication adherence in Indian epilepsy patients p. 501
Shrawan Kumar, Mamta Bhushan Singh, Amit Kumar, MV Padma Srivastava, Vinay Goyal
DOI:10.4103/aian.AIAN_925_20  
Purpose: While two-thirds of epilepsy patients can become seizure free on medical treatment, poor adherence to medication is a major problem to sustained remission and functional restoration. The aim of this study was to assess the prevalence and associated factors of antiepileptic drug (AED) non-adherence. Methods: We conducted a subgroup analysis based on results that emerged from a single center, cross-sectional study. Patients who were 18 years or older were included. The 4-item Morisky Medication Adherence Scale was used to measure adherence to AED (s). Multivariable logistic regression analysis was used to predict factors associated with AED non-adherence. Results: A total of 268 patients fulfilled inclusion criteria and were included in this subgroup analysis. Among the participants, 81 (30%) were non-adherent to medication. Three factors associated with non-adherence were AED polytherapy [OR: 4.5 (2.1-9.5) P = 0.001], drug related adverse events [OR: 3.9 (2.1-7.3) P = 0.001], and treatment duration exceeding 3 years [OR: 2.6 (1.3-5.0) P = 0.003]. Conclusion: About one-third patients were not compliant with their medication. If the treatment of patients is restricted to monotherapy as far as possible and patients are educated about duration of treatment and possible adverse effects of AEDs, non-adherence may be reduced.
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Alteration of mitochondrial function in oxidative stress in parkinsonian neurodegeneration: A cross-sectional study p. 506
Rizwana Qadri, Vinay Goyal, Madhuri Behari, Arulselvi Subramanian, Sudip Kumar Datta, Asok Kumar Mukhopadhyay
DOI:10.4103/aian.AIAN_392_20  
Context: Appropriate mitochondrial function and oxidative balance are critical to neuronal survival. Accumulation of reactive oxygen species leads to oxidative stress that can cause free radical damage to biomolecules of the cell components and the molecules in the cellular milieu that eventually lead to a variety of chronic diseases including neurodegenerative disorders. Mitochondrial dysfunction initiates neuronal apoptosis thereby leading to neurodegenerative diseases including Parkinson's disease (PD). Aim: To evaluate oxidative stress vis-a-vis mitochondrial function (Cytochrome C oxidase activity) in PD patients, Parkinson plus syndrome (PPS) patients in comparison with healthy controls (HCs). Settings and Design: Cross-sectional Study Methods: We assessed oxidative stress by chemiluminescence using luminol, and cytochrome c oxidase activity (CCO) by CCO kit using spectrophotometry in PD patients (n = 80), PPS patients (n = 40), and HCs (n = 40). Statistical Analysis: Data were presented as number (%) or mean ± SD/median as approximate. Quantitative baseline variables were compared among the groups using one-way ANOVA and qualitative variables were compared using Chi-square test. The difference in median was compared using Kruskal–Wallis test followed by Post-hoc Bonferronni correction. Results: Compared to HCs (Median 7.53 ± 15.58 RLU/sec/cell), ROS level in PD (14.13 ± 29.5), and PPS (17.43 ± 15.91) patients was significantly higher (P = 0.0029: HC vs, PD & P = 0.0500: HC vs. PPS). Also, ROS in PD patients (14.13 ± 29.5) was higher that PPS patients (17. 43 ± 15.91) but the difference was not statistically significant (P = 0.84). The CCO activity was found to be diminished in PD (Median: 0.025 ± 0.013 units/ml) and PPS patients (0.027 ± 0.008) in comparison to HCs (0.117 ± 0.049). Conclusion: Mitochondrial dysfunction and oxidative stress is associated with PD and PPS and may play an important role in etiopathogenesis. Though the cause–effect conundrum has not been comprehensively probed but addressing oxidative stress and mitochondrial damage may serve as an adjunctive therapy for PD and PPS. Iron metabolism as reflected in the red cell indices may aid in differentiating PD from PPS.
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Clinical evaluation of decompressive craniectomy in malignant middle cerebral artery infarction using 3d area and volume calculations p. 513
Serkan Kitis, Serdar Çevik, Kevser B Köse, Arzu Baygül, Serhat Cömert, Ülkün Ü Ünsal, Meliha G Papaker
DOI:10.4103/aian.AIAN_518_20  
Objective: We aimed to measure the craniectomy area using three-dimensional (3D) anatomic area and volume calculations to demonstrate that it can be an effective criterion for evaluating survival and functional outcomes of patients with malignant middle cerebral artery (MCA) infarction. Material and Methods: The patients diagnosed with malignant ischemic stroke between 2013 and 2018, for which they underwent surgery due to deterioration in their neurological function, were retrospectively reviewed. Radiological images of all patients were evaluated; total brain tissue volume, ischemic brain tissue volume, total calvarial bone area, and decompression bone area were measured using 3D anatomical area and volume calculations. Results: In total, 45 patients (27 males and 18 females) had been treated with decompressive craniectomy (DC). The removed bone area was found to be significantly related to the outcome in patients with MCA infarction. The average decompression bone area and mean bone removal rate for patients who died after DC were 112 ± 27 cm2 and 20%, whereas these values for surviving patients were 149 ± 29 cm2 and 26% (P = 0.001), respectively. At the 6-month follow-up, the average decompression bone area and mean bone removal rate for patients with severe disability were 126 ± 30 cm2 and 22.2%, whereas these values for patients without severe disability were 159 cm2 ± 26 and 28.4% (P = 0.001), respectively. Conclusion: In patients with malignant MCA infarction, the decompression area is associated with favorable functional outcomes, first, survival and second, 6-month modified Rankin scale score distribution after craniectomy.
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Anteroposterior stability: A determinant of gait dysfunction and falls in spinocerebellar ataxia p. 518
VS Ganapathy, Tittu T James, Mariamma Philip, Nitish Kamble, Amitabh Bhattacharya, Pradnya Dhargave, Pramod Kumar Pal
DOI:10.4103/aian.AIAN_1090_20  
Background: Establishing an association between gait variability and direction specific balance indices may help in identifying the risk of falls in patients with spinocerebellar ataxia (SCA) which may help in developing an appropriate intervention. This study is intended to identify the association between balance and gait parameters especially gait variability in these patients. Methods: Patients with genetically confirmed SCA (n = 24) as well as controls (n = 24) who met the study criteria were recruited. Gait was assessed using the GAITRite system and balance was assessed using dynamic posturography (Biodex) to record direction-specific dynamic balance indices. Disease severity was assessed using international cooperative ataxia rating scale (ICARS). Results: The mean age of the SCA group (38.83 ± 13.03 years) and the control group (36.38 ± 9.09 years) were comparable. The age of onset of illness was 32 ± 10.62 years and duration of 5.67 ± 3.62 years. The mean ICARS was 45.10 ± 16.75. There was a significant difference in the overall balance index (OBI), anterior–posterior index (API), medial/lateral index (MLI) between SCA patients (4.56 ± 2.09, 3.49 ± 1.88, 2.94 ± 1.32) and the controls (2.72 ± 1.25, 2.08 ± 0.85, 1.85 ± 0.97). However, correlation was observed only between gait stability and balance parameters in API direction. Conclusions: There was an increased anteroposterior oriented balance deficit in patients with SCA, which was significantly correlating with the gait parameters. The balance training intervention may focus on improving anteroposterior direction to prevent falls and improving walking efficiency.
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High-resolution vessel wall imaging in primary angiitis of central nervous system p. 524
Soumya Sundaram, P Naveen Kumar, Dev Prakash Sharma, Chandrasekharan Kesavadas, Sapna Erat Sreedharan, B Arun Prasad, PN Sylaja
DOI:10.4103/aian.AIAN_106_21  
Background: High-resolution vessel wall imaging (HRVWI) can aid in differentiating the various intracranial vasculopathies, but has been sparingly used in the diagnosis of primary angiitis of central nervous system (PACNS). This study is aimed to describe the vessel wall imaging characteristics of PACNS. Materials and Methods: Patients with confirmed diagnosis of PACNS according to the Calabrese and Mallek criteria who had abnormal HRVWI were included in this retrospective descriptive study. Magnetic resonance image of brain, conventional four-vessel cerebral digital subtraction angiogram, and HRVWI were read by a neuroradiologist. The vessel wall parameters assessed were T1W and T2W appearances, pattern of wall thickening and contrast enhancement, and remodeling index. Results: HRVWI done in 21 patients with PACNS yielded abnormality in 20 (95.2%) who were included in the analysis. The mean age at presentation was 42.55 ± 9.48 years and 14 (70%) were males. The median number of vessels involved were four (range 2–12). The commonest vessels affected were proximal middle cerebral artery (70%) and internal carotid artery (55%). Vessel wall thickening was concentric, eccentric, and absent in 12 (60%), 1 (5%), and 7 (35%) patients, respectively. Vessel wall enhancement was diffuse in 17 (85%), eccentric in 1 (5%), and absent in 2 (10%) patients. One patient had T2W hyperintense stenotic lesion. Remodeling index was negative in 11 (55%) patients. Conclusion: Distinctive vessel wall appearances were observed by HRVWI in PACNS, concentric vessel wall thickening and enhancement being more frequent. Hence, HRVWI can be considered as an additional noninvasive imaging modality in the diagnosis of PACNS.
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Electrophysiological evaluation of audiovestibular pathway dysfunction in parkinson's disease and its correlates: A case control study p. 531
Nikith Ampar, Anish Mehta, Rohan R Mahale, Mahendra Javali, R Pradeep, Purushottam Acharya, Rangasetty Srinivasa
DOI:10.4103/aian.AIAN_1011_20  
Background: Parkinson's disease (PD) is associated with brainstem dysfunction causing non-motor symptoms. Vestibular evoked myogenic potential (VEMP) and brainstem auditory evoked potential (BAEP) are electrophysiological tests to assess the vestibular and auditory pathways in the brainstem. Objectives: To study the abnormalities of cervical VEMP (cVEMP) and BAEP in PD and to correlate the findings with the symptoms related to brainstem involvement. Patients and Methods: cVEMP and BAEP were recorded in 25 PD patients and compared 25 age matched controls. The PD patients were assessed with the following clinical scales: REM Sleep Disorder Screening Questionnaire (RBD-SQ), Epworth Sleepiness Scale (ESS), mini-BESTest, Geriatric Depression Scale (GDS-15) and MMSE (Mini-mental state examination). The P13 and N23 peak latencies and the P13/N23 amplitude of cVEMP, the latencies of waves I, III and V, and the inter-peak latencies (IPL) of waves I-III, III-V and I-V of BAEP were measured. Results: The PD patients showed prolonged latencies and reduced amplitude in cVEMP responses. They had abnormal BAEP in the form of prolonged absolute latencies of wave V, followed by wave III and I–V IPL with no significant difference in waves I and I–III IPL. The cVEMP abnormality was correlated directly with RBD-SQ and inversely with mini-BESTest scores. There were no correlations between cVEMP/BAEP abnormality and disease severity, GDS-15, ESS and MMSE. Conclusion: PD is associated with cVEMP and BAEP abnormalities that suggest auditory and vestibular pathway dysfunction in the brainstem and cVEMP correlates with the symptoms of brainstem degeneration like RBD and postural instability.
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Teleneurorehabilitation among person with parkinson's disease in India: The initial experience and barriers to implementation p. 536
Divyani Garg, Ritu Majumdar, Sonal Chauhan, Ravi Preenja, Jasmine Parihar, Alvee Saluja, Rajinder K Dhamija
DOI:10.4103/aian.AIAN_127_21  
Objective: We aimed to assess the feasibility of teleneurorehabilitation (TNR) among persons with Parkinson's disease (PD), considering difficulties imposed by the COVID-19 pandemic in access to healthcare, particularly in low-resource settings. The feasibility of TNR in India has not been formally assessed so far. Methods: We conducted a single-center, prospective cohort study at a tertiary center in India. Persons with PD with Hoehn & Yahr (H&Y) stages 1-2.5, who were not enrolled into any formal exercise program, were offered TNR as per a predesigned program for 12 weeks. Baseline and post-intervention assessment included Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), part II and III, Parkinson's Disease Questionnaire (PDQ)-8 and Non-Motor Symptoms Scale (NMSS). We assessed adherence to TNR and problems expressed by patients/caregivers by means of open-ended surveys addressing barriers to rehabilitation. Results: We recruited 22 for TNR. Median age (interquartile range [IQR]) was 66.0 (44.0-71.0) years; 66.7% were H&Y stage 2.0. One patient died of COVID-19-related complications. Of the remaining 21, 14 (66.7%) had adherence of ≥75%; 16/21 (76.2%) patients had problems with attending TNR sessions as the family shared a single phone. Slow Internet speed was an issue among 13/21 (61.9%) of the patients. Other issues included lack of rapport, migration to distant hometowns and motor-hand impairment. Conclusion: Multiple challenges were faced in implementing a telerehabilitation program among persons with PD, exacerbated by the COVID-19 pandemic. These barriers were present at various levels: recruitment, adherence issues and maintenance. Future TNR programs must address these concerns.
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Comparison of five different electrophysiological criteria for childhood guillain barre syndrome p. 542
Ananthanarayanan Kasinathan, Arushi G Saini, Renu Suthar, Lokesh Saini, Jitendra K Sahu, Pratibha Singhi, Sunit Singhi, Naveen Sankhyan
DOI:10.4103/aian.AIAN_721_20  
Background: Internationally approved electrodiagnostic criteria for Guillain Barre syndrome lack in children. We intended to compare the diagnostic accuracy of the currently available five electrophysiological criteria for childhood Guillain Barre Syndrome (GBS) at the time of sentinel assessment. Methodology: In this single-center study, data of children diagnosed with GBS between January 2013 to December 2017 were retrieved. Patient charts were reviewed for clinical features, electrophysiological recordings. The electrodiagnostic results (4 motor nerves and two sensory nerves in upper limbs and lower limbs) were reanalyzed and were classified based on Dutch group; Ho; Hadden; Hughes and Rajabally criteria for GBS. Results: During this study period, of the 205 children with clinical features of GBS, 15 children had incomplete electrophysiological data, and four children were excluded due to missing data. The mean age of onset of the 186 children enrolled was 77 months; the median duration from symptom onset to electrodiagnostic evaluation was seven days; pure motor and motor-sensory form of GBS was seen in 71 and 115 children. Based on the Hadden criteria, a demyelinating pattern was noted in 57 children; axonal in 37; Inexcitable in 84 and Equivocal in 8 children. The sensitivity of the various criteria ranged from 71% to 100% for demyelination, 97% to 100% for axonal. The degree of agreement using Hadden and Rajabally criteria for Equivocal subtypes was 0.93. Conclusions: The Rajabally criteria showed the best sensitivity, specificity and diagnostic accuracy for electrodiagnosis of GBS in children when compared against Hadden criteria.
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Genotype-phenotype correlation of the childhood-onset bethlem myopathy in the mediterranean region of Turkey p. 547
Muhammet G Kutluk, Naz Kadem, Omer Bektas, Nadide C Randa, Gökcen O Tuncer, Pelin Albayrak, Tuba Eminoglu, Serap T Teber
DOI:10.4103/aian.AIAN_1182_20  
Objectives: Collagen-VI-related myopathies are caused by both dominant and recessive mutations in the three collagen-VI-related genes (COL6A1, COL6A2, and COL6A3) and present as two different major clinical entities; Bethlem myopathy and Ullrich congenital muscular dystrophy. Methods: In this study, we evaluated the clinical, pathologic, and genetic features of 8 patients with Bethlem myopathy from 3 families. Results: We inspected disease course differences with age and mutations. Different variants in COL6A1 and COL6A2 genes were detected. Muscle MRI of the lower limbs showed a specific pattern of muscle involvement with variable severity of fatty infiltration. One family had essential hypertension. Conclusion: Genotype-phenotype correlation studies are critical in determining gene or mutation-targeted therapies, patient follow-up, severity and progression prediction, and genetic counselling.
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The association of myositis specific antibodies in patients with inflammatory myositis: Preliminary data in Indian patients p. 552
Natasha Vijay Negalur, Gayatri G Ekbote, Dhiren N Raval, Dhaval V Tanna, Wasim S Kazi, Muzaffar A Bindroo, Durgarao J Yadavalli, Rajiva Gupta
DOI:10.4103/aian.AIAN_1151_20  
Context: Autoantibodies have a role in the diagnosis and prognosis in Autoimmune Inflammatory Myositis (AIM). Aims: The aim of this work was to study the prevalence and clinical correlation of myositis specific and associated antibodies (MSA and MAA) in AIM. Setting and Design: This was a cross-sectional observational study. Methods and Materials: Consecutive AIM patents were divided into groups as dermatomyositis (DM), polymyositis (PM), CTD-associated myositis (CTD-M), cancer-associated myositis (CAM) and juvenile myositis (JM). Their data along with serum samples were collected after obtaining informed consent. Sera was analyzed for IgG antibodies against Jo-1, PL-7, PL-12, EJ, SRP, Mi-2, MDA-5, TIF1γ, SAE1, SAE2, NXP2 and SSA/R052kD using the microELISA technique. The institutional ethics committee approved the study. Statistical Analysis: SPSS software (version 24.0) was used. P value < 0.05 was considered statistically significant. Results: There were 48 patients (DM = 19, PM = 19, CTD-M = 5, CAM = 2, JM = 3) included. MSA were positive in 37.5% patients. Antibodies against Mi-2 were present in 6 (12.5%), Jo-1 in 5 (10.4%), 2 (4.1%) each had PL-7 and SRP antibodies. One patient (2%) each had MDA-5, NXP2 and TIf1g antibodies. Jo-1 antibody was associated with mechanic's hands and ILD. There was a significant association of rash in the Mi-2 group with none of the patients having ILD. Malignancy screening was negative in NXP2 and TIF1g antibody-positive patients. Ro52 was the most common MAA (33.3%) and was associated with mechanic's hand. Conclusion: MSA was present in almost 40% of the cohort. Anti Jo-1 antibody was associated with mechanic's hands and ILD. None of the Mi-2 patients had ILD, which may point to a protective role of this antibody for ILD. The association of newer antibodies in Indian patients needs to be further studied in larger cohorts.
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Relationship between cerebral perfusion on Arterial Spin Labeling (ASL) MRI with brain volumetry and cognitive performance in mild cognitive impairment and dementia due to Alzheimer's disease p. 559
Shania Soman, Sheelakumari Raghavan, PG Rajesh, Ravi Prasad Varma, Nandini Mohanan, Sushama S Ramachandran, Bejoy Thomas, Chandrasekharan Kesavadas, Ramshekhar N Menon
DOI:10.4103/aian.AIAN_848_20  
Context: Cerebral blood flow (CBF) measurement using arterial spin labelling (ASL) MRI sequences has recently emerged as a prominent tool in dementia research. Aims: To establish association between quantified regional cerebral perfusion and gray matter (GM) volumes with cognitive measures in mild cognitive impairment (MCI) and early Alzheimer's Dementia (AD), using three dimensional fast spin echo pseudo-continuous ASL MRI sequences. Settings and Design: Hospital-based cross-sectional study. Methods and Material: Three age-matched groups, i.e., 21 cognitively normal healthy controls (HC), 20 MCI and 19 early AD patients diagnosed using neuropsychological tests and who consented for multimodality 3T MRI were recruited for the study. Statistical Analysis Used: Statistical parametric mapping and regions of interest (ROI) multivariate analysis of variance was used to ascertain differences between patients and controls on MRI-volumetry and ASL. Linear regression was used to assess relationship between CBF with GM atrophy and neuropsychological test measures. Results: Compared to HC, patients with MCI and AD had significantly lower quantified perfusion in posterior cingulate and lingual gyri, over hippocampus in MCI, with no differences noted between MCI and AD. Atrophy over the middle temporal gyrus and hippocampus differentiated AD from MCI. No significant positive correlations were noted between perfusion and GM volumes in ROI with the exception of temporal neocortex. Significantly positive coefficient b-value (p < 0.01) were apparent between global cognition with CBF in precuneus, temporal neocortex and precuneus volume, with negative b-values noted between medial temporal CBF for global cognition and recall scores. Conclusions: ROI-based CBF measurements differentiated MCI and AD from HC; volumetry of medial and neocortical temporal GM separates AD from MCI. Correlations between CBF and neuropsychology are variable and require further longitudinal studies to gauge its predictive utility on cognitive trajectory in MCI.
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Neurosyphilis, A true chameleon of neurology p. 566
Shripad S Pujari, Rahul V Kulkarni, Dhananjay Duberkar, Satish Nirhale, Dattatraya Nadgir, Pramod Dhonde, Tejas Sakale, Prafulla Shembalkar, Chandrashekhar Meshram
DOI:10.4103/aian.AIAN_28_21  
Background: Neurosyphilis (NS) is a rarely encountered scenario today. Manifestations are heterogeneous, and their characteristics have changed in the antibiotic era. A differential diagnosis of NS is not commonly thought of even with relevant clinical-radiological features, as it mimics many common neurological syndromes. Objectives: To study the manifestations of NS in the present era and the process of diagnosis. Method: The data of ten patients with NS was collected and analyzed. Their background data, clinical features, investigations, the process of reaching the diagnosis, management and outcomes were recorded. Observations and Results: The manifestations of NS in our cohort included six patients with cognitive decline/encephalopathy and one each with meningitis with cranial nerve palsies, cerebellar ataxia, myelitis and asymptomatic NS. The presence of Argyll Robertson pupil helped to clinch diagnosis in one patient. Treponemal tests were ordered in two patients only after alternative etiologies were looked at, to begin with, whereas in six patients treponemal test was requested as a part of standard workup for dementia/ataxia. Conclusions: NS dementia and behavior changes are mistaken for degenerative, vascular, nutritional causes, autoimmune encephalitis or prion disease. Meningitis has similarities with infective (tubercular), granulomatous (sarcoidosis, Wegener's), collagen vascular disease and neoplastic meningitis, and myelitis simulates demyelination or nutritional myelopathy (B12 deficiency). Rarely, NS can also present with cerebellar ataxia. Contemplate NS as one of the rare causes for such syndromes, and its early treatment produces good outcomes.
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Transient Ischemic Attack (TIA) Incidence with Geographic Information Systems (GIS) mapping for stroke prevention interventions p. 573
Cynthia Felix, Paramdeep Kaur, Ivy A Sebastian, Gagandeep Singh, Monika Singla, Shavinder Singh, Clarence J Samuel, Shweta J Verma, Jeyaraj D Pandian
DOI:10.4103/aian.AIAN_699_20  
Objectives: GIS mapping as a public health tool has been increasingly applied to chronic disease control. While evaluating TIA incidence from an existing regional stroke registry in Ludhiana city, India, we aim to apply the innovative concept of regional TIA GIS mapping for planning targeted stroke prevention interventions. Methods: TIA patient data was obtained from hospitals, scan centers and general practitioners from March 2010 to March 2013 using WHO-Stroke STEPS based surveillance as part of establishing a population-based stroke registry in Ludhiana city. From this registry, patients with TIA (diagnosed by MRI image-based stroke rule-out, or clinically) were chosen and data analyzed. Results: A total of 138 TIA patients were included in the final analysis. The annual TIA incidence rate for Ludhiana city was 7.13/100,000 (95% confidence interval: 5.52 to 8.74) for 2012-2013. Mean age was 58.5 ± 13.9 years (range: 22-88 years) and 87 (63%) were men. Majority of the TIA cases had anterior circulation TIAs. Hypertension (87.4%) was the most common risk factor. Using Geographic Information System (GIS) mapping, high TIA incidence was seen in central, western, and southern parts and clustering of TIA cumulative incidence was seen in the central part of Ludhiana city. Conclusion: Incidence rate of TIA was lower than that expected from a low- and middle-income country (LMIC). TIA GIS mapping, looking at regional localization, can be a novel option for developing targeted, cost-effective stroke prevention programs.
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Early post-stroke seizures in acute ischemic stroke: A prospective cohort study p. 580
Ayush Agarwal, Jyoti Sharma, MV Padma Srivastava, Rohit Bhatia, Mamta Bhushan Singh, Anu Gupta, Awadh K Pandit, Rajesh Singh, Roopa Rajan, Sadanand Dwivedi, Ashish Upadhyay, Ajay Garg, Venugopalan Y Vishnu
DOI:10.4103/aian.AIAN_1283_20  
Introduction: Stroke is the most common cause of epilepsy in the adult population. Post-stroke seizures (PSSs) are classified into early-onset seizures (ES) and late-onset (LS). ES can significantly affect the clinical outcome and occurrence of LS. Methods: We analyzed data from a prospective cohort of acute ischemic stroke patients between June 2018 and May 2020 in a neurology unit at a tertiary hospital. We screened all acute stroke patients and included consecutive patients older than 18 years of age, presenting with acute, first-ever neuroimaging-confirmed ischemic stroke. We excluded patients with a previous stroke, transient ischemic attacks, hemorrhagic stroke, cerebral venous thrombosis, prior history of seizures, or any other epileptogenic comorbidity. ES were classified as spontaneous seizures occurring within 1 week of the stroke. The main outcome assessed was the occurrence of ES. The secondary outcome was to determine predictors of ES and create an ES prediction score. Results: We screened 432 patients; of them, 291 were enrolled. ES occurred in 37 patients (12.7%). Cortical location (OR: 4.2), large artery disease subtype (OR: 2.9), mRS at presentation (OR: 1.4), use of anticoagulants (OR: 2.6), and hypertension (OR: 0.3) were significantly associated with the occurrence of ES. Patients with ES had a statistically significant worse clinical outcome at 3 months follow-up (P = 0.0072). Conclusion: We could formulate an ES prediction tool using the following components: (a) cortical location, (b) large vessel stroke, (c) mRS at admission, (d) anticoagulant use, and (e) presence of hypertension. This tool might help in treating patients at high risk for ES with prophylactic ASD, thereby preventing seizures and their complications.
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CASE REPORT Top

Pallidal deep brain stimulation for KMT2B related dystonia in an Indian patient p. 586
Roopa Rajan, Kanwaljeet Garg, Arti Saini, Mukesh Kumar, BK Binukumar, Vinod Scaria, Rajeev Aggarwal, Anu Gupta, VY Vishnu, Ajay Garg, Mamta Bhushan Singh, Rohit Bhatia, Achal K Srivastava, MV Padma Srivastava, Manmohan Singh
DOI:10.4103/aian.AIAN_1316_20  
Outcomes of pallidal stimulation in KMT2B dystonia have been infrequently reported prospectively. We report the six-month outcomes of bilateral GPi DBS in an Asian Indian patient with early-onset generalized dystonia associated with a novel heterozygous variant in the KMT2B gene.
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IMAGES IN NEUROLOGY Top

A case of paroxysmal kinesigenic myoclonus with episodic piloerection p. 589
CJ Suresh Chandran, Anoop Sugunan, Joseph Shibu
DOI:10.4103/aian.AIAN_1124_20  
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Transmantle heterotopia or closed lip schizencehaly: A diagnostic dilemma p. 590
Nishant Nayyar, Dinesh Sood, Preeti T Kapila, Narvir S Chauhan, Varsha Patial
DOI:10.4103/aian.AIAN_33_21  
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Tram track sign in sturge-weber syndrome Type 3 p. 592
Biswamohan Mishra, Rajesh Kumar Singh, Ajay Garg, Deepti Vibha, Ramanujam Bhargavi, Manjari Tripathi
DOI:10.4103/aian.AIAN_69_21  
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LETTERS TO THE EDITOR Top

Epilepsy care- awarness, cost and availability of antiepileptic drugs p. 593
Khichar Shubhakaran
DOI:10.4103/aian.AIAN_926_20  
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Post-mumps extrapyramidal syndrome in a young child p. 593
Rajesh Verma, Rajarshi Chakraborty
DOI:10.4103/aian.AIAN_846_20  
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Vivid visual hallucinations in the visually impaired: Charles bonnet syndrome—An analog to the “Phantom-Limb Phenomenon” p. 596
Scot Muirden
DOI:10.4103/aian.AIAN_878_20  
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Multiple lower cranial nerve palsies due to nasopharyngeal actinomycosis masquerading as a tumour – A diagnostic conundrum p. 597
Madhavi Karri, Balakrishnan Ramasamy, Santhosh Perumal
DOI:10.4103/aian.AIAN_867_20  
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Pediatric acute-onset neuropsychiatric syndrome with capgras syndrome p. 600
Indar Kumar Sharawat, Prateek Kumar Panda, Ravi Gupta
DOI:10.4103/aian.AIAN_959_20  
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What percentage of patients with duchene muscular dystrophy are potentially treatable with gene therapies? p. 601
Gouri Rao Passi, Manjari Paharia, Shree Prakash Jaiswal
DOI:10.4103/aian.AIAN_806_20  
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Association of autism spectrum disorder with pre-symptomatic duchenne muscular dystrophy: Isolated elevation of transaminases as a diagnostic clue p. 603
Prateek K Panda, Deep Shikha, Indar K Sharawat
DOI:10.4103/aian.AIAN_900_20  
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A novel mutation in the CLCN1 gene causing autosomal recessive myotonia congenita in siblings p. 605
Kamalesh Chakravarty, Vivek Lal, Sucharita Ray
DOI:10.4103/aian.AIAN_970_20  
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Takotsubo cardiomyopathy as a presenting manifestation of listeria rhombencephalitis p. 607
Valappil V Ashraf, K Abdul Salam, D Arunkumar, K Shajudeen
DOI:10.4103/aian.AIAN_800_20  
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Association of infantile spasms and hypsarrhythmia with primary microcephaly- Three case reports p. 609
Laxmi Khanna, Praveen Kumar, Ankkita Sharma Bhandari, M Gourie-Devi
DOI:10.4103/aian.AIAN_909_20  
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Progressive multifocal leukoencephalopathy as the initial presentation in an apparently healthy child p. 612
Rajni Farmania, Suvasini Sharma, Rahul Handa, Anju Seth
DOI:10.4103/aian.AIAN_818_20  
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Spinocerebellar ataxia Type 12 with an atypical ethnicity: A report of 2 families p. 614
Vikram Venkappayya Holla, Shweta Prasad, Nitish Kamble, Pramod Kumar Pal
DOI:10.4103/aian.AIAN_930_20  
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Cerebral Malaria in A Patient with COVID-19 p. 616
Ananth Ram, Sandeep Padmanabhan, Anup R Warrier, Arun Wilson
DOI:10.4103/aian.AIAN_860_20  
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Acute onset chronic inflammatory demyelinating polyneuropathy with prostatic malignancy p. 617
Puja Rani, Roopa Sharma, Priyanka Tater, Sandeep Sekhon
DOI:10.4103/aian.AIAN_971_20  
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Clinical presentation and outcome of children with brain abscess: Author's reply p. 620
Rajniti Prasad, John Biswas, Kulwant Singh, Om Prakash Mishra, Ankur Singh
DOI:10.4103/aian.AIAN_859_20  
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Florid brain calcification in a child with X-linked adrenoleukodystrophy: What does it signify? p. 620
Singanamalla Bhanudeep, Priyanka Madaan, Arushi Gahlot Saini, Sameer Vyas, Lokesh Saini
DOI:10.4103/aian.AIAN_974_20  
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Post Covid-19 guillain barre syndrome p. 622
Boby Varkey Maramattom, Ann Noble Zachariah, Hanna Angel Meleth, Zachariah T Zachariah, KM Mathew
DOI:10.4103/aian.AIAN_853_20  
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Bickerstaff brainstem encephalitis with isolated acute bilateral ophthalmoplegia: An unusual presentation p. 624
Amit Kumar Bagaria, Arvind Vyas, Vaibhav Mathur, Chandrajeet Singh Ranawat, Mridula Singh
DOI:10.4103/aian.AIAN_823_20  
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Neurology exams during the COVID-19 pandemic p. 626
Rajeswari Aghoram, Vaibhav Wadwekar, Sunil K Narayan, Pradeep P Nair, S Ramkumar, Sourabh Jain
DOI:10.4103/aian.AIAN_816_20  
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A case of moyamoya syndrome with isolated Protein S deficiency unmasked by pregnancy p. 628
Arka P Chakraborty, Souvik Dubey, Peyalee Sarkar, Shambaditya Das, Biman K Ray, Alak Pandit
DOI:10.4103/aian.AIAN_833_20  
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Abnormal saccades differentiate adolescent onset variant ataxia telangiectasia from other myoclonus dystonia p. 630
Ajith Cherian, Mitesh Chandarana, Ashish Anand Susvirkar, KP Divya, Udit U Saraf, Syam Krishnan
DOI:10.4103/aian.AIAN_619_20  
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COVID-19 and acute ischemic stroke; An Indian experience p. 632
Rahul Kulkarni, Shripad Pujari, Nilesh Palasdeokar, Sujit Jagtap, Sameer Jog, ZA Khan
DOI:10.4103/aian.AIAN_897_20  
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Contrast-induced encephalopathy – An unusual complication following endovascular interventions in the anterior circulation p. 635
Vivek Murumkar, Sameer Peer, Shumyla Jabeen, Richa S Chauhan, Jitender Saini, Ramalingaiah H Aravinda, Vivek Lanka, Girish B Kulkarni
DOI:10.4103/aian.AIAN_913_20  
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Epileptic seizure in patients with COVID-19: Two cases p. 639
Gonca Koksaldi Sahin, Akkan Avci, Onder Yesiloglu, Begum Seyda Avci, Hilmi Erdem Sumbul, Ilker Ozturk, Burcak Cakır Pekoz, Hayri Çınar, Salim Satar
DOI:10.4103/aian.AIAN_651_20  
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OBITUARY Top

Shraddhanjali: Dr. Ashok Panagariya p. 642
Bhawna Sharma, Parul Dubey, Madhuparna Paul
DOI:10.4103/aian.aian_671_21  
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