Search

Niemann-Pick disease type C (NPC), is a rare lysosomal storage disorder, which has a variable presentation based on the age of onset. We describe five adult/adolescent-onset NPC cases presenting with a range of movement disorders along with vertical supranuclear gaze palsy as part of the clinical presentation. A diagnostic delay of 4–17 years from the symptom onset was found in this case series. A high index of clinical suspicion in adult/adolescent patients presenting with vertical supranuclear gaze palsy along with various movement disorder phenomenology can help in the early diagnosis of NPC.

Pallavi Mishra,1 Ajay S Vamadevan,1,2 Ambuj Roy,3 Rohit Bhatia,4 Nitish Naik,3 Sandeep Singh,3 Gideon Senyo Amevinya,5 Ernest Amoah Ampah,5 Yolanda Fernandez,6 Caroline Free,7 Amos Laar,5 Dorairaj Prabhakaran,1,8,9 Pablo Perel,6 Helena Legido-Quigley10,11 1Health Systems Unit, Centre for Chronic Disease Control, New Delhi, India; 2Goa Institute of Management, Goa, India; 3Department of Cardiology, All India Institute of Medical Science, New Delhi, India; 4Department of Neurology, All India Institute of Medical Science, New Delhi, India; 5Department of Population, Family & Reproductive Health, School of Public Health, University of Ghana, Accra, Ghana; 6Centre for Global Chronic Conditions, London School of Hygiene and Tropical Medicine, London, UK; 7Department of Population Health, London School of Hygiene and Tropical Medicine, London, UK; 8Department of Non-Communicable Disease Epidemiology, London School of Hygiene and Tropical Medicine, London, UK; 9Research and Policy, Public Health Foundation of India, Gurugram, India; 10Saw Swee Hock School of Public Health, National University of Singapore, Singapore; 11Department of Health Services Research & Policy, London School of Hygiene and Tropical Medicine, London, UKCorrespondence: Pablo PerelCentre for Global Chronic Conditions, London School of Hygiene & Tropical Medicine (LSHTM), Keppel Street, London, WC1E 7HT, UKTel +44 20 7636 8636Email Pablo.Perel@lshtm.ac.ukIntroduction: In 2016, cardiovascular diseases (CVDs) led to 17.9 million deaths worldwide, representing 31% of all global deaths. CVDs are the leading cause of mortality worldwide and significant barriers to achieving the sustainable development goals. Modern medicines have been significant in improving health outcomes. However, non-adherence to medication is one of the reasons behind adverse health-related outcomes among patients suffering from atherosclerotic cardiovascular disease in low- and middle-income countries.Patients and Methods: This qualitative study was conducted at two tertiary care hospitals in India and Ghana. A total of 35 in-depth interviews were conducted with atherosclerosis cardiovascular disease (ASCVD) patients. The data were analysed thematically using the Capability Opportunity and Motivation (COM-B) framework.Findings: The findings were summarised under three important broad themes of the COM-B framework: capability, opportunity and behaviour. Under capability, comprehension of disease, medication schedule, and unplanned travel affected adherence among patients. Cost of medication, insurance and access were the critical factors under opportunity, which negatively influenced medication adherence. Mood, beliefs about treatment and outcome expectations under motivation led to non-adherence among patients. Apart from these factors, some important health system factors such as health care experience and trust in the facilities and reliance on alternative medication also affected adherence in both countries.Conclusion: This study has highlighted that the health system factors have dominantly influenced adherence to medication in India and Ghana. In India, we found participants to be satisfied with their health care provided at the government hospitals. However, limited time for consultation, lack of well-stocked pharmacy and unclear prescription negatively influenced adherence among participants in India and Ghana. The study emphasises that the health system needs to be strengthened, and the patients’ belief system needs to be explored to address the issue of medication adherence in LMICs.Keywords: non-communicable diseases, atherosclerotic cardiovascular disease, public health, qualitative research

A 42-year-old businessman had a 27-year history of multiple complaints. He had been born of a non-consanguineous marriage, but his siblings had young-onset hypertension. When aged 15 years, he developed an ulcerated skin lesion on the medial right thigh with right foot drop. This was presumptively treated as tuberculosis with corticosteroids and antituberculous therapy; the skin lesion resolved in 1–2 weeks and the foot drop recovered within 6–8 weeks. He stopped antituberculous therapy after 1 month. When aged 24 years, he developed a left foot drop that improved with oral corticosteroids in 3–4 weeks. However, on corticosteroid withdrawal he developed fever, left foot ulceration and abdominal pain. Biopsy of the skin ulcer was reported as ‘systemic lupus erythematosus-like’. His symptoms resolved with tapering corticosteroids over 4–6 months. He was also diagnosed and treated for hypertension at this time. When aged 30 years, he developed similar complaints. A rheumatologist prescribed monthly cyclophosphamide for 6 months with symptom resolution. He was then lost to follow-up for 6 years. When aged 36 years he presented with fever, abdominal and testicular pain, with skin ulcerations in the lower limbs. His viral markers for HIV, hepatitis B, hepatitis C were negative and a CT aortogram was normal. His condition responded well to pulsed corticosteroids and mycophenolate mofetil. He had further recurrent relapses every 1–2 years and tried a combination of multiple immunosuppressants: mycophenolate, azathioprine and cyclophosphamide. When aged 40 years, he had another relapse (fever, abdominal pain, left foot drop and weight loss) with significant (22 kg) unintentional weight loss over 2 months. CT scan of abdomen showed a left perirenal haematoma, with splenic and bilateral renal cortical infarcts. CT aortic angiogram showed multiple aneurysms in medium-sized abdominal aortic visceral branches. Chest X-ray and two-dimensional echocardiography were unremarkable. Upper gastrointestinal endoscopy identified oesophageal candidiasis, whereas colonoscopy showed multiple …

Background: Teleneurology consultations can be highly advantageous since neurological diseases and disabilities often limit patient's access to health care, particularly in a setting where they nee...

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an astrocytopathy with a predilection for the optic nerve, spinal cord, and brainstem. In this ambispective study, we evaluate clinical characteristics, responses to therapy, and disability outcomes in patients with NMOSD. Methods: Patients diagnosed as NMOSD and following up for at least 1 year at a tertiary care center in India were recruited. Patient data were collected ambispectively from January 2012 until December 2018. Results: A total of 106 patients (29M/77F) with NMOSD were evaluated. The mean age of onset was 29 (±11.6) years. About 77 patients (72.64%) were positive for the AQP4 antibody. Age of onset was higher for those presenting with an opticospinal syndrome (34.2 years) as compared to either isolated longitudinally extensive transverse myelitis (LETM) (30 years) or optic neuritis (ON) (25.3 years). The most common syndrome at onset was LETM in 57 patients (53.77%) followed by ON in 31 patients (29.24%). Azathioprine was the most common immunotherapy (83.96%) prescribed followed by rituximab (7.54%) and mycophenolate mofetil (1.88%). There was a significant decrease in the number of relapses post-azathioprine (P < 0.001). Out of 67 patients with ON, 21 (31.34%) had complete recovery while 17 (25.37%) patients had a severe deficit at a 3-month follow-up. Out of 92 patients with a motor deficit, 49 (53.26%) patients had a partial motor deficit at a 6-month follow-up. The severe visual deficit at baseline and female gender predicted poor visual and motor recovery, respectively. Conclusion: This is the largest descriptive study on patients with NMOSD from India. Relapse rates were similar irrespective of the clinical presentation, age, gender, and disease course. Treatment with immunosuppressive treatment significantly affected the disease course.

Purpose To compare epilepsy-related injuries in untreated or inadequately treated patients and patients on adequate treatment. Methods In a cross-sectional case-control study, seizure-related injuries in patients who were either on no treatment or inadequate treatment were compared with another group of patients receiving appropriate evidence-based epilepsy treatment. The inadequately treated patients or ‘cases’ were drawn from an outreach epilepsy clinic while the adequately treated patients or ‘controls’ were recruited from a tertiary care facility providing comprehensive epilepsy management. Results The odds of injury were eight times higher in inadequately treated patients or cases compared to the adequately treated patients or controls. After adjusting for gender, epilepsy duration, seizure frequency, current medication, and number of AEDs, the odds of injury were 15. 8 times higher in the cases. Major injuries such as burns, fractures, and tooth injuries were also higher in the cases. Conclusion Untreated or inadequately treated epilepsy patients have a significantly higher risk of injuries. With adequate treatment, some of the risks of injury can be mitigated.

Background and Purpose Various neurological findings including stroke in patients with coronavirus disease 2019 (COVID-19) have been described, although no clarity exists regarding the nature and pattern of this association. This systematic review aims to report the characteristics of stroke in patients with COVID-19.Methods Three authors independently searched Web of Science, Embase, Scopus, and PubMed starting from inception up to May 22, 2020. The data for individual patients was extracted where available from published reports including clinical and laboratory parameters and analysed for any significant associations between variables.Results We identified 30 relevant articles involving 115 patients with acute or subacute stroke with COVID-19. The mean±standard deviation age was 62.5±14.5 years. Stroke was ischemic in majority of the patients (101 [87.8%]). Hypertension (42 [42%]), dyslipidaemia (24 [26.1%]), and diabetes (23 [23.2%]) were the major vascular risk factors. Most of the patients (80 [85.1%]) had COVID-19 symptoms at the time of stroke with a median interval of 10 days to stroke from the diagnosis of COVID-19. Three-fourths (86 [74.8%]) of the patients were critically ill which frequently delayed the diagnosis of stroke. High levels of D-dimer, and ferritin were observed in these patients. Patients with COVID-19 and stroke had a high mortality (47.9%). Factors associated with mortality were intensive care unit admission, having two or more vascular risk factors, particularly smoking and high levels of D-dimer, C-reactive protein, and lactate dehydrogenase.Conclusions The association between stroke and COVID-19 is probably multifactorial including an amalgamation of traditional vascular risk factors, proinflammatory and a prothrombotic state. Prospectively collected data is required in the future to confirm this hypothesis.

BACKGROUND: Anticholinergic drugs are associated with significant cognitive and other adverse events in older adults, including those with Parkinson's disease (PD). Anticholinergic effects are considered lesser in younger individuals and the burden and outcomes in younger patients with PD are unknown. OBJECTIVES: To determine the cumulative anticholinergic burden in a cohort of younger of patients with PD and to correlate the same with cognitive impairment and freezing of gait (FOG). METHODS: We conducted a cross‐sectional study to identify the cumulative anticholinergic burden from medications prescribed to patients with PD. Two standard scales, the Anticholinergic Cognitive Burden (ACB) scale and the ACB score, were used to calculate the anticholinergic burden from prescriptions. We identified commonly prescribed drugs contributing to anticholinergic effects and correlated the cumulative ACB score with cognitive impairment (Movement Disorder Society–Unified Parkinson's Disease Rating Scale item 1.1) and FOG (Movement Disorder Society–Unified Parkinson's Disease Rating Scale items 2.13 and 3.11). RESULTS: We recruited 287 patients with PD (68.9% male) with a mean age of 56.9 ± 11.8 years and a duration of symptoms 6.3 ± 6.9 years. Median ACB score was 4 (range 0–12). A total of 164 (58.4%) patients had total ACB score > 3. ACB score > 3 was independently associated with cognitive impairment (Odds Ratio, 2.55; 95% confidence interval, 1.43–4.53; P

Background Patients in the neurointensive care unit have high utilization of devices, thereby increased chance of getting device-associated infection (DAI). Central line-associated bloodstream infection (CLABSI) remains one of the most important DAI. Education remains an important part of the hospital infection control and improves the infection-control practices. Materials and methods To evaluate the effectiveness of a quality initiative in reducing incidence of CLABSI, a prospective study (January 2017-December 2018) was done estimating CLABSI incidence before and after the intervention. Continuous teaching and training for hand hygiene practice and central-line catheter hub care were used as the tool for this study. Results The quality improvement (QI) initiative achieved a 48% reduction in the CLABSI rate from the baseline rate of 8.7 to 4.5 per 1000 catheter days. The overall mortality showed a reduction from 1.5 to 0.05% during the post-intervention period. There was a significant improvement in compliance with the hand hygiene practice and catheter hub care in the post-intervention period. Discussion and conclusion This study demonstrates adherence to hand hygiene and catheter hub care with continuous teaching, training, and supervision was highly effective in reducing the CLABSI rate. Clinical significance Central line-associated bloodstream infection is one of the most important DAI causing significant morbidity and mortality in critically ill patient. Our findings support that continuous educational intervention of hand hygiene with and training on the catheter hub care are two most important preventive measures in the reduction of CLABSI incidence. How to cite this article Mohapatra S, Kapil A, Suri A, Pandia MP, Bhatia R, Borkar S, et al. Impact of Continuous Education and Training in Reduction of Central Line-associated Bloodstream Infection in Neurointensive Care Unit. Indian J Crit Care Med 2020;24(6):414-417.

Chronic migraine is a disease of altered cortical excitability. Repetitive transcranial magnetic stimulation provides a novel non-invasive method to target the nociceptive circuits in the cortex. Motor cortex is one such potential target. In this study, we targeted the left motor cortex using fMRI-guided neuronavigation. Twenty right-handed patients were randomized into real and sham rTMS group. Baseline subjective pain assessments were done using visual analog scale (VAS) and questionnaires: State-Trait Anxiety Inventory, Becks Depression Inventory, and Migraine Disability Assessment (MIDAS) questionnaire. Objectively, pain was assessed by means of thermal pain thresholds using quantitative sensory testing. For corticomotor excitability parameters, resting motor thresholds and motor-evoked potentials were mapped. For rTMS total, 600 pulses in 10 trains at 10 Hz with an intertrain interval of 60 s were delivered in each session. Ten such sessions were given 5 days per week over 2 consecutive weeks. The duration of each session was 10 min. Real rTMS was administered at 70% of Resting MT. All the tests were repeated post-intervention and after 1 month of follow-up. There are no studies reporting the use of fMRI-based TMS for targeting the motor cortex in CM patients. We observed a significant reduction in the mean VAS rating, headache frequency, and MIDAS questionnaire in real rTMS group which was maintained after 1 month of follow-up. Ten sessions of fMRI-based rTMS over the left motor cortex may provide long-term pain relief in CM, but further studies are warranted to confirm our preliminary findings.

Background and Purpose— Definitions of significant hematoma expansion traditionally focus on changes in intraparenchymal volume. The presence of intraventricular hemorrhage (IVH) is a predictor of poor outcome, but current definitions of hematoma expansion do not include IVH expansion. We evaluated whether including IVH expansion to current definitions of hematoma expansion improves the ability to predict 90-day outcome. Methods— Using data from the PREDICT-ICH study (Predicting Hematoma Growth and Outcome in Intracerebral Hemorrhage Using Contrast Bolus CT), we compared a standard definition of hematoma expansion (≥6 mL or ≥33%) to revised definitions that includes new IVH development or expansion (≥6 mL or ≥33% or any IVH; ≥6 mL or ≥33% or IVH expansion ≥1 mL). The primary outcome was poor clinical outcome (modified Rankin Scale score, 4–6) at 90 days. Diagnostic accuracy measures were calculated for each definition, and C statistics for each definition were compared using nonparametric methods. Results— Of the 256 patients eligible for primary analysis, 127 (49.6%) had a modified Rankin Scale score of 4 to 6. Sensitivity and specificity for the standard definition (n=80) were 45.7% (95% CI, 36.8–54.7) and 82.9% (95% CI, 75.3–88.9), respectively. The revised definition, ≥6 mL or ≥33% or any IVH (n=113), possessed a sensitivity of 63.8% (95% CI, 54.8–72.1) and specificity of 75.2% (95% CI, 66.8–82.4). Overall accuracy was significantly improved with the revised definition ( P =0.013) and after adjusting for relevant covariates, was associated with a 2.55-fold increased odds (95% CI, 1.31–4.94) of poor outcome at 90 days. A second revised definition, ≥6 mL or ≥33% or IVH expansion ≥1 mL, performed similarly (sensitivity, 56.7% [95% CI, 47.6–65.5]; specificity, 78.3% [95% CI, 40.2–85.1]; aOR, 2.40 [95% CI, 1.23–4.69]). Conclusions— In patients with mild-to-moderate ICH, including IVH expansion to the definition of hematoma expansion improves sensitivity with only minimal decreases to specificity and improves overall prediction of 90-day outcome.

Background and Purpose— Patients with intracerebral hemorrhage (ICH) are often subject to rapid deterioration due to hematoma expansion. Current prognostic scores are largely based on the assessment of baseline radiographic characteristics and do not account for subsequent changes. We propose that calculation of prognostic scores using delayed imaging will have better predictive values for long-term mortality compared with baseline assessments. Methods— We analyzed prospectively collected data from the multicenter PREDICT study (Prediction of Hematoma Growth and Outcome in Patients With Intracerebral Hemorrhage Using the CT-Angiography Spot Sign). We calculated the ICH Score, Functional Outcome in Patients With Primary Intracerebral Hemorrhage (FUNC) Score, and modified ICH Score using imaging data at initial presentation and at 24 hours. The primary outcome was mortality at 90 days. We generated receiver operating characteristic curves for all 3 scores, both at baseline and at 24 hours, and assessed predictive accuracy for 90-day mortality with their respective area under the curve. Competing curves were assessed with nonparametric methods. Results— The analysis included 280 patients, with a 90-day mortality rate of 25.4%. All 3 prognostic scores calculated using 24-hour imaging were more predictive of mortality as compared with baseline: the area under the curve was 0.82 at 24 hours (95% CI, 0.76–0.87) compared with 0.78 at baseline (95% CI, 0.72–0.84) for ICH Score, 0.84 at 24 hours (95% CI, 0.79–0.89) compared with 0.76 at baseline (95% CI, 0.70–0.83) for FUNC, and 0.82 at 24 hours (95% CI, 0.76–0.88) compared with 0.74 at baseline (95% CI, 0.67–0.81) for modified ICH Score. Conclusions— Calculation of the ICH Score, FUNC Score, and modified ICH Score using 24-hour imaging demonstrated better prognostic value in predicting 90-day mortality compared with those calculated at presentation.

Endometriosis is a category of lesions and fibroids which occur in the innermost lining of the uterus that is the endometrial layer and spreads to the other parts of the neighboring tissues. It is characterized by inflammation, pelvic pain, menstrual bleeding, and Dysmenorrhea. Endometriosis exists in 3 forms based on its spreadability. i.) superficial peritoneal lesions ii.) deep infiltrating lesion and iii.) cyst. Traditional treatments involve are NSAIDS, Oral contraceptives, Aromatase inhibitors, and GnRH agonists. Elagolix emerges out to be the latest potent drug that acts by inhibiting the GnRH receptor. Unlike other allopathy medicine, it is not an asymptomatic pain reliever but acts on the root cause of the disease. FDA has approved it for specific treatment of endometriosis in July 2018. It is believed that endometriosis occurs by an increase in the production of estrogen. Elagolix competitively binds to the GnRH receptors and prevents the binding of the Gonadotropin hormone flowing through blood capillaries from the Hypothalamus to the pituitary gland and thereby stops the formation of Oestrogen in the reproductive system which ultimately ceases in the proliferation of endometrial layers and tissue spread. In estrogen biosynthesis, the aromatase P450 enzyme plays a vital role. Elagolix belongs to BCS class III and available in the oral dosage form of 150, 200mg. It is rapidly absorbed and Cmax reaches within 1hour. The plasma protein binding is 80%. In this review, various aspects related to Elagolix sodium have been summarised, which include pathophysiology, Mode of Action, Structure-activity relationship, Pharmacokinetics, pharmacodynamics, and clinical studies with minimal side effects.

This study aims to determine the impact of yoga as an adjunct to standard therapy versus standard therapy alone on the symptomatic burden in patients with recurrent vasovagal syncope (VVS).There is a significant reduction in the quality of life (QoL) of patients with recurrent VVS. Existing management therapies have been largely ineffective. Recent trials have demonstrated the efficacy of yoga in diseases with autonomic imbalance, suggesting its possible utility in VVS.Patients with recurrent VVS were randomized to receive either a specialized yoga training program in addition to current guideline-based therapy (intervention arm, group 1) or current guideline-based therapy alone (control arm, group 2). The primary outcome was a composite of the number of episodes of syncope and presyncope at 12 months. Secondary outcomes included QoL assessment by World Health Organization Quality of Life Brief Field questionnaire (WHOQoL-BREF) scores and Syncope Functional Status Questionnaire scores at 12 months, head up tilt test, and heart rate variability at 6 weeks.A total of 55 patients underwent randomization. The mean number of syncopal or presyncopal events at 12 months was 0.7 ± 0.7 in the intervention arm compared to 2.52 ± 1.93 in the control arm (P0.01). In the intervention arm, 13 (43.3%) patients remained free of events versus 4 (16.0%) patients in the control arm (P = 0.02). QoL at 12 months showed significant improvement of all Syncope Functional Status Questionnaire scores and 2 domains of WHOQoL-BREF scores (P0.05).Yoga as adjunctive therapy is superior to standard therapy alone in reducing the symptomatic burden and improving QoL in patients with recurrent VVS.

Introduction Micro-embolic signals (MESs) detected using transcranial Doppler (TCD) help in risk stratification in stroke patients. A systematic review and meta-analysis were performed to estimate the prevalence of MES and its association with stroke recurrence, functional outcome, and mortality in different stroke subtypes. Methods A comprehensive literature search was conducted before 26th January 2021 in PubMed, Embase, Google Scholar, Cochrane Library, and ClinicalTrials.gov . Studies were identified that used TCD to detect MES in stroke/TIA patients. Pooled prevalence and odds ratio (OR) along with 95% confidence interval (95% CI) were calculated for different outcome measures. The entire statistical analysis was conducted in R version 3.6.2. Findings Fifty-eight studies involving 5123 patients (1329 MES+, 3794 MES−) were included in our meta-analysis. The pooled prevalence of MES among all acute stroke/TIA patients was 30% (95% CI 25-34%). The pooled prevalence adjusted after the trim-and-fill analysis among all acute stroke/TIA patients was 18% (95% CI 14-23%). The prevalence of MES was high among all stroke subtypes except in patients with small vessel disease (SVD). In patients with new-onset stroke/TIA, the presence of MES was associated with a high risk of recurrence of cerebral ischemia (OR 4.03; 95% CI 2.38-6.82). Although no significant association was observed for the presence of MES with increased mortality (OR 2.37; 95% CI 0.75-7.50) and poor functional outcome (OR 2.11; 95% CI 0.20-22.50) among patients with new-onset stroke/TIA, this could only be determined in a smaller sample size of 477 patients. Conclusions Our meta-analysis showed a 30% prevalence of MES following acute stroke/TIA. The presence of MES increased the chance of recurrence of cerebral ischemia but was not associated with poor functional outcomes and mortality in the studied subgroup.

Objective Prognostic scores help in predicting mortality and functional outcome post intracerebral hemorrhage (ICH). We aimed to validate the ICH and ICH-GS scores in a cohort of Indian patients with ICH and observe the impact of any surgical intervention on prognostication. Methods This was an ambispective observational study of primary ICH cases enrolled between January 2014 and April 2018. Observed mortality on ICH and ICH GS scores for the entire cohort and individually for the medically and surgically managed patients was compared to the published mortality in the original derivation cohorts. Results 617 patients, (464 retrospective and 153 prospective) of ICH were included. In hospital mortality and 30-day mortality was 28.7% and 28.5% respectively. There was a significant association of increasing mortality with increasing ICH and ICH-GS scores. Area under receiver operating characteristic curve for 30-day mortality was 75.9% and 74.1% for ICH and ICH-GS scores respectively. However, mortality observed at individual scores was significantly less than previously reported. Among the surgically intervened patients (n = 265), both the expected mortality at baseline and discriminative ability of ICH and ICH-GS scores for 30-day mortality was significantly reduced following surgical intervention (ROC in surgically intervened groups: 59.9 (52.6-67.2) and 63(56-70) for ICH and ICH-GS scores respectively). Conclusions Although ICH and ICH-GS scores are valid in Indian population, mortality at individual scores is lower than previously reported. Mortality prediction using ICH and ICH GS scores is significantly modified by surgical interventions. Thus, newer prognostic tools which incorporate surgical intervention need to be developed and validated in future.

Purpose To compare long-term treatment outcomes in epilepsy patients from a single-visit outreach clinic on the Lifeline Express (LLE) with a conventional hospital (AIIMS) based epilepsy clinic in India. Methods Using a cross-sectional observational study design, consecutive epilepsy patients from fifteen LLE clinics conducted from 2009 to 2014 were compared to epilepsy patients registered in the same duration at the AIIMS epilepsy clinic. The primary outcome was to determine if patients were still taking AEDs. To determine current AED status, patients from the LLE clinic were contacted telephonically. For the AIIMS patients, hospital records were reviewed and phone calls made to those patients who had not followed-up for more than a year. Results In the 5 years under review, 1923 and 1257 patients had consulted at the LLE and AIIMS clinics respectively. Long-term outcomes were available for analysis in 688 AIIMS and 531 LLE clinic patients. Of the AIIMS patients, 581(87%) were continuing AEDs, 49(7%) had discontinued AEDs after being seizure-free for at least 5 years, 39(6%) had discontinued AEDs without medical advice and 19(2.8%) were dead. Outcomes in 531 LLE patients revealed that 351(72%) continued to be on AEDs, 34(7%) had discontinued AEDs on advice, 106 (22%) had discontinued AEDs without any medical advice and 40 (7.5%) were dead. The treatment gap in the LLE patients was reduced from 49% at first contact to 22% at follow-up 2–8 years later. Conclusions Even single-visit epilepsy clinics may be an effective option for reducing treatment gap in limited-resource regions of the world.

Background and Purpose: Differentiating between neurocysticercosis (NCC) and neurotuberculosis has serious therapeutic implications and this distinction relies heavily on neuroimaging. Few case reports discuss the conglomeration of ring-enhancing lesions (RELs) in patients with solitary NCC. The aim of our study is to describe the imaging findings of conglomerate RELs in a cohort of patients with solitary NCC, emphasizing the frequency of conglomeration. Materials and Methods: This retrospective study included 100 patients with solitary NCC. Two neuroradiologists analyzed contrast-enhanced computed tomography (CT) images regarding morphology, enhancement pattern, location, number of lesions, and degree of perilesional edema. The solitary lesions were classified as solitary discrete RELs (SD-RELs) when a well-defined lesion was seen and solitary conglomerate RELs (SC-RELs) when two or more ring lesions or ring/rings plus disc lesions were present contiguously. Follow-up CT scans were evaluated for the resolution of lesions and surrounding edema. Results: Out of 100 patients, 42 were SD-RELs and 58 were SC-RELs. No statistically significant difference was found between both groups in terms of age of presentation, clinical presentation, lesion size and location, and degree of perilesional edema. Larger lesions (>10 mm) were more likely to show scolex and were associated with greater degree of edema in both subgroups. During follow-up, 13 patients had new lesions (SD-RELs-5, SC-RELs-8). In SD-RELs, follow-up lesions were in the same location in four patients and new location in one; and in SC-RELs, lesions were in the same location in seven and in new location in one case. Conclusion: Conglomeration of RELs is a common finding in patients with solitary NCC.

Central nervous system (CNS) melioidosis is a rare neurological infectious disease which carries a high mortality. We describe a previously healthy middle-aged female, who presented to us with left-sided hemiparesis and was on antitubercular therapy from a previous presumed diagnosis of CNS tuberculoma. Non-characteristic imaging picture, multiple negative body fluid cultures, and positive Cerebrospinal fluid galactomannan led to a further delay in diagnosis. Gram stain of the tissue obtained from brain biopsy revealed Gram-negative rods in “safety pin” appearance. By picking up the colonies that appeared on blood agar and MacConkey agar, the identification of the clinical isolates was performed using VITEK® matrix (BioMérieux, Marcy-L’Etoile, France)-assisted laser desorption ionization time-of-flight mass spectrometry (VITEK MALDI TOF MS database version 3.2) which revealed Burkholderia pseudomallei. After the institution of appropriate treatment, she survived but with significant morbidity. A high index of suspicion should be kept for such previously healthy individuals belonging to non-endemic areas, where presentation is suspicious of an infective etiology, but not improving despite appropriate therapy. This may help in early recognition and institution of recommended treatment so that mortality can be avoided.

Background and Purpose: Occurrence of stroke has been reported among patients with COVID-19. The present study compares clinical features and outcomes of stroke patients with and without COVID-19. Methods: The COVID-19 Stroke Study Group (CSSG) is a multicentric study in 18 sites across India to observe and compare the clinical characteristics of patients with stroke admitted during the current pandemic period and a similar epoch in 2019. The present study reports patients of stroke with and without COVID-19 (CoVS and non-CoVS, respectively) seen between February 2020 and July 2020. Demographic, clinical, treatment, and outcome details of patients were collected. Results: The mean age and gender were comparable between the two groups. CoVS patients had higher stroke severity and extent of cerebral involvement on imaging. In-hospital complications and death were higher among CoVS patients (53.06% vs. 17.51%; P < 0.001) and (42.31% vs. 7.6%; P < 0.001), respectively. At 3 months, higher mortality was observed among CoVS patients (67.65% vs. 13.43%; P < 0.001) and good outcome (modified Rankin score [mRS]: 0–2) was seen more often in non-CoVS patients (68.86% vs. 33.33%; P < 0.001). The presence of COVID-19 and baseline stroke severity were independent predictors of mortality. Conclusions: CoVS is associated with higher severity, poor outcome, and increased mortality. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and baseline stroke severity are independent predictors of mortality.

Background and Purpose: Cerebral small vessel disease (CSVD) markers have not been widely studied in relation to hematoma volume and growth in hypertensive intracerebral hemorrhage (ICH). The objectives to assess the relationship of white matter hyperintense lesions (WMHL), microbleeds (MBs), and cortical siderosis (CSS) with hematoma volume, hematoma expansion (HE), and 3 months outcome in patients with hypertensive ICH. Methods: All consecutive acute hypertensive supratentorial ICH presenting to the emergency were prospectively recruited. Baseline and 24 hours computed tomography (CT) to assess hematoma volume and magnetic resonance imaging (MRI) for CSVD markers were performed in all subjects. WMHL (graded using Fazekas's scale), MBs, and CSS were assessed and compared with baseline variables and outcomes. All the images were assessed by an experienced stroke neurologist/neuroradiologist. Results: One hundred and fifty-seven patients were screened and 60 were included. Mean age was 54.08 ± 11.57 years and 47 (78%) were males. Of 60, 19 (28.1%) had HE, 31 (51.6%) had major bleed (>30 ml), and 28 (47.46%) had poor 3 month outcome (mRS 4-6). On univariate analysis, high grade WMHL was associated with greater HE [odds ratio (OR): 2.65, confidence interval (CI) 1.48–4.72, P = 0.001), greater proportion with volume >30 ml (OR: 7.16, CI: 1.09–47.13, P = 0.001) and poor outcome (OR: 2.1, CI: 0.05–3.27, P = 0.001). MBs were associated with poor outcome (P = 0.029) but not with HE/volume. CSS was related to HE (P = 0.031), a large volume bleed (P = 0.023), and poor outcome (P = 0.021). On multivariate model, only WMHL independently predicted HE (P = 0.034), greater proportion with bleed volume >30 ml (P = 0.041), and poor outcome (P = 0.042). Conclusions: WMHL in MRI serves as a predictor of hematoma expansion, a large volume bleed, and poor outcome in hypertensive ICH and may be incorporated into existing prediction models.

Summary Background A systematic understanding of the burden of neurological disorders at the subnational level is not readily available for India. We present a comprehensive analysis of the disease burden and trends of neurological disorders at the state level in India. Methods Using all accessible data from multiple sources, we estimated the prevalence or incidence and disability-adjusted life-years (DALYs) for neurological disorders from 1990 to 2019 for all states of India as part of the Global Burden of Diseases, Injuries, and Risk Factors Study 2019. We assessed the contribution of each neurological disorder to deaths and DALYs in India in 2019, their trends in prevalence or incidence and DALY rates over time, and heterogeneity between the states of India. We also assessed the Pearson correlation coefficient between Socio-demographic Index (SDI) of the states and the prevalence or incidence and DALY rates of each neurological disorder. Additionally, we estimated the contribution of known risk factors to DALYs from neurological disorders. We calculated 95% uncertainty intervals (UIs) for the mean estimates. Findings The contribution of non-communicable neurological disorders to total DALYs in India doubled from 4·0% (95% UI 3·2–5·0) in 1990 to 8·2% (6·6–10·2) in 2019, and the contribution of injury-related neurological disorders increased from 0·2% (0·2–0·3) to 0·6% (0·5–0·7). Conversely, the contribution of communicable neurological disorders decreased from 4·1% (3·5–4·8) to 1·1% (0·9–1·5) during the same period. In 2019, the largest contributors to the total neurological disorder DALYs in India were stroke (37·9% [29·9–46·1]), headache disorders (17·5% [3·6–32·5]), epilepsy (11·3% [9·0–14·3]), cerebral palsy (5·7% [4·2–7·7]), and encephalitis (5·3% [3·7–8·9]). The crude DALY rate of several neurological disorders had considerable heterogeneity between the states in 2019, with the highest variation for tetanus (93·2 times), meningitis (8·3 times), and stroke (5·5 times). SDI of the states had a moderate significant negative correlation with communicable neurological disorder DALY rate and a moderate significant positive correlation with injury-related neurological disorder DALY rate in 2019. For most of the non-communicable neurological disorders, there was an increase in prevalence or incidence from 1990 to 2019. Substantial decreases were evident in the incidence and DALY rates of communicable neurological disorders during the same period. Migraine and multiple sclerosis were more prevalent among females than males and traumatic brain injuries were more common among males than females in 2019. Communicable diseases contributed to the majority of total neurological disorder DALYs in children younger than 5 years, and non-communicable neurological disorders were the highest contributor in all other age groups. In 2019, the leading risk factors contributing to DALYs due to non-communicable neurological disorders in India included high systolic blood pressure, air pollution, dietary risks, high fasting plasma glucose, and high body-mass index. For communicable disorders, the identified risk factors with modest contributions to DALYs were low birthweight and short gestation and air pollution. Interpretation The increasing contribution of non-communicable and injury-related neurological disorders to the overall disease burden in India, and the substantial state-level variation in the burden of many neurological disorders highlight the need for state-specific health system responses to address the gaps in neurology services related to awareness, early identification, treatment, and rehabilitation. Funding Bill & Melinda Gates Foundation; and Indian Council of Medical Research, Department of Health Research, Ministry of Health and Family Welfare, Government of India.

Antiparasitic treatment improves the prognosis for neurocysticercosis (NCC)-induced seizures. However, patients with high lesion loads are typically denied the possible benefit of cysticidal therapy because of fear of complications, and such patients are not represented in clinical trials involving cysticidal therapy. We provide proof of concept for combination treatment with dual antiparasitic therapy and corticosteroids in patients with diffuse lesions, including starry sky patterns, or calcific NCC. The safety and efficacy of treating patients with high lesion loads or calcific NCC should be tested in a randomized controlled trial.

IntroductionThe lockdown due to COVID-19 pandemic led to temporary closure of routine hospital services. This prompted the initiation of teleconsult follow-up in our department. The study outlines the experience of tele-follow-up at a tertiary care teaching hospital in India, and the perspective of neurologists about this novel approach.MethodsThe tele-follow-up was started from 26th March 2020. Data of follow up appointments was provided by the medical record section. The faculty and senior residents conducted the tele-follow-up. Communication was made via voice calls. The data for initial ten days was analyzed to find the utility and experience of the new service.ResultsIn the initial ten working days, data of 968 patients was provided for tele-follow-up. A successful communication was made in 50.3% patients (contact with patients: 27.7% and family members 22.6%). The phone numbers which were not contactable/invalid/not available constituted 36.8% of the data. A total of 35 faculty and residents conducted the tele-follow-up. The utility of tele-follow-up was perceived as good by 71.4% of neurologists. Majority of neurologists (71.4%) observed that >90% of patients were continuing medications. Patients outside the city constituted 50-75% of the list. The survey revealed that all neurologists felt the need to continue tele-follow-up for far off stable patients post lock down and resumption of regular outpatient services.ConclusionThe survey established the feasibility and utility of teleconsult for follow up of patients with neurological diseases who were attending the regular outpatient services before the lock down.

Stroke care in India has evolved rapidly in the last decade with a focus on stroke awareness, prevention, rapid triage, treatment, and rehabilitation. But acute stroke care and poststroke rehabilitation in the country have limitations owing to the economic constraints and poor access to health care. The SARS‐CoV‐2/COVID‐19 pandemic has made stroke care even more challenging. We outline the unfavorable circumstances in stroke care induced by the pandemic; propose mitigating measures; crisis management; and provide a comparative evaluation of stroke care between India and the United States during the pandemic. There is a need for public health systems in both developed and developing countries to improve awareness, implement proper strategies of triage, acute treatment, well‐defined rehabilitation plans, telemedicine services, and virtual check‐ins., We outline the unfavorable circumstances in stroke care induced by the SARS‐CoV‐2/COVID‐19 pandemic, propose mitigating measures, crisis management, and provide a comparative evaluation of stroke care between India and United States during the pandemic.

Knowledge related to SARS-CoV-2 or 2019 novel coronavirus (2019-nCoV) is still emerging and rapidly evolving. We know little about the effects of this novel coronavirus on various body systems and its behaviour among patients with underlying neurological conditions, especially those on immunomodulatory medications. The aim of the present consensus expert opinion document is to appraise the potential concerns when managing our patients with underlying CNS autoimmune demyelinating disorders during the current COVID-19 pandemic.

The ongoing pandemic of COVID-19 is a global public health emergency. This has led to challenges for healthcare facilities to optimally manage other important medical emergencies. Stroke is an important public health emergency with significant mortality and morbidity. Timely treatment of acute stroke is critical to prevent disability. The current expert consensus statement on behalf of the Indian Stroke Association outlines the issues and suggestions related to the management of stroke during this ongoing COVID-19 pandemic.

Background: Lack of compliance to medication and uncontrolled risk factors are associated with increased risk of recurrent stroke and acute coronary syndrome in patients with recent stroke. Multimodal patient education may be a strategy to improve the compliance to medication and early adoption of nonpharmacological measures to reduce the vascular risk factor burden in patients with stroke. We thus aim to develop multilingual short messaging services (SMS), print, and audio-visual secondary stroke prevention patient education package. The efficacy of the package will be tested in a randomized control trial to prevent major cardiovascular and cerebrovascular events. Methods: In the formative stage, intervention materials (SMS, video, and workbook) were developed. In the acceptability stage, the package was independently assessed and modified by the stakeholders involved in the stroke patient care and local language experts. The modified stroke prevention package was tested for implementation issues (implementation stage). Results: Sixty-nine SMS, six videos, and workbook with 11 chapters with 15 activities were developed in English language with a mean ± SD SMOG index of 9.1 ± 0.4. A total of 355 stakeholders including patients (24.8%), caregivers (24.8%), doctors (10.4%), nurses (14.1%), local language experts (2.8%), physiotherapists (13.2%), and research coordinators (9.8%) participated in 10 acceptability stage meetings. The mean Patient Education Material Assessment Tool understandability score in all languages for SMS, video scripts, and workbook was 95.2 ± 2.6%, 95.2 ± 4.4%, and 95.3 ± 3.6%, respectively. The patients [n = 20, mean age of 70.3 ± 10.6 years and median interquartile range (IQR) baseline NIHSS 1 (0–3)] or the research coordinators (n = 2) noted no implementation issues at the end of 1 month. Conclusion: An implementable complex multilingual patient education material could be developed in a stepwise manner. The efficacy of the package to prevent major adverse cardiovascular events is being tested in the SPRINT INDIA study.

Governments have imposed lockdowns in the wake of the COVID-19 pandemic. Hospitals have restricted outpatient clinics and elective services meant for non-COVID illnesses. This has led to patients facing unprecedented challenges and uncertainties. This study was carried out to assess patients' concerns and apprehensions about the effect of the lockdown on their treatments.An ambispective, observational cross-sectional single centre study was conducted. Patients were contacted telephonically and requested to answer a structured questionnaire. Their responses were documented and summarized as frequency and proportions.A total of 727 patients were interviewed. Epilepsy (32%) was the most common neurological illness in our cohort followed by stroke (18%). About half the patients and/or their caregivers reported health-related concerns during the lockdown. The primary concern was how to connect with their treating neurologist if need arose. Forty-seven patients (6.4%) had drug default. Among patients on immunomodulatory treatments, only eight patients had drug default. High compliance rates were also observed in the stroke and epilepsy cohorts. Of the 71 patients who required emergency care during the lockdown, 24 could reach our hospital emergency. Fourteen patients either had a delay or could not seek emergency care. Two-thirds of our patients found the telemedicine experience satisfactory.The ongoing pandemic will continue to pose challenges to both physicians and patients. Patients in follow-up may need to be contacted regularly and counselled regarding the importance of maintaining drug compliance. Telemedicine can be used to strengthen the healthcare delivery to patients with non-COVID illnesses.

To study impact of COVID-19 pandemic on frequency, clinical/electrophysiological profile and treatment outcomes in pediatric Guillain-Barré syndrome (GBS).GBS is the most frequent cause of pediatric acute flaccid paralysis. The effect of the COVID-19 pandemic on pediatric GBS is unclear in the literature.We conducted an ambispective, multicentric, cohort study involving 12 of 27 centres in GBS Consortium, during two periods: pre-COVID-19 (March-August 2019) and during COVID-19 (March-August 2020). Children ≤12 years who satisfied National Institute of Neurological Diseases and Stroke criteria for GBS/variants were enrolled. Details pertaining to clinical/laboratory parameters, treatment and outcomes (modified Rankin Scale (mRS) at discharge, GBS Disability score at discharge and 3 months) were analysed.We enrolled 33 children in 2019 and 10 in 2020. Children in 2020 were older (median 10.4 [interquartile range 6.75-11.25] years versus 5 (2.5-8.4) years;The COVID-19 pandemic led to a marked decline in pediatric GBS presenting to hospitals. Antecedent illnesses, clinical and electrophysiological profile of GBS remained largely unchanged from the pre-pandemic era.

Stroke is a leading cause of death and disability worldwide. Aspirin is the most commonly used antiplatelet drug in both primary and secondary prevention of cerebrovascular and cardiovascular diseases. A proportion of patients may have stroke recurrence while they are on treatment with aspirin, giving rise to term aspirin resistance or aspirin failure. Studies have suggested that such recurrence could partly be attributed to biochemical aspirin resistance, with an estimated prevalence ranging between 5% and 65% among patients with ischemic stroke in the published studies. Common methods to evaluate laboratory aspirin resistance include light transmission aggregometry, PFA-100, VerifyNow-Aspirin assay, serum thromboxane B2, and urinary 11-dehydrothromboxane B2. Aspirin resistance is multifactorial in origin and involves diverse environmental and genetic factors, including single-nucleotide polymorphisms, miRNAs, drug interactions, and co-morbid risk factors. The current review overviews the concept of aspirin resistance, its evaluation and relationship with stroke recurrence, its outcome, and its implications on stroke management in the future.

Pompe disease (PD) is a lysosomal storage disorder characterized by glycogen accumulation in muscle, with infantile-onset (IOPD) and late-onset (LOPD) types. Nineteen cases of PD were diagnosed over a 14-year period on muscle biopsy by ultrastructural examination. Pools of glycogen (intralysosomal and cytoplasmic) and excessive phagocytosis were seen in myofibers on electron microscopy. Glycogen was noted in endothelial cells in IOPD. Although PD accounts for a small fraction of muscle diseases, timely accurate diagnosis is imperative as it is treatable. Ultrastructural examination is necessary to confirm the diagnosis in cases with non-diagnostic light microscopic features, especially in adult LOPD patients.

Purpose: To study the retinal nerve fiber layer (RNFL) and ganglion cell layer (GCL) changes on optical coherence tomography in early multiple sclerosis (MS) patients. Methods: A prospective cohort study was conducted at a tertiary care center. Patients of early MS (expanded disability status scale

LETM is a common syndrome and the diagnosis of CNS lymphoma is not usually considered in the list of differentials. Primary CNS lymphoma can present as longitudinally extensive transverse myelopathy. Failure to suspect and evaluate leads to delay in diagnosis and treatment. PCNSL may be non contrast enhancing on gadolinium enhanced MRI. CSF analysis should be done preferably before starting corticosteroids as it is usual practice in treatment of transverse myelitis, as steroids may lead to transient improvement and mask the correct diagnosis. Repeated CSF examinations may be needed to clinch the diagnosis.

Purpose Our objective was to assess how telephonic review of outpatients with stable epilepsy compared with conventional face-to-face clinic management. Methods We constructed a randomized parallel group study of suitable patients attending our Epilepsy Clinic and compared telephonic review with conventional clinic visit based management. Primary outcomes were the percentage of patients with breakthrough seizures and total number of breakthrough seizures. We also compared cost, patient satisfaction and numbers defaulting. Results A total of 465 patients were randomized and 429 were included in the final analysis. There was no significant difference in breakthrough seizures between the two groups. Mean time spent in the consultation was 10min in the telephone group (FT) and 22h in the face-to-face group (FC) and cost was INR 865 more expensive on an average in the FC group. Satisfaction was over 90% in the FT group. Significantly more people in the FC group were lost to follow-up. Conclusion This study provides Class I evidence that the number of stable epilepsy patients who have breakthrough seizures and the total number of breakthrough seizures remain the same irrespective of whether patients are reviewed telephonically or face-to-face in the clinic. Clinicians managing epilepsy patients should consider using telephonic review for selected patients. Telephonic reviews have the potential of effectively reducing the secondary treatment gap in millions of patients who do not have easy access to doctors.

We report the case of a pregnant female presenting with behavioral change and hallucinations followed by focal seizures with impaired awareness. EEG revealed generalized slowing interspersed with extreme delta-brush pattern and MRI brain was normal. Both Serum and CSF anti-N-methyl-d-aspartate receptor (NMDAR) antibodies were positive. Patient had a prolonged hospital stay with full recovery and delivered a healthy baby, highlighting the significance of early diagnosis and management in this disorder.

Objectives Stroke constitutes a significant public health problem in developing countries. Caregivers provide an important support system for patient care but usually lack knowledge and skill to attend their stroke patients. We assessed whether a caregiver-directed educational intervention would reduce hospital-acquired complications and improve stroke patients’ outcomes. Materials and methods We randomly assigned two Neurology inpatient wards to receive either standard care or an educational intervention. The coprimary outcomes included incidence of hospital-acquired complications and in-hospital mortality. Secondary outcomes included the modified Rankin Scale and mortality at three months. Results Among 164 patients recruited, 82 received intervention, and standard care each. The mean (Standard deviation) Glasgow coma scale of patients was 11.01 (3.4), and National Institute of Health Stroke Scale was 19.17 (8.54). The incidence of complications (72 in the intervention versus 81 in the control group; p=0.56) was not different. Ten patients (12.2%) in the intervention group and 16 (19.5%) in the control group (p=0.20) died in-hospital. Twenty patients (27.8%) in the intervention and twelve (18.2%) in the control group attained modified Rankin Scale 0-2 at three months (p=0.12). The mortality at three months (20 [24.4%] in the intervention versus 25 [30.5%] in the control group) was not different (p=0.38). The intervention group had fewer complications (42 versus 68 in the control group; p=0.01) during the initial ten days of hospital stay, but adjusted analysis revealed no difference. Conclusion A structured educational intervention did not reduce the incidence of hospital-acquired complications, mortality, or morbidity. However, there was a trend towards fewer complications in the initial days of hospital stay. Extended hospital stay, caregiver fatigue, and dilution of the intervention over time might be reasons for the apparent lack of effect. Clinical trial registration-URL http://www.ctri.nic.in . Unique identifier: CTRI/2018/11/016312.