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We wanted to evaluate if optical coherence tomography angiography OCTA findings could predict the functional outcome in extracranial carotid artery atherosclerotic disease (ECAD) associated stroke. This exploratory study was performed on adults with acute ischaemic stroke due to ECAD at 3–6 weeks following stroke onset with risk factor matched controls without carotid artery stenosis. Twenty-three stroke patients (cases) and 23 controls were enrolled. There was significant difference between cases and controls in deep vessel density at the macula (p =.0007) and in radial peripapillary capillary perfusion density (RPCPD) at the optic nerve head (ONH) (p =.0007). Statistically significant difference was noted in the total superficial vessel density (SVD) at the macula (SVD within 1 standard deviation [SD] versus SVD beyond 1 SD of control data) in the ipsilateral eye and functional outcome at 3 months (poor versus very good outcome, modified Rankin scale [mRS] 0–1 versus mRS 2–6, respectively; p =.0361). There was statistically insignificant correlation between the RPCPD at the ONH and the National Institutes of Health Stroke Scale score at admission, mRS at discharge, and mRS at 3 months following stroke onset (r =.33, r =.35, r =.39; p =.11, p =.09, p =.06, respectively). The findings of this exploratory study suggested that OCTA findings may predict 3 month outcomes in cases of ECAD-related stroke and could be useful in decision making in future intervention studies as to whether intervene or not in patients having critical or non-critical ECAD for preventing stroke. [ABSTRACT FROM AUTHOR]

Background and Aims: Tenecteplase is used as the standard of care treatment for thrombolysis in acute ischemic stroke (AIS) patients within 4.5 h of symptom onset. Documented reports were less certain to claim the benefits of it in an extended window period. EAST-AIS (CTRI/2022/03/040718) trial is designed to determine the success rate of thrombolysis in an extended window period for good clinical outcomes. Study Design: It is a randomized, placebo-controlled trial of tenecteplase administered within 4.5-24 h of stroke onset (with or without large vessel occlusion) based on evidence of salvageable tissue through baseline computed tomography perfusion (CTP) or magnetic resonance imaging (MRI) scan. Criteria of patient inclusion are as follows: patients of both genders (male and female), age >18 years, pre-stroke modified Ranking Scale (mRS) <2, baseline NIHSS >5, CTP showing penumbra--ischemic core ratio >1.8, absolute difference in volume >10 ml, and ischemic core volume <70 ml. The sample size for the study is 100 patients: 50 in the tenecteplase arm (0.25 mg/kg body weight; maximum- 25 mg) and 50 in the placebo arm (controls). Study Outcomes: The study's primary objective is safety endpoints along with the efficacy of tenecteplase assessed using the mRS score at 90 days of stroke onset. Conclusion: The result obtained from EAST-AIS will determine the safety and efficacy of tenecteplase injection administered 4.5-24 h following the symptom onset for AIS patients within the territory of Internal Carotid Artery (ICA), Middle Cerebral Artery (MCA), or Anterior Cerebral Artery (ACA) occlusion. [ABSTRACT FROM AUTHOR]

Background: Acute kidney injury (AKI) is prevalent in patients with acute stroke. Although AKI is linked to poor clinical outcomes, data about its incidence and effect on stroke outcomes is limited. Methods: This was a prospective observational study carried out at a single tertiary care center that analyzed the data of 204 consecutive subjects with acute ischemic stroke and intracerebral hemorrhage. Considering serum creatinine at admission as the baseline, AKI was defined as a rise in serum creatinine value of 0.3 mg/dl over 48 h or a percentage increase of at least 50% from baseline over 7 days during hospitalization. The primary outcome was to measure the prevalence of AKI in patients with acute stroke. Secondary outcome measures were all-cause mortality, duration of hospital stay, need for dialysis, and comparison of outcomes in ischemic and hemorrhagic stroke. For both the stroke subtypes, we employed a multivariate logistic regression model, with AKI and hospital mortality being the outcomes. Covariates included gender, age, ventilatory requirement, duration of hospital stay, and National Institutes of Health Stroke Scale score at admission. Results: There were 144 cases of ischemic stroke with 12 deaths (8.3%) and 60 cases of intracranial hemorrhage (ICH) with 22 deaths (36.7%). The mean age was 55 years, 72.6% were males, and AKI complicated 34% of ischemic stroke and 66.7% of ICH hospitalizations. AKI was linked to increased hospital mortality from ischemic stroke (odds ratio [OR] 27.21, 95% CI 3.39-218.13) and hemorrhagic stroke (OR 5.12, 95% CI 1.29-20.28) in multivariate analysis stratified by stroke type. Conclusions: AKI complicates stroke frequently and increases hospital mortality. Additional studies are required to assess if the association is causal and if remedies to prevent AKI would decrease mortality. [ABSTRACT FROM AUTHOR]

The article focuses on cervical artery dissection (CAD), a condition where blood collects between the arterial wall layers, leading to a false lumen and mural hematoma. Topics include the multifactorial causes and symptoms of CAD, diagnostic imaging features like the crescent sign and double lumen, and the management approach involving antithrombotic therapy, with a particular focus on a study analyzing demographic and outcome data from CAD patients in India.

The gold standard for facioscapulohumeral muscular dystrophy (FSHD) genetic diagnostic procedures was published in 2012. With the increasing complexity of the genetics of FSHD1 and 2, the increase of genetic testing centers, and the start of clinical trials for FSHD, it is crucial to provide an update on our knowledge of the genetic features of the FSHD loci and renew the international consensus on the molecular testing recommendations. To this end, members of the FSHD European Trial Network summarized the evidence presented during the 2022 ENMC meeting on Genetic diagnosis, clinical outcome measures, and biomarkers. The working group additionally invited genetic and clinical experts from the USA, India, Japan, Australia, South‐Africa, and Brazil to provide a global perspective. Six virtual meetings were organized to reach consensus on the minimal requirements for genetic confirmation of FSHD1 and FSHD2. Here, we present the clinical and genetic features of FSHD, specific features of FSHD1 and FSHD2, pros and cons of established and new technologies (Southern blot in combination with either linear or pulsed‐field gel electrophoresis, molecular combing, optical genome mapping, FSHD2 methylation analysis and FSHD2 genotyping), the possibilities and challenges of prenatal testing, including pre‐implantation genetic testing, and the minimal requirements and recommendations for genetic confirmation of FSHD1 and FSHD2. This consensus is expected to contribute to current clinical management and trial‐readiness for FSHD. [ABSTRACT FROM AUTHOR]

Introduction: Guidelines recommend screening older people (> 60–65 years) with type 2 diabetes (T2D) for cognitive impairment, as it has implications in the management of diabetes. The Montreal Cognitive Assessment (MoCA) is a sensitive test for the detection of mild cognitive impairment (MCI) in the general population, but its validity in T2D has not been established. Methods: We administered MoCA to patients with T2D (age ≥ 60 years) and controls (no T2D), along with a culturally validated neuropsychological battery and functional activity questionnaire. MCI was defined as performance in one or more cognitive domains ≥ 1.0 SD below the control group (on two tests representing a cognitive domain), with preserved functional activities. The discriminant validity of MoCA for the diagnosis of MCI at different cut-offs was ascertained. Results: We enrolled 267 patients with T2D and 120 controls; 39% of the participants with T2D met the diagnostic criteria for MCI on detailed neuropsychological testing. At the recommended cut-off on MoCA (< 26), the sensitivity (94.2%) was high, but the specificity was quite low (29.5%). The cut-off score of < 23 showed an optimal trade-off between sensitivity (69.2%), specificity (71.8%), and diagnostic accuracy (70.8%). The cut-off of < 21 exhibited the highest diagnostic accuracy (74.9%) with an excellent specificity (91.4%), a good positive and negative predictive value (78.5% and 73.7%, respectively). Conclusions: The recommended screening cut-off point on MoCA of < 26 has a suboptimal specificity and may increase the referral burden in memory clinics. A lower cut-off of < 21 on MoCA maximizes the diagnostic accuracy. Interactive Visual Abstract available for this article. Plain Language Summary: Type 2 diabetes (T2D) is a risk factor for cognitive dysfunction which potentially impacts diabetes self-management skills. Guidelines recommend screening older adults with diabetes for early detection of cognitive impairment. For screening cognitive impairment in busy endocrine clinics, we need a test that is easy and rapid to administer, sensitive enough to pick the cognitive deficits of T2D and at the same time gives less false-positive outcomes. The Montreal Cognitive Assessment (MoCA) scale is a widely available cognitive screening tool, but there are no studies evaluating its discriminant properties in people with diabetes. We evaluated the performance metrics of MoCA in this population. We found mild cognitive impairment in four out of ten participants with T2D at or above 60 years of age. At the recommended cut-off on MoCA (< 26), the sensitivity was high, but the specificity quite low. We found better diagnostic accuracy at lower cut-offs (20/21), with high specificity but a lower sensitivity. At this cut-off, approximately one out of five people screened using MoCA would require detailed neuropsychological testing, and four out of five who undergo detailed evaluation would have true cognitive impairment. [ABSTRACT FROM AUTHOR]

Background and aims: Early neurological deterioration (END) in acute ischemic stroke (AIS), patients is defined as clinical worsening or recurrence during first 72 h after onset of AIS. We have conducted this study to determine the association between END and functional outcome at 3 months of onset of AIS along with associated risk factors of END in AIS cases. Methodology: This study was conducted after approval of Institute Ethics Committee. Two hundred three consecutive patients were admitted from September 2020 to January 2022 at a tertiary care hospital. One hundred ninety patients were included in the study; patients were divided into two groups: (1) early neurological deterioration (END) and (2) non-early neurological deterioration (non-END). Patients were followed-up either telephonically or in person at approximately 3 months using modified Rankin Scale 0–6. All the clinically significant prognostic markers and p < 0.10 variables were considered significant in univariate analysis; P < 0.05 were considered statistically significant for the multivariate analysis. Results: Out of 190 cases included in the cohort 34/190 (17.8%) cases showed END with mean age (56.56 (± 16.6)) and males (20/34 (58.8%)). END was independently associated with high blood glucose at admission (OR = 1.015; P = 0.002; 95%CI = 1.005–1.024) and low serum albumin (OR = 0.208; P = 0.002; 95%CI = 0.077–0.562). Patients with END showed poor functional outcome (mRS > 2) at end of 3 months (32 (94.1%); P < 0.001) and death was also statistically significant (22 (64.7%); P < 0.001) as compared to AIS cases having non-END. Conclusion: Our study showed END may be associated with poor functional outcome in AIS patients. Higher blood glucose at admission and low serum albumin may be statistically significant causing END. Future prospective cohort with larger sample size may confirm the findings. [ABSTRACT FROM AUTHOR]

Purpose: Inter-rater reliability is a critical aspect of stroke image interpretation. This study aims to investigate inter-rater reliability between neurologists and neuro-radiologist when assessing Alberta Stroke Program Early CT Score (ASPECTS) and Intracerebral Haemorrhage (ICH) scores using a mobile application (SMART INDIA App – by neurologists) and the Picture Archiving and Communication System (PACS – by neuro-radiologist). Methods: Adult patients diagnosed with ischemic or haemorrhagic stroke were included in this study. Two Neurologists (R1 and R2) assessed the ASPECTS and ICH scores by viewing the SMART INDIA App. A neuroradiologist expert (R3) assessed the same using PACS. Kappa statistics are presented for agreement between the Raters. Results: 100 consecutive patients each of Acute Ischemic stroke (AIS) and ICH were included. A significant agreement in the total ASPECTS between the Raters 1 and 2 (0.85(95% CI—0.775,0.926)), Raters 1 and 3(0.76(95% CI—0.671,0.857)) and Raters 2 and 3(0.76(95% CI—0.673,0.857)) was noted. A good agreement was ascertained between Rater 1 and 3, even though the devices were different. A similar excellent agreement was also noted in assessing the ICH score. The study's findings indicate that neurologists using different devices and platforms demonstrated good to excellent agreement with the neuro-radiologist (considered the gold standard) when estimating ASPECTS and ICH scores. Conclusion: The interpretations of neurologists using the SMART INDIA App can be deemed valid and reliable from the neuroimaging viewpoint. These results contribute to our understanding of the feasibility and reliability of app-based image evaluation in the assessment of stroke. [ABSTRACT FROM AUTHOR]

Stroke is the second leading cause of death and a major cause of disability worldwide. Stroke severity scales serve as reliable means to track a patient's neurological deficit, predict outcome, and guide treatment decisions in clinical practice. The National Institute of Health Stroke Scale (NIHSS) was introduced over 30 years ago, marking a significant milestone in the field of stroke. Over the years, there have been notable advancements in acute stroke care. Despite several modifications made to NIHSS, none has yet succeeded in effectively capturing all the complex effects of a stroke. This review focuses on the pitfalls of NIHSS and emphasizes the need for a quick and comprehensive clinical and upgraded version of the stroke severity rating scale. [ABSTRACT FROM AUTHOR]

There is a growing awareness of the significance of using minimum clinically important differences (MCIDs) in stroke research. An MCID is the smallest change in an outcome measure that is considered clinically meaningful. This review is the first to provide a comprehensive summary of various scales and patient-reported outcome measures (PROMs) used in stroke research and their MCID values reported in the literature, including a concise overview of the concept of and methods for determining MCIDs in stroke research. Despite the controversies and limitations surrounding the estimation of MCIDs, their importance in modern clinical trials cannot be overstated. Anchor-based and distribution-based methods are recommended for estimating MCIDs, with patient self-evaluation being a crucial component in capturing the patient's perspective on their health. A combination of methods can provide a more comprehensive understanding of the clinical relevance of treatment effects, and incorporating the patient's perspective can enhance the care of stroke patients. [ABSTRACT FROM AUTHOR]

Facioscapulohumeral muscular dystrophy (FSHD) is the second most common muscular dystrophy in adults, and it is associated with local D4Z4 chromatin relaxation, mostly via the contraction of the D4Z4 macrosatellite repeat array on chromosome 4q35. In this study, we aimed to investigate the use of Optical Genome Mapping (OGM) as a diagnostic tool for testing FSHD cases from the UK and India and to compare OGM performance with that of traditional techniques such as linear gel (LGE) and Pulsed-field gel electrophoresis (PFGE) Southern blotting (SB). A total of 6 confirmed and 19 suspected FSHD samples were processed with LGE and PFGE, respectively. The same samples were run using a Saphyr Genome-Imaging Instrument (1-color), and the data were analysed using custom EnFocus FSHD analysis. OGM was able to confirm the diagnosis of FSHD1 in all FSHD1 cases positive for SB (n = 17), and D4Z4 sizing highly correlated with PFGE-SB (p < 0.001). OGM correctly identified cases with mosaicism for the repeat array contraction (n = 2) and with a duplication of the D4Z4 repeat array. OGM is a promising new technology able to unravel structural variants in the genome and seems to be a valid tool for diagnosing FSHD1. [ABSTRACT FROM AUTHOR]

Primary coenzyme Q10 (CoQ10) deficiency is a rare genetic disorder that affects mitochondrial respiration and can lead to various symptoms such as cerebellar ataxia, intellectual disability, seizures, and more. The most common form of this disorder is primary CoQ10 deficiency-4 (COQ10D4), which is caused by a variant in the CoQ8A gene. This article presents a case study of a young male with slowly progressive cerebellar ataxia and dystonia caused by primary CoQ10 deficiency-4. Early supplementation of CoQ10 may result in favorable outcomes, so a timely diagnosis is essential. [Extracted from the article]

Azathioprine (AZA) has demonstrated efficacy in multiple randomized control trials (RCTs) for Relapsing-Remitting Multiple Sclerosis (RRMS). However, we still need comparative real-world data with other first-line disease-modifying therapies (DMTs). We aimed to assess AZA's effectiveness regarding relapses, disability progression, time to the first relapse, magnetic resonance imaging (MRI) activity, and safety compared with other approved first-line DMTs in an Indian population in a real-world setting. We conducted a single-center prospective study of treatment-naive RRMS patients between 2017 and 2019. We evaluated the effects of AZA and other approved DMTs on clinical and radiological measures. Among 192 eligible patients (F:M ratio 2.84:1), 68 patients (35.4%) were on AZA, 68 patients (35.4%) were on dimethyl fumarate (DMF), 32 patients (16.7%) on interferon (IFN beta-1a), and 16 patients (8.3%) on teriflunomide (TFL). Four treatment groups were comparable: AZA v/s DMF v/s TFL v/s IFN beta-1a. In primary outcomes, there was no significant difference between the groups in terms of change in the Expanded Disability Status Scale (EDSS) score at three months (p-value = 0.169), six months (p-value = 0.303), 12 months (p-value = 0.082), and 24 months (p-value = 0.639), the number of relapses (p-value = 0.229), and time to the first relapse (p-value > 0.05 in all groups). In the secondary outcome, there was no significant difference between the treatment groups on serial MRI parameters used according to "Magnetic Resonance Imaging in Multiple Sclerosis" (MAGNIMS) 2016 criteria (p-value > 0.05). In safety outcomes, leukopenia was significantly more common in the AZA group (p-value = 0.025), flu-like symptoms (p-value = 0.0001), and injection site reactions (p-value = 0.035) were significantly more common in the IFN beta-1a group. Our study suggests AZA is as effective as other approved DMTs and a good alternative as a first-line treatment for multiple sclerosis's clinical and radiological activity in real-world settings on short follow-up. Based on these results, more randomized controlled trials of AZA v/s DMF or other DMTs are needed for more robust outcomes. [ABSTRACT FROM AUTHOR]

Background: Neuropathic Tremor (NT) is a postural/kinetic tremor of the upper extremity, often encountered in patients with chronic neuropathies such as paraprotein‐associated and hereditary neuropathies. Objectives: To describe the clinical and electrophysiological features of NT in a previously underrecognized setting‐ during recovery from Guillain‐Barré Syndrome (GBS). Methods: Patients with a documented diagnosis of GBS in the past, presenting with tremor were identified from review of clinical records. Participants underwent structured, videotaped neurological examination, and electrophysiological analysis using tri‐axial accelerometry‐surface electromyography. Tremor severity was assessed using the Fahn‐Tolosa‐Marin Tremor Rating Scale. Results: We describe the clinical and electrophysiological features of 5 patients with GBS associated NT. Our cohort had a fine, fast, and slightly jerky postural tremor of frequency ranging from 8 to 10 Hz. Dystonic posturing and overflow movements were noted in 4/5 patients. Tremor appeared 3 months–5 years after the onset of GBS, when patients had regained near normal muscle strength and deep tendon jerks were well elicitable. Electrophysiological analysis of tremor strongly suggested the presence of a central oscillator in all patients. Conclusion: NT is not limited to chronic inflammatory or hereditary neuropathies and may occur in the recovery phase of GBS. The tremor is characterized by a high frequency, jerky postural tremor with dystonic posturing. Electrophysiological evaluation suggests the presence of a central oscillator, hypothetically the cerebellum driven by impaired sensorimotor feedback. [ABSTRACT FROM AUTHOR]

Antinuclear antibodies (ANA) are a diverse group of autoantibodies found in various systemic autoimmune disorders. They represent a key diagnostic marker in the diagnosis of connective tissue disorders (CTD). Although many techniques exist, ANA by indirect immunofluorescence remains the gold standard for diagnosing CTDs. Neurologists should be aware of the type of assay used for detection and the advantages and disadvantages of using each method. Through this article, we aimed to review the methodological aspects of the detection of ANA and its subtypes and their clinical relevance in various neurologic disorders. [ABSTRACT FROM AUTHOR]

Background: Stroke is a leading cause of death and disability globally. Over the last decade, digital health and related technology has emerged as a useful adjunct in the management of persons with stroke, particularly with the development of a large number of mobile phone applications dedicated to various aspects of stroke. However, whether social media can provide similar key support in stroke is an intriguing question. In this systematic review, we aimed to the scope and limits of social media platforms in care and research pertinent to persons with stroke. Methods: PubMed database was searched using Medical Subject Headings terms and exploded keywords. The search retrieved 556 abstracts, which were screened by two reviewers. Of these, 14 studies met the review inclusion criteria. Given the small number of studies and heterogeneity of outcomes, quantitative analysis was not possible. The review was registered on PROSPERO (CRD42022324384). Results: The social media platforms employed by the included studies comprised YouTube (n = 5), Twitter (n = 5), Facebook (n = 2), both Twitter and Facebook (n = 1), and WhatsApp (n = 1). Four assessed quality and accuracy of videos on YouTube available for stoke patients and caregivers. Three used social media to research link between role of gender and stroke descriptors on social media platforms, and one studied Twitter-derived racial/ethnic perceptual construction on the occurrence of cardiovascular disease. Three studies described use of social media by stroke survivors, in post-stroke care and engagement. 11 studies were assessed to be of "fair" quality and three were assessed to be of "poor" quality. Conclusions: Limited preliminary data of low quality indicates that social media is used by persons with stroke and their caregivers, and may be harnessed as a tool of education and research. Future studies must address the current lack of high-quality evidence for the use of social media in stroke care. [ABSTRACT FROM AUTHOR]

Background: Bacopa monnieri (BM), a commonly used herb, has shown neuroprotective effects in animal and in vitro studies; but human studies on patients with Alzheimer's Disease (AD) have been inconclusive. Further high-quality trials are required to conclusively state the utility of BM in AD and other neurodegenerative dementias. Methods: In the present study, we did a narrative review of the current challenges in designing clinical trials of BM in dementia and their evidence-based recommendations. Results: Many facets of the BM trials need improvement, especially effect size and sample size estimation. Current assessment and outcomes measures need a more holistic approach and newer scales for diagnosing and monitoring prodromal AD. The stringent guidelines in CONSORT and STROBE are often considered difficult to implement for clinical trials in ayurvedic medications like BM. However, adherence to these guidelines will undoubtedly improve the quality of evidence and go a long way in assessing whether BM is efficacious in treating AD/prodromal AD patients and other neurodegenerative dementias. Conclusion: Future studies on BM should implement more randomized controlled trials (RCTs) with an appropriate sample size of accurately diagnosed AD/prodromal AD patients, administering a recommended dosage of BM and for a pre-specified time calculated to achieve adequate power for the study. Researchers should also develop and validate more sensitive cognitive scales, especially for prodromal AD. BM should be evaluated in accordance with the same rigorous standards as conventional drugs to generate the best quality evidence. [ABSTRACT FROM AUTHOR]

Background: Social cognition is the study of how people make sense of themselves and others. Impairment in several domains of social cognition is increasingly being recognized in Parkinson's disease (PD). Objectives: We aimed to study multiple domains of social cognition in Indian PD patients using a culturally appropriate, validated instrument. Methods: We recruited 52 individuals with PD and 31 healthy volunteers (HV) and used the Social Cognition Rating Tools in Indian Setting (SOCRATIS) tool to assess theory of mind (ToM), attributional biases and social cue perception. Quality of life (QoL) was assessed using the PDQOL scale. Results: Baseline characteristics were comparable between PD and HV. The mean (SD) FOT index (first order ToM index) was 0.86(0.18) in PD and 0.99(0.07) in HV [P < 0.001]. The PD group showed higher Externalizing Bias [EB, 4.42(3.91)], compared to HV [1.58(3.22), P = 0.001]. The mean (SD) Faux Pas Composite Index (FPCI ALT) was 0.69(0.09) in PD and 0.78(0.13) in HV [P < 0.001]. Social cognition indices were not associated with QoL in PD. Clinical parameters—age, gender, HAM‐D, MOCA, education, levodopa equivalent daily dose of medication, number of PD drugs and trihexyphenidyl use did not predict social cognition. Conclusion: PD patients were less successful than age, gender matched controls in understanding social situations and other's thought processes and had higher tendency to attribute undesirable events to external causes. Deficits in social cognition did not impair the quality of life. [ABSTRACT FROM AUTHOR]

Cerebral collateral circulation refers to the auxiliary vascular structures which compensate cerebral blood flow when it has been compromised due to stenosis or occlusion of the principal supplying arteries. They play a vital role in sustaining blood flow to the ischemic areas in acute, subacute or chronic phases of ischemic stroke or TIA. Good collateral circulation has shown protective effects towards a favorable functional outcome and a lower risk of recurrence of stroke. The benchmark mechanical thrombectomy trials utilized these collateral scoring methods to guide patient selection and prognosticate favorable outcome models. This shows a promising future of the collateral circulation for extending the time frame of the reperfusion therapies by optimally guiding patient selection and moving from a "time window" to a "tissue window." [ABSTRACT FROM AUTHOR]

OBJECTIVE: To develop an automated algorithm to detect, quantify, and differentiate between tremor using pen-on-paper spirals. METHODS: Patients with essential tremor (n = 25), dystonic tremor (n = 25), Parkinson’s disease (n = 25), and healthy volunteers (HV, n = 25) drew free-hand spirals. The algorithm derived the mean deviation (MD) and tremor variability from scanned images. MD and tremor variability were compared with 1) the Bain and Findley scale, 2) the Fahn–Tolosa–Marin tremor rating scale (FTM–TRS), and 3) the peak power and total power of the accelerometer spectra. Inter and intra loop widths were computed to differentiate between the tremor. RESULTS: MD was higher in the tremor group (48.9±26.3) than in HV (26.4±5.3; p < 0.001). The cut-off value of 30.3 had 80.9% sensitivity and 76.0% specificity for the detection of the tremor [area under the curve: 0.83; 95% confidence index (CI): 0.75, 0.91, p < 0.001]. MD correlated with the Bain and Findley ratings (rho = 0.491, p = 0 < 0.001), FTM–TRS part B (rho = 0.260, p = 0.032) and accelerometric measures of postural tremor (total power, rho = 0.366, p < 0.001; peak power, rho = 0.402, p < 0.001). Minimum Detectable Change was 19.9%. Inter loop width distinguished Parkinson’s disease spirals from dystonic tremor (p < 0.001, 95% CI: 54.6, 211.1), essential tremor (p = 0.003, 95% CI: 28.5, 184.9), or HV (p = 0.036, 95% CI: -160.4, -3.9). CONCLUSION: The automated analysis of pen-on-paper spirals generated robust variables to quantify the tremor and putative variables to distinguish them from each other. SIGNIFICANCE: This technique maybe useful for epidemiological surveys and follow-up studies on tremor. [ABSTRACT FROM AUTHOR]

Background and Purpose: We aimed to explore the characteristics, clinical features, predictors of seizure, and associated clinical outcomes in patients with cerebral venous thrombosis (CVT). Methods: We enrolled patients with CVT from January 2014 to July 2020. Prospectively patients were recruited from December 2018. We analyzed predictors of seizures and associated good functional outcomes (modified Rankin Scale, mRS: 0-2) using multivariable logistic regression. Results: We enrolled 153 patients with CVT in which 77 (50%) had presented with a seizure. The median age was 31 years (IQR 16 46), and the majority were men (73.2%). Focal to bilateral tonic clonic was the most common seizure type (27%), followed by generalized seizures (22%). None of the patients had status epilepticus. Antiseizure medications (ASM) were used in 71% of patients at diagnosis, 42% having received them prophylactically. Supratentorial parenchymal involvement was seen in 72% of seizure patients compared to 38% in those without, and superior sagittal sinus was most commonly involved. Percentage of patients who achieved good clinical outcome (mRS 0 2) at 3 months did not vary significantly between both groups. The only predictor for seizures with CVT was the presence of a parenchymal lesion (OR 3.75, 95% CI 1.79 7.85), whereas seizure occurrence (OR 12.55, 95% CI 1.53 102.59) was associated with statistically significant risk for recurrent seizures, by multiple logistic regression analysis. Seizure occurrence was not associated with adverse functional outcomes. Conclusion: Seizures at presentation occurred in 50% of patients with CVT which was associated with a parenchymal lesion in the brain. There was no association between seizure at presentation and clinical outcome. [ABSTRACT FROM AUTHOR]

Objectives: Unbiased and full disclosure of trial results is vital to evidence‐based medicine. Non‐publication and selective publication leads to publication bias and unrealistic risk–benefit ratio. In the present study, we aim to determine the publication rate of clinical trials related to neurology registered with the Clinical Trial Registry of India (CTRI), compare the characteristics of published and unpublished trials, and evaluate the adherence of investigators to ethics‐approved criteria and outcomes. Materials and Methods: A cross‐sectional search using the keyword "neurology" was carried out in CTRI registry. Two independent investigators searched Pubmed, Medline, Scopus, and Google Scholar for published manuscripts. The final literature search occurred in November 2021. Results: Out of 325 trials, 102 trials were published (31.4%). Ninety‐one trials were beyond 3 years of expected time of trial completion and were still unpublished. Randomized trials had a slightly higher publication rate than non‐randomized ones (56% vs. 46%, p =.223); however the difference was not statistically significant. Majority of trials sponsored by pharmaceutical companies were not published, while majority of those sponsored by non‐pharmaceutical institutions were published (34.5% vs. 69.3%, p <.001). Feedback to CTRI about trial status was particularly poor (31.5% ‐ informed vs. 68.5% ‐ not informed, p <.001). 52 (50.9%) and 65 (63.7%) of the 102 published trials had changed the registered inclusion and exclusion criteria, respectively, in the CTRI registry compared to those in the published manuscript. In 29 (28.3%) of the 102 trials, the primary outcome did not match with that registered in the CTRI and in 73 (57.8%) trials, the secondary outcomes did not match. Conclusion: A large proportion of neurology registered trials are still unpublished, with a majority of pharmaceutical company–sponsored trials not being published. There is scope for improving the provisions in CTRI for enlisting trial results, that may prevent publication bias and also ensure the investigators adhere to the pre‐specified ethics approved trial procedures and outcomes. [ABSTRACT FROM AUTHOR]

Background and Aims: Bruns-Garland syndrome (BGS) continues to be a contentious topic even 100 years after its discovery. Its lifelong incidence is 1% amongst diabetic individuals and affects middle aged-elderly individuals with type 2 diabetes mellitus (usually not poorly controlled). Methods: The data presented in this review was collated from studies published on PubMed, MEDLINE and Google Scholar in October 2021. The search words included: "Bruns-Garland syndrome", "diabetic amyotrophy" and "diabetic lumbosacral radiculoplexus neuropathy" and "proximal diabetic neuropathy". Results: The exact pathophysiology is debatable but an ischemic pathology (non-systemic microvasculitis) is most plausible. Its cardinal symptoms include acute onset of severe proximal lower extremity pain followed by weakness and wasting, some sensory loss, weight loss and autonomic symptoms. Conclusion: The prognosis is good as most patients improve to near- normal strength with pain cessation within 18 months of onset. [ABSTRACT FROM AUTHOR]

Anoctaminopathies are a group of autosomal recessive skeletal muscle disorders with various clinical phenotypes, caused by anoctamin 5 (ANO5) gene mutations and the abnormal expression of ANO5 protein. Patients with recessive mutations in ANO5 present with variable symptoms ranging from asymptomatic hyperCKemia and exercise-induced myalgia to proximal and/or distal muscle weakness. Here, we describe the clinical, pathological, and molecular findings of two unrelated patients with ANO5-related muscular dystrophy (MD). Ninety-six histologically identified MD cases were subjected to next-generation sequencing using a customized panel of 54 genes (IIlumina Design Studio). Two patients were diagnosed with ANO5-related MD. One patient had a pathogenic homozygous mutation of c.1406G>A in exon 14, while the other patient had a novel heterozygous mutation of c.2141C>G in exon 19 of ANO5 gene. Both showed two different phenotypes (limb girdle MD and Miyoshi myopathy) and histomorphological patterns. Muscle biopsy of one patient in addition showed amyloid deposit in the walls of interstitial blood vessels. ANO5-related MD is a heterogeneous disease with different clinical phenotypes as well as genotypes. All muscle biopsies with unclassified muscular dystrophies should be subjected to Congo red stain. The results of this study suggest that screening for ANO5 gene should represent an early step in the diagnostic work-up of the patients with undiagnosed MD and persistent asymptomatic hyperCKemia, even when muscle biopsy histomorphology is normal. [ABSTRACT FROM AUTHOR]

Purpose: Drawing differential diagnoses to a Neuro-ophthalmology clinical scenario is a difficult task for a neurology trainee. The authors conducted a study to determine if a mobile application specialized in suggesting differential diagnoses from clinical scenarios can complement clinical reasoning of a neurologist in training.Methods: A cross-sectional multicenter study was conducted to compare the accuracy of neurology residents versus a mobile medical app (Neurology Dx) in drawing a comprehensive list of differential diagnoses from Neuro-ophthalmology clinical vignettes. The differentials generated by residents and the App were compared with the Gold standard differential diagnoses adjudicated by experts. The prespecified primary outcome was the proportion of correctly identified high likely gold standard differential diagnosis by residents and App.Results: Neurology residents (n = 100) attempted 1500 Neuro-ophthalmology clinical vignettes. Frequency of correctly identified high likely differential diagnosis by residents was 19.42% versus 53.71% by the App (P < 0.0001). The first listed differential diagnosis by the residents matched with that of the first differential diagnosis adjudicated by experts (gold standard differential diagnosis) with a frequency of 26.5% versus 28.3% by the App, whereas the combined output of residents and App scored a frequency of 41.2% in identifying the first gold standard differential correctly. The residents correctly identified the first three and first five gold standard differential diagnosis with a frequency of 17.83% and 19.2%, respectively, as against 22.26% and 30.39% (P < 0.0001) by the App.Conclusion: A ruled based app in Neuro-ophthalmology has the potential to complement a neurology resident in drawing a comprehensive list of differential diagnoses. [ABSTRACT FROM AUTHOR]

Background: Disability evaluation as per World Health Organization includes assessment of impairments, activity limitations, and participation restriction, which unfortunately is not assessed by the existing guidelines of disability in India.Aim: The aim of this study wasto comparea new study criterion with the existing guidelines for assessing cognitive disability for chronic neurological conditions.Methodology: A cross-sectional pilot study was conducted on 41 participants. They were assessed on an existing guideline (Gazette India 200,1 assessing physical domain and Intelligence Quotient[IQ]) and study criterion assessing three aspects of cognitive capacity: IQ, neurocognitive functioning, and QOL.Results: The existing guideline underestimated 84% of cases for disability. The average percentage of disability measured by the study criterion was 33.2% more as compared to existing guidelines with S.D of 26.6.Conclusion: Cognitive capacity assessment is an important element to be measured in chronic neurological disability certification. However, a large sample is required to make an affirmative claim for the same. [ABSTRACT FROM AUTHOR]

Hirayama disease is a relatively unusual cause of cervical myelopathy characterized by uni- or bilateral asymmetric weakness, fasciculations and atrophy of the distal upper limbs, mostly seen in young males. The insidious weakness progresses over 3-5 years, after which it stops. Hirayama disease results from the abnormal anterior shifting of the posterior cervical dura that occurs during cervical flexion, leading to cord impingement. Electrophysiologic studies reveal denervation of C7-T1 myotomes and absence of sensory involvement. Dynamic magnetic resonance imaging of the cervical spine is essential in confirming the diagnosis. Treatment is aimed at primarily avoiding neck flexion, which is achieved with the help of a cervical brace; however, progressive disease calls for surgical intervention. Although the disease is self-limiting, it causes many functional impairments in affected individuals. In this review, we describe the epidemiology, pathophysiology, clinical manifestations, imaging characteristics, electrophysiologic findings, differential diagnoses and updates in the treatment of Hirayama disease. This review also aims to improve the awareness of this disease among clinicians, enabling early suspicion, diagnosis and management. [ABSTRACT FROM AUTHOR]

Social media platforms, such as Twitter, are increasingly used by clinicians, including neurologists. Twitter represents a convenient means to reach out to fellow clinicians, researchers and patients alike. To make maximum use of these platforms, they must be used pragmatically and within the ambit of ethical principles that guide all medical practice. As neurologists, familiarizing ourselves with the technical know-how and potential uses of Twitter empowers us best to use it as a valuable clinical and networking tool. Twitter offers diverse formats for a neurologist, thus enabling the creation and dissemination of specific types of information in the form of tweetorials, journal clubs, conference-based tweeting, tweetchats and the like. In this practical step-by-step guide, we demonstrate how to harness Twitter for myriad academic uses. [ABSTRACT FROM AUTHOR]

Disease modification trials in dementia and mild cognitive impairment (MCI) have not met with success. One potential criticism of these trials is the lack of sensitive outcome measures. A large number of outcome measures have been employed in dementia and MCI trials. This review aims to describe and analyze the utility of cognitive/clinical outcome measures in Alzheimer's disease (AD) and MCI trials. Methods: A PubMed search was conducted using relevant MeSH terms and exploded keywords. The search was confined to English language publications of human studies from the last five years which describe the latest trends in the use of outcome measures. Results: Despite broad use, the outcome measures employed are heterogeneous, with little data on correlations between scales. Another problem is that most studies are over-reliant on clinician/researcher assessment and cognitive outcomes, and there is a definite lack of stakeholder input. Finetuning of the paradigm is also required for people with early-stage disease, mild to moderate disease, and advanced dementia, as the outcome measures in these subgroups have varying relevance. Disease modification/prevention is an appropriate goal in early disease, whereas palliation and freedom from discomfort are paramount in later stages. The outcome measures selected must be suitable for and sensitive to these particular care goals. Although there is a shift to enrich MCI cohorts using a biomarker-based approach, the clinical relevance of such outcome measures remains uncertain. Conclusions: Outcome measures in dementia/MCI trials remain inhomogeneous and diverse, despite extensive use. Outcome measures fall within several paradigms, including cognitive, functional, quality-of-life, biomarker-based, and patient-reported outcome measures. The success of future disease-modifying trials is reliant to a large extent on the selection of outcome measures which combine all outcomes of clinical relevance as well as clinical meaning. Outcome measures should be tied to the type and stage of dementia and to the specific interventions employed. [ABSTRACT FROM AUTHOR]

(1) Background: During the second wave of COVID-19, India faced a rapid and sudden surge of not only COVID19-delta variant cases but also mucormycosis, making the infection even more fatal. We conducted a study to determine factors associated with the occurrence of mucormycosis in patients with COVID-19. (2) Methods: This case–control study comprised 121 patients; 61 cases (mucormycosis with COVID-19) and 60 controls. Patients were included from April 10, 2021 onwards. Follow-up was conducted after about 90 days and health status was recorded based on the modified Rankin Scale (mRS). (3) Results: Mucormycosis with COVID-19 cases had a median (IQR) age of 49 (43–59) years with 65.6% males and were older (95% CI 1.015–1.075; p = 0.002) than in the control group with median (IQR) 38 (29–55.5) years and 66.6% males. Baseline raised serum creatinine (OR = 4.963; 95% CI 1.456–16.911; p = 0.010) and D-dimer (OR = 1.000; 95% CI 1.000–1.001; p = 0.028) were independently associated with the occurrence of mucormycosis in COVID-19 patients. Additionally, diabetes mellitus (OR = 26.919; 95% CI 1.666–434.892; p = 0.020) was associated with poor outcomes and increased mortality in patients with mucormycosis with COVID-19 as per the multivariable analysis. A total of 30/61 mucormycosis patients had intracranial involvement. (4) Conclusions: The study observed elevated levels of baseline raised creatinine and D-dimer in mucormycosis pa-tients with COVID-19 as compared to the control group. However, future studies may be conducted to establish this cause–effect relationship. [ABSTRACT FROM AUTHOR]

Introduction: Two pharmacological possibilities exist for an acute ischemic stroke (AIS): recanalization of the occluded artery and neuroprotection from ischaemic injury, the latter's efficacy being debatable. We sought to determine whether administration of Citicoline immediately after recanalization therapy for AIS would improve clinical and radiological outcome at three months compared to standard treatment alone. Patients and methods: CAISR was a single centre, randomized, placebo-controlled, parallel-group trial with blinded endpoint assessment. It was approved by the All India Institute of Medical Sciences Institutional ethics committee and registered at the Clinical Trial Registry of India (CTRI/2018/011900). We recruited participants with AIS undergoing recanalization therapy and randomly assigned them to receive either Citicoline or placebo in 1:1 ratio. Citicoline arm patients received Citicoline 1gm BD intravenously for three days, followed by oral citicoline 1gm BD for 39 days. Placebo arm patients received 100ml intravenous normal saline for three days, followed by multivitamin tablet BD for 39 days. All patients received standard of care. Outcome: Blinded assessors did the follow-up assessment at six weeks (MRI Brain-stroke volume) and three months (NIHSS 0–2, mRS 0–2 and Barthel index> = 95). Results: The infarct volume decreased from week 1 to week 6 by 2.6 cm3 on placebo versus 4.2 cm3 on Citicoline (p-0.483). The OR for achieving NIHSS 0–2, mRS 0–2 and Barthel index> = 95 with Citicoline was found to be 0.96(95%CI 0.39–2.40), 0.92(95%CI 0.40–2.05) and 0.87(95%CI 0.22–2.98) respectively. Conclusion: CAISR was the first to evaluate the role of Citicoline, when used immediately after recanalization therapy, when the penumbral tissue is the most susceptible either to be protected from injury or become ischemic. We did not find any significant difference between the Citicoline or placebo arms with respect to either our primary or secondary outcomes. [ABSTRACT FROM AUTHOR]

Background: One of the major challenges is to deliver adequate health care in rural India, where more than two-thirds of India's population lives. There is a severe shortage of specialists in rural areas with one of the world's lowest physician/population ratios. There is only one neurologist per 1.25 million population. Stroke rehabilitation is virtually nonexistent in most district hospitals. Two innovative solutions include training physicians in district hospitals to diagnose and manage acute stroke ('Stroke physician model') and using a low-cost Telestroke model. We will be assessing the efficacy of these models through a cluster-randomized trial with a standard of care database maintained simultaneously in tertiary nodal centers with neurologists. Methods: SMART INDIA is a multicenter, open-label cluster-randomized trial with the hospital as a unit of randomization. The study will include district hospitals from the different states of India. We plan to enroll 22 district hospitals where a general physician manages the emergency without the services of a neurologist. These units (hospitals) will be randomized into either of two interventions using computer-generated random sequences with allocation concealment. Blinding of patients and clinicians will not be possible. The outcome assessment will be conducted by the blinded central adjudication team. The study includes 12 expert centers involved in the Telestroke arm by providing neurologists and telerehabilitation round the clock for attending calls. These centers will also be the training hub for "stroke physicians" where they will be given intensive short-term training for the management of acute stroke. There will be a preintervention data collection (1 month), followed by the intervention model implementation (3 months). Outcomes: The primary outcome will be the composite score (percentage) of performance of acute stroke care bundle assessed at 1 and 3 months after the intervention. The highest score (100%) will be achieved if all the eligible patients receive the standard stroke care bundle. The study will have an open-label extension for 3 more months. Conclusion: SMART INDIA assesses whether the low-cost Telestroke model is superior to the stroke physician model in achieving acute stroke care delivery. The results of this study can be utilized in national programs for stroke and can be a role model for stroke care delivery in low- and middle-Income countries. (CTRI/2021/11/038196) [ABSTRACT FROM AUTHOR]

The article presents case studies related to Myelin Oligodendrocyte glycoprotein antibody syndrome and seizures. Topics include a 12‑year‑old girl presented with acute onset unsteadiness and imbalance while walking and diminution of vision from both eyes; and a 13‑year‑old boy presented with recurrent episodes of focal neurological deficits associated with pure motor paraparesis with stiffness of limbs, and horizontal binocular diplopia which were responsive to corticosteroids.

During the second wave of COVID-19 pandemic, there is a sudden increase in number of cases mucormycosis infection in India. This communication by the Tropical Neurology subsection expert group of the Indian Academy of Neurology (IAN) describes the clinical and diagnostic features, treatment of the disease and gives recommendations about the ways forward. [ABSTRACT FROM AUTHOR]

This article is a reply to a previous article on neuropathic tremor in Guillain Barré Syndrome. The authors thank the previous authors for their interest and highlight an important treatment option for neuropathic tremor following Guillain Barré Syndrome. They discuss the similarities between their series of patients and the patient reported by the previous authors, as well as the different treatment responses observed. The authors also provide a pathophysiological explanation for the potential effectiveness of pregabalin in treating neuropathic tremor. They conclude by suggesting that propranolol, pregabalin, and gabapentin may be initial medications to be tried for neuropathic tremors, but further studies are needed to understand the natural history and therapeutic response of this disorder. [Extracted from the article]

Background: We estimated plasma amyloid-peptides levels (Aβ1-42 and Aβ1-40) as diagnostic biomarker of Alzheimer's disease (AD) and evaluated its association with clinical severity and 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) Z score of the different brain regions in the Indian population. Patients and Methods: A case-control study was conducted. Diagnostic and statistical manual-IV, Dubois, and NIA-AA criteria were used for the diagnosis of AD. The plasma Aβ1-42 and Aβ1-40 concentration and 18F-FDG PET Z score were estimated for different brain regions. Results: Forty-seven cognitive impairment patients (AD = 29, mild cognitive impairment = 18) and 33 age-matched controls were enrolled. Plasma Aβ1-42 level was significantly higher in the AD group compared to controls (P = 0.046) and a cut-off >5.7 ng/mL has a specificity of 96.9%, sensitivity of 27.6%, positive predictive value 88.9%, and negative predictive value 60.4% for differentiating AD patients from controls. Significant correlation was seen between Aβ1-40/Aβ1-42 ratio and 18F-FDG PET Z score in the bilateral-parietal, temporal, frontal-association area, and posterior-cingulate areas. Conclusion: As a diagnostic biomarker of AD, plasma Aβ1-42 level showed good specificity but low sensitivity in the Indian population. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Carotid artery stenting (CAS) involves dilatation of carotid bulb which can trigger a series of neuronal responses resulting in hemodynamic depression that might influence the outcome of the procedure. This is a frequent but underdiagnosed complication of CAS. Although it is mild, transient and self-limiting in majority of cases, it can result in significant morbidity and mortality if persistent. Neurologists should be aware of the predisposing factors and management of this common complication. In patients who present with stroke following carotid stenting, neurologists should be aware of hypoperfusion secondary to hemodynamic depression as a cause of stroke apart from the stent thrombosis and occlusion. [ABSTRACT FROM AUTHOR]

A case study of an 83‑year‑old gentleman with non‑valvular atrial fibrillation, diabetes presented with a right parietal subdural hematoma following a traumatic fall. Topics include magnetic resonance imaging (MRI) of the brain revealed a reduction in the midline shift without any evidence of vascular insult; and Valproate and phenytoin were not used in this patient due to their Cytochrome P450 (CYP) inhibiting and inducing properties for multiple comorbidities and treatments.