Search

Paraneoplastic neurologic syndromes are cancer-associated, immune-mediated neurologic manifestations that may involve any part of the nervous system. They usually present with characteristic neurologic features and should be considered in high-risk phenotypes such as limbic encephalitis, encephalomyelitis, rapidly progressive cerebellar syndrome, opsoclonus–myoclonus, sensory neuronopathy, enteric neuropathy, and Lambert–Eaton myasthenic syndrome. The diagnosis is made by antibody positivity in the serum or cerebrospinal fluid, in the presence of an appropriate clinical phenotype. Findings on antibody testing by immunoblot should always be verified by immunofluorescence. We report a rare case of sensory neuronopathy with triple paraneoplastic antibody positivity (anti-Hu, anti-collapsing response-mediator protein 5, and anti-amphiphysin) on immunoblot but only anti-Hu positivity on immunofluorescence. The presence of lower facial dyskinesias should raise the possibility of an immune-mediated neurologic syndrome in the appropriate clinical context.

Autoimmune autonomic disorders encompass a spectrum of disorders mediated by immune responses directed against the autonomic nervous system, including the peripheral and central autonomic pathways. While centrally mediated autoimmune autonomic disorders primarily can cause autonomic hyperactivity, peripherally mediated disorders are more common and can cause either locally confined or global autonomic failure. These disorders are often underrecognized owing to vague and varied clinical signs and symptoms. The discovery of specific autoantibodies in the past decade has caused a growing recognition of autoimmune causes for these disorders. The management is also complex, as these disorders often manifest with generalized symptoms, are difficult to diagnose, invoke challenges involving robust establishment of an autoimmune syndrome, and are rare. This article presents an overview of autonomic disorders that have a suspected autoimmune etiology, as well as recent advancements in their diagnosis and management.

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.

Neurosarcoidosis (NS) is a protean illness with multiple clinical and radiological presentations giving it the moniker of “a chameleon” or the great mimic. NS can present as a wide spectrum of neurological syndromes localizing both to the central and peripheral nervous systems. The absence of a diagnostic serum test makes it difficult to diagnose with certainty and remains largely a histopathological diagnosis and one of exclusion. A high index of suspicion should be there in suspecting NS, and it should always be excluded among patients presenting with acute to subacute neurological deficits.

Peripheral demyelinating polyneuropathy, central dysmyelination, Waardenburg syndrome, and Hirschsprung’s disease is a rare genetic disorder caused by de novo variants in the SOX10 gene. The SOX10 gene is expressed in the neural crest cells during early embryonic development and in the glial cells of the peripheral and central nervous systems during late embryonic development, as well as in adults. Here, we describe our findings in a 9-month-old male infant presenting with failure to thrive, global developmental delay, seizures, hypotonia, heterochromia iridis, hypopigmented skin macules, pendular nystagmus, Hirschsprung’s disease, and hearing impairment. Nerve conduction studies were suggestive of sensorimotor demyelinating polyneuropathy. Brain magnetic resonance imaging showed diffuse hypomyelination. Targeted genetic testing revealed a novel stop-loss variant in the SOX10 gene (NM_006941.4). This case highlights the importance of clinical phenotyping that can aid in targeted genetic testing.

Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disorder because of the persistence of mutated measles virus in the central nervous system. Till date, no curative therapy has been established for SSPE. Multiple drugs have been tried to modify the disease process but have shown mild to moderate benefit at best. It is also challenging to attribute the relative success of some strategies described in single case reports because of the known phenomenon of spontaneous improvement in 5% of patients with SSPE. Critical gaps in understanding the pathophysiological processes involved exist. Current therapies such as interferon alfa require invasive strategies for administration by the intraventricular or intrathecal route, with varying dosage regimens. Oral therapies such as isoprinosine and ribavirin are expensive and not readily available in resource-constrained settings. Most of the evidence so far favors the use of combinational regimens. In this viewpoint, we critically summarize the current evidence on disease-modifying strategies in the context of our region.

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.

Tuberculosis involving the spinal cord is associated with high mortality and disabling long-term sequelae. Although tuberculous radiculomyelitis is the most frequent complication, pleomorphic clinical manifestations exist. Diagnosis can be challenging among patients with isolated spinal cord tuberculosis due to diverse clinical and radiological presentations. The principles of management of tuberculosis of the spinal cord are primarily derived from, and dependent upon, trials on tuberculous meningitis (TBM). Although facilitating mycobacterial killing and controlling host inflammatory response within the nervous system remain the primary objectives, several unique features require attention. The paradoxical worsening is more frequent, often with devastating outcomes. The role of anti-inflammatory agents such as steroids in adhesive tuberculous radiculomyelitis remains unclear. Surgical interventions may benefit a small proportion of patients with spinal cord tuberculosis. Currently, the evidence base in the management of spinal cord tuberculosis is limited to uncontrolled small-scale data. Despite the gargantuan burden of tuberculosis, particularly in lower and middle-income countries, large-scale cohesive data are surprisingly sparse. In this review, we highlight the varied clinical and radiological presentations, performance of various diagnostic modalities, summarize data on the efficacy of treatment options, and propose a way forward to improve outcomes in these patients.

Normal pressure hydrocephalus (NPH) is the most frequently occurring form of hydrocephalus among adults. It is characterized clinically by the classical triad, called Hakim's triad, comprising gait issues, cognitive impairment, and urinary problems. NPH may be primary or idiopathic (iNPH) or secondary. Characteristic neuroimaging features occur, which are vital to diagnosis. Diagnostic criteria in the form of Japanese guideline and Congress of Neurological Surgeons 2005 guidelines have been devised, and broadly, are based on a constellation of clinical and neuroimaging features, in association with cerebrospinal fluid (CSF) testing. CSF tap test, extended lumbar drainage, and CSF infusion tests are invasive diagnostic tests. CSF tap test and extended lumbar drainage are used to demonstrate clinical reversibility with CSF drainage, and patients who demonstrate this are candidates for CSF shunting. However, due to the low negative predictive value of these tests, potential response to shunting cannot be negated among patients who do not respond to CSF drainage. Various shunting procedures are used for treatment, including ventriculoperitoneal, lumboperitoneal, and ventriculoatrial shunts. Endoscopic third ventriculostomy has also been attempted with limited success. Among the clinical features, gait abnormalities are most responsive to shunting. Persistent long-term response to shunting has been reported. Patients need to be meticulously followed up after the shunting procedure, to assess clinical and neuroimaging response, and detect possible shunt-related complications, especially CSF over-drainage. Early treatment is associated with better prognosis, and it is crucial to recognize and treat this condition before the development of severe symptoms.

Background: Subacute sclerosing panencephalitis (SSPE) is a progressive neurodegenerative disorder of children and young adults. In the setting of the COVID-19 pandemic, we have observed increasing admissions of SSPE and a change in the clinical profile, mainly younger age of presentation, i.e., children under the age of 5 years. Clinical Description: We presented the clinical and investigational characteristics of 20 children who were diagnosed as SSPE during the period March 2020–March 2021 in our hospital, based on the Dyken criteria. The mean age was observed to be 3 years 9 months and male-to-female ratio of 1:1. Most children presented with developmental regression and myoclonic jerks. The clinical details and investigations were presented and a few atypical cases were discussed. Management: All the patients were treated symptomatically: anticonvulsant drugs, nutritional rehabilitation and supported feeding, occupational therapy, and palliative therapy. A trial of isoprinosine was started in all the patients, but was discontinued by most due to the expense. There were no mortalities. Conclusion: The change in profile of SSPE to an earlier onset in the setting of the pandemic should make the clinician keep a high index of suspicion of this disorder in children under the age of five years who present with unexplained developmental regression or acute encephalopathy, before the onset of typical myoclonus.

Writer’s cramp (WC) is a focal task-specific dystonia that affects the fingers, hands, and forearms. It interferes with an individual’s ability to write, causing professional disability. In this systematic review, we discuss the epidemiology, pathophysiology, clinical features, and management of WC. In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we included publications until February 5, 2022, from the PubMed database. In addition, we elaborate on the clinical assessment and selection of appropriate muscles for botulinum neurotoxin therapy through pictorial illustrations. The prevalence of WC is underestimated because medical attention is only sought by those with professional disability. The clinical presentation of WC is heterogeneous. In some patients, dystonia is manifested only during writing tasks (simple WC), while in others, several manual tasks are affected in addition to writing (complex WC). Some patients have semirhythmic movements of the hand with no apparent postural abnormality during writing (writing tremor). The assessment of the pattern of dystonia is confounded by the compensatory movements a patient adopts while writing. There are several pharmacological, nonpharmacological, and surgical options for the management of WC. However, there are no randomized controlled trials supporting the efficacy of oral medications. The efficacy of physiotherapy, occupational therapy, and noninvasive brain stimulation is debatable. Neurosurgical procedures, although reportedly effective, are seldom justified in focal task-specific dystonia. Botulinum neurotoxin is considered an effective treatment option based on several prospective studies and few randomized controlled trials. However, WC may be the most challenging indication for this therapy because writing is a complex manual task. Furthermore, given the heterogeneity, clinicians often face difficulties in discerning the exact abnormality and selecting the appropriate muscles for treatment.

Introduction/Aims: Studies conducted during the coronavirus disease 2019 (COVID-19) pandemic have reported varied data regarding the incidence of Guillain–Barre syndrome (GBS). The present study investigated demographic and clinical features, management, and outcomes of patients with GBS during a specified period of the COVID-19 pandemic, and compared these features to those of GBS in the previous year. Methods: A multicenter, ambispective cohort study including 26 centers across India was conducted. Data from a pre-COVID-19 period (March 1 to August 31, 2019) were collected retrospectively and collected ambispectively for a specified COVID-19 period (March 1 to August 31, 2020). The study was registered with the Clinical Trial Registry India (CTRI/2020/11/029143). Results: Data from 555 patients were included for analysis: pre-COVID-19 (n = 334) and COVID-19 (n = 221). Males were more commonly affected during both periods (male:female, 2:1). Gastroenteritis was the most frequent antecedent event in 2019 (17.4%), whereas fever was the most common event in 2020 (10.7%). Paraparesis (21.3% versus [vs.] 9.3%, P = 0.001) and sensory involvement (51.1% vs. 41.3%; P = 0.023) were more common during COVID-19 in 2020, whereas back pain (26.3% vs. 18.4%; P = 0.032) and bowel symptoms (20.7% vs. 13.7%; P = 0.024) were more frequent in the pre-COVID period. There was no difference in clinical outcomes between the two groups in terms of GBS disability score at discharge and 3 months after discharge. Independent predictors of disability in the pre-COVID period included areflexia/hyporeflexia, the requirementfor intubation, and time to bulbar weakness; in the COVID-19 period, independent predictors included time from onset to admission, intubation, and intubation requirement. The mortality rate was 2.3% during the entire study period (13/555 cases). Discussion: Results of this study revealed an overall reduction in the frequency of GBS during the pandemic. The lockdown likely reduced the risk for antecedent infections due to social distancing and improved hygiene, which may have resulted in the reduction of the frequency of GBS.

Background: Among the primary environmental issues affecting global health, air pollution is considered the leading cause of concern. Globally, around 800,000 deaths were attributed to air pollution according to WHO. Evidence suggests that there has been a strong association of air pollution with stroke. Approximately, 25% of stroke mortality was due to air pollution according to a study in 2013. Objective: The aim of this review was to analyze the association between stroke, intracerebral hemorrhage, and subarachnoid hemorrhage and air pollution and its burden globally with a special focus on South Asia along with its association with the COVID-19 pandemic. Results: There is growing research data linking air pollution to cardiovascular disorders including stroke. Short-term and long-term air pollution exposures have been shown to increase stroke incidence in epidemiological data. Air pollution, both gaseous and particle, show a strong and tight temporal relationship with stroke hospitalizations and death. The link between ICH and SAH to air pollution is less strong and less well studied as compared to ischemic stroke. Stroke and air pollution both are highly prevalent in South Asia. It is possible that the high prevalence of stroke in south Asia may be linked to the high frequency of air pollution in addition to other conventional risk factors. Decreased stroke admissions and mortality and reduced cardiovascular mortality reported during coronavirus disease 2019 (COVID) lockdown may be attributable to decreased levels of air pollution. Conclusion: Even though air pollution poses a significant threat to human health, a great number of countries still fail to achieve internationally agreed air quality standards. Air pollution should be recognized among the most significant controllable risk factors for cardiovascular and cerebrovascular disease prevention and treatment.

Background: Spinocerebellar ataxia is a neurodegenerative disease. Information on comparative assessment of quality of life (QoL) among SCAs, particularly SCA 12, is scarce. We aimed to compare health-related QoL in SCA 1, 2 and 12. Methods: We conducted a cross-sectional study among individuals with genetically-confirmed SCAs. Ataxia severity was assessed using Brief Ataxia Rating Scale (BARS), independence in activities of daily living (ADL) using Katz index (Katz ADL) and depression using Beck's Depression Inventory-II (BDI-II). QoL was assessed via Short Form Health Survey version 2.0 (SF-36). Results: We enrolled 89 individuals (SCA1 = 17, SCA2 = 43, SCA12 = 29; 56% males). Mean age at onset (41.0 ± 11.6 for SCA12 versus 24.9 + 7.0 for SCA1 and 28.8 ± 9.8 years for SCA2) was significantly higher among SCA12. SCA12 had lower BARS (mean score 4.1 ± 4.5 versus 10.6 ± 4.6 for SCA1 and 12.5 ± 4.5 for SCA2). SCA12 scored better on all SF-36 subdomains including Physical (PCS) and Mental Component Summary (MCS) scores. PCS score amongst SCA12 was 44.4 ± 9.0 versus 30.4 ± 9.1 for SCA1 and 33.3 ± 8.9 for SCA2. MCS score for SCA12 was 51.4 ± 11.4 versus 41.8 ± 11.5 for SCA1 and 41.8 ± 11.2 for SCA2. SCA12 had lower mean BDI scores (5.0 ± 6.0) versus SCA1 (9.5 ± 11.6) and SCA2 (10.9 ± 10.3). BARS and BDI emerged as significant predictors of most SF-36 subdomains. Conclusions: Our study suggests that despite older age and comparable disease duration, SCA12 patients experience better QoL, less severe depression and ataxia versus SCA1 and SCA2. Severity of ataxia and depression are significant predictors of QoL among the three SCA types.

Air pollution, the most prevalent form of pollution worldwide, is associated with a wide range of neurological disorders, including neurodegenerative conditions, stroke, autism, depression, and developmental delay. There is accumulating evidence on the association between air pollution and headache disorders, especially migraine. Many classical and non-classical air pollutants have been associated with headache, including particulate matter, nitrogen dioxide, sulfur dioxide, ozone, carbon monoxide, as well as polycyclic aromatic hydrocarbons and volatile organic compounds. There has also been research on the impact of biomass fuels on health-related symptoms, including headache, which form an important source of air pollution in our country. The exact mechanisms underlying headache pathophysiology vis-à-vis air pollution are not precisely defined but include triggering of neuroinflammation and activation of the transient receptor potential ankyrin 1 (TRPA1)-associated pathways. Evidence from different regions of the world indicates a significant association between headache incidence and prevalence, and occurrence of air pollution. Despite growing data, research on adverse effects of air pollution on headache disorders remains limited, and appropriate outcome measures are not holistically defined in these studies. Due to the rapid advancement of the scourge of air pollution, there is a pressing need to expand the arena of research, specifically focused on pathological mechanisms, impact on health and quality-of-life parameters, as well as broader global ramifications.

Man and environment have a strong connection with each other for their functioning. Environmental toxins which can be natural or manmade result in the loss of this balance by causing systemic inflammatory response within the human body, with the brain being the most affected target end-organ. These problems are more prominent in Third World countries, where environmental regulations laws are either relaxed or non-existent. These neurotoxins play a very important aetiological role in the manifestation of various neurodegenerative diseases, neurodevelopmental disorders and psychiatric disorders. Environmental neurotoxicity results from inhibition of mitochondrial activity, excess oxidative stress leading to neuroinflammation, and promoting apoptosis and neuronal cell death. Having the know-how of these neurotoxins will provide insight into the process of neurodegeneration and will result in further designing of studies to delve into processes and mechanisms of neuronal regeneration and axonal sprouting. This review highlights the various central nervous system disorders associated with exposure to environmental neurotoxins and discusses the way forward to prevent or halt the process of neurodegeneration.

Objective: To study impact of COVID-19 pandemic on frequency, clinical/electrophysiological profile and treatment outcomes in pediatric Guillain-Barré syndrome (GBS). Background: 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. Methods: 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. Results: 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; P = 0.022) and had more sensory symptoms (50% versus 18.2%; P = 0.043). The 2020 group had relatively favourable mRS at discharge (median 1 (1–3.5) versus 3 (2–4); P = 0.042) and GBS disability score at 3 months (median 0 (0–0.75) versus 2 (0–3); P = 0.009) compared to 2019. Multivariate analysis revealed bowel involvement (P = 0.000) and ventilatory support (P = 0.001) as independent predictors of disability. No child in 2020 had preceding/concurrent SARS-CoV2 infection. Conclusions: 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.

Although acquired manganese neurotoxicity has been widely reported since its first description in 1837 and is popularly referred to as “manganism,” inherited disorders of manganese homeostasis have received the first genetic signature as recently as 2012. These disorders, predominantly described in children and adolescents, involve mutations in three manganese transporter genes, i.e., SLC30A10 and SLC39A14 which lead to manganese overload, and SLC39A8, which leads to manganese deficiency. Both disorders of inherited hypermanganesemia typically exhibit dystonia and parkinsonism with relatively preserved cognition and are differentiated by the occurrence of polycythemia and liver involvement in the SLC30A10-associated condition. Mutations in SLC39A8 lead to a congenital disorder of glycosylation which presents with developmental delay, failure to thrive, intellectual impairment, and seizures due to manganese deficiency. Chelation with iron supplementation is the treatment of choice in inherited hypermanganesemia. In this review, we highlight the pathognomonic clinical, laboratory, imaging features and treatment modalities for these rare disorders.

Psychogenic non-epileptic seizure (PNES) is a common disorder that imitates epileptic seizures and has its etiological roots in psychological distress. Due to its “epileptic” similarity, it is often dealt with not only by mental health professionals but also by physicians, pediatricians and neurologists. There is a growing consensus towards the psychotherapeutic treatment of the disorder, albeit a lack of clarity in choosing a gold-standard approach. This paper seeks to serve as a compendium of different psychotherapeutic approaches and their efficacy in the management of PNES. The paper employed the search strategy by selecting the keywords: “Psychogenic Non-Epileptic Seizures (PNES) and psychosocial management”, “PNES Treatment approach”, “PNES and psychotherapy” in PUBMED, EBSCO host, PsycINFO, and SCOPUS database. Eventually, specific therapies were cross-searched with PNES for an exhaustive review. Several studies were found employing various psychotherapeutic approaches for the treatment of PNES in pilot studies, randomized controlled, or open uncontrolled trials. Cognitive Behavior Therapy was demonstrated as an efficacious treatment for PNES in a randomized controlled trial (RCT). Other approaches that were effective in ameliorating the symptoms were psychodynamic therapies or psychoeducation based group therapies. Some therapies like Novel Integrative psychotherapy, Eye Movement Desensitisation Therapy and Mindfulness-based therapies require further exploration in larger clinical trials. The findings demonstrate that psychological intervention for PNES is a promising alternative treatment approach with a need for more RCTs with a larger sample and robust methodology for better generalization.

Background: The challenges being faced during the lockdown period may worsen motor or non-motor symptoms in Parkinson's disease (PD). Objective: This study was undertaken to investigate the impact of lockdown on the disease activity, caregiver perceptions and the quality of life of patients with PD. Materials and Methods: This cross-sectional study was conducted from June till September 2020. Sixty-four patients with PD and caregivers were interviewed telephonically after obtaining consent. The responses were recorded by means of a structured questionnaire. Non-motor symptoms scale (NMSS) and the Parkinson Disease Questinnaire-8 (PDQ-8) were applied. PDQ-8 severity index (PDQ-8 SI) scores were expressed as percentage of the raw PDQ-8 score of the total score. Data were analsyed by using SPSS version 20.0. Results: Of 64 patients, 39 (60.9%) were men and 25 (39.1%) were women. The overall median age of the patients was 65 (55.25–69.75) years. The median duration was 48 (30–84) months. Twenty-six (40.6%) patients reported symptomatic worsening during the lockdown period. Slowness in activities of daily living and walking worsened in 15 (57.7%) and 14 (53.8%) patients, respectively, while tremors increased in 12 (46.2%) patients. Mood and sleep disturbances were the most common non-motor symptoms to worsen. Increase in non-motor symptoms and the NMSS total score were independent predictors of PDQ-8 scores. Increase in non-motor symptoms during the lockdown was an independent predictor of the highest quartile of PDQ-8 SI scores. Conclusions: Motor and non-motor symptoms have worsened in patients with PD during the lockdown. The increase in non-motor symptoms was independently associated with poorer quality of life among patients with PD during the lockdown.

Glutaric aciduria type 1 is an autosomal recessive disorder caused by mutations in GCDH gene on chromosome 19 leading to the deficiency of glutaryl-CoA dehydrogenase which causes an abnormal metabolism of lysine, hydroxylysine and tryptophan with resultant accumulation of glutaric acid and 3-hydroxy glutaric acid. Usual presentations include macrocephaly with recurrent dystonic episodes, along with developmental regression. The diagnosis is based on characteristic magnetic resonance imaging finding of widening of sylvian fissures and urinary tandem mass spectroscopic analysis of excess glutarylcarnitine and hydroxyglutaric acid. Management includes lysine-free diet and carnitine supplementation.

Introduction: Scoring systems to predict outcomes in pediatric status epilepticus (SE) are limited. We sought to assess usefulness of the END-IT score in pediatric SE. Methodology: We conducted a retrospective study at a tertiary hospital in New Delhi, India. Children aged 1 month–18 years who presented with seizure for ≥5 min/actively convulsing to emergency were enrolled. END-IT score was calculated and correlated with outcome at discharge using Pediatric Overall Performance Category (POPC) scale, in-hospital mortality, and progression to refractory and super-refractory SE (SRSE). Results: We enrolled 140 children (mean age 5.8 years; 67.1% males). Seven children died and 15 had unfavorable outcomes. The predictive accuracy of END-IT at a cutoff of > 2: for unfavorable outcome (POPC score ≥3) was: sensitivity 0.73 (95% CI: 0.45–0.92), specificity 0.94 (95% CI: 0.89–0.98), PPV 0.61 (95% CI: 0.36–0.83), NPV 0.97 (95% CI: 0.92–0.99), positive likelihood ratio (13.09), F1 score (0.666); for death: sensitivity 0.86 (95% CI: 0.42–0.99), specificity 0.91 (95% CI: 0.85–0.95), PPV 0.33 (95% CI: 0.13–0.59), NPV 0.99 (95% CI: 0.96–1.00), F1 score (0.48); for RSE: sensitivity 0.80 (95%CI: 0.28–0.99), specificity 0.90 (95% CI: 0.83–0.94), PPV 0.22 (95% CI: 0.06–0.48) NPV 0.99 (95% CI: 0.96–1.00), F1 score (0.35); for SRSE: sensitivity 0.67 (95% CI: 0.22–0.96) specificity 0.75 (95% CI: 0.66–0.82), PPV 0.22 (95% CI: 0.06–0.48) NPV 0.98 (95% CI: 0.94–0.99), F1 score (0.33). Conclusion: We demonstrate utility of the END-IT score to predict short-term outcomes as well as progression to refractory and SRSE for the first time among children with SE.

Objective: We aimed to assess the feasibility of teleneurorehabilitation (TNR) among persons with Parkinson's disease (PD), considering difficulties imposed by the COVID-19 pandemic in access to healthcare, particularly in low-resource settings. The feasibility of TNR in India has not been formally assessed so far. Methods: We conducted a single-center, prospective cohort study at a tertiary center in India. Persons with PD with Hoehn & Yahr (H&Y) stages 1-2.5, who were not enrolled into any formal exercise program, were offered TNR as per a predesigned program for 12 weeks. Baseline and post-intervention assessment included Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), part II and III, Parkinson's Disease Questionnaire (PDQ)-8 and Non-Motor Symptoms Scale (NMSS). We assessed adherence to TNR and problems expressed by patients/caregivers by means of open-ended surveys addressing barriers to rehabilitation. Results: We recruited 22 for TNR. Median age (interquartile range [IQR]) was 66.0 (44.0-71.0) years; 66.7% were H&Y stage 2.0. One patient died of COVID-19-related complications. Of the remaining 21, 14 (66.7%) had adherence of ≥75%; 16/21 (76.2%) patients had problems with attending TNR sessions as the family shared a single phone. Slow Internet speed was an issue among 13/21 (61.9%) of the patients. Other issues included lack of rapport, migration to distant hometowns and motor-hand impairment. Conclusion: Multiple challenges were faced in implementing a telerehabilitation program among persons with PD, exacerbated by the COVID-19 pandemic. These barriers were present at various levels: recruitment, adherence issues and maintenance. Future TNR programs must address these concerns.

Ketogenic dietary therapies (KDTs) have been in use for refractory paediatric epilepsy for a century now. Over time, KDTs themselves have undergone various modifications to improve tolerability and clinical feasibility, including the Modified Atkins diet (MAD), medium chain triglyceride (MCT) diet and the low glycaemic index treatment (LGIT). Animal and observational studies indicate numerous benefits of KDTs in paediatric neurological conditions apart from their evident benefits in childhood intractable epilepsy, including neurodevelopmental disorders such as autism spectrum disorder, rarer neurogenetic conditions such as Rett syndrome, Fragile X syndrome and Kabuki syndrome, neurodegenerative conditions such as Pelizaeus-Merzbacher disease, and other conditions such as stroke and migraine. A large proportion of the evidence is derived from individual case reports, case series and some small clinical trials, emphasising the vast scope for research in this avenue. The term 'neuroketotherapeutics' has been coined recently to encompass the rapid strides in this field. In the 100th year of its use for paediatric epilepsy, this review covers the role of the KDTs in non-epilepsy neurological conditions among children.

Research in amyotrophic lateral sclerosis (ALS), a neurodegenerative condition, has seen advancement in several key areas of research in 2020. These include a thrust to move the classification of ALS from a neuromuscular condition to a neurodegenerative condition, due to research suggesting involvement of cortical areas, and early cortical hyperexcitability. A new criterion for ALS, called the Gold Coast criterion, has been described. The Gold Coast criteria have removed the categories of possible, probable, and definite ALS, to make the diagnosis of ALS more inclusive and permit enhanced eligibility of patients in clinical trials for ALS. New biomarkers, both imaging and protein based, have been described. Advances in therapy have also occurred, with a large phase II clinical trial reporting benefits with sodium phenylbutyrate-taurursodiol.