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The spectrum of movement disorders associated with anti N-Methyl-d-Aspartate-Receptor (NMDAR) encephalitis is myriad, particularly in children, possibilities of which were investigated from two tertiary care centres.A retrospective study was conducted in two tertiary referral centres in Eastern India, analysing data of 8 paediatric patients diagnosed as anti NMDAR encephalitis, presenting with one or more movement disorders (MDs).All the patients were of Bengali ethnicity with a median age of 9 years (3-16 years) and with female predilection (62.5%). CSF pleocytosis was a common feature in all. Seizures were described in 62.5%% of patients with a solitary patient exhibiting abnormalities on brain imaging. 3 out of 8 (37.5%) of patients presented with a single MD while the remaining had more than one type. Oro-linguo-facial dyskinesias and dystonia (37.5% each) were the most common movement type followed by chorea (12.5%). Complex stereotypies, myoclonus and facial tics were noted in one patient each. All patients received pulse methyl prednisolone. Escalation to second line therapy in form of rituximab was done for 5 patients (62.5%). Following immunotherapy, hyperkinetic movements resolved in 50% of patients, with persistence of movements in one (12.5%). A mortality of 37.5% was noted. Median duration of follow up was 26 months, during which none of the patients had evidence of systemic neoplasm.MDs are a core feature of anti NMDAR encephalitis, particularly in the paediatric age group, understanding and characterization of which, is the key to early diagnosis and effective therapy.

Background: In April 2020, a novel syndrome termed multisystem inflammatory syndrome in children (MIS-C), related to SARS-CoV-2 infection was first described. This syndrome has a wide spectrum of systemic involvement, has shown to include the nervous system as well. Our study aims to obtain a baseline clinical and demographic profile of varied neurological manifestations of MIS-C. It also aims to delineate a profile of short-term outcome in these patients with regards to residual neurological sequelae. Methods: A single-centre, retrospective, observational and hospital based study for 5 months was conducted among patients in the age group from 1 month to 12 years, with MIS-C with co-existing neurological symptoms. The subjects who had pre-existing neurological diseases were excluded from the study. A total of 34 subjects were collected. Post-discharge, each patient was followed up for a period of 1 month. The residual neurological deficits, if any, at discharge and follow-up. Results: The neurological complication found were Acute Symptomatic Seizure (29.4%), Aseptic Meningitis (23.5%), Encephalitis (11.8%), Guillain-Barré Syndrome (11.8%), Miller-Fisher Syndrome (2.9%), ADEM (8.8%), Autoimmune Encephalitis (8.8%) and Acute Haemorrhaegic Stroke (2.9%). 11.8% expired and 50% required P.I.C.U admissions. 23.5% had residual neurological deficit at discharge and 8.8% at follow-up after 1 month of discharge. Conclusions: In spite of great variability in manifestations, prognosis is favourable if early aggressive treatment is initiated.

Polymer nanocomposite is a new kind of material that offers to substitute traditionally filled polymers. The nanomaterial polymer matrix inter-phase area increases drastically due to the inherent high surface-to-volume ratio resulting in remarkably enhanced properties compared to the pristine polymers or their conventional counterpart filled nanocomposites. Nanocomposites have several novel properties such as nonlinear optical properties, electronic conductivity and luminescence. Therefore, their use has been projected in many areas like chemical sensors, polymer electrolyte membrane fuel cell (PEMFCs), electroluminescent devices, batteries, electrocatalysis, smart windows and memory devices. PEMFCs embody a potential candidate for electrochemical energy generation in the twenty-first century due to their better efficiency and environmentally friendly nature. Proton exchange/Polymer electrolyte membrane (PEM) plays a vital role in the PEMFCs. Currently, PEM like Nafion and Flemions are widely used in PEMFC, which have certain drawbacks such as fuel cross-over through the membrane, low operating temperature, and high cost. The researchers from several laboratories across the globe have put their extreme effort into preparing a novel polymer electrolyte membrane with high proton conductivity, better long-term stability, improved thermal stability, high peak power density (PPD), and less fuel crossover with minimum cost. The advent of nanotechnology has brought a new scope to this research area. The hybrid (organic polymer with inorganic nanoparticle) nanocomposite membrane has developed into an exciting alternative to the conventional polymer membrane applications. It provides an exclusive blend of inorganic and organic properties and helps to overcome the drawbacks of align="center"pristine polymer membranes. In this book chapter, we have focused on different nanomaterials and their effect is analyzed in polymer electrolyte nanocomposite membranes for PEMFC applications.

BACKGROUND Though epilepsy remains a significant problem for children and adolescents in our country, studies delineating the clinical profile and response to treatment in childhood epilepsy are lacking. The current study was carried out for obtaining a baseline profile and to predict the response to treatment in childhood epilepsy in India that may be helpful in planning management strategies from a public health point of view. METHODS Patients with clinical suggestion of active epilepsy (N = 141) from one month to 12 years, were enrolled into the study over a period of 1 year (February 2010 to January 2011) from the out-patient department and epilepsy clinic of Bangur Institute of Neurology. Detailed history was taken along with neurological examination. Electroencephalography (EEG) and neuroimaging (MRI / CT scan) were done on all patients. Each patient included in the study was kept in followup for a period of 6 months and their response to the treatment was recorded. RESULTS About 48.9 % (N = 69) patients had localisation related epilepsy while the rest had generalised epilepsy. Of those with generalised epilepsy, generalised tonicclonic seizures (GTCS) was by far the most common type. Of those with focal EEG activity, the highest proportion (50 %), were localised to the temporal lobe. Symptomatic aetiology accounted for 59.6 % (N = 84) of the patients. 20.6 % (N = 29) had poor response to treatment at 6 months follow-up. Abnormal neuroimaging (OR = 6.708) and abnormal EEG (OR = 6.357) were effective factors in predicting poor response to treatment. CONCLUSIONS Our study highlights the need to link specialised epilepsy services with primary health centres for early detection and treatment. EEG is an essential cost-effective modality in determining seizure localisation and response to treatment. KEYWORDS Paediatric, Epilepsy, Clinical Profile, Response to Treatment

Subacute Sclerosing Panencephalitis (SSPE) is a very rare and deadly complication of measles infection, with its incidence being signicantly higher in developing countries. Our retrospective analysis of four patients highlights another ominous trend of this deadly disease, i.e, early age at onset leading to a rapid progress of symptoms. Four patients, presenting to the pediatrics department of a tertiary care hospital of eastern India, from February 2020 To December 2020, diagnosed as SSPE, by the modied Dyken's criterion, were analyzed. Paired CSF and serum samples were taken from all the patients and were analyzed for measles-specic IgG in serum (dilution 1:404) & CSF (dilution 1:2) using measles specic IgG ELISA (Enzyme-linked immunosorbent assay) kit. All the patients were under 6 years of age with a mean latency period of 2.5 years. All had contracted measles infection before receiving the scheduled rst dose of vaccine at 9 months of age. Treatment was initiated according to the institutional protocol, keeping in mind the affordability. However, follow-up of the patients reected the rapid progression of the disease. High morbidity and mortality is associated with SSPE in developing countries like India, where treatment availability and affordability becomes a major issue. In order to prevent this disease, it is necessary to prevent measles infection. Recent studies have recorded early disappearance of maternal measles-specic antibodies thus pointing towards the need to vaccinate early. Our case series emphasizes the need to revamp the current vaccination strategies in India with particular focus on re-scheduling the rst dose to 6 months of age.

Correction for ‘Rational design of microporous polybenzimidazole framework for efficient proton exchange membrane fuel cells’ by Harilal et al., J. Mater. Chem. A, 2022, https://doi.org/10.1039/d2ta00734g.

In April 2020, a novel syndrome in children and adolescents, termed multisystem inflammatory syndrome in children (MIS-C), related to SARS-CoV-2 (severe acute respiratory syndrome corona virus 2) infection was first described in the United Kingdom and Italy. In this case series, we analysed 6 patients who had moderate to severe respiratory system manifestations at presentation, closely resembling the acute infection. A retrospective analysis of 6 pediatric patients, diagnosed as MIS-C, presenting with predominant respiratory system manifestations between December 2020 to October 2021 was undertaken. All the 6 patients presented with fever and predominant respiratory symptoms which mainly included difficulty in breathing, while two patients had accompanying chest pain. Gastrointestinal symptoms were found to be the most common accompanying systemic involvement. The inflammatory markers were found to be deranged in all the patients. COVID-19 RT-PCR was negative and serology for COVID-19 IgG was positive in all the patients. All the patients were successfully discharged. All were followed up 1 month after discharge. Residual respiratory morbidity was found in none of them. Our case series highlights the importance of distinguishing active covid-19 infection from MIS-C especially in the setting of overlapping clinical features. Timely diagnosis of MIS-C will also prevent unnecessary antibiotic usage.

Endogenous Cushing’s syndrome (CS) is rare in infancy. Bilateral micronodular adrenocortical disease (BMAD), either primary pigmented nodular adrenocortical disease or the non-pigmented isolated micronodular adrenocortical disease is an important aetiology of CS in this age group, which requires bilateral adrenalectomy for cure. BMAD may be isolated, or a component of Carney complex. Isolated sporadic BMAD without other systemic manifestations poses a diagnostic challenge. Paradoxical cortisol response to dexamethasone suggests, while adrenal histopathology and mutational analysis of the culprit genes confirm BMAD. BMAD was suspected in 6-year-old infant with midnormal adrenocorticotrophic hormone, inconclusive adrenal and pituitary imaging and paradoxical increase in cortisol following high dose of dexamethasone. Exome sequencing revealed heterozygous c.354+1G>C (5′ splice site) variant in the myosin heavy chain gene (MYH8), located in chromosome 17. This particular variant has not been reported in the literature. In view of suspected phenotype and its absence in the population databases, the variant was classified as pathogenic.

Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency (11-BHD) and primary glucocorticoid resistance syndrome (PGRS) are two relatively uncommon causes of gonadotropin-releasing hormone-independent isosexual male precocity; PGRS, however, is considerably rarer than 11-BHD. Other than serum and urinary cortisol, which are elevated in PGRS and low/low–normal in 11-BHD, both of these conditions are indistinguishable by clinical, biochemical or radiological parameters. In 11-BHD, oxidation of 11-deoxycortisol (11-DOC) to cortisol is impaired, resulting in accumulation of 11-DOC and other cortisol precursors. 11-DOC shares structural homology with cortisol, and falsely elevated serum cortisol values are observed in older generation immunoassays (Siemens ADVIA Centaur) due to antibody cross-reactivity. 11-BHD, thus, may be misdiagnosed as PGRS. Structure-based cortisol assays are not widely available in low-income countries. Hence, immunoassays using highly specific antibodies against cortisol are required to ensure assay selectivity. Newer generation analysers probably are effective alternatives to liquid chromatography–tandem mass spectrometry in conditions associated with 11β-hydroxylase defect.

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