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Infantile epileptic spasms syndrome (IESS) is a devastating developmental epileptic encephalopathy (DEE) consisting of epileptic spasms, as well as one or both of developmental regression or stagnation and hypsarrhythmia on EEG. A myriad of aetiologies are associated with the development of IESS; broadly, 60% of cases are thought to be structural, metabolic or infectious in nature, with the remainder genetic or of unknown cause. Epilepsy genetics is a growing field, and over 28 copy number variants and 70 single gene pathogenic variants related to IESS have been discovered to date. While not exhaustive, some of the most commonly reported genetic aetiologies include trisomy 21 and pathogenic variants in genes such as TSC1, TSC2, CDKL5, ARX, KCNQ2, STXBP1 and SCN2A. Understanding the genetic mechanisms of IESS may provide the opportunity to better discern IESS pathophysiology and improve treatments for this condition. This narrative review presents an overview of our current understanding of IESS genetics, with an emphasis on animal models of IESS pathogenesis, the spectrum of genetic aetiologies of IESS (i.e., chromosomal disorders, single-gene disorders, trinucleotide repeat disorders and mitochondrial disorders), as well as available genetic testing methods and their respective diagnostic yields. Future opportunities as they relate to precision medicine and epilepsy genetics in the treatment of IESS are also explored. [ABSTRACT FROM AUTHOR]

AbstractCymbopogon martinii essential oil (Palmarosa essential oil, PRO) has a wide range of applications in food, medicine, and cosmetics. Because of its hydrophobicity, instability, and volatility, the best option to safeguard this oil is to encapsulate it using nanoemulsion technology. In this work, we synthesize O/W (oil/water) nanoemulsions from C. martini essential oil and analyze their biological activity. The study uses co-surfactants like ethanol and surfactants like tween 80 and sodium dodecyl sulfate to create O/W nanoemulsions. The nanoemulsions were investigated using UV, fluorescence, and FT-IR fingerprinting techniques in addition to parameters such as pH and conductivity. Additionally, the study explored the biological effects of the nanoemulsions, including their antibacterial, anti-inflammatory, anticancer, and anti-diabetic capabilities. The pharmacokinetics of drug release was also examined. By using GC-FID analysis, it has been determined that geraniol is the primary component of PRO. The concentrations of total phenolic, total flavonoid, and total condensed tannin were found to be 2.91 mg/g GAE, 9.29 mg/g RE, and 3.78 mg/g VAE, respectively. Palmarosa essential oil nanoemulsions (PNE) were discovered to have 93.24% encapsulation efficiency. The nanoemulsions remained stable and spherical even after 50 days of storage. PNE was synthesized with a mean diameter of 460.55 ± 98.65 nm, a polydispersity index (PDI) value of 0.45 ± 0.04, and a zeta potential measurement of 0.1711 ± 0.05 mV. According to a drug kinetic analysis, PNE displayed a first-order model with an R2 value of 0.930. PNE revealed a dose-dependent 92% inhibition of α-amylase. Additionally, PNE demonstrated potent anti-bacterial activity against the investigated pathogens that varied from 38% to 94%. In the same way, PNE demonstrated potent anti-fungal efficacy against the studied fungal infections, with a range of 50–95%. Anticancer research showed that the PNE-containing cells had a percent viability of 21.29% after 24 hours and 14.92% after 48 hours, respectively. The article explores the possible applications of nanoemulsions made from palmarosa essential oil in the cosmetic, food, and medicinal sectors. [ABSTRACT FROM AUTHOR]

Hypercholesterolemia plays a significant role in the formation of gallstones more important being the cholesterol gallstones. Lipid parameters including total cholesterol, triglycerides, high density lipoproteins and low-density lipoproteins show a significant relation with cholesterol gallstones. Patients with cholesterol type of gallstones have lipid profile parameters towards undesirable ranges as compared to other stones. [ABSTRACT FROM AUTHOR]

Gallbladder disease is a common health problem throughout the world. The commonest reason is gallstones accounting for over 90% of cases. Gallstones can occur in any age group of both the sexes. Incidence of gallstones increases with age and is more common in female population including both symptomatic and asymptomatic cases. The present study was conducted to assess the age and sex distribution of cholelithiasis cases in tertiary hospital of northern india. [ABSTRACT FROM AUTHOR]

Cerium oxide (CeO2) is a material with unique dielectric properties that make it a promising candidate for various applications. High electrical resistance and a wide bandgap of pristine CeO2 limit its applicability in photovoltaics and photo‐electrocatalysis. CeO2 is co‐doped with transition metals (Fe+2/Fe+3 and Cu+2), which reduces its optical bandgap energy and electrical resistance. This study aims to investigate the dielectric relaxation behavior and charge‐carrier dynamics of Fe, Cu–CeO2. The enhanced charge‐carrier dynamics in the Fe, Cu–doped CeO2 compared to pristine CeO2 are reported. Using temperature‐dependent electrochemical impedance spectroscopy (TD‐EIS), the dielectric relaxation and carrier dynamics in pristine CeO2 and Fe, Cu–doped CeO2 in the temperature range 313–473 K along with the modulus spectroscopy are investigated. It is observed that Z′ values reduced with the temperature, thus showing the negative temperature coefficient of resistance in the frequency range 6.28–1.005 × 105 radians s−1. Furthermore, a correlated study of –Z″ and M″ shows the charge‐carrier relaxation behavior changes from ideal Debye type to non‐Debye type with temperature rise in Fe, Cu–doped CeO2. [ABSTRACT FROM AUTHOR]

Aim: We performed a systematic review and network meta-analysis (NMA) to obtain comparative effectiveness estimates and rankings of non-surgical interventions used to treat infantile spasms.Method: All randomized controlled trials (RCTs) including children 2 months to 3 years of age with infantile spasms (with hypsarrhythmia or hypsarrhythmia variants on electroencephalography) receiving appropriate first-line medical treatment were included. Electroclinical and clinical remissions within 1 month of starting treatment were analyzed.Results: Twenty-two RCTs comparing first-line treatments for infantile spasms were reviewed; of these, 17 were included in the NMA. Both frequentist and Bayesian network rankings for electroclinical remission showed that high dose adrenocorticotropic hormone (ACTH), methylprednisolone, low dose ACTH and magnesium sulfate (MgSO4 ) combination, low dose ACTH, and high dose prednisolone were most likely to be the 'best' interventions, although these were not significantly different from each other. For clinical remission, low dose ACTH/MgSO4 combination, high dose ACTH (with/without vitamin B6 ), high dose prednisolone, and low dose ACTH were 'best'.Interpretation: Treatments including ACTH and high dose prednisolone are more effective in achieving electroclinical and clinical remissions for infantile spasms.What This Paper Adds: Adrenocorticotropic hormone and high dose prednisolone are more effective than other medications for infantile spasms. Symptomatic etiology decreases the likelihood of remission even after adjusting for treatment lag. [ABSTRACT FROM AUTHOR]

This scientific commentary refers to 'Randomized placebo-controlled crossover trial of memantine in children with epileptic encephalopathy' by Schiller I et al. i (https://doi.org/10.1093/brain/awac380). Nine patients (33%) in the memantine group achieved the primary outcome compared to only two patients (7%) in the placebo group ( I P i < 0.04).[4] Of the nine responders, six had >50% seizure reduction. Trend reductions in autism probability were seen, but with both memantine and placebo, while measures of functional impairment trended lower with memantine but not placebo. [Extracted from the article]

The contamination of environmental sully with antibiotics is regarded as a major problem today and predictable to attain more recognition in near future. However, human intervention resulting in antibiotic consumption is being enhancing all around the world. Our review of literature revealed the role of microbiome in sully and how antibiotic resistant genes raised. The structure of antibiotics basically influenced by natural components such as biotic and abiotic push which shifts based on different soils. Therefore, management of microbiome in soil and their expression studies were distinctively revealed. The assessment of antibiotic resistance genes with help of next generation sequencing provided a clear comprehension on genome and transcriptome of the bacterial genes. Thus, interaction of microbiome with soil can also be well understood. The current findings in our study will guide every researcher to follow logical protocol in analyzing microbiota composition is covered as well and also to understand its metagenomic and sequenced with next-generation sequencer which helps to comprehend the diverse micro-flora present in soil and its operation. Finally, later progresses in bioinformatics computer program, flow of work, and applications for analyzing metagenomic information are put in a nutshell. [ABSTRACT FROM AUTHOR]

In the present work, fluorescence anisotropy studies of BODIPY (pyrromethene 546 or C14H17BF2N2) dye have been performed and found that it has a potential to be used as a thermal probe to measure the temperature of microfluid. It is well-known that a dye rotates in its excited state, so to control the molecular rotation of the dye in the excited state, sorbitol is used in the solution. It has been found that adding sorbitol, fluorescence anisotropy increases, fluorescence lifetime decreases. It has been found that adding sorbitol, temperature sensitivity increases. To study the effect of -CH2 groups on fluorescence properties, another BODIPY (pyrromethene 597 or C22H33BF2N2) dye is also used, as C14H17BF2N2 and C22H33BF2N2 have similar structures with a difference of only -8CH2 groups. It has been found that C22H33BF2N2 has superior properties over C14H17BF2N2, i.e., pyrromethene 597 shows larger temperature sensitivity as compared to pyrromethene 546, even without the addition of sorbitol. [ABSTRACT FROM AUTHOR]

Background: Infantile apneic seizures (IASs) are unexpected life-threatening events. We aimed to determine the utility of prolonged video-electroencephalography (vEEG) and heart rate variability (HRV) in IAS.Methods: The study included seven infants with apneic seizures captured by vEEG, percutaneous oxygen saturation (SpO2), and electrocardiography (ECG). Interictal, preictal, and postictal HRV of patients and N2 sleep HRV of 10 age-matched controls were determined.Results: We analyzed seven vEEGs (duration = 17 to 87 hours) of seven patients aged three to 13 months (mean onset age of apneic event = 6.3 months). Fifteen apneic seizures (one to five per infant) were captured. The initial apneic seizure was captured at 7.5 to 76 hours (mean = 36.6 hours) after vEEG initiation. Ictal rhythmic delta/theta/fast waves were seen over temporal (five patients), central (one), and diffuse areas (one). Ictal SpO2 decreased between 1.5% and 90% (mean = 47.9%). Ictal decreased heart rate (HR) (six seizures) and ictal increased HR (14) was detected. Both decreased and increased HR was observed (five). The preictal low-frequency (LF)/high-frequency (HF) ratio was significantly higher than the interictal LF/HF ratio (P = 0.048). Preictal (P = 0.048), and postictal (P = 0.019) root mean square of successive differences (RMSSDs) of patients were lower than the sleep RMSSD of controls. These results indicated dominant sympathetic activity. RMSSD from interictal to preictal periods tended to be higher in IAS with decreased HR than in IAS with increased HR alone (P = 0.066). The postictal RMSSD showed tendency to be higher in IAS with decreased HR than in IAS with increased HR alone (P = 0.088). The decreased HR and increased RMSSD suggested not only sympathetic activity but also escalated parasympathetic activity in IAS.Conclusions: Infants with unexpected apneic events should be monitored with prolonged vEEG, SpO2, and ECG. Abnormal HRV in infants with apneic seizures might indicate additional autonomic dysregulation in IAS. [ABSTRACT FROM AUTHOR]

BACKGROUND: Users with Severe Speech and Motor Impairment (SSMI) often use a communication chart through their eye gaze or limited hand movement and care takers interpret their communication intent. There is already significant research conducted to automate this communication through electronic means. Developing electronic user interface and interaction techniques for users with SSMI poses significant challenges as research on their ocular parameters found that such users suffer from Nystagmus and Strabismus limiting number of elements in a computer screen. This paper presents an optimized eye gaze controlled virtual keyboard for English language with an adaptive dwell time feature for users with SSMI. OBJECTIVE: Present an optimized eye gaze controlled English virtual keyboard that follows both static and dynamic adaptation process. The virtual keyboard can automatically adapt to reduce eye gaze movement distance and dwell time for selection and help users with SSMI type better without any intervention of an assistant. METHODS: Before designing the virtual keyboard, we undertook a pilot study to optimize screen region which would be most comfortable for SSMI users to operate. We then proposed an optimized two-level English virtual keyboard layout through Genetic algorithm using static adaptation process; followed by dynamic adaptation process which tracks users' interaction and reduces dwell time based on a Markov model-based algorithm. Further, we integrated the virtual keyboard for a web-based interactive dashboard that visualizes real-time Covid data. RESULTS: Using our proposed virtual keyboard layout for English language, the average task completion time for users with SSMI was 39.44 seconds in adaptive condition and 29.52 seconds in non-adaptive condition. Overall typing speed was 16.9 lpm (letters per minute) for able-bodied users and 6.6 lpm for users with SSMI without using any word completion or prediction features. A case study with an elderly participant with SSMI found a typing speed of 2.70 wpm (words per minute) and 14.88 lpm (letters per minute) after 6 months of practice. CONCLUSIONS: With the proposed layout for English virtual keyboard, the adaptive system increased typing speed statistically significantly for able bodied users than a non-adaptive version while for 6 users with SSMI, task completion time reduced by 8.8% in adaptive version than nonadaptive one. Additionally, the proposed layout was successfully integrated to a web-based interactive visualization dashboard thereby making it accessible for users with SSMI. [ABSTRACT FROM AUTHOR]

Children and adults with genetic generalized epilepsy may have focal clinical seizure symptoms as well as electroencephalographic (EEG) findings. This may pose a diagnostic challenge to clinicians, especially when concomitant focal neuroimaging findings exist and the epilepsy is medically refractory. We sought to highlight the challenges that clinicians may face through the description of 2 children with suspected genetic generalized epilepsy who had both focal seizure symptoms and EEG/neuroimaging findings and underwent invasive EEG monitoring. Ultimately, invasive monitoring failed to demonstrate a focal origin for the seizures in both cases, and instead confirmed the presence of genetic generalized epilepsy. We demonstrate that ≥3-Hz generalized monomorphic spike and waves are less likely to represent secondary bilateral synchrony, that focal neuroimaging findings may not always be causal and that repeated hyperventilation is an essential activation procedure for genetic generalized epilepsy. [ABSTRACT FROM AUTHOR]

Aim: To determine whether epilepsy surgery improved health-related quality of life (HRQoL) and whether seizure freedom after surgery mediated the improvement in HRQoL.Method: This multicenter cohort study compared HRQoL after epilepsy surgery to pharmacological management in children with drug-resistant epilepsy (DRE). HRQoL was measured using the Quality of Life in Childhood Epilepsy (QOLCE) questionnaire at baseline and 1-year follow-up. The mediator between treatment type and HRQoL was seizure freedom.Results: Two hundred and thirty-seven patients were recruited (surgery group: n=147 [92 males, 45 females]; pharmacological group: n=90 [53 males, 37 females]). Mean age at seizure onset was 6 years (SD 4y 4mo) in the surgical group and 6 years 1 month (SD 4y) in the pharmacological group. The odds ratio of seizure freedom was higher for the surgery versus pharmacological group (β=4.24 [95% confidence interval {CI}: 2.26-7.93], p<0.001). Surgery had no direct effect on total QOLCE score at 1-year (β=0.24 [95% CI -2.04 to 2.51], p=0.839) compared to pharmacological management, but had an indirect effect on total QOLCE that was mediated by seizure freedom (β=0.92 [95% CI 0.19-1.65], p=0.013), adjusting for baseline total QOLCE score. Surgery had a direct effect on improving social function (p=0.043), and an indirect effect on improving physical function (p=0.016), cognition (p=0.042), social function (p=0.012) and behavior (p=0.032), mediated by seizure freedom.Interpretation: Greater seizure freedom achieved through epilepsy surgery mediated the improvement in HRQoL compared to pharmacological management in children with DRE.What This Paper Adds: Seizure freedom is higher after pediatric epilepsy surgery compared to pharmacologically managed epilepsy. Surgery indirectly improves health-related quality of life (HRQoL) mediated by seizure freedom compared to pharmacological management. Surgery has a direct effect on improving social function relative to pharmacological management. Baseline HRQoL was an important predictor of HRQoL after treatment. [ABSTRACT FROM AUTHOR]

Background: The safety of hemispherectomy between staged cardiac procedures is unknown and not previously reported. Method: Retrospective review of a case with drug-resistant epilepsy due to stroke following bidirectional cavopulmonary connection (BDCPC). Results: This report describes the first case of a successful pediatric peri-insular functional hemispherectomy in the setting of a BDCPC. A discussion of the complex preoperative planning from both a cardiac and neurological perspective is presented. Considerations regarding hemispherectomy and its effects on the cardiac physiology, and perioperative considerations are emphasized in clinical decision making. Conclusions: A multidisciplinary approach was critical in this child which led to a successful outcome. [ABSTRACT FROM AUTHOR]

Congenital disorders of glycosylation (CDG) are multisystemic inherited metabolic disorders with marked phenotypic variability. The most frequent described type is PMM2-CDG (earlier known as CDG Type Ia) which presents either with pure neurologic features or with combined neurologic and systemic features. The classical presentation is characterized by varied combinations of developmental delay, hypotonia, ataxia, dysmorphism, inverted nipples, and abnormal fat distribution. Strokelike episodes and seizures are known acute complications that usually occur on a background of developmental delay, ataxia, or dysmorphism. We report here a developmentally normal young girl who presented with isolated strokelike episodes and was diagnosed to have CDG Type Ia. This condition should be kept in the differentials of unexplained strokelike episodes in children. The diagnosis has important therapeutic and prognostic implications. [ABSTRACT FROM AUTHOR]

Background: Guillain-Barre syndrome (GBS) is a common acquired polyneuropathy in children.Aim: To describe the clinical and serial electrophysiological features along with short-term outcomes of children with GBS in north India.Setting and Design: This was a prospective study conducted at a tertiary care pediatric hospital in north India.Materials and Methods: Consecutive children, aged 2 to 18 years, with GBS, presenting within 4-weeks of onset of weakness, diagnosed on clinical and/or electrophysiological grounds, were enrolled. The enrolled children underwent a detailed clinical-assessment followed by nerve conduction studies. Repeat nerve conduction studies were performed after 2-weeks of the first study to determine changes in the electrophysiological subtype. The patients were followed up for 3 months.Results: Thirty-six children were studied. The mean age at presentation was 5.1 years [standard deviation (SD): 2.1]. The mean medical research council (MRC)-sum-score at admission was 24.1 (SD: 10.4). Thirty-three children (91%) had loss of ambulation, 24 (66%) had cranial nerve involvement, and 6 (16.6%) required ventilation. At presentation, 20 had acute motor axonal neuropathy (AMAN), 13 had acute inflammatory demyelinating polyneuropathy (AIDP), 2 had in-excitable nerves, and 1 had normal findings. Four children, initially diagnosed as AIDP, had AMAN with reversible conduction failure on the repeat study. The final classification was AMAN in 25 (69.4%; 95% confidence interval (CI), 51.9-83.7%) and AIDP in 9 children (25%; 95% CI, 12.1-42.2%). Only one patient was nonambulatory at a 3-month follow-up (n = 32). The Erasmus GBS outcome score was 2 in 2 (5.6%), 3 in 5 (13.9%), 4 in 26 (72.2%), and 5 in 3 (8.3%) patients.Conclusions: The serial electrophysiological studies were helpful in establishing the final correct diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study is to describe the clinical characteristics, multimodality imaging findings, and clinical course of iris varices.Methods: Retrospective, noncomparative, observational case series of 28 eyes of 26 patients with iris varices, diagnosed between 2007 and 2017, has been used.Results: The mean (±SD) age was 58.3 ± 12.5 years (median 57.5, range 37-81). Patients were 57.7% male and 27% hypertensive. Varices were bilateral in two patients. The mean and median visual acuities were both 20/20 (range 20/16-20/40). Intraocular pressures were 16 mmHg (10-23 mmHg). Secondary glaucoma did not occur. The inferotemporal iris quadrant was affected in 75%. A single varix was seen in 64% and 36% appeared multiple. Varix orientation was radial in 57% and circumferential in 21%. Combined radial and circumferential varix orientation was noted in 18%. One had independent radial and circumferential varices in separate quadrants. A single episcleral sentinel blood vessel directed to the varix was present in 36%. Ultrasound biomicroscopy (UBM) showed a slightly increased mean iris thickness of 0.8 mm and multiple echolucent iris stromal vascular channels. Iris angiography showed no leakage of dye. Managed by observation over a mean follow-up of 37.7 months (range, 3-129), 96.4% eyes were stable and one (3.6%) regressed. No corectopia, ectropion uveae, hyphema, or metachronous anterior segment benign or malignant tumors occurred.Conclusion: Iris varix is primarily located in the inferotemporal quadrant and not associated with dysmorphic pupillary findings, progression, secondary glaucoma, or malignancy. Iris varices were benign vasculopathies with no associated ocular or vision-related morbidity. [ABSTRACT FROM AUTHOR]

Highlights • Extra-operative mapping (penfield technique) frequently leads to after-discharges and seizures. • Intra-operative motor-mapping (IODCS-SDG) using "train of five" provided more mapping information. • No stimulation-provoked seizures were noted during IODCS-SDG. Abstract Objectives The objective of this study was to review our experience with intra-operative "train of five" stimulation using subdural grid for motor mapping in children undergoing epilepsy surgery evaluation. Methods Twenty consecutive children below 18-years of age with drug-refractory epilepsy who underwent invasive-EEG monitoring using subdural-grid placement and intra-operative motor mapping using direct cortical stimulation by sub-dural grid electrodes (IODCS-SDG) at our institution between January-2016 and June-2017 were reviewed. Stimulation was delivered through the subdural-grid electrodes using a train-of-five pulses and muscle responses were recorded by motor-evoked-potentials (MEPs). Intra-operative direct cortical stimulation delivered through a ball-tipped probe (IODCS-probe) and extra-operative motor-mapping (EODCS-SDG) were also performed. Results IODCS-SDG was completed in 20 patients and subsequent EODCS-SDG was done in 17/20 patients. MEP responses were more commonly obtained in the deltoid (19/20), extensor-digitorum-communis (20/20) and first-dorsal-interosseus (19/20). The median thresholds varied between 40 V and 60 V for the six muscle groups. The respective IODCS-probe thresholds tended to be similar. No stimulation-provoked seizures or anaesthesia-related complications were noted during IODCS-SDG. EODCS-SDG could not be completed in 4/17 children and mapping data obtained was frequently inadequate. Nine patients demonstrated 100% concordance between IODCS-SDG and EODCS-SDG for the common mapped body regions. Stimulation-provoked seizures during EODCS-SDG were seen in 6/17 (35.3%) and after-discharges in 7/17 (41.2%) children. Conclusions IODCS-SDG could be performed safely in children with drug refractory epilepsy undergoing invasive EEG monitoring. Significance IODCS-SDG may be a useful adjunct to EODCS-SDG in motor mapping for children. [ABSTRACT FROM AUTHOR]

Background: Epilepsy in children negatively impacts on caregiver quality of life (QOL). The study aimed to evaluate the relationships between patient factors [demographics, clinical factors, health-related quality of life (HRQL)], contextual factors (socio-economic factors), caregiver mood, and caregiver QOL, and whether family factors mediate the relationship between patient HRQL and caregiver QOL.Methods: Children aged 4-18 years with medically intractable epilepsy were enrolled. Patient demographics, clinical data, patient HRQL [measured using Quality of Life in Childhood Epilepsy Questionnaire (QOLCE)], socio-economic factors, caregiver anxiety and depression, and family factors (adaptation, resources and demands) were assessed. Caregiver QOL was measured using CarerQol, which evaluates care-related QOL, incorporating the negative and positive effects of caregiving.Results: One hundred and eighty-one children were studied. In bivariable regression analysis, higher patient HRQL (QOLCE) (β = 0.54, p < 0.001) and household income (β = 10.49, p = 0.019) were associated with higher caregiver QOL. Higher depression (β = − 2.48, p < 0.001) and anxiety (β = − 2.04, p < 0.001) were associated with lower caregiver QOL. Seizure severity and other socio-economic factors did not influence caregiver QOL (all p > 0.05). In multivariable regression analysis, higher QOLCE (β = 0.21, p = 0.001), lower depression (β = − 1.07, p < 0.001) and lower anxiety (β = − 1.19, p < 0.001) were associated with higher caregiver QOL. Family demands and resources moderated the relationship between patient HRQL and caregiver QOL.Conclusions: Patient HRQL and caregiver mood were more important correlates of caregiver QOL than seizure severity in medically intractable epilepsy. The findings are significant in delineating variables (caregiver mood and family factors) that are potentially modifiable, and show promise for improving caregiver QOL. [ABSTRACT FROM AUTHOR]

Background: A retrospective analysis using chromosomal microarray in syndromic patients with intellectual disability from genetic clinics of a tertiary healthcare center in India was conducted.Aim: To identify the spectrum of chromosomal abnormalities detected on microarray analysis.Settings and Design: Cases were identified among those with intellectual disability with dysmorphism attending genetic clinics of a tertiary care center.Patients and Methods: All patients attending genetic clinics over a 3-year period were analyzed. Clinical profile and baseline investigations were noted on a predesigned proforma. Among the 65 studied cases, there were 12 cases suggested to be having Prader-Willi syndrome (PWS), 27 cases with DiGeorge/velocardiofacial syndrome (DGS), and 1 case with Williams-Beuren syndrome (WBS). These were detected by fluorescent in situ hybridization (FISH) analysis with specific probes and were excluded from the final analysis. Chromosomal microarray analysis (CMA; single-nucleotide polymorphism-based array-comparative genomic hybridization) was performed as per the clinical indication in selected patients with dysmorphism, microcephaly, mental retardation, and/or multiple malformations. These patients had a negative result on FISH analysis.Results: In suspected patients with PWS, FISH and methylation testing confirmed six cases to be really PWS. FISH also detected five cases of DGS and one case of WBS. These were excluded from the final analysis. Among the 18 cases tested by CMA, in 13 patients, abnormalities with potential clinical significance were identified. Genetic counseling was done in all these cases. Prenatal diagnosis was done in one family.Conclusion: In cases with dysmorphism with or without mental retardation or cardiac defect, advanced studies such as CMA can lead to a definitive diagnosis. Genetic counseling is mandatory in all these cases and a prenatal diagnosis is also feasible in selected families. [ABSTRACT FROM AUTHOR]

Essential oil from Melaleuca alternifolia (also known as Tea tree essential oil, TTO) is used as traditional medicine and used as therapeutic in medicine, food and cosmetic sectors. However, this oil is highly unstable, volatile and prone to oxidation which limits its practical use. The objective of this study was synthesis of tea tree oil based O/W (oil/water) nanoemulsions (tea tree essential oil nanoemulsions, TNE) and evaluation of its biological potential. Physiological characterization was carried out using UV, fluorescent, and FT-IR techniques. Various biological activities such as anticancerous, antidiabetic and anti-inflammatory were also estimated. Pharmacokinetics study on TNE was carried out. Encapsulation efficiency of nanoemulsions was found to be 83%. Nanoemulsions were spherical in shape with globule size 308nm, zeta potential -9.42 and polydispersity index was 0.31. Nanoemulsions were stable even after 50days of storage at different temperatures. Anti-oxidant potential of TNE was conducted by various assays and IC50 were: Nitric oxide radical scavenging activity:225.1, DPPH radical scavenging activity:30.66, Iron chelating assay:38.73, and Iron reducing assay:39.36. Notable anticancer activity was observed with the percent cell viability of HeLa cells after treatment with 1, 2 and 5µl of TNE was 82%, 41% and 24%, respectively. Antidiabetic study revealed that TNE inhibited -amylase in a dose-dependent manner, with 88% inhibition at its higher volume of 250µl. Drug kinetic study revealed that nanoemulsions exhibited first-order model. Based on this, the possible role of M. alternifolia oil-based nanoemulsions in cosmetic, food, and pharma sectors has been discussed. [ABSTRACT FROM AUTHOR]

The article presents the discussion on Infantile spasms syndrome (ISS) being a severe epileptic encephalopathy characterized by epileptic spasms, developmental stagnation and/or regression. Topics include treatment of ISS showing the use of anti‑seizure medications and hormonal therapy in the form of prednisolone or adrenocorticotropic hormone; and determining other forms of steroid therapy being used for the treatment of ISS.

Background: West syndrome is a catastrophic epilepsy syndrome characterized by infantile spasms, hypsarrhythmia, and developmental arrest or regression.Aim: The aim of this study was to explore the role of pyridoxine in the management of infantile spasms.Setting and Design: This was a pilot, randomized, open-label trial conducted at a tertiary level hospital from November 2012 to March 2014.Materials and Methods: Children aged 3 months to 3 years presenting with infantile spasms in clusters (at least 1 cluster/day) with hypsarrhythmia or its variants on electroencephalogram (EEG) were enrolled. The study participants were randomized to receive either oral prednisolone (4 mg/kg/day) alone or 30 mg/kg/day of pyridoxine with oral prednisolone. The primary outcome measure was the proportion of children who achieved spasm freedom for 48 h on day-14 after treatment initiation, as per parental reports, in both the groups. The adverse effects were also monitored. The study was registered with clinicaltrials.gov (ClinicalTrials.gov Identifier: NCT01828437).Results: Sixty-two children were randomized into the two groups with comparable baseline characteristics. The proportion of children with spasm cessation on day-14 was similar in the two groups (39 vs. 37%, P = 0.98). The adverse effects were comparable in both the groups.Conclusions: The combination of pyridoxine with oral prednisolone was not found to be a beneficial therapy as compared to prednisolone alone in the treatment of infantile spasms in this pilot study. However, high dose pyridoxine may be safe in children with infantile spasms. [ABSTRACT FROM AUTHOR]

BACKGROUND: Guidelines recommend ABO-identical platelet (PLT) transfusions. Hemolytic reactions after a minor ABO-incompatible PLT transfusion have escalated due to single-donor platelets (SDP) containing ABO-incompatible plasma. Avoiding such events by examining titers or performing plasma reduction is cumbersome. The introduction of platelet additive solutions (PAS) has enabled to reduce these reactions by avoiding passive transfer of isoagglutinin. Our aim was to study antibody titers (anti-A, anti-B) in "O" SDP by adding PAS at source and the quality parameters with reference to viability, morphology, and metabolism. MATERIALS AND METHODS: Group "O" SDP (n = 50) were prepared on a standard cell separator. PAS in a ratio of 70:30 (PAS: plasma) was added at source under sterile conditions (study arm). The units were studied on day of collection (day 0) and day 4 and compared with SDP containing 100% plasma (control arm). A titer study was performed after PAS addition. RESULTS: In the study group, the median antibody titers (anti-A, anti-B) reduced from 128 to16, post-PAS addition (P < 0.001). Morphology scores were superior in PAS platelet concentrates (P < 0.001). Metabolic parameters pO2 and pCO2 were similar in the two arms signifying good unit storage and stable oxygen consumption (P > 0.05). Lactate levels, glucose consumption rate, and lactate production rates were significantly low in study arm showing the advantage of PAS. CONCLUSION: O group SDPs can be prepared with PAS and the beneficial effects were significant with respect to antibody titers. Quality parameters were well maintained. Availability of PAS units has benefitted patients. [ABSTRACT FROM AUTHOR]

Introduction: Hyperleukocytosis (HL) and leukostasis seen in myeloid leukemias are a medical emergency. We present a case series of ten such patients in a 4-year period. Sixteen therapeutic leukocyte reduction (TLR) were done in ten cases along with other supportive measures. The American Society for Apheresis supports the routine implementation of TLR in cases of HL secondary to myeloid leukemias with signs of leukostasis. Materials and Methods: The procedures were performed on the intermittent flow cell separator after discussion with the treating physician about patient's condition. Clinical, demographic, analytical, and technical variables were reviewed retrospectively and the patients were followed up at the end of 4 years. Descriptive analysis was performed for all variables, and relationships between quantitative variables and categorical variables were determined by applying the Student's t-test. Results: The mean age of presentation was 34 years. Priapism was the most common symptom followed by respiratory distress and neurological disturbances. After an average of 1.6 TLR procedures, the mean leukocyte count reduction achieved was 39.9% along with symptomatic relief. The mean survival at 4-year follow-up was 12.8 months and the overall mortality was 20%. Acute myeloid leukemia patients presented with lower mean platelet counts compared to chronic myeloid leukemia patients; however, the platelet loss in the final product was minimized. Conclusion: TLR is a safe and effective therapy for leukoreduction in hematological malignancies in our experience. [ABSTRACT FROM AUTHOR]

Background and Objectives: The occurrence of hemolysis due to transfusion of ABO plasma-incompatible platelets (PLTs) is challenging. There has been no consensus for critical antibody titers in the transfusion community. This study was conducted to understand the trends of anti-A and anti-B antibody titer levels in O group donors and to identify any specific patterns of distribution in relation to age and gender. Materials and Methods: A total of 1635 Group O PLT donors were randomly selected for this prospective study. Serial 2-fold doubling dilutions were prepared for each sample to calculate the titer of anti-A and anti-B in a standard 96 well micro-plate. Tube technique was used for comparison with the microplate method for 100 samples. Results: Out of 1635 donors, 1430 (87.46%) were males and 205 (12.54%) were females. The median titer for anti-A and anti-B was 128 with range from 4 to 2048. Spearman's correlation coefficient for microplate versus tube technique was estimated to be 0.803 (P < 0.01, two-tailed). 57.12% and 51.19% of all donors had titers ≥128 for anti-A and anti-B, respectively. The geometric mean of anti-A and anti-B was 155.7 and 137.28, respectively. The titers were significantly higher (P < 0.001) in female donors. An inverse relation between titer levels and age was seen. Conclusion: Microplate can be used to perform titers in resource-constrained settings. Screening for critical titers in O group donors is essential as they are more implicated in hemolytic transfusion reactions. In the absence of a global consensus on this topic, institutes may need to formulate their own guidelines on handling ABO plasma-incompatible PLT transfusions. [ABSTRACT FROM AUTHOR]

Hereditary myoclonus-dystonia (DYT 11) is caused by the epsilon-sarcoglycan (SGCE) mutation. The clinical details and investigations of cases diagnosed with myoclonus-dystonia were reviewed. We describe 5 patients (3 families) with myoclonus-dystonia diagnosed at our center. Majority of the patients had the classical phenotype with few atypical features (adult-onset disease and onset in lower limbs). Four patients carried a mutant variant in the SGCE-gene. A diagnosis of myoclonus-dystonia should be considered in cognitively normal patients with early-onset myoclonus (that may occur both at rest and/or action) with or without dystonia and with or without psychiatric-disturbances. [ABSTRACT FROM AUTHOR]

A 39-year-old female presented with a painless yellow-pink tumor on her right eye. High-frequency ultrasound imaging revealed an epibulbar lesion with homogenous low internal reflectivity and no evidence of intraocular invasion. The patient underwent excisional biopsy leading to a pathology diagnosis of myxoma. Additional surgical margins as well as adjuvant cryotherapy margins were followed by extensive conjunctival repair. Herein, we report on a conjunctival myxoma with unique ultrasonographic findings. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study is to retrospectively collect and then describe the clinico-radiographical profile of confirmed cases of leukodystrophy who presented over a 5-year period to a tertiary care teaching hospital in North India. Materials and Methods: The case records of 80 confirmed cases of leukodystrophy were reviewed and the cases have been described in terms of their clinical presentation and neuroimaging findings. Results: The cases have been grouped into five categories: Hypomyelinating, demyelinating, disorders with vacuolization, cystic, and miscellaneous. The commonest leukodystrophies are megalencephalic leukoencephalopathy with subcortical cysts (MLC), Pelizaeus-Merzbacher disease (PMD), and metachromatic leukodystrophy (MLD). A notable proportion of hypomyelinating disorders were uncharacterized. Conclusions: Leukodystrophies at this point of time have no definite cure. They have a progressively downhill clinical course. Early diagnosis is imperative for appropriate genetic counseling. A simplified approach to diagnose common leukodystrophies has also been provided. It is important to develop a registry, which can provide valuable epidemiological data to prioritize research in this field, which has many unanswered questions. [ABSTRACT FROM AUTHOR]

Objectives To explore the existing evidence for anti-convulsant drugs and their routes of administration in treating acute seizures in children and adults when intravenous access is not available. Methods All major databases including Medline via Ovid, PubMed, Cochrane CENTRAL, Embase, and Google Scholar were searched till May 2015. Randomized and quasi-randomized controlled trials comparing two anti-convulsant drugs (at least one comparator being administered through non-intravenous route) for treatment of acute seizures were included. Outcome measures Primary outcome measure was proportion of children with clinical seizure cessation within 10 min of drug administration. Secondary outcome measures were time taken to clinical seizure cessation from the time of admission and from the time of drug administration, and incidence of significant adverse effects. Results Out of the 19,165 citations, 26 studies were finally included. Regarding the primary outcome measure, the quality of evidence was ‘moderate’ for following 3 comparisons: buccal midazolam being superior to per-rectal diazepam (RR 1.14; 95% CI, 1.06–1.24), intra-nasal lorazepam being same as intravenous lorazepam (RR 1.04; 95% CI, 0.89–1.22) and intramuscular paraldehyde (RR 1.22; 95% CI, 0.99–1.52). The quality of evidence was ‘very-low’ for 1 comparison: per-rectal lorazepam being superior to per-rectal diazepam (RR 3.17; 95% CI, 1.63–6.14). The quality of evidence was ‘low’ for following 2 comparisons: sub-lingual lorazepam being inferior to rectal diazepam (RR 0.71; 95% CI, 0.62–0.81), and intranasal midazolam being superior to per-rectal diazepam (RR 1.14; 95% CI, 1.05–1.25). The rest of the comparisons did not show any difference, but the quality of evidence was ‘low’ to ‘very low’. The time to seizure cessation after drug administration was lower in the intravenous group. However, time to seizure cessation after presentation (includes time for drug administration) was lower in the non-intravenous group. Significant adverse effects were infrequently reported and when present, were similar in both the groups. Conclusions When intravenous access is not available, non-intravenous routes of administration of benzodiazepines should be considered for the control of acute seizures in children/adults. The preference may be guided by availability, expertise and social preference. [PROSPERO No: CRD42015019012]. [ABSTRACT FROM AUTHOR]

This paper presents a system based on Seismocardiography (SCG) to monitor the heart sound signal for the long-term. It uses an accelerometer, which is of small size and low weight and, thus, convenient to wear. Such a system should also be robust to various noises which occur in real life scenarios. Therefore, a detailed analysis is provided of the proposed system and its performance is compared to the performance of the Phoncardiography (PCG) system. For this purpose, both signals of five subjects were simultaneously recorded in clinical and different real life noisy scenarios. For the quantitative analysis, the detection rate of fundamental heart sound components, S1 and S2, is obtained. Furthermore, a quality index based on the energy of fundamental components is also proposed and obtained for the same. Results show that both the techniques are able to acquire the S1 and S2, in clinical set-up. However, in real life scenarios, we observed many favourable features in the proposed system as compared to PCG, for its use for long-term monitoring. [ABSTRACT FROM PUBLISHER]

A series of novel quinoline–oxadiazole hybrid compounds was designed based on stepwise rational modification of the lead molecules reported previously, in order to enhance bioactivity and improve druglikeness. The hybrid compounds synthesized were screened for biological activity against Mycobacterium tuberculosis H 37 Rv and for cytotoxicity in HepG2 cell line. Several of the hits exhibited good to excellent anti-tuberculosis activity and selectivity, especially compounds 12m , 12o and 12p , showed minimum inhibitory concentration values <0.5 μM and selectivity index >500. The results of this study open up a promising avenue that may lead to the discovery of a new class of anti-tuberculosis agents. [ABSTRACT FROM AUTHOR]

This study describes the clinical characteristics, treatment, and outcome of children with West syndrome in a tertiary care hospital in north India. Overall, 310 case records diagnosed from January 2009 to June 2012 were reviewed. The median age of onset of spasms was 5 months (interquartile range = 2.5-7 months). The predominant underlying etiology was perinatal cerebral ischemia (55%). Adrenocorticotropic hormone or oral steroids were received by 92% children, of whom 43% became seizure free. Median lag time for appropriate treatment initiation was significantly less in patients who became seizure free as compared to those with persisting seizures (11 vs 15 months, P = .001) soon after receiving treatment of choice. Later age at onset of spasms was associated with a favorable seizure outcome (P = .03). In a resource-limited setting, unawareness along with treatment costs and repeated visits to the hospital are significant obstacles to optimum management. [ABSTRACT FROM AUTHOR]

Polydimethyl siloxane (PDMS) and Silica (SiO 2 ) nanoparticle composite blocks of three different batches (CB1–CB3) made by varying the size of SiO 2 nanoparticles (NP), are studied for the degree of hydrophilization and retainability after oxidation by contact angle measurements (CA) and force distance spectroscopy (FDS) using Atomic Force Microscope (AFM). While CA measurements have shown high hydrophilization and retainability for CB3, F-D spectroscopy has reiterated the observation and has shown long range interactive forces and high Debye length of the electrostatic double layer formed. These results are in agreement with the radius ratio rule of binary sphere system for high density packing in the composite and thereby for strong hydrophilization and retainability due to reinforcement and restricted diffusion of uncured polymer. [ABSTRACT FROM AUTHOR]

Titanium dioxide (TiO2) is a highly stable, photosensitive, and nontoxic metal oxide. The physical and chemical properties of TiO2 electrodes have been explored extensively for the last few decades. However, a detailed and comparative study of the dielectric relaxation and carrier kinetics with the dopants is still lacking. Herein, the crystallinity of TiO2 electrodes decreased with the dopants is reported. The doping of copper (Cu) in TiO2 reduces its bandgap to 2.98 eV from 3.22 eV. The improved charge carrier conduction at high frequencies (ω = 103–106 radians s−1) with the doping is noticed. The charge transfer resistance (Rct) for the electrons is minimum for Cu–TiO2 (Rct = 34.11 Ω) and Cu, Zn, and N–TiO2 (Rct = 27.44 Ω) compared to pristine and Cu and Zn–TiO2. Further, the high electrical permittivity is associated with the polar nature of electrodes. The correlation analysis of impedance and modulus spectroscopy data provides a prodigious picture of the charge carrier relaxation and conduction mechanism. A shift in the dielectric relaxation process from Debye type to non‐Debye type is observed after doping due to defect‐mediated fast relaxation of polarization. [ABSTRACT FROM AUTHOR]

The article discusses the case of a 14-year-old girl with anti-N-methyl-D-aspartate antibodies-receptor (NMDAR) encephalitis. Topics include the treatment given to the girl while in the hospital, seizures as a common presentation in an anti-NMDA encephalitis, and the presence of extreme delta brushes (EDP) in the electroencephalogram (EEG) of individuals with anti-NMDA encephalitis.

There is scarcity of data on spectrum and outcome of invasive filamentous fungal infections (IFIs) in children with Type 1 diabetes (T1D) from developing countries. A retrospective review of medical records of children with T1D hospitalized with IFI over the past decade at the Pediatric Endocrinology and Diabetes Unit of a large tertiary care hospital of North India was performed with an aim to study their clinical spectrum, hospital course and final outcome. Of the 10 patients studied, nasal/paranasal involvement was seen in 6 and pulmonary involvement in 5 patients. One patient developed disseminated disease. Majority of the identified mycoses belonged to Class Zygomycetes Order Mucorales. Early surgery along with antifungal therapy helped limit the extension of infection and achieve a good outcome in majority of patients. Two patients died; one with a late diagnosis of pulmonary mucormycosis and the other with disseminated disease. The longterm morbidity in the survivors was minimal. In conclusion, rapid diagnosis followed by a multimodal approach involving aggressive surgical debridement, appropriate antifungal therapy and control of hyperglycemic state is the key to good outcome in this otherwise lethal infection. [ABSTRACT FROM AUTHOR]

There is scanty data regarding the efficacy and tolerability of the modified Atkins diet in children with Lennox-Gastaut syndrome. This study was a retrospective review of children with Lennox-Gastaut syndrome treated with the modified Atkins diet from May 2009 and March 2011. The diet was initiated in those children who persisted to have daily seizures despite the use of at least 3 appropriate antiepileptic drugs. Twenty-five children were started on a modified Atkins diet, restricting carbohydrate intake to 10 g/d. After 3 months, 2 patients were seizure-free, and 10/25 children had >50% reduction in seizure frequency. At 6 months, of 11 patients on the diet, 3 were seizure free and 8 had >50% reduction in seizure frequency. At 1 year, all 9 children on diet had >50% reduction in seizure frequency. The side effects of the diet were mild. The modified Atkins diet was found to be effective and well tolerated in children with Lennox-Gastaut syndrome. [ABSTRACT FROM PUBLISHER]

Background: Valproate is a commonly used anticonvulsant drug. Uridine 5'-diphospho (UDP)-glucuronosyltransferase (UGT) contributes to around 50% of valproate metabolism and its polymorphisms may be important for explaining the considerable variation in valproate levels in patients with epilepsy. Aim: This study was aimed to analyze the genetic polymorphisms of UGT1A6 in Indian children with epilepsy and their potential influence on the pharmacokinetics of valproate. Setting and Design: This cross-sectional study was carried out in the Department of Pediatrics, All India Institutes of Medical Sciences (AIIMS), New Delhi, between March 2011 and July 2012. Materials and Methods: Children aged 3-12 years diagnosed with epilepsy on valproate monotherapy for at least 1 month were enrolled. They underwent a detailed clinical examination. The UGT1A6 polymorphisms were detected by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Random samples were checked by genetic sequencing. The steady-state plasma concentrations of valproate were measured by High Performance Liquid Chromatography (HPLC) and associated with UGT1A6 polymorphisms. Results: A total of 80 children were studied. The prevalence of UGT1A6 T19G was as follows: TT (45%), TG (38.8%), and GG (16.3%); that of UGT1A6 A541G was: AA (48.8%), AG (38.8%), and GG (12.5%); and that of UGT1A6 A552C was: AA (43.8%), AC (40%), and CC (16.3%). The association between valproate doses or standardized serum valproate concentration and the various UGT1A6 genotypes could not be studied reliably in this small study population. Conclusions: The frequencies of UGT1A6 geneotypes and alleles were reported in the study population. [ABSTRACT FROM AUTHOR]

To diagnose health status of the heart, heart monitoring systems use heart signals produced during each cardiac cycle. Many types of signals are acquired to analyze heart functionality and hence several heart monitoring systems such as phonocardiography, electrocardiography, photoplethysmography and seismocardiography are used in practice. Recently, focus on the at-home monitoring of the heart is increasing for long term monitoring, which minimizes risks associated with the patients diagnosed with cardiovascular diseases. It leads to increasing research interest in portable systems having features such as signal transmission capability, unobtrusiveness, and low power consumption. In this paper we intend to provide a detailed review of recent advancements of such heart monitoring systems. We introduce the heart monitoring system in five modules: (1) body sensors, (2) signal conditioning, (3) analog to digital converter (ADC) and compression, (4) wireless transmission, and (5) analysis and classification. In each module, we provide a brief introduction about the function of the module, recent developments, and their limitation and challenges. [ABSTRACT FROM AUTHOR]

Neurocysticercosis, parasitic infestation of the central nervous system by the Taenia solium larvae, is a major public health problem, primarily in the developing countries. Seizures are the primary clinical manifestation which could be acute (secondary to active lesions) or remote symptomatic (due to calcified lesions). Cysticidal therapy is the standard of care for solitary parenchymal active neurocysticerci. However treatment related side effects and tendency to spontaneous resolution raises concern from time to time whether cysticidal therapy is actually required. This is a retrospective case record analysis of two groups of patients with solitary parenchymal neurocysticerci (group A; 171 patients between 2000 and 2004 who did not receive cysticidal therapy, group B; 512 patients between 2008 and 2013 who received cysticidal therapy). Group B had significantly more radiological resolution of lesions whereas group A reported significantly more seizure recurrences on antiepileptics. There was no significant difference in occurrence of calcification in the two groups. Overall patients with calcified lesions had significantly more breakthrough seizures. Well designed prospective studies should be planned in future to understand the mechanism underlying the epileptogenicity of calcified lesions and how they are linked to host and environment factors. [ABSTRACT FROM AUTHOR]

The prevalence and the burden of vincristine-induced neuropathy have been poorly documented in childhood acute lymphoblastic leukemia survivors. This cross-sectional study was carried out at a tertiary care center in northern India from October 2011 to June 2012. Eighty consecutive acute lymphoblastic leukemia survivors aged 5 to 18 years, within 3 years of completion of their chemotherapy, were enrolled. After clinical evaluation, detailed nerve conduction studies were performed and the reduced version of the Total Neuropathy Score was calculated. The mean age at the time of evaluation was 11.2 ± 3.2 years. 33.75% had neuropathy electrophysiologically. Symmetric motor axonal polyneuropathy was the most common pattern of involvement seen in 19 (23.8%) children. There was significant improvement with time, as revealed by lower prevalence of neuropathy with increasing interval following vincristine injection. 33.75% of the children had Reduced version of Total Neuropathy Score ≥ 1. [ABSTRACT FROM AUTHOR]

The ketogenic diet is a high-fat, low-carbohydrate, and restricted protein diet that is useful in patients with refractory epilepsy. The efficacy of the ketogenic diet is better than most of the new antiepileptic drugs. Other modifications of the diet are also beneficial, such as the modified Atkins diet and the low glycemic index treatment. There is a lack of awareness of the ketogenic diet as a treatment modality for epilepsy amongst pediatricians and neurologists. In this review, the use of the ketogenic diet and other dietary treatments in refractory epilepsy is discussed. The Indian experience with the use of these dietary treatments is also briefly reviewed. [ABSTRACT FROM AUTHOR]

Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome characterized by classical cutaneous and ocular lesions with central nervous system anomalies. We describe an infant with classical encephalocraniocutaneous lipomatosis characterized by probable naevus psiloliparus, frontal subcutaneous lipomas, ocular limbal dermoids and arachnoid cysts, and ventriculomegaly. He also had giant congenital nevus with leptomeningeal melanosis. This case represents a rare association between encephalocraniocutaneous lipomatosis and neurocutaneous melanosis. [ABSTRACT FROM AUTHOR]

Abstract: Split resonances are a common appearance in superconducting cavities and are studied here on a specific example. A five cell superconducting cavity suitable for the envisioned electron-hadron collider eRHIC was designed and will be used in the upcoming “Coherent electron Cooling” Proof-of-Principle experiment. The superconducting cavity is intended for high-current applications, and beam stability depends on minimizing the Higher Order Modes (HOM). This was attempted in the design phase and will be done with appropriate mode dampers in operation. The design was implemented as a copper cavity and two Niobium cavities were fabricated. The availability of the copper model provided a convenient opportunity to confirm the design and to study potentially nefarious HOMs. Selected high-Q resonances were investigated via bead pull measurements. The appearance of split resonances impeded their study and suitable methods of HOM identification are presented in this report. [Copyright &y& Elsevier]

The modified Atkins diet is a less restrictive variation of the ketogenic diet. This diet is started on an outpatient basis without a fast, allows unlimited protein and fat, and does not restrict calories or fluids. Recent studies have shown good efficacy and tolerability of this diet in refractory epilepsy. In this review, we discuss the use of the modified Atkins diet in refractory epilepsy. [ABSTRACT FROM AUTHOR]

Abstract: A series of novel arylquinoline derivatives was designed retaining significant pharmacophoric features and three dimensional geometry of bedaquiline. In silico ADME study was performed to assess drug likeness and toxicity profiles of the designed molecules. The compounds were evaluated for activity against Mycobacterium tuberculosis H37Rv using Resazurin Microtitre Assay (REMA) plate method and cytotoxicity in VERO C1008 cell line. Several of the synthesized compounds exhibited good antituberculosis activity and selectivity, especially compounds, 12i (MIC: 5.18μM and MIC/CC50: 152.86) and 12l (MIC: 5.59μM and MIC/CC50: 160.57). The study opens up a new platform for the development of arylquinoline based drugs for treating tuberculosis. [Copyright &y& Elsevier]