Landscape of genetic infantile epileptic spasms syndrome-A multicenter cohort of 124 children from India.

OBJECTIVE: Literature on the genotypic spectrum of Infantile Epileptic Spasms Syndrome (IESS) in children is scarce in developing countries. This multicentre collaboration evaluated the genotypic and phenotypic landscape of genetic IESS in Indian children. METHODS: Between January 2021 and June 2022, this cross-sectional study was conducted at six centers in India. Children with genetically confirmed IESS, without definite structural-genetic and structural-metabolic etiology, were recruited and underwent detailed in-person assessment for phenotypic characterization. The multicentric data on the genotypic and phenotypic characteristics of genetic IESS were collated and analyzed. RESULTS: Of 124 probands (60% boys, history of consanguinity in 15%) with genetic IESS, 105 had single gene disorders (104 nuclear and one mitochondrial), including one with concurrent triple repeat disorder (fragile X syndrome), and 19 had chromosomal disorders. Of 105 single gene disorders, 51 individual genes (92 variants including 25 novel) were identified. Nearly 85% of children with monogenic nuclear disorders had autosomal inheritance (dominant-55.2%, recessive-14.2%), while the rest had X-linked inheritance. Underlying chromosomal disorders included trisomy 21 (n = 14), Xq28 duplication (n = 2), and others (n = 3). Trisomy 21 (n = 14), ALDH7A1 (n = 10), SCN2A (n = 7), CDKL5 (n = 6), ALG13 (n = 5), KCNQ2 (n = 4), STXBP1 (n = 4), SCN1A (n = 4), NTRK2 (n = 4), and WWOX (n = 4) were the dominant single gene causes of genetic IESS. The median age at the onset of epileptic spasms (ES) and establishment of genetic diagnosis was 5 and 12 months, respectively. Pre-existing developmental delay (94.3%), early age at onset of ES (<6 months; 86.2%), central hypotonia (81.4%), facial dysmorphism (70.1%), microcephaly (77.4%), movement disorders (45.9%) and autistic features (42.7%) were remarkable clinical findings. Seizures other than epileptic spasms were observed in 83 children (66.9%). Pre-existing epilepsy syndrome was identified in 21 (16.9%). Nearly 60% had an initial response to hormonal therapy. SIGNIFICANCE: Our study highlights a heterogenous genetic landscape and phenotypic pleiotropy in children with genetic IESS.

Melatonin and Triclofos Sodium to Execute Sleep Electroencephalography in Pediatric Patients: A Cost-Effectiveness Analysis Alongside a Randomized Controlled Trial.

OBJECTIVES: To evaluate cost-effectiveness between oral sedatives melatonin and triclofos sodium used to perform sleep electroencephalography (EEG) in pediatric patients and develop a criterion for resource allocation by the institution. METHODS: This prospective study was conducted alongside the randomized controlled trial conducted at a tertiary care hospital, wherein pediatric patients arriving for sleep EEG were randomized to receive melatonin dosed at 0.3 mg/kg (< 10 kg), 3 mg (10-15 kg), and 6 mg (> 15 kg) and triclofos at 50 mg/kg (maximum dose 1 g, 6 mo to 11 y; 2 g, 11 to 18 y) after due consent. The cost-effectiveness analysis was performed from the healthcare institution's perspective. Successful EEG and abnormal EEG were the effectiveness parameters. RESULTS: Two hundred twenty-eight patients were divided equally between two groups. Melatonin (N = 114) and triclofos (N = 114) recorded successful EEG in 89.4% and 91.2% patients and abnormal EEG in 49% and 42.3% patients, respectively (p > 0.05). The total direct cost incurred was INR 1881.75 (USD 26.6) and INR 2772.5 (USD 39.2) for melatonin and triclofos, respectively (p < 0.05). The cost-effectiveness ratio-1 (CER-1) for melatonin and triclofos per successful EEG recorded was INR 18.45 (USD 0.39) and INR 26.66 (USD 0.58), respectively. The CER-2 for melatonin and triclofos per abnormal EEG detected was INR 37.64 (USD 0.53) and INR 63.01 (USD 0.89), respectively. CONCLUSIONS: Melatonin is more cost-effective than triclofos when charged based on individual dose requirements. Hospitals, diagnostic centers, healthcare institutions may consider resource-utilization-based costing system for cost-effective allocation of resources.

Compliance with status epilepticus management protocol and effect on clinical outcomes in children with status epilepticus.

BACKGROUND: Guidelines for the management of status epilepticus (SE) aid in rationalising the treatment for a better clinical outcome; however, published literature regarding the use of antiepileptics and compliance is limited, even after the availability of a consensus guideline. OBJECTIVES: To evaluate the use of antiepileptics in children with SE and to analyse the effect of compliance with the Status Epilepticus Management Protocol on clinical outcomes. METHODS: An open-label non-randomised prospective observational study was conducted in children with SE aged 1 month to 14 years for 8 months in a tertiary care teaching hospital. The recommended antiepileptics, sequence of drug administration and time frames of management offered to paediatric patients were assessed for compliance with the Status Epilepticus Management Protocol adopted in our hospital. Comparison of clinical outcomes (hospital stay, intubation, refractory and super-refractory SE, duration of SE cessation, functional motor deficits and cognitive decline) between compliant and non-compliant patients was assessed. RESULTS: A total of 40 patients were included in the study, of which 28 (70%) were boys. All the patients received midazolam nasal spray in the triage area. Only 18% of the patients received rescue benzodiazepine (BZD) antiepileptic drug (AED) therapy in pre-hospital settings. Median time (p25-p75) of administration of first-line AED (BZD) and second-line AED (non-BZD) was 11 (8-15) min and 30 (22-35) min, respectively. Administration of continuous infusion (IV midazolam) was delayed at 57 (45-69) min. Compliance with the Status Epilepticus Management Protocol was seen in 24 (60%) patients. Non-compliance with the treatment protocol in relation to the time frame significantly prolonged the length of hospital stay (9 vs 4 days, p=0.0008) and SE duration from first assessment (115 vs 50 min; p=0.005). At discharge, the proportion of patients returning to their functional baseline was significantly different in the compliant and non-compliant patient groups (79% vs 44%). There were no deaths. CONCLUSION: Rescue therapy in the pre-hospital setting needs attention. There was full compliance with the Status Epilepticus Management Protocol for choice of AED and sequence of AED therapy. Non-compliance in treatment management within time frames significantly affected the length of hospital stay, duration of SE and clinical outcome.

Clinical Profile and Treatment Outcomes of Hypermanganesemia with Dystonia 1 and 2 among 27 Indian Children.

Background: Hypermanganesemia with dystonia 1 and 2 (HMNDYT1 and 2) are rare, inherited disorders of manganese transport. Objectives: We aimed to describe clinical, laboratory features, and outcomes among children with HMNDYT. Methods: We conducted a retrospective multicenter study involving tertiary centers across India. We enrolled children between 1 month to 18 years of age with genetically confirmed/clinically probable HMNDYT. Clinical, laboratory profile, genetic testing, treatment details, and outcomes scored by treating physicians on a Likert scale were recorded. Results: We enrolled 27 children (19 girls). Fourteen harbored SLC30A10 mutations; nine had SLC39A14 mutations. The SLC39A14 cohort had lower median age at onset (1.3 [interquartile range (IQR), 0.7-5.5] years) versus SLC30A10 cohort (2.0 [IQR, 1.5-5.1] years). The most frequent neurological features were dystonia (100%; n = 27), gait abnormality (77.7%; n = 21), falls (66.7%; n = 18), and parkinsonism (59.3%; n = 16). Median serum manganese (Mn) levels among SLC39A14 (44.9 [IQR, 27.3-147.7] mcg/L) cohort were higher than SLC30A10 (29.4 [17.1-42.0] mcg/L); median hemoglobin was higher in SLC30A10 (16.3 [IQR, 15.2-17.5] g/dL) versus SLC39A14 cohort (12.5 [8.8-13.2] g/dL). Hepatic involvement and polycythaemia were observed exclusively in SLC30A10 variants. A total of 26/27 children underwent chelation with disodium calcium edetate. Nine demonstrated some improvement, three stabilized, two had marked improvement, and one had normalization. Children with SLC39A14 mutations had poorer response. Two children died and nine were lost to follow-up. Conclusions: We found female predominance. Children with SLC39A14 mutations presented at younger age and responded less favorably to chelation compared to SLC30A10 mutations. There is emerging need to better define management strategies, especially in low resource settings.

Factors influencing HINTS exam usage by Canadian Emergency Medicine Physicians.

OBJECTIVES: The HINTS examination (head impulse, nystagmus, test of skew) is a bedside physical examination technique that can distinguish between vertigo due to stroke, and more benign peripheral vestibulopathies. Uptake of this examination is low among Emergency Medicine (EM) physicians; therefore, we surveyed Canadian EM physicians to determine when the HINTS exam is employed, and what factors account for its low uptake. METHODS: We designed and tested a 26-question online survey, and disseminated it via email to EM physicians registered with the Canadian Association of Emergency Physicians (CAEP), with 3 and 5-week reminder emails to increase completion. This anonymous survey had no incentives for participation, and was completed by 185 EM physicians, with post-graduate medical training in either Emergency Medicine or Family Medicine. The primary outcomes were the frequencies of various responses to survey questions, with secondary outcomes being the associations between participant characteristics and given responses. RESULTS: 88 respondents (47.8%) consistently use the HINTS examination in the work-up of vertigo, and 117 (63.7%) employ it in scenarios where its clinical utility is limited. The latter is more common among physicians working in non-academic settings, without 5-year EM residency training, and with greater years of practice (p < 0.01). The most frequent explanations for non-use were a lack of need for the HINTS examination, the lack of validation of the exam among EM physicians, and concerns surrounding the head-impulse test. CONCLUSIONS: Though HINTS exam usage is common, there is a need for education on when to apply it, and how to do so, particularly as concerns the head-impulse test. Our attached rubric may assist with this, but quality-improvement initiatives are warranted. Low uptake is partly due to the lack of validation of this examination among EM physicians, so effort should be made to conduct well-designed HINTS trials exclusively involving EM physicians.

RéSUMé: OBJECTIFS: L'examen HINTS (head impulse, nystagmus, test of skew) est une technique d'examen physique au chevet du patient qui permet de distinguer les vertiges dus à un accident vasculaire cérébral des vestibulopathies périphériques plus bénignes. Cet examen est peu pratiqué par les médecins en médecine d'urgence (MU). Nous avons donc mené une enquête auprès des médecins d'urgence canadiens afin de déterminer quand l'examen HINTS est utilisé et quels sont les facteurs qui expliquent sa faible utilisation. MéTHODES: Nous avons conçu et testé une enquête en ligne de 26 questions, et l'avons diffusée par courriel aux médecins urgentistes inscrits à l'Association canadienne des médecins d'urgence (ACMU), avec des courriels de rappel de trois et cinq semaines pour augmenter le taux de réponse. Cette enquête anonyme, qui ne comportait aucune incitation à la participation, a été remplie par 185 médecins de médecine d'urgence ayant suivi une formation médicale postuniversitaire en médecine d'urgence ou en médecine familiale. Les résultats primaires étaient les fréquences des diverses réponses aux questions de l'enquête, les résultats secondaires étant les associations entre les caractéristiques des participants et les réponses données. RéSULTATS: 88 répondants (47,8 %) utilisent systématiquement l'examen HINTS dans l'évaluation des vertiges, et 117 (63,7 %) l'emploient dans des scénarios où son utilité clinique est limitée. Cette dernière est plus fréquente chez les médecins travaillant dans des établissements non universitaires, n'ayant pas suivi une formation de 5 ans en résidence en médecine d'urgence et ayant un plus grand nombre d'années de pratique (p < 0,01). Les explications les plus fréquentes de la non-utilisation étaient le manque de nécessité de l'examen HINTS, le manque de validation de l'examen parmi les médecins de médecine d'urgence et les préoccupations concernant le test d'impulsion de la tête. CONCLUSIONS: Bien que l'utilisation de l'examen HINTS soit courante, il existe un besoin d'éducation sur le moment où il faut l'appliquer et sur la manière de le faire, en particulier en ce qui concerne le test d'impulsion de la tête. Notre rubrique ci-jointe peut vous aider à cet égard, mais des initiatives d'amélioration de la qualité sont justifiées. Le faible taux d'utilisation est en partie dû au manque de validation de cet examen parmi les médecins de la médecine d'urgence. Il faut donc s'efforcer de mener des essais HINTS bien conçus impliquant exclusivement des médecins de la médecine d'urgence.

Enteral lorazepam is a promising weaning strategy for midazolam-responsive febrile infection-related epilepsy syndrome (FIRES): a case series

OBJECTIVE: Prolonged and repetitive cycles of pharmacological coma, with midazolam or other general anaesthetics, is often the mainstay for seizure control in febrile infection-related epilepsy syndrome (FIRES). Here we present our experience of enteral lorazepam as an effective weaning substitute in midazolam-dependent patients. METHODS: This was a retrospective study based on a review of medical records of all FIRES patients who had received enteral lorazepam as a weaning substitute for midazolam, between January 2020 and July 2021. The patients were divided into an early group (lorazepam initiated after one failed attempt to wean off midazolam) and late group (lorazepam initiated after two or more failed attempts). The conversion from intravenous midazolam to enteral lorazepam was also calculated, and epilepsy outcome at follow-up was also assessed. RESULTS: Seven patients (five males) were eligible. The median age at onset of FIRES was seven years (range: 4-14). A median of six (range: 6-8) anti-seizure medications (ASMs) had failed (including clobazam in two and clonazepam in one) to control seizures. The early and late lorazepam groups were comparable regarding the maximum midazolam dose for seizure control, total ASMs tried and days to wean off midazolam. The median (range) duration of hospital stay was 27 days (22-46) in the early group, compared to 51 days (40-78) in the late group. The early group patients were also on fewer ASMs (median: 3;range: 3-5) compared to the late group (median: 5; range: 4-6) at discharge. Five patients were sedated with initial lorazepam dose, but this side effect resolved on dosage reduction. On follow-up, all seven patients had seizure recurrence. In four, seizures recurred on reducing lorazepam, however, in three of these patients, this was resolved by escalating the dose. SIGNIFICANCE: Enteral lorazepam can be an effective weaning substitute for midazolam-dependent children with FIRES. Early introduction of enteral lorazepam was associated with reduced duration of hospital stay.

Tubercular Bronchoesophageal Fistula in an Adolescent Girl.

Bronchoesophageal fistula is a rare complication of Mycobacterium tuberculosis in children. An adolescent girl who was diagnosed of tubercular mediastinal lymphadenopathy with associated bronchoesophageal fistula at presentation, is reported here. This 16-y-old girl presented with high-grade fever, cough, decreased appetite, weight loss for 3 mo, and breathlessness for 10 d. Chest radiograph revealed hilar lymphadenopathy with bilateral pleural effusion. GA GeneXpert was positive for mycobacterium and rifampicin sensitivity. Despite antitubercular therapy cough persisted and there was a history of dry cough with food intake, especially more on liquids. Bronchoscopy and CECT chest confirmed bronchoesophageal fistula in the right main bronchus just below the carina. Child continued on tube feeding and antitubercular therapy. After completion of intensive phase, child improved with resolution of clinical symptoms and scarring of tract on repeat bronchoscopy. It is concluded that in children with combination of mediastinal lymphadenopathy and persistent cough following intake of food needs careful evaluation for trachea/bronchoesophageal fistula.

Time Elapsed from Onset of Pediatric Convulsive Status Epilepticus to Antiepileptic Administration-An Experience of Single Institute.

Data regarding time elapsed from the onset of pediatric convulsive status epilepticus (CSE) to antiepileptic (AED) administration remains scarce after the adoption of standard treatment-guidelines in Indian healthcare settings. A prospective observational analysis was performed on 52 children presenting to an urban, academic tertiary care teaching hospital diagnosed with CSE and evolving to refractory CSE (RCSE). Time frames of AED administration were compared to the adopted 'Status Epilepticus Management Protocol'. Fifty-two patients [36 (69.2%) male] were enrolled, with a median age of 4.1 y. After CSE onset, the median (p25-p75) time until the administration of the first-line, second-line, and third-line therapy phases of AED doses were 30 (25-37) min, 68 (48-79) min, and 105 (100-135) min, respectively. The second dose of non-BZD AED was administered at a median (p25-p75) of 90 (71-95) min. Twenty-six (50%) patients received at least one continuous infusion. The time elapsed from CSE onset to AED administration and escalation from one class to another was delayed.

Clinical and Electrophysiological Factors Predicting Prolonged Recovery in Children with Guillain-Barré Syndrome.

OBJECTIVE: To compare clinical and nerve conduction studies (NCS) parameters predictive of outcome in children with acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). METHODS: In this prospective observational study, NCS was done on all children at admission and repeated before discharge. Functional status of patients was graded as per Hughes Disability score. These children were followed up till they achieved independent walking. Clinical and NCS criteria were compared between (a) AMAN and AIDP and (b) two subgroups of children with AMAN-those who achieved early (within 60 d) versus delayed (i.e., after 60 d) walking. RESULTS: Fifty-seven children were initially enrolled, first NCS showed inexcitable nerves in 10, AMAN in 29, acute motor-sensory axonal neuropathy (AMSAN) in 3, AIDP in 13, and 2 were normal. Subsequent NCS showed AMAN in 37, AIDP in 15, AMSAN in 3 patients. There were no deaths, 16 required ventilation. Follow-up till independent walking, was available for 40 patients. AMAN was associated with faster progression, greater peak disability, prolonged hospital stay, and delayed walking (p < 0.05). Asymmetrical nerve involvement predicted prolonged hospital stay as well as delayed walking. In the AMAN group, prolonged ulnar F-wave latencies were significantly associated with delayed walking (p = 0.02). CONCLUSION: Long term prognosis of pediatric GBS is generally satisfactory. AMAN, asymmetric involvement and prolonged ulnar F-wave latencies in children with AMAN were associated with delayed walking.

Efficacy and tolerability of Melatonin vs Triclofos to achieve sleep for pediatric electroencephalography: A single blinded randomized controlled trial.

PURPOSE: To compare Melatonin with Triclofos for efficacy (proportion of successful EEG, need of augmentation, sleep onset latency (SOL), yield of discharges, duration of sleep, presence and grade of artifacts) and tolerability (adverse effect profile). METHODS: A randomized trial was performed (block randomization). All children were advised regarding sleep deprivation, EEG technician administered the drug. EEG was labelled successful if at least 30 min of record could be obtained (sleep with or without awake state). Pediatric neurologist reported the EEG findings-sleep onset latency, epileptiform abnormalities and graded the artifacts (excess beta activity and movement artifacts if present). The parents were interviewed telephonically next day by a pediatric resident for any adverse effects. The parents, pediatric neurologist and pediatric resident were blinded for the drug given. RESULTS: 228 children were randomized (114 each received Melatonin and Triclofos). Both the groups were comparable at baseline for age group and demographic data. The proportion of successful EEG was 89.4% in Melatonin and 91.2% in Triclofos. First dose was effective in 64% in Melatonin and 63.15% in Triclofos group. Augmentation dose was needed in 25.4% in Melatonin and 28% in Triclofos group. Mean total sleep duration was 80 min after Melatonin and 82.39 after Triclofos administration. Adverse effects were observed in 6.14% of Melatonin and 8.65% of Triclofos group. None of the results were statistically significant. CONCLUSION: There was no significant difference between efficacy and tolerability of Melatonin and Triclofos. Melatonin can be safely used to achieve sleep for EEG in children.

Significant treatment gap and co-morbidities identified in an epidemiological survey of pediatric epilepsy in rural suburbs of India.

PURPOSE: A cross-sectional epidemiological survey of children was conducted in two rural clusters to estimate the point prevalence and study various aspects of childhood epilepsy. MATERIAL AND METHODS: In the first stage, a house-to-house survey was conducted by health workers using a screening questionnaire, which was pre-validated in a pilot study. All screen positive houses were visited by pediatric neurologist for detailed evaluation. Children with a clinical diagnosis of epilepsy underwent EEG and were evaluated for type of seizure, epilepsy syndrome, etiology, co-morbidities and treatment gap. Knowledge, attitude and practice regarding epilepsy was assessed amongst caregivers of the affected children. RESULTS: A total population of 75,455 population was screened, 19,181 children aged 2 months to 18 years were identified. Out of 355 screen positive children, 66 were diagnosed with epilepsy. The point prevalence of pediatric epilepsy was 3.44 per 1000 children. 53% had focal epilepsy, 31.8% had an identifiable epilepsy syndrome, 44% had at least one comorbidity. The etiology was identified in 68%, the commonest being perinatal brain insult. The magnitude of treatment gap was 45.45%, with significant deficits in knowledge. CONCLUSION: There are significant deficits in diagnosis and treatment of pediatric epilepsy among the rural population of India. The existing rural health care facilities need to be augmented to facilitate the timely diagnosis and optimum care of these children, including care of associated co-morbidities.

Thinking beyond infections in children with recurrent/persistent pneumonia.

Children with recurrent or persistent pneumonia often have underlying chronic cardiopulmonary disease, but few reports on this subject have been published. Children with isolated common cardiac diseases, uncomplicated bronchial asthma or with incomplete records were excluded. Of 4361 children followed during a five-year period, 107 were included in our study. Underlying causes were identified in 99.0%: immunodeficiency disorders (20.2%), cardiothoracic malformations (18.3%), syndromic conditions (14.4%), infections (10.6%) bronchiectasis (10.6%), gastro-oesophageal reflux disease (6.6%), interstitial lung disease (3.8%) and other miscellaneous conditions (15.4%). Thus, children with recurrent or persistent pneumonia should be carefully evaluated for an underlying aetiology, as early diagnosis and appropriate management will decrease morbidity and mortality in most of these children.

Association of Child Neurology (AOCN) - Indian Epilepsy Society (IES) Consensus Guidelines for the Diagnosis and Management of West Syndrome.

JUSTIFICATION: West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use. PROCESS: An expert group consisting of pediatric neurologists and epileptologists was constituted. Experts were divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed a consensus. The evidence was reviewed, and for areas where the evidence was not certain, the Delphi consensus method was adopted. The final guidelines were circulated to all experts for approval. RECOMMENDATIONS: Diagnosis should be based on clinical recognition (history/home video recordings) of spasms and presence of hypsarrhythmia or its variants on electroencephalography. A magnetic resonance imaging of the brain is the preferred neuroimaging modality. Other investigations such as genetic and metabolic testing should be planned as per clinico-radiological findings. Hormonal therapy (adrenocorticotropic hormone or oral steroids) should be preferred for cases other than tuberous sclerosis complex and vigabatrin should be the first choice for tuberous sclerosis complex. Both ACTH and high dose prednisolone have reasonably similar efficacy and adverse effect profile for West syndrome. The choice depends on the preference of the treating physician and the family, based on factors of cost, availability of infrastructure and personnel for daily intramuscular injections, and monitoring side effects. Second line treatment options include anti-epileptic drugs (vigabatrin, sodium valproate, topiramate, zonisamide, nitrazepam and clobazam), ketogenic diet and epilepsy surgery.

Efficacy, Tolerability and Serum Phenytoin Levels after Intravenous Fosphenytoin Loading Dose in Children with Status Epilepticus.

OBJECTIVE: To evaluate the efficacy and tolerability of intravenous fosphenytoin in children with status epilepticus, and resulting serum total phenytoin levels. METHODS: In this prospective study, 51 children aged less than 18 years received intravenous loading dose of fosphenytoin (18-20 mg/kg). Serum total phenytoin levels were estimated at 90 -100 minutes. Outcomes studied were (i) seizure control and local and/or systemic adverse effects in next 24 hours and (ii) phenytoin levels and its correlation with dose received, seizure control and adverse effects. RESULTS: The actual dose of fosphenytoin received varied from 15.1 to 25 mg/kg. Seizures were controlled in 45 (88%) children and, two required additional dose of 10 mg/kg. None of the children showed any local or systemic adverse effects. Serum total phenytoin levels were in the therapeutic range (10-20 µg/mL) in 12 (23.5%), sub-therapeutic in 16 (31.3%) and supra-therapeutic in 25 (49%) children. There was weak correlation of the phenytoin levels with dose of fosphenytoin received, seizure control, or adverse effects. CONCLUSIONS: Intravenous fosphenytoin loading dose of 20 mg/kg is effective in controlling seizures in 88% of children with status epilepticus, with a good safety profile. Seizure control and adverse effects appear to be independent of serum total phenytoin levels achieved.

Association of Child Neurology-Indian Epilepsy Society Consensus Document on Parental Counseling of Children with Epilepsy.

When a child is diagnosed with epilepsy, counseling regarding the same is done by the treating doctor. Most parents are frightened and have poor knowledge about epilepsy. Therapeutic advice including drug dosage, administration and side effects takes up the major part of physician's time, thereby neglecting important issues like home seizure management, follow up and others. These lacunae in knowledge require systematic patient and family education. To address these issues, an expert group meeting of pediatric neurologists and epileptologists in India along with social workers/epilepsy educators, legal experts, parents, and teachers was held. The various aspects regarding parental counseling in children with epilepsy were discussed and a consensus document was formulated. Here authors present the group consensus statement on counseling parents and caregivers of children with epilepsy. This document is intended to help physicians and pediatricians counsel the families when a child is diagnosed with epilepsy.

Short-term carbon dynamics in a temperate grassland and heathland ecosystem exposed to 104 days of drought followed by irrigation.

Temperate ecosystems are susceptible to drought events. The effect of a severe drought (104 days) followed by irrigation on the plant C uptake, its assimilation and input of C in soil were examined using a triple 13CO2 pulse-chase labelling experiment in model grassland and heathland ecosystems. First 13CO2 pulse at day 0 of the experiment revealed much higher 13C tracer uptake for shoots, roots and soil compared to the second pulse (day 44), where all plants showed significantly lower 13C tracer uptake. After the third 13CO2 pulse (day 70), very low 13C uptake in shoots led to a negligible allocation of 13C into roots and soil. During irrigation after the severe drought, the 13C tracer that was allocated in plant tissues during the second and third pulse labelling was re-allocated in roots and soil, as soon as the irrigation started. This re-allocation was higher and longer lasting in heathland compared to grassland ecosystems.

Studies on the structural changes during curing of epoxy and its blend with CTBN.

Cashew nut shell liquid (CNSL), an agricultural renewable resource material, produces natural phenolic distillates such as cardanol. Cardanol condenses with formaldehyde at the ortho- and para-position of the phenolic ring under acidic or alkaline condition to yield a series of polymers of novolac- or resol-type phenolic resins. These phenolic resins may further be modified by epoxidation with epichlorohydrin to duplicate the performance of such phenolic-type novolacs (CFN). The structural changes during curing of blend samples of epoxy and carboxyl terminated poly (butadiene-co-acrylonitrile) (CTBN) were studies by Fourier-transform infrared (FTIR) spectrophotometer. The epoxy samples were synthesized by biomass material, cardanol. Blend sample was prepared by physical mixing of CTBN ranging between 0 and 20weightpercent CTBN liquid rubber into cardanol-based epoxidized novolac (CEN) resin. The FTIR spectrum of uncured blend sample clearly indicated that there appeared a band in the region of 3200-3500cm-1 which might be due to the presence of phenolic hydroxyl group and OH group of the opened epoxide. Pure epoxy resin showed peaks near 856cm-1 which might be due to oxirane functionality of the epoxidized novolac resin. Both epoxy and its blend sample was cured with polyamine. The cure temperature of CEN resin was found to be decreased by the incorporation of CTBN. The decomposition behavior was also studied by thermogravimetric analyzer (TGA). Two-step decomposition behavior was observed in both epoxy and its blend samples.

EpiNet as a way of involving more physicians and patients in epilepsy research: Validation study and accreditation process.

OBJECTIVE: EpiNet was established to encourage epilepsy research. EpiNet is used for multicenter cohort studies and investigator-led trials. Physicians must be accredited to recruit patients into trials. Here, we describe the accreditation process for the EpiNet-First trials. METHODS: Physicians with an interest in epilepsy were invited to assess 30 case scenarios to determine the following: whether patients have epilepsy; the nature of the seizures (generalized, focal); and the etiology. Information was presented in two steps for 23 cases. The EpiNet steering committee determined that 21 cases had epilepsy. The steering committee determined by consensus which responses were acceptable for each case. We chose a subset of 18 cases to accredit investigators for the EpiNet-First trials. We initially focused on 12 cases; to be accredited, investigators could not diagnose epilepsy in any case that the steering committee determined did not have epilepsy. If investigators were not accredited after assessing 12 cases, 6 further cases were considered. When assessing the 18 cases, investigators could be accredited if they diagnosed one of six nonepilepsy patients as having possible epilepsy but could make no other false-positive errors and could make only one error regarding seizure classification. RESULTS: Between December 2013 and December 2014, 189 physicians assessed the 30 cases. Agreement with the steering committee regarding the diagnosis at step 1 ranged from 47% to 100%, and improved when information regarding tests was provided at step 2. One hundred five of the 189 physicians (55%) were accredited for the EpiNet-First trials. The kappa value for diagnosis of epilepsy across all 30 cases for accredited physicians was 0.70. SIGNIFICANCE: We have established criteria for accrediting physicians using EpiNet. New investigators can be accredited by assessing 18 case scenarios. We encourage physicians with an interest in epilepsy to become EpiNet-accredited and to participate in these investigator-led clinical trials.

A comprehensive review of epilepsy in the Arab world.

PURPOSE: We conducted a comprehensive review of the epidemiology of epilepsy in the Arab world. METHODS: Epidemiological literature about epilepsy from 22 countries of the Arab League was searched in French and English using several keywords (specific and wider) and combinations, individually for each country. The search was conducted on Google first and then on PubMed. The results are presented as counts, proportions, and medians along with 95% confidence intervals (CI). Unpaired t-test with unequal variance and regressions were performed, altogether and individually, for lifetime and active epilepsy prevalence as well as incidence. RESULTS: Google provided 21 prevalence, four camp and nine incidence estimates while PubMed provided ten such estimates; none of them was identified by Google. No epidemiological data about epilepsy was found from 10/22 countries. Excluding pediatric studies, 13 prevalence estimates from six countries were identified. Including pediatric studies, 21 estimates from nine countries were found. Median lifetime and active epilepsy prevalence were 7.5/1000 (95% CI 2.6-12.3, range 1.9-12.9) and 4.4/1000 (95% CI 2.1-9.3, range 2.1-9.3), respectively, excluding pediatric studies (1984-2014, N=244081). Median incidence was 56.0/100,000 (n=9, N=122484, 95% CI 13.7-147.9, range 10.4-190). CONCLUSION: The fact that no epidemiological data about epilepsy is available in the public domain for almost one half of all Arab countries offers opportunities for future research. This thorough review of existing literature demonstrates a prevalence of epilepsy three times higher than previously reported for this region. The median incidence is similar to other regions of the world, e.g. North America. Google yielded additional valuable sources not indexed in PubMed and provided pertinent references more quickly.