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Objectives There is shortage of specialists for the diagnosis of children with epilepsy, especially in resource limited settings. Existing INCLEN (International Clinical Epidemiology Network) instrument was validated for children aged 2–9 years. The current study validated modifications of the same including wider symptomatology and age group. Methods The Modified INCLEN tool was validated by a team of experts by modifying the existing tools (2–9 years) to widen the age range from 1 month to 18 years and include broader symptomatology in a tertiary care teaching hospital of North India between January and June 2015. A qualified medical graduate applied the candidate tool which was followed by gold standard evaluation by a Pediatric Neurologist (both blinded to each other). Results A total of 197 children {128 boys (65%) and 69 girls (35%)}, with a mean age of 72.08 (±50.96) months, completed the study. The sensitivity, specificity, positive and negative predictive value, positive and negative likelihood ratio of the modified epilepsy tool were 91.5% (84.5–96.1), 88.6% (80.0–93.5), 89.7% (81.9–95.3), 90.8% (83.7–95.7), 8 (6.6–9.8) and 0.09 (0.07–0.12) respectively. Significance The new modified diagnostic instruments for epilepsy is simple, structured and valid instruments covering 1month to 18 years for use in resource limited settings with acceptable diagnostic accuracy. All seizure semiologies as well as common seizure mimics like breath-holding spells are included in the tool. It also provides for identification of acute symptomatic and febrile seizures. [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]

There are conflicting reports in the literature about the influence of valproate on thyroid function. A cross-sectional study was performed to determine the prevalence of subclinical hypothyroidism in ambulatory children aged 3 to 15 years with controlled epilepsy receiving valproate monotherapy for at least 6 months. Fifty-seven consecutive children with controlled epilepsy on valproate monotherapy and 52 healthy age- and sex-matched control children were studied. Thyroid-stimulating hormone, free thyroxine, antithyroid peroxidase antibodies, and serum valproic acid levels were measured. There was a significantly high (P = .012) prevalence of subclinical hypothyroidism (26%) in those receiving valproate monotherapy compared with healthy controls (7.7%). Median duration of valproate therapy was significantly higher (P = .039) in the subclinical hypothyroidism group (21 months, range 6-36) compared with those without subclinical hypothyroidism (14 months, range 6-25). Results of the present study suggest higher prevalence of subclinical hypothyroidism in children with controlled epilepsy on long-term valproate monotherapy. [ABSTRACT FROM PUBLISHER]

Summary: Purpose: The ketogenic (lipid to non-lipid) ratio may play an important role in the efficacy and tolerability of ketogenic diets (KD). This study was planned to compare the efficacy and tolerability of 2.5:1 versus 4:1 lipid:non-lipid ratio KD in young children with refractory epilepsy. Methods: Children aged 6 months to 5 years with refractory epilepsy were enrolled. They were randomized to receive either a 4:1 or 2.5:1 ketogenic ratio diet, which was introduced using a non-fasting protocol. Seizure frequency, biochemical profile (liver and kidney function tests, fasting lipid profile, and spot urinary calcium–creatinine ratio), and adverse effects were recorded at three months in both groups. Results: Thirty eight children were enrolled, 19 in each group. At three months, 11 children (58%) in the 4:1 group and 12 (63%) in the 2.5:1 group had more than 50% reduction in seizures (p =0.78). Five children (26%) in the 4:1 group and four (21%) in 2.5:1 group became seizure free. There was no significant difference in the biochemical parameters between the two groups. Conclusion: 2.5:1 ratio KD is possibly as effective as 4:1 KD in controlling seizures and has fewer adverse effects. [Copyright &y& Elsevier]

This cross-sectional study was planned to study the blood ammonia levels in epileptic children on 2 dose ranges of valproic acid monotherapy. A total of 60 epileptic children aged 3 months to 12 years, that were on valproic acid monotherapy for at least 3 months, were enrolled and divided into 2 groups, a low-dose group (dose, 20-39 mg/kg/d [n = 32]) and a high-dose group (dose, 40-60 mg/kg/d [n=31]). Measurements of blood ammonia, serum valproic acid levels, and liver and renal function tests were performed. Blood ammonia levels were significantly higher in the high-dose group (median, 79.2 µg/dL; range, 55.23-114.29 µg/dL) as compared with the low-dose group (median, 47.2 µg/dL; range, 20.62-73.25 µg/dL). Blood ammonia levels significantly correlated with both the dose (r = .77, P < .001) and serum levels (r = .88, P < .001) of valproic acid. All the children with hyperammonemia were asymptomatic. There were no significant differences in the other biochemical parameters between the 2 groups. [ABSTRACT FROM AUTHOR]

Intractable epilepsies pose a therapeutic challenge. Precise localization of the epileptic focus is imperative before planning surgical intervention. Functional imaging is an important component of presurgical work-up. Positron emission topography is unavailable in developing countries; hence, the need to evaluate the available imaging modality, single photon emission computed topography (SPECT), was felt. We investigated 61 children with intractable epilepsy, identified by predefined criteria, by performing electroencephalography (EEG), magnetic resonance imaging (MRI), computed topography (CT), and ictal and interictal SPECT. The localizing value of ictal and interictal SPECT imaging for epileptic foci was correlated with clinical, electrophysiologic, and anatomic neuroimaging data. An ictal SPECT was obtained in 9, and interictal SPECT was performed in all (61). Ictal SPECT was localizing in 8 of 9 (88.8%). Interictal SPECT was localizing in a significantly higher proportion of patients (47.54%) than either the scalp EEG (16.39%) (P = .0003) or CT scan (21.56%) (P = .0046). Our data demonstrated that interictal and ictal SPECT identified more focal changes in children with intractable epilepsy than interictal EEG, CT, and probably MRI. The definitive proof of the SPECT-based findings being epileptogenic foci awaits correlation with intraoperative monitoring and postoperative follow-up. (J Child Neurol 2001;16:257–263). [ABSTRACT FROM AUTHOR]

The predominant reason for the discontinuation of low glycemic index therapy (LGIT) in children with epilepsy is the dietary restrictions imposed therein. This trial intended to compare the efficacy of daily and intermittent LGIT in children with drug-resistant epilepsy (DRE). This study was performed between February 2018 and January 2019 to compare the efficacy of daily and intermittent LGIT in children aged 1–15 years with DRE following 24 weeks of dietary therapy. Compliance, the difficulty faced by caregivers, adverse effects, impact on behaviour, and social quotient in both arms were compared. Children in the intermittent LGIT arm received a liberalized diet for two days every week (Saturday and Sunday), which also allowed medium glycemic index foods. Carbohydrate calories were allowed up to 20% of the total caloric requirement in the liberalized diet, as compared to only 10% in standard LGIT. Out of 132 children randomized (66 in each group), 122 completed 24 weeks follow up. Mean weekly seizure frequency reduction at 24 weeks in the intermittent LGIT group was comparable with that of the daily LGIT group in both intention-to-treat (ITT) and per-protocol analysis (−50.95%± 22.34% vs −47.16%± 23.41%, p=0.36 in ITT and −53.88%±20.54% vs −49.20%±21.87%, p=0.23) in per-protocol analysis for intermittent and daily LGIT group respectively). The proportion with ≥50% reduction in seizure frequency was also comparable between both groups (p=0.73 and 0.56 in ITT and per protocol analysis respectively). The proportion of patients with adverse events and satisfactory compliance rate also had a trend towards favoring intermittent LGIT (p=0.06 and 0.51, respectively), while caregiver difficulty was lower with intermittent LGIT (p=0.001). Intermittent LGIT is comparable to daily LGIT in terms of seizure frequency reduction after 24 weeks of dietary therapy. ClinicalTrials.gov (Registration number- NCT03464487, https://clinicaltrials.gov/ct2/show/NCT03464487). • Out of 132 children randomized, 122 children completed 12 weeks follow up. • Mean weekly seizure frequency reduction at 24 weeks was comparable in both the groups. • Adverse events and compliance rate also had a trend towards favoring intermittent LGIT. • Fewer caregivers faced difficulty with intermittent LGIT (p=0.001). [ABSTRACT FROM AUTHOR]

Purpose The aim of this study was to evaluate the efficacy of the modified Atkins diet in a randomized controlled trial in children with refractory epilepsy. Methods Children aged 2-14 years who had daily seizures despite the appropriate use of at least three anticonvulsant drugs were enrolled. Children were randomized to receive either the modified Atkins diet or no dietary intervention for a period of 3 months. The ongoing anticonvulsant medications were continued unchanged in both the groups. Seizure control at 3 months was the primary end point. Analysis was intention to treat. Adverse effects of the diet were assessed by parental reports (ClinicalTrials.gov Identifier: NCT00836836). Key Findings Among a total of 102 children, 50 were in the diet group and 52 in the control group. Four children discontinued the diet before the study end point, and three children in the control group were lost to follow-up. The mean seizure frequency at 3 months, expressed as a percentage of the baseline, was significantly less in the diet group: 59 ± 54 (95% confidence interval [ CI] 44-74.5) versus 95.5 ± 48 (95% CI 82-109), p = 0.003. The proportion of children with >90% seizure reduction (30% vs. 7.7%, p = 0.005) and >50% seizure reduction was significantly higher in the diet group (52% vs. 11.5%, p < 0.001). Constipation was the most common adverse effect among children on the diet (23, 46%). Significance The modified Atkins diet was found to be effective and well tolerated in children with drug-refractory epilepsy. [ABSTRACT FROM AUTHOR]

Summary: Purpose: Autonomic symptoms frequently occur during seizures. There are reports that the adult group of intractable epilepsy patients have different autonomic profile than the well controlled epilepsy, but there is no clear evidence in the children epilepsy group, therefore, we planned to study the autonomic profile in well controlled and refractory epilepsy children by recording the short-term heart rate variability (HRV). Methods: This cross sectional study was conducted in a tertiary care hospital between July 2008 and June 2009. Children with mean age of 9.1±3.3 years were enrolled. Three groups of children, 40 in each group namely, refractory epilepsy, well controlled epilepsy and normal control children were included. Children who had chronic systemic diseases and were on drugs that cause autonomic dysfunction were excluded. All children underwent short term heart rate variability testing. Data was analyzed in time domain and frequency domain. Results: pNN50 was significantly lower in children with refractory epilepsy than the well control as well as healthy controls. Rest of the parameters of time domain and frequency domain were comparable between the groups. Conclusions: Our study showed that parasympathetic activity is lower in refractory epilepsy children. However, autonomic tone is comparable in well control versus healthy controls. [Copyright &y& Elsevier]

Introduction Epilepsy has several comorbidities and associated stigma. Stigma associated with epilepsy is well known and prevalent worldwide. Surgical treatment is an established treatment for drug refractory epilepsy. Following surgery in children, it is possible that the stigma may reduce, but such an effect has not been studied earlier. Materials and methods Analysis of prospectively collected data was performed for pediatric patients at a single tertiary center for treating epilepsy. Child stigma scale, as described by Austin et al., was used to evaluate stigma both pre- and postoperatively. Analysis was done using Paired t test. Results In this study, following surgery, there was significant reduction of stigma (P < 0.001). This was proportional to the reduction in seizures, though there were 9 (30%) patients, who due to persistent neurodisability did not have any reduction of stigma despite having good seizure outcome. Conclusion Surgery in drug-resistant epilepsy helps in reducing stigma. Seizure reduction is probably not the only factor responsible for a change in stigma outcome. [ABSTRACT FROM AUTHOR]

Epilepsy of infancy with migrating focal seizures {a.k.a malignant migrating partial seizures of infancy (MMPSI)} is an uncommon epileptic encephalopathy with a poor prognosis. Migrating focal seizures with autonomic features, developmental stagnation and refractoriness to treatment are its key features. It is caused by genetic defects in various ion channels, most common being sodium activated potassium channel (KCNT1), found in up to 50% of cases. With advent of genetic diagnosis and precision medicine, many targeted therapies have been identified. Antagonist of KCNT1 coded ion channel like Quinidine has shown promising results in MMPSI. Here we report first mutation proven case of MMPSI from India. This child had a novel heterozygous missense mutation in exon10 of the KCNT1 gene (chr9:138650308; c.808C > C/G (p.Q270E)) which was pathogenic. Neither quinidine nor ketogenic diet could control his seizures. Ultimately, the child succumbed to his illness at nine months of age. [ABSTRACT FROM AUTHOR]

• Low glycemic index therapy diet is more efficacious in in children with drug resistant epilepsy. • Around one third of children with drug resistant epilepsy have at least 50 % reduction in seizure frequency with LGIT diet. • Low glycemic index therapy diet has very few adverse effects and majority of children find it acceptable. • Transient lethargy and vomiting occur only in minority and resolve spontaneously without requiring discontinuation of diet. • LGIT diet can be started successfully on outpatient basis without strict monitoring. A classic ketogenic diet, even though effective in children with drug-resistant epilepsy is not tolerated well by them and cumbersome to prepare. Low glycemic index therapy (LGIT), the least restrictive with minimal adverse effects among ketogenic dietary therapies has been proven effective in uncontrolled trials, but a placebo-controlled trial in this regard is still lacking. In this open-label randomized controlled study, we randomized children above age two years with drug-resistant epilepsy into two groups (LGIT and control groups). Patients in the LGIT group received an add-on low glycemic index diet for 3 months along with the ongoing antiepileptic drugs and the patients in the control group did not receive any dietary intervention. Seizure frequency was assessed from the seizure diary maintained by the parents. Diet compliance was assessed using the diet diary that was maintained by the parents for three days just before the scheduled monthly visits of the patients. Forty children with drug-refractory epilepsy (20 in each group) were enrolled. While 6/20 children in the LGIT arm have >50 % reduction in seizure frequency, none achieved this in the control arm (p = 0.02). The overall compliance with the low glycemic diet in the intervention group was 88.5 %. Out of six responders to LGIT, one child achieved seizure freedom and one achieved >90 % seizure reduction. Five continued LGIT further for a median duration of 8 months (range-4−12 months) successfully. The number needed to treat for more than 50 % seizure reduction was 3 and for more than 90 % seizure reduction was 10. The mean frequency of seizures for the intervention and control groups at three months of follow-up was not significantly different (p = 0.16), but the change in seizure frequency as compared to baseline was better in the intervention arm (p = 0.01). Three patients in the LGIT arm had non-serious adverse events (lethargy in two, vomiting in one). In children aged 2−8 years with drug-refractory epilepsy, the administration of LGIT along with ongoing anti-seizure medications (ASM) is more efficacious in reducing seizure frequency as compared to ASM alone. [ABSTRACT FROM AUTHOR]