ObjectiveThis systematic review and meta-analyses assessed seizure outcome following pediatric epilepsy surgery.MethodsMEDLINE, EMBASE, and Cochrane were searched for pediatric epilepsy surgery original research from 1990 to 2017. The outcome was seizure freedom at 12 months or longer follow-up. Using random-effects models, the effect sizes for controlled studies, uncontrolled studies on surgery locations (temporal lobe [TL], extratemporal lobe [ETL], or hemispheric surgery), pathologies, nonlesional epilepsy, and incomplete resection were estimated. Meta-regression assessed the relationship between age at surgery, age at seizure onset, and seizure outcome. Random-effects network meta-analysis was conducted for surgery locations.ResultsTwo hundred fifty-eight studies were included. Surgery achieved higher seizure freedom than medical therapy (odds ratio [OR] = 6.49 [95% confidence interval [CI]: 2.87–14.70], p < 0.001). Seizure freedom declined over time after surgery, from 64.8% (95% CI: 51.2%–76.4%; p = 0.034) at 1 year, to 60.3% (95% CI: 52.9%–67.4%; p = 0.007) at 5 years, and to 39.7% (95% CI: 28.4%–52.2%, p = 0.106) at 10 years. Seizure freedom was (1) highest for hemispheric surgery, followed by TL and ETL surgery, and (2) highest for tumor and lower for malformations of cortical development. Seizure freedom was lower for nonlesional than lesional epilepsy (OR = 0.54 [95% CI: 0.34, 0.88], p = 0.013) and incomplete than complete resection (OR = 0.13 [95% CI: 0.08, 0.21], p < 0.001). Age at surgery and age at seizure onset were associated with seizure freedom for mixed pathologies and surgery locations and TL surgery.ConclusionEpilepsy surgery was more effective than medical therapy to control seizures. Understanding seizure outcomes of different surgery locations, pathologies, nonlesional epilepsy, and incomplete resection will assist with presurgical counseling.

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.

No abstract

Epileptic encephalopathies refer to a group of disorders in which the unremitting epileptic activity contributes to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone, and these can worsen over time leading to progressive cerebral dysfunction. Several syndromes have been described based on their electroclinical features (age of onset, seizure type, and EEG pattern). This review briefly describes the clinical evaluation and management of commonly encountered epileptic encephalopathies in children.

No abstract

In adults, the Status Epilepticus Severity Score (STESS), a clinical score, has been shown to be a good predictor of outcome and treatment response. We devised a pediatric modification of this score: the Status Epilepticus in Pediatric patients Severity Score (STEPSS) and evaluated it in children with status epilepticus. Methods: In this prospective study, children aged 1 month to 18 years presenting with seizure duration ≥ 5 min or actively convulsing to the emergency room were enrolled. STEPSS score was calculated at the time of admission. Outcomes included death, the Pediatric Overall Performance Category (POPC) at discharge and treatment response. The diagnostic utility of the STEPSS score to predict unfavourable outcome was evaluated. Results: One-hundred and forty children (mean age 5.8 years) were enrolled. Seven children died and overall 15 children had an unfavourable outcome. The predictive accuracy of STEPSS at a cut-off of > 3: for unfavourable outcome (POPC score ≥ 3) -sensitivity (0.

No abstract

Objective: Through international collaboration, we evaluated the phenotypic aspects of a multiethnic cohort of KCNT1-related epilepsy and explored genotypephenotype correlations associated with frequently encountered variants. Methods: A cross-sectional analysis of children harboring pathogenic or likely pathogenic KCNT1 variants was completed. Children with one of the two more common recurrent KCNT1 variants were compared with the rest of the cohort for the presence of particular characteristics. Results: Twenty-seven children (15 males, mean age = 40.8 months) were included.Seizure onset ranged from 1 day to 6 months, and half (48.1%) exhibited developmental plateauing upon onset. Two-thirds had epilepsy of infancy with migrating focal seizures (EIMFS), and focal tonic seizures were common (48.1%). The most frequent recurrent KCNT1 variants were c.2800G>A; p.Ala934Thr (n = 5) and c.862G>A; p.Gly288Ser (n = 4). De novo variants were found in 96% of tested parents (23/24). Sixty percent had abnormal magnetic resonance imaging (MRI) findings. Delayed myelination, thin corpus callosum, and brain atrophy were the most common. One child had gray-white matter interface indistinctness, suggesting a malformation of cortical development. Several antiepileptic drugs (mean = 7.4/patient) were tried, with no consistent response to any one agent. Eleven tried quinidine; 45% had marked (>50% seizure reduction) or some improvement (25%-50% seizure reduction). Seven used cannabidiol; 71% experienced marked or some improvement. Fourteen tried diet therapies; 57% had marked or some improvement.When comparing the recurrent variants to the rest of the cohort with respect to developmental trajectory, presence of EIMFS, >500 seizures/mo, abnormal MRI, and treatment response, there were no statistically significant differences. Four patients died (15%), none of sudden unexpected death in epilepsy. Significance: Our cohort reinforces common aspects of this highly pleiotropic entity. EIMFS manifesting with refractory tonic seizures was the most common.Cannabidiol, diet therapy, and quinidine seem to offer the best chances of seizure reduction, although evidence-based practice is still unavailable. K E Y W O R D Scannabidiol, epilepsy of infancy with migrating focal seizures, KCNT1, ketogenic diet, microcephaly, quinidine