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Objectives: The profile of seizures in neurocutaneous syndromes is variable. We aimed to define the characteristics of epilepsy in children with neurocutaneous syndromes. Materials and Methods: Cross-sectional study over 18 months at a tertiary care pediatric hospital, including children with neurocutaneous syndromes aged between 1 and 15 years, using the 2017-International League Against Epilepsy classification. Results: In 119 children with neurocutaneous syndromes, 94 (79%) had epilepsy. In eight children with neurofibromatosis one with epilepsy, 5 (62.5%) had generalized motor tonic-clonic seizures, 1 (12.5%) had generalized motor epileptic spasms, 1 (12.5%) had generalized motor automatism, and 1 (12.5%) had a focal seizure. In 69 children with tuberous sclerosis complex with epilepsy, 30 (43.5%) had generalized motor epileptic spasms, 23 (33.3%) had focal seizures, and nine (13.0%) had generalized motor tonic-clonic seizures. In 14 children with Sturge-Weber syndrome with epilepsy, 13 (92.8%) had focal seizures, and 1 (7.2%) had generalized motor tonic seizures. Statistically significant associations were found between epilepsy and intellectual disability (P = 0.02) and behavioral problems (P = 0.00). Conclusion: Profiling seizures in children with neurocutaneous syndromes are paramount in devising target-specific treatments as the epileptogenesis in each syndrome differs in the molecular pathways leading to the hyperexcitability state. Further multicentric studies are required to unravel better insights into the epilepsy profile of neurocutaneous syndromes. [ABSTRACT FROM AUTHOR]

The neurodevelopmental outcomes in children with tuberous sclerosis complex (TSC) with epileptic spasms remain underdiagnosed and might be responsible for significant morbidity and mortality burdens, even after spasms abate. The study was a cross-sectional study over 18 months at a tertiary care pediatric hospital, involving 30 children with TSC who had epileptic spasms. They were assessed with Diagnostic and Statistical Manual of Mental Disorders-5 criteria for autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), and intellectual disability (ID), and childhood psychopathology measurement schedule (CPMS) for behavioral disorders. The median age at onset of epileptic spasms was 6.5 (1–12) months, and the age at enrolment was 5 (1–15) years. Of 30 children, 2 (6.7%) had only ADHD, 15 (50%) had only ID/GDD (global developmental delay), 4 (13.3%) had ASD and ID/GDD, 3 (10%) had ADHD and ID/GDD, and 6 (20%) had none. The median intelligence quotient/development quotient (IQ/DQ) score was 60.5 (20–105). CPMS assessment revealed significant behavioral abnormalities in almost half the children. Eight (26.7%) patients were completely seizure-free for at least 2 years, 8 (26.7%) had generalized tonic-clonic seizures, 11 (36.6%) had focal epilepsy, and 3 (10%) had evolved into Lennox-Gastaut syndrome. A high proportion of neurodevelopment disorders, including ASD, ADHD, ID/GDD, and behavioral disorders were seen in this pilot study with a small cohort of children with TSC with epileptic spasms. [ABSTRACT FROM AUTHOR]

Neonatal seizures are common manifestations of several neurological or systemic disorders and associated with high morbidity, mortality and poor short- and long-term developmental outcomes. It is important to determine the aetiology and factors that determine the poor outcome, more so in a newly developed setting. The early detection of predictors of poor outcome will help in planning acute management, counselling, follow-up and rehabilitation services. In this prospective observational study, we looked at the clinical profile, aetiology, short-term outcomes and predictors of poor outcome of neonatal seizures among out-born neonates. The common causes were hypoxic ischaemic encephalopathy, sepsis and metabolic disturbances. One-third of neonates had poor outcome. Abnormal neurological and cardiorespiratory examination at admission; low oxygen saturation, glucose and pH; and hypoxic ischemic encephalopathy-III were predictors of poor outcome. [ABSTRACT FROM AUTHOR]

Background and Purpose: Epstein–Barr virus (EBV) meningoencephalitis can have variable and nonspecific brain magnetic resonance imaging (MRI) findings in children. This study was done with the purpose of describing brain MRI findings in children with EBV meningoencephalitis. Materials and Methods: The study included 45 pediatric patients that presented with variable neurological symptoms and were found to have EBV meningoencephalitis based on positive EBV deoxyribonucleic acid (DNA) in the cerebrospinal fluid. All these patients had undergone brain MRI. Clinical and radiological features were evaluated. Results: Fever was a presenting feature in all cases. Signs of meningitis and raised intracranial pressure (ICP) were seen in 24 (53.3%) cases, encephalopathy in 15 (33.3%), and seizures were present in 33 (73.3%). MRI was abnormal in 29 (64.4%) patients. The cortical/subcortical pattern was diagnosed in 9 (20%) cases, white matter involvement in 7 (15.5%), basal ganglia in 5 (11.1%), thalamic involvement in 4 (8.8%), brain stem involvement in 2 (6.2%), substantia nigra involvement in 2 (4.4%), and cerebellar involvement in 2 (4.4%). Diffusion restriction was present in 11 (24.4%) cases and susceptibility changes in 7 (15.5%). Meningeal enhancement was present in 10 (22.2%) cases. In addition, brain abscess and subdural effusion/empyema were present in 1 (2.2%) case each. Conclusion: Pediatric EBV meningoencephalitis has varied clinicoradiological spectrum and there is no specific MRI pattern to characterize the meningoencephalitis on imaging. Common MRI findings include cortical-subcortical involvement, white matter changes, basal ganglia, and thalamic involvement. [ABSTRACT FROM AUTHOR]

Summary: This multicenter, randomized, blinded, controlled, trial investigated the efficacy and safety of levetiracetam compared with phenobarbital as a preferred treatment for neonatal seizures of any cause. The primary outcome variable was complete seizure freedom for 24 hours, assessed by independent review of the EEGs by 2 experts. Eighty percent of patients randomly assigned to phenobarbital remained seizure free for 24 hours, compared with 28% of patients randomly assigned to levetiracetam (P,.001; relative risk 0.35 [95% confidence interval: 0.22–0.56]; modified intention-to-treat population). A 7.5% improvement in efficacy was achieved with a dose escalation of levetiracetam from 40 to 60 mg/kg. More adverse effects were seen in subjects randomly assigned to phenobarbital (not statistically significant). The authors concluded that phenobarbital was more effective than levetiracetam for the treatment of neonatal seizures and higher rates of adverse effects were seen with phenobarbital treatment. [ABSTRACT FROM AUTHOR]

Toxic encephalopathy is an important differential diagnosis in a child with encephalopathy and seizures. Subtle circumstantial evidence and apt neuroimaging features can significantly contribute to management, especially in a case of accidental exposure. 2, 4-D (ethyl ester) poisoning is a rare diagnosis, despite the common usage of this toxic compound as weedicide in northern India. The clinical similarity to the anticholinesterase poisoning, especially in the setting of agrochemical exposure is the main cause of under-diagnosis with usually fatal outcomes. We present an interesting case of accidental 2, 4-D (ethyl ester) poisoning in a child with typical neuroimaging features. A review of the literature regarding neuroimaging patterns of bilaterally symmetrical signal abnormalities involving basal ganglia in brain magnetic resonance imaging (MRI) from the point of view of clinical significance, is also discussed. [ABSTRACT FROM AUTHOR]

The article present a case study of a 18‑month‑old girl with concerns of language delay and recurrent seizures from the age of 12 months. It mentions that on her examination, she was easily excitable and had frequent unprovoked episodes of laughter; and also mentions her electroencephalography (EEG) at 1 year of age showed runs of high amplitude generalized irregular delta waves with multifocal epileptiform discharges suggestive of hypsarrhythmia.

Objectives: To study the clinical profile, and outcome of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods: This is a retrospective case series of children <12 y of age, diagnosed with anti-NMDAR encephalitis at a tertiary care institute during the period, May 2013 through June 2015.Results: Twenty patients were tested for suspected anti-NMDAR encephalitis over this 2 y period. Of these, six children were positive for anti-NMDAR antibodies. Four of these six children had completed treatment and two are currently receiving immunotherapy. Behavioral changes, psychosis, seizures and oro-lingual-facial dyskinesia were the presenting features. Extreme irritability, insomnia and mutism were noted in all the children. The symptoms were persistent, and the course was progressive over 4-8 wk duration. Neuroimaging and electroencephalography were non-specific. Intravenous pulse methylprednisolone and immunoglobulins were used as first-line therapeutic agents. Only one patient responded to first line immunotherapy; five out of six children required second-line immunotherapy. One patient recovered following rituximab, and two patients showed a good response to cyclophosphamide pulse therapy; two patients are currently under treatment with second line immunotherapeutic agents. Tumor screen was negative in all children.Conclusions: Anti-NMDAR encephalitis is rare but a potentially treatable condition. Timely recognition is essential because treatment is entirely different from other viral encephalitis. Aggressive immunotherapy is the key to a favourable outcome. [ABSTRACT FROM AUTHOR]

A case study is presented of 5‑year‑old girl with an episode of acute onset lethargy, and confusion following an upper respiratory tract infection and recovered spontaneously over a period of 5 days. Pure tone audiometry showed profound bilateral sensorineural hearing impairment and brainstem evoked response audiometry was suggestive of bilateral profound hearing impairment. She was treatede for CAPOS syndrome associated with ATP1A3 gene.

Keywords: Epilepsy; Myoclonic absence; Hyperventilation; Electroencephalography; Valproate Epilepsy with myoclonic absences[1] refers to absence epilepsy with rhythmic 3 Hz myoclonic movements, which are associated with 3 Hz generalized spike-wave discharges on the EEG. Epilepsy with myoclonic absences is considered a pharmacoresistant epilepsy, but the combination of valproate and ethosuximide has yielded good results. [Extracted from the article]

• Phenobarbitone is a drug of first choice for controlling neonatal seizure. • The exact duration to prevent seizure recurrence is a matter of debate. • We compared seizure recurrence in phenobarbitone withdrawal and continued groups. • The seizure recurrence was similar in both the groups. The duration of hospital stay, neurological abnormality, and mortality were also similar. The long-term administration of phenobarbitone in neonates may be associated with adverse neurological outcome. The timing of stopping phenobarbitone maintenance after acute seizure control in neonates is a matter of debate. To study the effect of early withdrawal of phenobarbitone on recurrence of neonatal seizures. Open-label randomized controlled trial. Outborn neonates (≥34 weeks of gestation to <28 days of postnatal period) with seizures (n = 221) admitted to Neonatal unit in Pediatric emergency of a tertiary care hospital in north India over 1 year. After a loading dose of phenobarbitone (20 mg/kg), neonates who remained seizure free for at least 12 h were enrolled after written informed consent from parents, and randomized (computer generated block randomization) to 'phenobarbitone withdrawal group' (n = 112) where phenobarbitone maintenance was stopped and 'phenobarbitone continued group' (n = 109) where phenobarbitone maintenance was continued until discharge and further as per clinician's discretion. The primary outcome was seizure recurrence until discharge and secondary outcomes were time to reach full enteral feeds, duration of hospital stay, abnormal neurological status at discharge, and mortality in two groups. The baseline variables were comparable in 2 groups. The incidence of seizure recurrence was similar in the phenobarbitone withdrawal and phenobarbitone continued groups (50% vs. 37.6%, respectively, p = 0.078). Among secondary outcomes, the phenobarbitone withdrawal and continued groups had similar time to reach full enteral feeds (4.02 days vs. 4.2 days, p = 0.75), duration of hospital stay (6.3 days vs. 6.5 days, p = 0.23), abnormal neurological status at discharge (45.6% vs. 38%, p = 0.39), and mortality (11.6% vs. 8.3%, p = 0.50). Early withdrawal of phenobarbitone in neonatal seizures does not lead to a significant increase in the rate of seizure recurrence. [ABSTRACT FROM AUTHOR]