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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 (SpO 2 ), 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 SpO 2 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, SpO 2 , and ECG. Abnormal HRV in infants with apneic seizures might indicate additional autonomic dysregulation in IAS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Routine electroencephalogram (EEG) has many limitations, especially the inability to capture reported habitual events in question. A prolonged EEG with synchronized video (video-EEG) overcomes some of these limitations by improving the sensitivity, specificity and the diagnostic yield by attempting to record the habitual events when they are frequent and when indicated. Video-EEG is employed commonly for the diagnosis and classification of epilepsy/epilepsy syndromes, to distinguish between seizures and seizures mimickers, for pre-surgical evaluation and in the management of critically ill children. The duration of recording would vary depending on the indication and frequency of events. Ambulatory EEG is another cost effective and convenient alternative in certain circumstances. However, availability of the machines and expertise, accessibility, affordability and labor intensive nature of the procedure limit widespread use in India. This review explores the role of video-EEG in the management of children with epileptic and non-epileptic paroxysmal events with respect to routine clinical practice in India., (© 2021. Dr. K C Chaudhuri Foundation.)

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., (Copyright © 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

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]

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]

Objectives: Triclofos may be a better sedative in view of better palatability and less gastric irritation as compared to chloral hydrate. This study aimed to assess the efficacy of triclofos (a commonly used sedative in India) as a sedative for sleep electroencephalogram (EEG) study in children. Methods: This prospective observational study was carried out in a tertiary care pediatric center. Consecutive children aged 6 months to 5 years referred for sleep EEG evaluation were recruited. Their clinical details were noted in a proforma after an informed consent. After a trial for natural sleep, oral triclofos was administered. Sleep parameters and adverse effects were noted. Results: One‑hundred and sixty children were then enrolled. EEG was successfully recorded in 149 (93.1%) children. Median latency of sleep onset was 30 min and median duration of sleep was 90 min. The adverse effects in the following 24 h were mild and included dizziness, irritability, and vomiting. Conclusions: Oral triclofos was found to be an effective sedative for EEG in children with minimal adverse effects. [ABSTRACT FROM AUTHOR]

Context: Menkes disease is an X-linked multisystem disorder characterized by early onset of cerebral and cerebellar neurodegeneration, fair skin, hypopigmented sparse hair and connective tissue abnormalities. Aims: We aimed to evaluate the clinical, electrophysiological and radiological features of children with Menkes disease seen at our institute. Setting/Design: The medical records of children diagnosed with Menkes disease admitted in the pediatric neurology ward or attending the special pediatric neurology clinic at a tertiary care and a referral hospital in North India, from January 2010 to December 2012, were retrospectively reviewed. The clinical data of each case was subsequently summarized and reported. Statistical analysis used: Descriptive statistics were used. Results: During the study period, 1174 children were seen. Out of these, 6 cases were diagnosed as Menkes disease on the basis of clinical phenotype, low serum copper and ceruloplasmin and supportive neuroimaging. All the children were males and had disease onset within 3 months of age, with 4 children presenting in the neonatal period. Global developmental delay and refractory seizures were the predominant clinical symptoms. Two children had symptomatic West syndrome. Other seizure semiologies included tonic-clonic (4), myoclonic (2) and tonic seizures (1). The electroencephalographic abnormalities included hypsarrhythmia (2) and multifocal epileptiform discharges (3). The salient radiological features included white matter changes, temporal lobe abnormalities, global atrophy, subdural hygromas and tortuous cerebral blood vessels. Conclusions: Menkes disease should be suspected in a case of refractory early onset seizures especially in the presence of subtle clinical clues. The neuroimaging findings may further support the diagnosis. [ABSTRACT FROM AUTHOR]

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 fromthe 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. [ABSTRACT FROM AUTHOR]

The article discusses the case study of a 4.5-year-old boy with late infantile neuronal ceroid lipofuscinoses and reach the appropriate age when his parents noticed the decrease of his speech output and slurring. He undergoes on magnetic resonance imaging of the brain and was revealed that he had a mild cerebellar atrophy. The article discusses the neuronal ceroid lipofuscinoses (NCL), a common etiology for neurodegeneration in childhood.

A 7-year-old boy presented with episodic blindness for the last 2 months with occipital paroxysms and fixation-off sensitivity on electroencephalography (EEG). The clinico-EEG features were suggestive of idiopathic childhood occipital epilepsy of Gastaut. The interesting phenomenon of fixation-off sensitivity is discussed. [ABSTRACT FROM PUBLISHER]

The article presents a case study of 9‑year old boy presented with frequent brief paroxysmal events for the last six months. It mentions that diagnosis by electroencephalogram (EEG) showed benign EEG variants like 14‑ and 6‑Hz positive bursts and N waves which are predominantly seen during drowsiness, light and rapid eye movement (REM) sleep, and have maximal amplitude over the posterior temporal head region.

The article presents a case study of a three an half‑year‑old girl was taken to hospital due to daily seizures problem. It mentions the presence of cognitive decline, behavioural abnormalities, or abnormal body movements in patient. It also mentions that absence of seizures may be associated with myoclonic jerks and limited area of the face or neck.

The article presents a case study of a 4‑year‑old girl with speech delay and refractory seizures. It mentions that the patient has no adverse perinatal events, had normal motor milestones, and could speak only a few bisyllables. It also mentions the use of magnetic resonance imaging (MRI) and electroencephalogram (EEG) for examine the patient.

Cognitive flexibility encompasses the ability to efficiently shift focus and forms a critical component of goal-directed attention. The neural substrates of this process are incompletely understood in part due to difficulties in sampling the involved circuitry. We leverage stereotactic intracranial recordings to directly resolve local-field potentials from otherwise inaccessible structures to study moment-to-moment attentional activity in children with epilepsy performing a flexible attentional task. On an individual subject level, we employed deep learning to decode neural features predictive of task performance indexed by single-trial reaction time. These models were subsequently aggregated across participants to identify predictive brain regions based on AAL atlas and FIND functional network parcellations. Through this approach, we show that fluctuations in beta (12–30 Hz) and gamma (30–80 Hz) power reflective of increased top-down attentional control and local neuronal processing within relevant large-scale networks can accurately predict single-trial task performance. We next performed connectomic profiling of these highly predictive nodes to examine task-related engagement of distributed functional networks, revealing exclusive recruitment of the dorsal default mode network during shifts in attention. The identification of distinct substreams within the default mode system supports a key role for this network in cognitive flexibility and attention in children. Furthermore, convergence of our results onto consistent functional networks despite significant inter-subject variability in electrode implantations supports a broader role for deep learning applied to intracranial electrodes in the study of human attention. [ABSTRACT FROM AUTHOR]

Absence seizures associated with myoclonic phenomena have been associated with 4 seizure types. Recently, a new seizure type of neck myoclonia with absences was described. We present a case of 9-year-old girl who presented with abnormal head shaking and vacant stare for the past 5 months with an ictal electroencephalograph (EEG) record showing 3-Hz spike-and-wave discharges. The seizures were easily controlled with valproate and clobazam. Neck myoclonia with absences might be a new idiopathic generalized epilepsy syndrome in development. [ABSTRACT FROM PUBLISHER]

Objectives This study aimed to test the hypothesis that high-dose prednisolone (4 mg/kg/day) may be more efficacious than usual-dose (2 mg/kg/day) prednisolone for spasm resolution at 14-days in children with infantile spasms. Methods This was a randomized, open-label-trial conducted at a tertiary-level-hospital from February-2012 to March-2013. Children aged 3-months to 2-years presenting with infantile spasms in clusters (at least 1 cluster/day) with hypsarrhythmia or its variants on EEG were enrolled. The study participants were randomized to receive either high-dose prednisolone (4 mg/kg/day) or the usual-dose (2 mg/kg/day) prednisolone. The primary outcome measure was the proportion of children who achieved spasm freedom for 48-h at day-14 after treatment initiation as per parental reports in both the groups. The adverse effects were also monitored. The study was registered with the clinicaltrials.gov (ClinicalTrials.gov Identifier: NCT01575639). Results Sixty-three children were randomized into the two groups with comparable baseline characteristics. The proportion of children with spasm cessation on day-14 was significantly higher in the high-dose group as compared to the usual-dose group (51.6% vs. 25%, p = 0.03). The absolute risk reduction was 26.6% (95% confidence interval 11.5-41.7%) with number needed to treat being 4. The adverse effects were comparable in both the groups. Conclusions High-dose prednisolone (4 mg/kg/d) was more effective than low-dose prednisolone (2 mg/kg/d) in achieving spasm cessation at 14-days (as per parental reports) in children with infantile spasms. [ABSTRACT FROM AUTHOR]