OBJECTIVE: The dysregulation of brain iron homeostasis is closely relevant to a multitude of chronic neurological disorders. This study employed quantitative susceptibility mapping (QSM) to detect and compare whole-brain iron content between childhood epilepsy with centrotemporal spikes (CECTS) children and typically developing children.
METHODS: 32 children with CECTS and 25 age- and gender-matched healthy children were enrolled. All participants were imaged with 3.0-T MRI to acquire the structural and susceptibility-weighted data. The susceptibility-weighted data were processed using STISuite toolbox to obtain QSM. The magnetic susceptibility difference between the two groups was compared using voxel-wise and region of interest methods. Multivariable linear regression, controlling for age, were employed to investigate the associations between the brain magnetic susceptibility and age at onset.
RESULTS: Lower magnetic susceptibility was mainly observed in sensory- and motor-related brain regions in children with CECTS, including bilateral middle frontal gyrus, supplementary motor area, midcingulate cortex, paracentral lobule and precentral gyrus, the magnetic susceptibility of right paracentral lobule, right precuneus and left supplementary motor area were found to have positive correlation with the age at onset.
CONCLUSIONS: This study suggests that the potential iron deficiency in certain brain regions is associated with CECTS, which might be helpful for further illumination of potential pathogenesis mechanism of CECTS.

UNASSIGNED: Estimate sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of EEG findings: centrotemporal spikes, photoparoxysmal response, asymmetric photic driving, and asymmetric sleep spindles, for epilepsy phenotype and presence of structural brain abnormalities.
UNASSIGNED: In this case-control study we reviewed children referred for EEG over a 4-year period, with at least one of centrotemporal spikes, photoparoxysmal response, asymmetric photic driving, or asymmetric sleep spindles. This cohort was analyzed in combination with a research database of pediatric patients with seizures.
UNASSIGNED: Centrotemporal spikes had 100% sensitivity for childhood epilepsy with centrotemporal spikes or atypical childhood epilepsy with centrotemporal spikes, but lower specificity (70%) and PPV (58%). Photoparoxysmal response had high specificity (92%) and NPV (92%) for genetic generalized epilepsy. Asymmetric photic driving had low sensitivity for structural brain abnormalities (17%), with specificity 80%. In contrast, asymmetric sleep spindles had much higher sensitivity and specificity, 44% and 97%, respectively.
UNASSIGNED: Although centrotemporal spikes are classically associated with childhood epilepsy with centrotemporal spikes, these discharges are seen in other conditions. Photoparoxysmal response is highly indicative of a genetic generalized epilepsy, though may be seen in other epilepsy phenotypes. Relative attenuation of sleep spindles is a more reliable indicator of structural brain malformation than asymmetric photic driving.
UNASSIGNED: The quantitative diagnostic utility of EEG findings should be considered when incorporating these results into clinical decision-making.

Previous studies have demonstrated that language impairments are frequently observed in patients with benign epilepsy with centrotemporal spikes (BECTS). However, how BECTS affects language processing in the Chinese population remains unclear. With the use of functional magnetic resonance imaging (fMRI) in an overt picture-naming task, the present study examined functional connectivity in 27 children with BECTS and 26 healthy controls. The results indicated that children with BECTS showed altered functional connectivity associated with speech production between the left precuneus and the right cerebellum, between the right precuneus and the bilateral thalamus and the left superior temporal gyrus, between the right cuneus and the right postcentral gyrus and the right inferior parietal lobule, and between the right cerebellum and right middle frontal gyrus. Collectively, the findings in this study demonstrate the abnormal functional connectivity basis of speech production in Chinese children with BECTS, providing clues to understanding the brain mechanisms of language-related network in patients with BECTS.

The 2017 International League Against Epilepsy classification has defined a three-tier system with epilepsy syndrome identification at the third level. Although a syndrome cannot be determined in all children with epilepsy, identification of a specific syndrome provides guidance on management and prognosis. In this paper, we describe the childhood onset epilepsy syndromes, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features. Based on the 2017 Classification of Seizures and Epilepsies, some syndrome names have been updated using terms directly describing the seizure semiology. Epilepsy syndromes beginning in childhood have been divided into three categories: (1) self-limited focal epilepsies, comprising four syndromes: self-limited epilepsy with centrotemporal spikes, self-limited epilepsy with autonomic seizures, childhood occipital visual epilepsy, and photosensitive occipital lobe epilepsy; (2) generalized epilepsies, comprising three syndromes: childhood absence epilepsy, epilepsy with myoclonic absence, and epilepsy with eyelid myoclonia; and (3) developmental and/or epileptic encephalopathies, comprising five syndromes: epilepsy with myoclonic-atonic seizures, Lennox-Gastaut syndrome, developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep, hemiconvulsion-hemiplegia-epilepsy syndrome, and febrile infection-related epilepsy syndrome. We define each, highlighting the mandatory seizure(s), EEG features, phenotypic variations, and findings from key investigations.

OBJECTIVE: Childhood epilepsy with centrotemporal spikes (CECTS) is known as age-limited focal epilepsy syndrome in childhood. Lacosamide is a third-generation antiepileptic drug. This study aimed to evaluate the efficacy of lacosamide monotherapy for the treatment of CECTS.
METHODS: We enrolled 18 patients (6 girls and 12 boys) who met the following criteria: 1) the age of onset of the seizures was between 3 and 13 years of age; 2) showing at least hemifacial and/or oropharyngeal seizures; 3) interictal discharges in central and/or middle temporal electrodes; 4) no intellectual disability; 5) treatment duration of lacosamide monotherapy over 6 months. We retrospectively collected and analyzed clinical data and treatment information. We evaluated the seizure occurrences during 0-3, 4-6, and 7-12 months from the treatment initiation and the last 6 months of the follow-up. We also evaluated the outcomes as seizure-free if the patients developed no seizures both over 6 months and 3 times of pretreatment mean seizure interval at the last follow-up.
RESULTS: Of the patients, 39%, 67% and 72% were seizure-free during 0-3, 4-6, and 7-12 months from treatment initiation, respectively. Finally, 83% of the patients achieved seizure freedom. Seizure freedom was achieved in 72% during the first 4 months of treatment. All patients continued lacosamide monotherapy during the study, although four patients showed transient fatigue or somnolence.
CONCLUSIONS: Lacosamide showed good efficacy for controlling seizures with fewer adverse effects, and therefore may be a good candidate as a first-line medication for the treatment of new-onset CECTS.

Rolandic epilepsy is the most common form of epileptic encephalopathy, characterized by sleep-potentiated inferior Rolandic epileptiform spikes, seizures, and cognitive deficits in school-age children that spontaneously resolve by adolescence. We recently identified a paucity of sleep spindles, physiological thalamocortical rhythms associated with sleep-dependent learning, in the Rolandic cortex during the active phase of this disease. Because spindles are generated in the thalamus and amplified through regional thalamocortical circuits, we hypothesized that: 1) deficits in spindle rate would involve but extend beyond the inferior Rolandic cortex in active epilepsy and 2) regional spindle deficits would better predict cognitive function than inferior Rolandic spindle deficits alone. To test these hypotheses, we obtained high-resolution MRI, high-density EEG recordings, and focused neuropsychological assessments in children with Rolandic epilepsy during active (n = 8, age 9-14.7 years, 3F) and resolved (seizure free for > 1 year, n = 10, age 10.3-16.7 years, 1F) stages of disease and age-matched controls (n = 8, age 8.9-14.5 years, 5F). Using a validated spindle detector applied to estimates of electrical source activity in 31 cortical regions, including the inferior Rolandic cortex, during stages 2 and 3 of non-rapid eye movement sleep, we compared spindle rates in each cortical region across groups. Among detected spindles, we compared spindle features (power, duration, coherence, bilateral synchrony) between groups. We then used regression models to examine the relationship between spindle rate and cognitive function (fine motor dexterity, phonological processing, attention, and intelligence, and a global measure of all functions). We found that spindle rate was reduced in the inferior Rolandic cortices in active but not resolved disease (active P = 0.007; resolved P = 0.2) compared to controls. Spindles in this region were less synchronous between hemispheres in the active group (P = 0.005; resolved P = 0.1) compared to controls; but there were no differences in spindle power, duration, or coherence between groups. Compared to controls, spindle rate in the active group was also reduced in the prefrontal, insular, superior temporal, and posterior parietal regions (i.e., \"regional spindle rate\", P < 0.039 for all). Independent of group, regional spindle rate positively correlated with fine motor dexterity (P < 1e-3), attention (P = 0.02), intelligence (P = 0.04), and global cognitive performance (P < 1e-4). Compared to the inferior Rolandic spindle rate alone, models including regional spindle rate trended to improve prediction of global cognitive performance (P = 0.052), and markedly improved prediction of fine motor dexterity (P = 0.006). These results identify a spindle disruption in Rolandic epilepsy that extends beyond the epileptic cortex and a potential mechanistic explanation for the broad cognitive deficits that can be observed in this epileptic encephalopathy.

Objective: Childhood epilepsy with centrotemporal spikes (CECTS), the most common childhood epilepsy, still lacks longitudinal imaging studies involving antiepileptic drugs (AEDs). In order to examine the effect of AEDs on cognition and brain activity. We investigated the neuromagnetic activities and cognitive profile in children with CECTS before and after 1 year of treatment. Methods: Fifteen children with CECTS aged 6-12 years underwent high-sampling magnetoencephalography (MEG) recordings before treatment and at 1 year after treatment, and 12 completed the cognitive assessment (The Wechsler Intelligence Scale for Children). Next, magnetic source location and functional connectivity (FC) were investigated in order to characterize interictal neuromagnetic activity in the seven frequency sub-bands, including: delta (1-4 Hz), theta (4-8 Hz), alpha (8-12 Hz), beta (12-30 Hz), gamma (30-80 Hz), ripple (80-250 Hz), and fast ripple (250-500 Hz). Results: After 1 year of treatment, children with CECTS had increased scores on full-scale intelligence quotient, verbal comprehension index (VCI) and perceptual reasoning index (PRI). Alterations of neural activity occurred in specific frequency bands. Source location, in the 30-80 Hz frequency band, was significantly increased in the posterior cingulate cortex (PCC) after treatment. Moreover, FC analysis demonstrated that after treatment, the connectivity between the PCC and the medial frontal cortex (MFC) was enhanced in the 8-12 Hz frequency band. Additionally, the whole-brain network distribution was more dispersed in the 80-250 Hz frequency band. Conclusion: Intrinsic neural activity has frequency-dependent characteristic. AEDs have impact on regional activity and FC of the default mode network (DMN). Normalization of aberrant DMN in children with CECTS after treatment is likely the reason for improvement of cognitive function.

OBJECTIVE: To estimate anti-seizure medication (ASM) treatment burden and its effects on health-related quality of life (HRQOL) in new-onset childhood epilepsy with centrotemporal spikes (CECTS) using different treatment approaches in Kazakhstan.
METHODS: Forty-three patients were followed prospectively during 2015 to 2020 for at least 2 years. Patients were divided into three groups: (1) history of ≤3 seizures (n = 32); (2) ≥4 seizures (n = 6); (3) cerebral palsy coexisting with CECTS (n = 5). The first group was subdivided into treated (n = 8) and observed (n = 24) subgroups. The shortened Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55) was completed by parents after 6 months of follow-up.
RESULTS: At the end of the study, all children had a sustained remission from seizures for at least 2 years. Differences were identified in emotional, social, and physical subscales between patients in the low seizure frequency group. Signs of low self-esteem, anxiety, depression, limited social interaction owing to pharmacotherapy, painful medical procedures, and stigma were reasons for decreased HRQOL in the treated subgroup. Overall HRQOL in treated (89.2 ± 5.2) patients was significantly decreased compared with observed children with low seizure frequency (98.0 ± 3.0).
CONCLUSIONS: ASM therapy does not necessarily improve and may decrease HRQOL in children with low seizure frequency CECTS.

UNASSIGNED: Cerebral palsy is the most frequent motor disability in childhood and is associated with a higher incidence of seizure disorders. In many instances, it is recognized that motor difficulties, as well as seizures, are from the same underlying brain lesion. However, self-limited childhood epilepsies, being a common group of epilepsy syndromes, would be expected to occur in patients with cerebral palsy merely on chance association and be unrelated to the structural brain imaging abnormality causing the motor impairment. Differential diagnosis in this case is important determining the long-term prognosis and need for anticonvulsant treatment.
UNASSIGNED: Here, we report two patients with cerebral palsy combined with epilepsy, whose age at onset, seizure semiology and electroclinical features were similar to children with self-limited childhood-specific seizure disorders (childhood epilepsy with centrotemporal spikes and Panayiotopoulos syndrome).
UNASSIGNED: These cases highlight the importance of comprehensive differential diagnosis of seizures in cerebral palsy. Co-existence of age-dependent focal epilepsies with an underlying brain pathology as white matter injury, not affecting the cerebral cortex, might take place in the case of children with impaired motor skills. With health systems increasingly utilizing clinical pathways, it is important to consider the possibility of a self-limited childhood epilepsy and avoid aggressive and unnecessary medication treatment in children with cerebral palsy.

Epilepsy can now be diagnosed even in the presence of one unprovoked seizure or if the diagnosis of an epilepsy syndrome can be made. Epilepsy syndromes represent a specific set of seizure types and electroencephalographic and imaging features that tend to have age-dependent features, triggers, and prognosis. Epilepsy syndromes are the third and final level of epilepsy diagnosis, after classification of seizure and epilepsy types. Some epilepsy syndromes are self-limiting and pharmacoresponsive and others are pharmacoresistant and associated with poor developmental outcomes (epileptic and developmental encephalopathy). Features and management of 7 common age-dependent pediatric epilepsy syndromes are described.