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Object Epilepsy surgery is an effective treatment for selected patients with focal intractable epilepsy. Complete removal of the epileptogenic zone significantly increases the chances for postoperative seizure-freedom. In complex surgical candidates, delineation of the epileptogenic zone requires a long-term invasive video/EEG from intracranial electrodes. It is especially challenging to achieve a complete resection in deep brain structures such as opercular-insular cortex. We report a novel approach utilizing intraoperative visual detection of stereotactically implanted depth electrodes to inform and guide the extent of surgical resection. Methods We retrospectively reviewed data of pediatric patients operated in Motol Epilepsy Center between October 2010 and June 2020 who underwent resections guided by intraoperative visual detection of depth electrodes following SEEG. The outcome in terms of seizure- and AED-freedom was assessed individually in each patient. Results Nineteen patients (age at surgery 2.9–18.6 years, median 13 years) were included in the study. The epileptogenic zone involved opercular-insular cortex in eighteen patients. The intraoperative detection of the electrodes was successful in seventeen patients and the surgery was regarded complete in sixteen. Thirteen patients were seizure-free at final follow-up including six drug-free cases. The successful intraoperative detection of the electrodes was associated with favorable outcome in terms of achieving complete resection and seizure-freedom in most cases. On the contrary, the patients in whom the procedure failed had poor postsurgical outcome. Conclusion The reported technique helps to achieve the complete resection in challenging patients with the epileptogenic zone in deep brain structures.

Epilepsy surgery fails in 30% of patients with focal cortical dysplasia (FCD). The seizure persistence after surgery can be attributed to the inability to precisely localize the tissue with an endogenous potential to generate seizures. In this study, we aimed to identify the critical components of the epileptic network that were actively involved in seizure genesis.The directed transfer function was applied to intracranial EEG recordings and the effective connectivity was determined with a high temporal and frequency resolution. Pre-ictal network properties were compared with ictal epochs to identify regions actively generating ictal activity and discriminate them from the areas of propagation.Analysis of 276 seizures from 30 patients revealed the existence of a seizure-related network reconfiguration in the gamma-band (25-170 Hz; p 0.005) - ictogenic nodes. Unlike seizure onset zone, resecting the majority of ictogenic nodes correlated with favorable outcomes (p 0.012).The prerequisite to successful epilepsy surgery is the accurate identification of brain areas from which seizures arise. We show that in FCD-related epilepsy, gamma-band network markers can reliably identify and distinguish ictogenic areas in macroelectrode recordings, improve intracranial EEG interpretation and better delineate the epileptogenic zone.Ictogenic nodes localize the critical parts of the epileptogenic tissue and increase the diagnostic yield of intracranial evaluation.

OBJECTIVEIn this study, the authors aimed to determine 1) whether the use of intraoperative electrocorticography (ECoG) affects outcomes and complication rates of children undergoing resective epilepsy surgery; 2) which patient- and epilepsy-related variables might influence ECoG-based surgical strategy; and 3) what the predictors of epilepsy surgery outcomes are.METHODSOver a period of 12 years, data were collected on pediatric patients who underwent tailored brain resections in the Motol Epilepsy Center. In patients in whom an abnormal ECoG pattern (e.g., spiking, suppression burst, or recruiting rhythm) was not observed beyond presurgically planned resection margins, the authors did not modify the surgical plan (group A). In those with significant abnormal ECoG findings beyond resection margins, the authors either did (group B) or did not (group C) modify the surgical plan, depending on the proximity of the eloquent cortex or potential extent of resection. Using Fisher’s exact test and the chi-square test, the 3 groups were compared in relation to epilepsy surgery outcomes and complication rate. Next, multivariate models were constructed to identify variables associated with each of the groups and with epilepsy surgery outcomes.RESULTSPatients in group C achieved significantly lower rates of seizure freedom compared to groups A (OR 30.3, p < 0.001) and B (OR 35.2, p < 0.001); groups A and B did not significantly differ (p = 0.78). Patients in whom the surgical plan was modified suffered from more frequent complications (B vs A+C, OR 3.8, p = 0.01), but these were mostly minor (duration < 3 months; B vs A+C, p = 0.008). In all cases, tissue samples from extended resections were positive for the presence of the original pathology. Patients with intended modification of the surgical plan (groups B+C) suffered more often from daily seizures, had a higher age at first seizure, had intellectual disability, and were regarded as MR-negative (p < 0.001). Unfavorable surgical outcome (Engel class II–IV) was associated with focal cortical dysplasia, incomplete resection based on MRI and/or ECoG findings, negative MRI finding, and inability to modify the surgical plan when indicated.CONCLUSIONSIntraoperative ECoG serves as a reliable tool to guide resection and may inform the prognosis for seizure freedom in pediatric patients undergoing epilepsy surgery. ECoG-based modification of the surgical plan is associated with a higher rate of minor complications. Children in whom ECoG-based modification of the surgical plan is indicated but not feasible achieve significantly worse surgical outcomes.

Background Neurodegeneration with brain iron accumulation constitutes a group of rare progressive movement disorders sharing intellectual disability and neuroimaging findings as common denominators. Beta-propeller protein-associated neurodegeneration (BPAN) represents approximately 7% of the cases, and its first signs are typically epilepsy and developmental delay. We aimed to describe in detail the phenotype of BPAN with a special focus on iron metabolism. Material and methods We present a cohort of paediatric patients with pathogenic variants of WD-Repeat Domain 45 gene (WDR45). The diagnosis was established by targeted panel sequencing of genes associated with epileptic encephalopathies (n = 9) or by Sanger sequencing of WDR45 (n = 1). Data on clinical characteristics, molecular-genetic findings and other performed investigations were gathered from all participating centres. Markers of iron metabolism were analysed in 6 patients. Results Ten children (3 males, 7 females, median age 8.4 years) from five centres (Prague, Berlin, Vogtareuth, Tubingen and Cologne) were enrolled in the study. All patients manifested first symptoms (e.g. epilepsy, developmental delay) between 2 and 31 months (median 16 months). Seven patients were seizure-free (6 on antiepileptic medication, one drug-free) at the time of data collection. Neurological findings were non-specific with deep tendon hyperreflexia (n = 4) and orofacial dystonia (n = 3) being the most common. Soluble transferrin receptor/log ferritin ratio was elevated in 5/6 examined subjects; other parameters of iron metabolism were normal. Conclusion Severity of epilepsy often gradually decreases in BPAN patients. Elevation of soluble transferrin receptor/log ferritin ratio could be another biochemical marker of the disease and should be explored by further studies.

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Objective The aim of this study is the investigation of early-life EEG background abnormalities or “dysmature” traits in infants with tuberous sclerosis complex (TSC) and their capacity to predict autism spectrum disorder or neurodevelopmental outcome. Methods EEG data were prospectively collected from TSC patients during the EPISTOP trial (NCT02098759). Subjects were younger than 4 months, and ASD risk and neurodevelopmental outcome were assessed at the age of 2 years. The EEG at the first visit was analyzed by means of Multiscale Entropy (MSE), multifractality (MFA), amplitude integrated EEG features and topological indices of the EEG network. These features were associated with both ASD and abnormal Bayley outcome of the infants using linear discriminant analysis. Results The classification of the ASD patients shows that MFA and MSE had the best discrimination performances, with an area under the ROC curve AUC (MFA) = 0.74 and AUC(MSE) = 0.79 respectively, and kappa scores of Kappa(MFA) = 0.48 and Kappa(MSE) = 0.26. Concerning both abnormal Bayley outcome and ASD, the developmental abnormalities detection shows that entropy and fractal features outperform the other subsets of attributes and the multiclass analysis shows that those features can also discriminate patients with ASD from patients with only developmental abnormalities (Kappa(MFA) = 0.41 and Kappa(MSE) = 0.36). Conclusion Quantitative EEG analysis shows that a dysmature EEG, i.e. a signal with higher fractal regularity and lower entropy, is associated with autism spectrum disorder or abnormal Bayley outcome at 2 years of age.

This article describes the research results aimed at distance education during the Covid-19 pandemic and closing schools and its symmetry with the classical state in terms of time, difficulty, and the mental and physical condition of students. An important aspect is therefore to maintain the symmetry of attitudes to teaching in face-to-face form and distance form. In terms of the eight-year gymnasium in the Czech Republic, students&rsquo, attitudes to the teaching subject informatics were investigated. The main research questions in our study dealt with whether students felt equally balanced regarding the amount of tasks and time taken for home preparation during the Covid-19 outbreak compared with the time before the quarantine and their condition (both mental and physical) during the Covid-19 outbreak. The research was conducted using an anonymous questionnaire, which was answered by 110 out of 180 students. According to the results, it is evident that students felt that during the distance education, there are more tasks compared to face-to-face ones. Students also claimed to spend more time learning at distance education than at school. On the other hand, they agreed that the self-education schedule is suitable for them. In terms of the questionnaire, their condition (both mental and physical) was also evaluated, which was slightly above the average.

AIM We aimed to identify early predictors of intractable epilepsy, intellectual disability (ID) and autism spectrum disorders (ASD) in the cohort of TSC patients initially diagnosed with cardiac rhabdomyomas (CR). METHOD Over the period of twelve years we prospectively obtained clinical, neuropsychological, electrophysiological and neuroimaging data in a group of 22 TSC patients (9 females, 13 males) with the pre/perinatal diagnosis of CR, included to the study at the time of diagnosis. Afterwards, we statistically determined variables associated with ID, ASD and intractable epilepsy. RESULTS Development of ID was predicted by severe epilepsy (a higher number of anti-epileptic drugs used), a higher number of dysplastic lesions on MRI, and abnormal background activity on EEG (p

Chirurgicka lecba epilepsie představuje významnou terapeutickou alternativu u pacientů s farmakorezistentni epilepsii. Klinika dětske neurologie 2. LF UK a FN Motol se v ramci Centra pro epilepsie Motol zabýva chirurgickou lecbou epilepsie u děti od roku 2000. K 1. 11. 2017 zde bylo realizovano 263 výkonů u 232 pacientů. V nasem clanku shrneme zakladni principy epileptochirurgie, předoperacni diagnostiky a představime výsledky epileptochirurgickeho programu dětske casti Centra pro epilepsie Motol vcetně aktualnich trendů. 80,7 % děti v nasem souboru je dva roky po operaci bez zachvatů. Zakladnim předpokladem uspěchu epileptochirurgie zůstava vcasne provedeni výkonu, ktere pro dětske pacienty představuje navic sanci na zachovani jejich kognitivniho potencialu.

OBJECTIVEResective epilepsy surgery is an established treatment method for children with focal intractable epilepsy, but the use of this method introduces the risk of postsurgical motor deficits. Electrical stimulation mapping (ESM), used to define motor areas and pathways, frequently fails in children. The authors developed and tested a novel ESM protocol in children of all age categories.METHODSThe ESM protocol utilizes high-frequency electric cortical stimulation combined with continuous intraoperative motor-evoked potential (MEP) monitoring. The relationships between stimulation current intensity and selected presurgical and surgery-associated variables were analyzed in 66 children (aged 7 months to 18 years) undergoing 70 resective epilepsy surgeries in proximity to the motor cortex or corticospinal tracts.RESULTSESM elicited MEP responses in all children. Stimulation current intensity was associated with patient age at surgery and date of surgery (F value = 6.81, p < 0.001). Increase in stimulation current intensity predicted postsurgical motor deficits (F value = 44.5, p < 0.001) without effects on patient postsurgical seizure freedom (p > 0.05).CONCLUSIONSThe proposed ESM paradigm developed in our center represents a reliable method for preventing and predicting postsurgical motor deficits in all age groups of children. This novel ESM protocol may increase the safety and possibly also the completeness of epilepsy surgery. It could be adopted in pediatric epilepsy surgery centers.