Epileptiform discharges in the context of self-limited pediatric focal epilepsy (EDSelFEC) in pediatric hemispherotomy patients: Role of white matter abnormalities.

Objective: To investigate the frequency of epileptiform discharges associated with self-limited focal epilepsy (EDSelFEC) in children who have undergone a hemispherotomy and to evaluate whether patients with coexistence of EDSelFEC and structural hemispheric epilepsies differ from patients without coexistence of EDSelFEC and whether there are differences between the two groups with regard to preoperative management and postoperative outcome.

Methods: Data on 131 children who underwent a hemispherotomy between January 1999 and January 2015 were retrieved from the Epilepsy center's epilepsy surgery database. Children with EDSelFEC were compared with children without EDSelFEC with respect to epileptogenic hemispheric pathology, family history, age at epilepsy onset, timing of surgery, lesion laterality, preoperative cognitive function, response to sodium channel blocker antiepileptic medication, and surgical outcome.

Subacute Degeneration of Fibers After Vertical Parasagittal Hemispherotomy.

Purpose: After vertical parasagittal hemispherotomy a restricted diffusion is often seen ipsilaterally and even distant from the adjacent resection margin. This retrospective cohort study analyses the anatomic site and the time course of the diffusion restriction after vertical parasagittal hemispherotomy.

Methods: Fifty-nine patients were included into this study, all of them having had one pre-operative and at least one post-operative MRI, including diffusion imaging at b‑values of 0 and 1000 s/mm with a calculated ADC.

Outcome of Epilepsy Surgery in MRI-Negative Patients Without Histopathologic Abnormalities in the Resected Tissue.

Background And Objective: Patients with presumed nonlesional focal epilepsy-based on either MRI or histopathologic findings-have a lower success rate of epilepsy surgery compared with lesional patients. In this study, we aimed to characterize a large group of patients with focal epilepsy who underwent epilepsy surgery despite a normal MRI and had no lesion on histopathology. Determinants of their postoperative seizure outcomes were further studied.

Brain expression profiles of two antisense RNAs in children and adolescents with epilepsy.

Objective: Heterozygous mutations within the voltage-gated sodium channel α subunit () are responsible for the majority of cases of Dravet syndrome (DS), a severe developmental and epileptic encephalopathy. Development of novel therapeutic approaches is mandatory in order to directly target the molecular consequences of the genetic defect. The aim of the present study was to investigate whether cis-acting long non-coding RNAs (lncRNAs) of are expressed in brain specimens of children and adolescent with epilepsy as these molecules comprise possible targets for precision-based therapy approaches.

Ganglioglioma with adverse clinical outcome and atypical histopathological features were defined by alterations in PTPN11/KRAS/NF1 and other RAS-/MAP-Kinase pathway genes.

Exome-wide sequencing studies recently described PTPN11 as a novel brain somatic epilepsy gene. In contrast, germline mutations of PTPN11 are known to cause Noonan syndrome, a multisystem disorder characterized by abnormal facial features, developmental delay, and sporadically, also brain tumors. Herein, we performed a deep phenotype-genotype analysis of a comprehensive series of ganglioglioma (GG) with brain somatic alterations of the PTPN11/KRAS/NF1 genes compared to GG with common MAP-Kinase signaling pathway alterations, i.

DNA methylation-based classification of malformations of cortical development in the human brain.

Malformations of cortical development (MCD) comprise a broad spectrum of structural brain lesions frequently associated with epilepsy. Disease definition and diagnosis remain challenging and are often prone to arbitrary judgment. Molecular classification of histopathological entities may help rationalize the diagnostic process.

Multilobar unilateral hypoplasia with emphasis on the posterior quadrant and severe epilepsy in children with FCD ILAE Type 1A.

Objective: Focal cortical dysplasia (FCD) Type 1 and its three subtypes have yet not been fully characterized at the clinical, anatomopathological, and molecular level (International League Against Epilepsy [ILAE] FCD classification from 2011). We aimed to describe the clinical phenotype of patients with histopathologically confirmed FCD1A obtained from a single epilepsy center between 2002 and 2016.

Methods: Medical records were retrieved from the hospital's archive.

[Hemispherotomy in pediatric epilepsy surgery-Surgical, epileptological and functional aspects].

Hemispherotomies represent a major part of surgical interventions for epilepsy in childhood (16-21%). The anatomical resection has been replaced by minimally invasive disconnection techniques with lower perioperative mortality and fewer postoperative complications. Today the procedure is not only carried out from the lateral aspect via the Sylvian fissure/insula but also via a vertical parasagittal approach.

Efficacy and mid-term outcome of middle meningeal artery embolization with or without burr hole evacuation for chronic subdural hematoma compared with burr hole evacuation alone.

Background: Chronic subdural hematoma (CSDH) is a common neurosurgical condition with high recurrence rates. Repeated microbleedings from fragile neo-vessels supplied by peripheral branches of the middle meningeal artery (MMA) are believed to be responsible for the growth and recurrence of CSDH. Thus, MMA embolization might be a promising method to prevent re-bleedings and recurrences.

Deep Brain Stimulation in KMT2B-Related Dystonia: Case Report and Review of the Literature With Special Emphasis on Dysarthria and Speech.

KMT2B-related dystonia is a progressive childhood-onset movement disorder, evolving from lower-limb focal dystonia into generalized dystonia. With increasing age, children frequently show prominent laryngeal or facial dystonia manifesting in dysarthria. Bilateral deep brain stimulation of the globus pallidus internus (GPi-DBS) is reported to be an efficient therapeutic option.

Dysmorphic neurons as cellular source for phase-amplitude coupling in Focal Cortical Dysplasia Type II.

Objective: Reliable localization of the epileptogenic zone is necessary for successful epilepsy surgery. Neurophysiological biomarkers include ictal onsets and interictal spikes. Furthermore, the epileptic network shows oscillations with potential localization value and pathomechanistic implications.

Network for Therapy in Rare Epilepsies (NETRE): Lessons From the Past 15 Years.

In 2005, twork for herapy in are pilepsies (NETRE)-was initiated in order to share treatment experiences among clinicians in patients with rare epilepsies. Here we describe the structure of the rapidly growing NETRE and summarize some of the findings of the last 15 years. NETRE is organized in distinct groups (currently >270).

Sacrificing one visual hemifield during pediatric epilepsy surgery: Effects on visual search.

Objectives: To investigate early and late effects of planned surgically acquired homonymous hemianopias on visual search in children and adolescents.

Methods: This prospective study included five patients (5y 5 m-18y 0 m; 2 girls) with pharmaco-refractory epilepsies in whom one visual hemifield was sacrificed as part of the surgical strategy, and, as controls, seven patients (5y 11 m-18y 0 m; 6 girls) undergoing epilepsy surgeries not affecting the visual fields. Visual search was studied using the "Table Test", which is an everyday life-like visual search test.

Correlates of intellectual development before and after hemispherotomy: an analysis of 75 children and adolescents.

This study describes the intellectual development of 75 children and adolescents who underwent hemispherotomy. Furthermore, we aimed to reveal predicting factors on pre- and postsurgical development with a focus on the role of aetiology. We analysed presurgical and six-month postsurgical developmental and intellectual data of 75 patients (age range: 0.

Presurgical Language fMRI in Children, Adolescents and Young Adults : A Validation Study.

Purpose: To validate four established, child-friendly functional magnetic resonance imaging (fMRI) language tasks (word chain task [WCT], vowel identification task [VIT], synonym task [SYT] and beep story task [BST]) in a predominantly pediatric cohort.

Methods: Intracarotid amobarbital procedures (IAP) (n = 17) and unchanged language after hemispherotomy (n = 6) were used as gold standards. The fMRI activations of nine regions of interest (ROI) in the frontal, temporal and parietal lobes as well as in the cerebellum were visually assessed in 23 fMRI examinations (in total 117 fMRI task sessions) of 23 patients (age range 10.

Uncovering homonymous visual field defects in candidates for pediatric epilepsy surgery.

Objectives: Perimetry in children can be challenging due to low cooperation and short attention span. Especially during the pre-surgical work-up of children with pharmaco-refractory epilepsies, however, diagnosing homonymous visual field defects (HVFDs) can be crucial for planning surgical strategies. Here, we evaluated "campimetry" for visual field testing in children.

Genomic DNA methylation distinguishes subtypes of human focal cortical dysplasia.

Objectives: Focal cortical dysplasia (FCD) is a major cause of drug-resistant focal epilepsy in children, and the clinicopathological classification remains a challenging issue in daily practice. With the recent progress in DNA methylation-based classification of human brain tumors we examined whether genomic DNA methylation and gene expression analysis can be used to also distinguish human FCD subtypes.

Methods: DNA methylomes and transcriptomes were generated from massive parallel sequencing in 15 surgical FCD specimens, matched with 5 epilepsy and 6 nonepilepsy controls.

Electrode placement accuracy in robot-assisted epilepsy surgery: A comparison of different referencing techniques including frame-based CT versus facial laser scan based on CT or MRI.

Background: Precise robotic or stereotactic implantation of stereoelectroencephalography (sEEG) electrodes relies on the exact referencing of the planning images in order to match the patient's anatomy to the stereotactic device or robot. We compared the accuracy of sEEG electrode implantation with stereotactic frame versus laser scanning of the face based on computed tomography (CT) or magnetic resonance imaging (MRI) datasets for referencing.

Methods: The accuracy was determined by calculating the Euclidian distance between the planned trajectory and the postoperative position of the sEEG electrode, defining the entry point error (EPE) and the target point error (TPE).

Neurologic phenotypes associated with / mutations: Expanding the spectrum of disease.

Objective: To characterize the neurologic phenotypes associated with mutations and to seek genotype-phenotype correlation.

Methods: We analyzed clinical, EEG, and neuroimaging data of 44 new and 55 previously reported patients with mutations.

Results: Childhood-onset focal seizures, frequently complicated by status epilepticus and resistance to antiepileptic drugs, was the most common phenotype.

Mesial Temporal Sclerosis in SCN1A-Related Epilepsy: Two Long-Term EEG Case Studies.

Patients with temporal lobe epilepsy (TLE) due to mesial temporal sclerosis (MTS) are eligible candidates for resective epilepsy surgery. We report on 2 male patients aged 4 years with suspected TLE due to MTS who were referred for presurgical evaluation. Both patients came to medical attention within the first year of life suffering from febrile status epileptici and subsequent unprovoked seizures.

Age-related MR characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE).

Background: Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a newly described, rare histopathologic entity detected in resected brain tissue of patients with refractory epilepsies. It shows a predominantly frontal localization causing a difficult-to-treat epilepsy with onset usually in early childhood. Histologically, MOGHE is characterized by blurred gray-white-matter boundaries with increased numbers of heterotopic neurons in the subcortical white matter and increased density of oligodendroglia.

Histopathological Findings in Brain Tissue Obtained during Epilepsy Surgery.

Background: Detailed neuropathological information on the structural brain lesions underlying seizures is valuable for understanding drug-resistant focal epilepsy.

Methods: We report the diagnoses made on the basis of resected brain specimens from 9523 patients who underwent epilepsy surgery for drug-resistant seizures in 36 centers from 12 European countries over 25 years. Histopathological diagnoses were determined through examination of the specimens in local hospitals (41%) or at the German Neuropathology Reference Center for Epilepsy Surgery (59%).

Predicting hand function after hemidisconnection.

Hemidisconnections (i.e. hemispherectomies or hemispherotomies) invariably lead to contralateral hemiparesis.