Effect of Genetic Defects in a Cortical Circuit Model Associated with Childhood Absence Epilepsy

2021 ◽  
pp. 477-487
Author(s):  
Maliha Ahmed ◽  
Sue Ann Campbell
Author(s):  
Anita N. Datta ◽  
Laura Wallbank ◽  
Peter K. H. Wong

AbstractObjectiveBenign epilepsy of childhood with central temporal spikes (BECTS) and absence epilepsy are common epilepsy syndromes in children with similar age of onset and favorable prognosis. However, the co-existence of the electrocardiogram (EEG) findings of rolandic spike and 3 Hz generalized spike-wave (GSW) discharges is extremely rare, with few cases reported in the literature. Our objective was to characterize the EEG findings of these syndromes in children in our center and review the electro-clinical features.MethodsAll EEGs at BC Children’s Hospital are entered in a database, which include EEG findings and clinical data. Patients with both centro-temporal spikes and 3 Hz GSW discharges were identified from the database and clinical data were reviewed.ResultsAmong the 43,061 patients in the database from 1992 to 2017, 1426 with isolated rolandic discharges and 528 patients with isolated 3 Hz GSW discharges were identified, and 20 (0.05%) patients had both findings: 3/20 had BECTS, and subsequently developed childhood absence epilepsy and 17/20 had no seizures characteristic for BECTS. At follow-up, 17 (85%) were seizure-free, 1 (5%) had rare, and 2 (10%) had frequent seizures.ConclusionsThis is the largest reported group of patients to our knowledge with the co-existence of rolandic and 3 Hz GSW discharges on EEGs in one institution, not drug-induced. As the presence of both findings is extremely rare, distinct pathophysiological mechanisms are likely. The majority had excellent seizure control at follow-up, similar to what would be expected for each type of epilepsy alone.


2010 ◽  
Vol 14 (1) ◽  
pp. 93-96 ◽  
Author(s):  
Alberto Verrotti ◽  
Giovanna Di Marco ◽  
Rosanna la Torre ◽  
Francesco Chiarelli

2017 ◽  
Vol 21 (3) ◽  
pp. 570-575 ◽  
Author(s):  
Alberto Verrotti ◽  
Sara Casciato ◽  
Alberto Spalice ◽  
Marco Carotenuto ◽  
Pasquale Striano ◽  
...  

2004 ◽  
Vol 279 (11) ◽  
pp. 9681-9684 ◽  
Author(s):  
Houman Khosravani ◽  
Christophe Altier ◽  
Brett Simms ◽  
Kevin S. Hamming ◽  
Terrance P. Snutch ◽  
...  

2017 ◽  
Author(s):  
Marwan Abdellah ◽  
Juan Hernando ◽  
Nicolas Antille ◽  
Stefan Eilemann ◽  
Henry Markram ◽  
...  

AbstractBackground We present a software workflow capable of building large scale, highly detailed and realistic volumetric models of neocortical circuits from the morphological skeletons of their digitally reconstructed neurons. The limitations of the existing approaches for creating those models are explained, and then, a multi-stage pipeline is discussed to overcome those limitations. Starting from the neuronal morphologies, we create smooth piecewise watertight polygonal models that can be efficiently utilized to synthesize continuous and plausible volumetric models of the neurons with solid voxelization. The somata of the neurons are reconstructed on a physically-plausible basis relying on the physics engine in Blender.Results Our pipeline is applied to create 55 exemplar neurons representing the various morphological types that are reconstructed from the somatsensory cortex of a juvenile rat. The pipeline is then used to reconstruct a volumetric slice of a cortical circuit model that contains ∼210,000 neurons. The applicability of our pipeline to create highly realistic volumetric models of neocortical circuits is demonstrated with an in silico imaging experiment that simulates tissue visualization with brightfield microscopy. The results were evaluated with a group of domain experts to address their demands and also to extend the workflow based on their feedback.Conclusion A systematic workflow is presented to create large scale synthetic tissue models of the neocortical circuitry. This workflow is fundamental to enlarge the scale of in silico neuroscientific optical experiments from several tens of cubic micrometers to a few cubic millimeters.


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