The SRA collaborative network brings together individually unique projects linked by a common goal of rapid translation of research discoveries into clinical practice of SUDEP diagnosis, risk detection and prevention. Until now, there has not been an organized clinical network specifically focused on individuals at high risk for SUDEP. Moreover, there is a lack of unified clinical monitoring algorithms that will stratify SUDEP risk i patient population, allow in-depth correlative analysis with brain morphometric studies and genetic investigations, and inform model systems of premature epilepsy mortality. We therefore propose a Clinical Network Core (SRA-CNC) that consists of four principal components: (1) a Pediatric Clinical Network (PCN) aimed at recruitment, physiological monitoring, and sample collection in children affected by Dravet syndrome, (2) an Adult Clinical Network (ACN) focused on high SUDEP risk adult patients based on the combined phenotype of epilepsy, cardiac arrhythmias and respiratory dysfunction, (3) Analysis Center entails physiological, morphometric, and epidemiological evaluations of the human data, and (4) Data integration and storage. The SRA-CNC will perform four main functions;(1) It will establish infrastructure, logistical and regulatory support and implement standard operating procedures for case ascertainment, scope and mode of physiological monitoring and data collection, and procurement of biological specimens;(2) it will provide problem focused expert analysis of the (i) clinical and epidemiological data, (ii) ictal, cardiac, and respiratory physiology, and (iii) imaging based morphometry;(3) It will optimize resource utilization and facilitate data exchange, analysis, and communication among participating centers;and (4) SRA-CNC will translate investigational procedures into clinical practice in the form of public education and evidence driven recommendations. The Clinical Network Core is an essential component of the proposed SUDEP Center Without Wall piloted by the investigators of SUDEP Research Alliance as it will inform and facilitate current and future research translational projects in humans and i the individualized model systems.
The SRA-Clinical Network Core is the first expert driven network unifying comprehensive clinical, physiological, and structural analysis of a large population of pediatric and adult epilepsy patients at high SUDEP risk. It is designed for reciprocal support of patient centered research in SUDEP genetic (Project 1), physiologic (Project 2), cellular (Project3), and model biological (Project 4) mechanisms
|Goldman, Alica M; Behr, Elijah R; Semsarian, Christopher et al. (2016) Sudden unexpected death in epilepsy genetics: Molecular diagnostics and prevention. Epilepsia 57 Suppl 1:17-25|
|Lhatoo, Samden; Noebels, Jeffrey; Whittemore, Vicky et al. (2015) Sudden unexpected death in epilepsy: Identifying risk and preventing mortality. Epilepsia 56:1700-6|
|Goldman, Alica M (2015) Mechanisms of sudden unexplained death in epilepsy. Curr Opin Neurol 28:166-74|