Sudden unexpected death in the young has a devastating impact on families, care providers, and the community, and attracts significant public and media attention. The most common diagnoses that increase risk for sudden death in the young (SDY) include Dravet Syndrome, other genetic forms of epilepsy, hypertrophic cardiomyopathy, coronary artery anomalies of wrong sinus origin, arrhythmogenic right ventricular cardiomyopathy, and ion channelopathies. In up to 30% of cases of sudden cardiac death in the young (SCD), no specific diagnosis is found (autopsy negative). SCD has been documented at all ages (approximately 10-15% of sudden infant death syndrome cases may be due to ion channelopathies) and may be associated with competitive athletics. Similarly, sudden unexpected death in epilepsy (SUDEP) has been documented at all ages, and several risk factors have been identified (some cases of SUDEP may be due to ion channelopathies). Noncompliance of anticonvulsant medication usage and polytherapy in those with refractory epilepsy are also risk factors of SUDEP. SDY is a critical public health and scientific issue. A young life suddenly cut short represents many lost productive years. Further, there is significant scientific disagreement, fueled by lack of evidence, about the best approach to prevent SDY. Some experts support the implementation of cardiovascular (i.e. electrocardiogram) screening programs in infants, athletes, those diagnosed with epilepsy, or all children, to identify at-risk individuals and prevent SCD and SUDEP. However, rational policy development in the area of screening and prevention is currently limited by the lack of prospectively defined epidemiological data, including incidence rates and etiology for SDY. Creation of a registry for Sudden Death in the Young represents the first prospective, comprehensive evaluation of SCD and SUDEP in the United States. It will provide the opportunity to estimate incidence more precisely than any previous study and to establish an infrastructure for future expanded research use. The Centers for Disease Control currently oversees the Sudden Unexpected Infant Death (SUID) Case Registry, a state-level surveillance system for deaths involving individuals from birth to one year of age. This system includes data from death certificates, child death review, medical records, death scene investigations, and pathology reports. The Registry enhances the activities of the National Center for Child Death Review and utilizes their case reporting system, but with a standardized reporting form specific to SUID. A pilot program began in 2009, and now includes seven states (Colorado, Georgia, Michigan, Minnesota, New Hampshire, New Jersey and New Mexico). The purpose of this Memo of Understanding is to expand the current efforts of the CDC?s SUID Case Registry in the number of states funded, the age range of cases collected, and the type of data collected. Specifically, NINDS support is for the expansion of the case ascertainment to include cases of SUDEP in individuals under the age of 24 in up to 15 states.
