SUDEP in the North American SUDEP Registry

Abstract

ObjectiveTo obtain medical records, family interviews, and death-related reports of sudden unexpected death in epilepsy (SUDEP) cases to better understand SUDEP.MethodsAll cases referred to the North American SUDEP Registry (NASR) between October 2011 and June 2018 were reviewed; cause of death was determined by consensus review. Available medical records, death scene investigation reports, autopsy reports, and next-of-kin interviews were reviewed for all cases of SUDEP. Seizure type, EEG, MRI, and SUDEP classification were adjudicated by 2 epileptologists.ResultsThere were 237 definite and probable cases of SUDEP among 530 NASR participants. SUDEP decedents had a median age of 26 (range 1–70) years at death, and 38% were female. In 143 with sufficient information, 40% had generalized and 60% had focal epilepsy. SUDEP affected the full spectrum of epilepsies, from benign epilepsy with centrotemporal spikes (n = 3, 1%) to intractable epileptic encephalopathies (n = 27, 11%). Most (93%) SUDEPs were unwitnessed; 70% occurred during apparent sleep; and 69% of patients were prone. Only 37% of cases of SUDEP took their last dose of antiseizure medications (ASMs). Reported lifetime generalized tonic-clonic seizures (GTCS) were <10 in 33% and 0 in 4%.ConclusionsNASR participants commonly have clinical features that have been previously been associated with SUDEP risk such as young adult age, ASM nonadherence, and frequent GTCS. However, a sizeable minority of SUDEP occurred in patients thought to be treatment responsive or to have benign epilepsies. These results emphasize the importance of SUDEP education across the spectrum of epilepsy severities. We aim to make NASR data and biospecimens available for researchers to advance SUDEP understanding and prevention.

A human amygdala site that inhibits respiration and elicits apnea in pediatric epilepsy

“…who have never had a GTCS (6)(7)(8). To prevent such devastating outcomes, it will be critical to identify the mechanisms underlying SUDEP in children as well as adults and in various types of epilepsy.…”

Genetic generalized and focal epilepsy prevalence in the North American SUDEP Registry

“…In investigative death reporting, slightly more decedents with GGE were discovered with evidence of an unwitnessed recent GTCS, based on a tongue bite or blood on pillow, urinary incontinence, and unusual body positioning (i.e., falling out of bed). This, in conjunction with the many varied groups and presentations of SUDEP, 22 may indicate that each epilepsy subgroup has different risk factors or ultimate SUDEP mechanism.…”

“…Standard protocol approvals, registrations, and patient consents Full NASR intake and interview methodology was reported, 22 and all NOK of participants provided informed consent to participate in this study. This study was approved by the New York University Langone Medical Center Institutional review board.…”

Proteomic and Transcriptomic Analyses of the Hippocampus and Cortex in SUDEP and High-Risk SUDEP Cases

“…Human Brain Tissue for Proteomics: Post-mortem brain tissue from epilepsy patients who died from SUDEP or other causes was obtained through the North American SUDEP Registry (NASR) with approval by the New York University School of Medicine Institutional Review Board (IRB). Causes of death were classified (OD, DF) into non-SUDEP epilepsy and SUDEP (definite SUDEP, definite SUDEP plus, and probable SUDEP) (4,5). Lifetime GTCS history was determined from interviews and medical records, representing the best estimate for each case and as described previously (5).…”

“…Studies on SUDEP epidemiology, risk factors, mechanisms, and prevention have advanced our understanding, although detailed pathophysiological understanding remains limited (5)(6)(7). After a GTCS, prolonged (i.e.…”