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Sudden unexpected death in epilepsy
Published in Helen Whitwell, Christopher Milroy, Daniel du Plessis, Forensic Neuropathology, 2021
Christopher Milroy, Daniel du Plessis
Sudden unexplained death in childhood (SUDC) is defined as the sudden and unexpected death in a child older than 1 year that remains unexplained after a complete autopsy, death scene investigation and ancillary testing (Kinney et al. 2012). Population-based studies subsequently reported a significant association amongst febrile seizures, hippocampal maldevelopment and sudden death. This led to the proposal that this SUDC subset represented a new entity, termed ‘hippocampal maldevelopment associated with sudden death’ (HMASD). HMASD was subsequently defined as focal granular cell bilamination (FGCB) in the dentate gyrus (Figure 13.3) with or without hippocampal asymmetry and/or malrotation (Kinney et al. 2009). Cases of a personal and/or family history of febrile seizures, but without HMASD, were classified as sudden unexplained death in childhood with febrile seizure phenotype (SUDC-FS) and the remaining cases, without HMASD, febrile seizures or an explained cause of death were classified as SUDC (Kinney et al. 2012). The explained group consisted of children in whom known/explained causes of sudden death were found such as infections or cardiac abnormalities (Hefti et al. 2016).
Congenital Central Hypoventilation Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
LO-CCHS is suspect when an older individual (>1 month of age, late childhood, or adulthood) meets the criteria above for the newborn or shows apparent life-threatening events and cyanosis during sleep, recurrent severe pulmonic infections with related hypoventilation, unexplained seizures, respiratory depression after antiseizure medication, sedation, or anesthesia, unexplained neurocognitive delay with history of prior cyanosis, unexplained nocturnal hypercarbia and hypoxemia, unresolved central alveolar hypoventilation after treatment for obstructive sleep apnea, unresponsiveness to hypercarbia or hypoxemia (prolonged underwater swimming), pneumonia, sudden and unexpected death (sudden infant death syndrome [SIDS] or sudden unexplained death of childhood [SUDC], especially if there is a family history of CCHS).
Sudden unexpected death in epilepsy: ongoing challenges in finding mechanisms and prevention
Published in International Journal of Neuroscience, 2018
Doungporn Ruthirago, Parunyou Julayanont, Amputch Karukote, Mohamed Shehabeldin, Kenneth Nugent
In children, SUDEP has several similar characteristics with sudden infant death syndrome (SIDS) and sudden unexplained death in childhood (SUDC). All three conditions often occur during sleep in male patients found lying in prone position, and routine autopsies do not reveal other causes of death. Hypothetical mechanisms of SIDS involve cardiac, respiratory and arousal system dysfunction, and there is evidence for correlation with defects in the serotonin system, which are similar to SUDEP [11]. Some cases diagnosed as SIDS and SUDC may actually be SUDEP without a prior diagnosis of epilepsy. In adult and geriatric populations, without investigation, SUDEP may be incorrectly called sudden cardiac death, cardiorespiratory failure or other more common conditions. These unclear boundaries cause difficulties in epidemiological studies of SUDEP.