A 20-month-old girl with a complex chromosomal disorder had first presentation of febrile status epilepticus and was admitted to the hospital. Two days after her initial seizure, she died suddenly and unexpectedly during a video EEG monitoring study. An advanced analysis of the physiologic changes in the hours and minutes leading up to death was undertaken. The electrocardiography over the last 19 minutes of life was reviewed, and the R-R intervals were manually measured. Heart rate variability was assessed through calculation of the SD of the R-R intervals and the root mean square of successive differences over successive 100 beat periods. Instantaneous heart rate, SD of the R-R intervals, the root mean square of successive differences, and oxygen saturation were plotted against time over the last 19 minutes of life. Diffuse cerebral suppression on EEG was observed 10 minutes before death, followed minutes later by severe bradycardia and increased heart rate variability. Although the child did not meet criteria for a diagnosis of epilepsy, the sequence of physiologic changes leading up to death suggests a pathophysiology similar to sudden unexplained death in epilepsy. A comparable pattern of diffuse cerebral suppression preceding parasympathetic overactivity has been suggested in some rare cases of adults who have experienced sudden unexplained death in epilepsy during video EEG monitoring.
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