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Sudden Death After Febrile Seizure Case Report: Cerebral Suppression Precedes Severe Bradycardia Kenneth A

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Sudden Death After Febrile Seizure Case Report: Cerebral Suppression Precedes Severe Bradycardia Kenneth A Sudden Death After Febrile Seizure CaseKenneth A. Myers, Report: MD, PhD, a, b Robyn E. McPherson, Cerebral MD, MSc, b Robin Clegg,Suppression MD,c Jeffrey Buchhalter, MD, PhD b Precedes Severe Bradycardiaabstract 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 aEpilepsy Research Centre, Austin Health, University of intervals were manually measured.‍ Heart rate variability was assessed through Melbourne, Melbourne, Victoria, Australia; and bDivision calculation of the SD of the R-R intervals and the root mean square of successive of Neurology and cSection of Cardiology, Department of Pediatrics, Alberta Children’s Hospital, University of differences over successive 100 beat periods.‍ Instantaneous heart rate, SD of Calgary, Calgary, Alberta, Canada 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 Dr Myers conceived the study, collected the data, prepared figures, and drafted the initial cerebral suppression on EEG was observed 10 minutes before death, followed manuscript; Dr McPherson assisted with data minutes later by severe bradycardia and increased heart rate variability.‍ collection and edited the manuscript; Dr Clegg Although the child did not meet criteria for a diagnosis of epilepsy, the sequence reviewed the electrocardiography recording and the patient’s cardiac history and edited the of physiologic changes leading up to death suggests a pathophysiology similar to manuscript; Dr Buchhalter reviewed the EEG sudden unexplained death in epilepsy.‍ A comparable pattern of diffuse cerebral recording and edited the manuscript; and all suppression preceding parasympathetic overactivity has been suggested in authors approved the final manuscript as submitted and agree to be accountable for all aspects of the some rare cases of adults who have experienced sudden unexplained death in work. epilepsy during video EEG monitoring.‍ DOI: https:// doi. org/ 10. 1542/ peds. 2016­ 2051 Accepted for publication Feb 23, 2017 Address correspondence to Kenneth A. Myers, MD, PhD, Epilepsy Research Centre, Melbourne Brain Febrile status epilepticus is defined 3reported in children than in adults, as a febrile seizure or series of febrile potentially because many adult risk Centre, Austin Health, 245 Burgundy St, Heidelberg, ≥ VIC 3084, Australia. E­mail: [email protected] seizures without intervening1 recovery factors are less relevant for young lasting 30 minutes.‍ Although children (eg, alcohol use, living alone, PEDIATRICS (ISSN Numbers: Print, 0031­4005; Online, febrile status is a medical emergency, poor adherence to medication).‍ 1098­4275). the mortality risk seems low.‍ The Copyright © 2017 by the American Academy of The current article presents the case of Pediatrics FEBSTAT (Consequences of Prolonged a young girl who died suddenly during FINANCIAL DISCLOSURE: The authors have Febrile Seizures in Childhood) study video EEG monitoring (VEM), days longitudinally followed up 119 children indicated they have no financial relationships after her first presentation with febrile relevant to this article to disclose. with febrile status epilepticus and status epilepticus.‍ We conducted an FUNDING: No external funding. reported 2 deaths, neither of 2which was advanced analysis of the physiologic related to prolonged seizure.‍ POTENTIAL CONFLICT OF INTEREST: The authors changes occurring in the hours and have indicated they have no potential conflicts of Death after a seizure is an important minutes before death.‍ interest to disclose. issue, as individuals with epilepsy have METHODS an increased risk of sudden death.‍ This To cite: Myers KA, McPherson RE, Clegg R, et al. phenomenon (ie, sudden unexpected Case Report Sudden Death After Febrile Seizure Case Report: death in epilepsy [SUDEP]) is a rare Cerebral Suppression Precedes Severe Brady­ ’ but tragic occurrence, with devastating cardia. Pediatrics. 2017;140(5):e20162051 effects on the individual s family A 20-month-old girl with a complicated and friends.‍ SUDEP is less commonly medical history underwent prolonged Downloaded from www.aappublications.org/news by guest on September 27, 2021 PEDIATRICS Volume 140, number 5, November 2017:e20162051 CASE REPORT Myers et al 2017 ROUGH GALLEY PROOF Sudden Death After Febrile Seizure Case https://doi.‍org/10.‍1542/peds.‍2016-2051 November 2017 Report: Cerebral Suppression Precedes Severe Bradycardia 5 140 Pediatrics RESULTS VEM during an admission after first girl was administered 2 L of 100% EEG presentation of convulsive febrile oxygen by nasal prongs and the status epilepticus’ .‍ dextrose infusion; serum electrolyte levels were normal that morning, For the majority of the VEM, the EEG The child s medical history was and glucose levels were normal showed diffuse, high-amplitude delta significant for a chromosomal throughout the day.‍ At 6.‍5 hours activity, with relative attenuation disorder involving distal monosomy into the recording, Code Blue was over the left hemisphere, consistent 9p, proximal monosomy 15q and – × called for cessation of breathing.‍ with a diffuse encephalopathy.‍ 16p13.‍11 duplication (arr(hg19) – Cardiopulmonary resuscitation was Frequent multifocal spikes were seen 9p24.‍3p23(204090 11371865) 1, ’ × briefly initiated but then stopped from the left frontal, right posterior 15q11.‍1q14(20686203 – per the family s wishes; the girl temporal, and left anterior temporal 39443222) 1, × subsequently died.‍ regions, suggesting a predisposition 16p13.‍11(14910213 Electrophysiologic Analysis for focal seizures from these regions.‍ 16194575) 3) and associated Occasional periods of diffuse dysmorphic features, bilateral attenuation lasting 2 to 5 seconds cleft palate, short webbed neck, During the VEM, electrocardiography were observed (Fig 1A), although congenital heart disease (mild (ECG) (lead I) and oxygen saturation the recording did not resemble burst valvular pulmonary stenosis and (finger sensor) were monitored.‍ suppression or hypsarrhythmia, trivial patent ductus arteriosus), and The EEG recording was reviewed patterns seen in some developmental severe bilateral bronchomalacia.‍ and the ECG tracing examined beat- epileptic encephalopathies.‍ She had a difficult medical by-beat, with each R-R interval course, experiencing respiratory manually measured and recorded No clinical or electrographic seizures insufficiency requiring ventilator over the 19 minutes leading up to were observed during 6.‍5 hours support, chronic hypoglycemia, and terminal asystole.‍ Instantaneous of VEM before death.‍ Ten minutes frequent infections, all of which heart rate (1/R-R interval) was before terminal asystole (T-10 had led to numerous prolonged calculated, as well as 2 heart rate minutes), there was an abrupt onset hospitalizations.‍ Neurologic variability measures (SD of the R-R of diffuse attenuation, which did not abnormalities included hypotonia interval [SDNN] and the root mean significantly improve (Fig 1B).‍ from birth and global developmental square of successive differences ECG delay.‍ An MRI of the brain as a [RMSSD]).‍ Increases in these heart neonate showed a simplified gyral rate variability (HRV) measures pattern, hypo-opercularization of were considered to represent a Single-lead ECG showed sinus rhythm the temporal lobes, and hypoplastic for the first 6 hours of recording, ’ shift toward parasympathetic over4 olfactory bulbs and tracts.‍ In sympathetic autonomic activity.‍ including up to the time of diffuse discussion with the child s family and Changes over time of SDNN and EEG suppression at T-10 minutes palliative care, a decision was made RMSSD were estimated by calculating (Fig 2A).‍ not to intubate or perform chest values for the 100 R-R intervals At T-8 minutes, profound sinus compressions in the event of acute surrounding each beat (50 intervals bradycardia was seen with deterioration.‍ before and 50 intervals after each ventricular escape beats (Fig 2B).‍ beat).‍ These values were plotted While in respite care, at 20 At T-7 minutes, ongoing ventricular against time, along with oxygen months of age, the child had her escape rhythm was established (Fig saturation values at 30-second first seizure (convulsive febrile 2C).‍ Sinus bradycardia was again intervals.‍ status epilepticus lasting 45 observed by T-4 minutes (Fig 2D).‍ minutes) and was admitted to the We did not measure PR, QRS, or QT The terminal rhythm was sinus hospital for treatment of vomiting intervals because such measurements bradycardia progressing to asystole.‍ and diarrhea associated with a differ when calculated from standard Heart rate was steady at 120 to 135 rotavirus infection.‍ A 50-minute 12-lead ECGs compared with single- beats/min until T-8 minutes, after 5 ∼ seizure occurred the next day, lead ECG recordings.‍ We attempted which the heart rate decreased and associated with hypoglycemia to assess the respiratory rate because plateaued at 70 beats/min (the (serum glucose level, 1.‍8 mmol/L), premorbid breathing abnormalities ventricular escape rate) (Fig 3).‍ and levetiracetam treatment was have been described6, 7 in previous SDNN
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