Abstract
Epidemiologic data suggest that SIDS is related to the sleep state, but exiguous literature has addressed infants who had been awake at the time of sudden catastrophic deterioration and subsequent death. The aims of this study are to: (1) Report five infants who were awake at the onset of the lethal event, and (2) Discuss potential lethal pathophysiological events that may lead to these circumstances. The demographic and pathologic profiles of these cases are similar to SIDS. Altered responses to severe hypotension, bradycardia, and apnea, perhaps elicited by aspiration and mediated by cerebellar and vestibular structures, might be involved in the pathogenesis of these deaths. Comprehensive medical history review, investigation of the circumstances of death, thorough postmortem examination with ancillary studies, and preservation of tissues for gene testing, are crucial to explaining these deaths. Careful attention should be given to the awake or sleep state immediately prior to the sudden clinical collapse, and death of infants; those who were awake should be reported to enhance understanding of this phenomenon.
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Froggatt P, Lynas M, MacKenzie G. Epidemiology of sudden unexpected death in infants (‘cot death’) in Northern Ireland. Br J Preventive Social Med 1971;25:119–34.
Beckwith JB. The sudden infant death syndrome. Curr Probl Pediatr 1973;3:1–36.
Krous HF, Beckwith JB, Byard RW, et al. Sudden infant death syndrome and unclassified sudden infant deaths: a definitional and diagnostic approach. Pediatrics 2004;114:234–8.
Coppoletta MM, Wolbach SB. Body length and organ weights of infants and children. Am J Pathol 1933;9:55–9.
Scholz DG, Kitzman DW, Hagen PT, Istrup DM, Edwards WD. Age-related changes in normal human hearts during the first 10 decades of life. Part I (Growth): a quantitative anatomic study of 200 specimens from subjects from birth to 19 years old. Mayo Clin Proc 1988;63:126–36.
Krous HF, Chadwick AE, Stanley C, Beckwith JB. Is SIDS associated with sleep? A report of six cases demonstrating difficulty in this determination. Forensic Sci Med Path 2005;1:179–85.
Krous HF, Haas EA, Masoumi H, Chadwick AE, Stanley C. A comparison of pulmonary intra-alveolar hemorrhage in cases of sudden infant death due to SIDS in a safe sleep environment or to suffocation. Forensic Sci Int 2007;172:56–62.
Hauck FR. Changing epidemiology. In: Byard RW, Krous HF, editors. Sudden infant death syndrome: problems, progress & possibilities. London: Arnold; 2001. pp. 31–57.
Blair PS, Fleming PJ, Bensley D, et al. Smoking and the sudden infant death syndrome: results from 1993–5 case–control study for confidential inquiry into stillbirths and deaths in infancy. Confidential Enquiry into Stillbirths and Deaths Regional Coordinators and Researchers. BMJ 1996;313:195–8.
Willinger M, James LS, Catz C. Defining the sudden infant death syndrome (SIDS): deliberations of an expert panel convened by the National Institute of Child Health and Human Development. Pediatr Pathol 1991;11:677–84.
Beckwith JB. Discussion of terminology and definition of the sudden infant death syndrome. In: Bergman AB, Beckwith JB, Ray CG, editors. Proceedings of the Second International Conference on the Causes of Sudden Death in Infants. Seattle: University of Washington Press; 1970. pp. 14–22.
Axelrod FB. Familial dysautonomia. Muscle Nerve 2004;29:352–63.
Glickstein JS, Axelrod FB, Friedman D. Electrocardiographic repolarization abnormalities in familial dysautonomia: an indicator of cardiac autonomic dysfunction. Clin Auton Res 1999;9:109–12.
Harper RM. Sudden infant death syndrome: a failure of compensatory cerebellar mechanisms? Pediatr Res 2000;48:140–2.
Harper RM. Autonomic control during sleep and risk for sudden death in infancy. Arch Ital Biol 2001;139:185–94.
Yates BJ, Holmes MJ, Jian BJ. Adaptive plasticity in vestibular influences on cardiovascular control. Brain Res Bull 2000;53:3–9.
Chong A, Murphy N, Matthews T. Effect of prone sleeping on circulatory control in infants. Arch Dis Child 2000;82:253–6.
Gozal D, Harper RM. Novel insights into congenital hypoventilation syndrome. Curr Opin Pulm Med 1999;5:335–8.
Wu CL, Denq JC, Harper CM, O’Brien PC, Low PA. Clinical and electrophysiologic attributes as predictors of results of autonomic function tests. Clin Auton Res 1998;8:347–51.
Panigrahy A, Filiano JJ, Sleeper LA, et al. Decreased kainate receptor binding in the arcuate nucleus of the sudden infant death syndrome. J Neuropathol Exp Neurol 1997;56:1253–61.
Panigrahy A, Filiano J, Sleeper LA, et al. Decreased serotonergic receptor binding in rhombic lip-derived regions of the medulla oblongata in the sudden infant death syndrome [In Process Citation]. J Neuropathol Exp Neurol 2000;59:377–84.
Panigrahy A, Filiano J, Sleeper LA, et al. Decreased serotonergic receptor binding in rhombic lip-derived regions of the medulla oblongata in the sudden infant death syndrome. J Neuropathol Exp Neurol 2000;59:377–84.
Kinney HC. Abnormalities of the brainstem serotonergic system in the sudden infant death syndrome: a review. Pediatr Dev Pathol 2005;8:507–24.
Poets CF, Meny RG, Chobanian MR, Bonofiglo RE. Gasping and other cardiorespiratory patterns during sudden infant deaths. Pediatr Res 1999;45:350–4.
Poets CF. Apparent life-threatening events and sudden infant death on a monitor. Paediatr Respir Rev 2004;5(Suppl A):S383–6.
Krous HF, Masoumi H, Haas EA, Chadwick AE, Stanley C, Thach BT. Aspiration of gastric contents in sudden infant death syndrome without cardiopulmonary resuscitation. J Pediatr 2007;150:241–6.
Harding R, Johnson P, Johnston BE, McClelland MF, Wilkinson AR. Proceedings: cardiovascular changes in new-born lambs during apnoea induced by stimulation of laryngeal receptors with water. J Physiol 1976;256:35P–6P.
Lutherer LO, Lutherer BC, Dormer KJ, Janssen HF, Barnes CD. Bilateral lesions of the fastigial nucleus prevent the recovery of blood pressure following hypotension induced by hemorrhage or administration of endotoxin. Brain Res 1983;269:251–7.
Chen CH, Williams JL, Lutherer LO. Cerebellar lesions alter autonomic responses to transient isovolaemic changes in arterial pressure in anaesthetized cats. Clin Auton Res 1994;4:263–72.
Welsh JP, Yuen G, Placantonakis DG, et al. Why do Purkinje cells die so easily after global brain ischemia? Aldolase C, EAAT4, and the cerebellar contribution to posthypoxic myoclonus. Adv Neurol 2002;89:331–59.
Khurana A, Thach BT. Effects of upper airway stimulation on swallowing, gasping, and autoresuscitation in hypoxic mice. J Appl Physiol 1996;80:472–7.
Stevens LH. Sudden unexplained death in infancy. Amer J Dis Child 1965;110:243–4.
Gardner AMN. Aspiration of food and vomit. Q J Med 1958;27:227–42.
Ackerman MJ, Siu BL, Sturner WQ, et al. Postmortem molecular analysis of SCN5A defects in sudden infant death syndrome. JAMA 2001;286:2264–9.
Arnestad M, Opdal SH, Vege A, Rognum TO. A mitochondrial DNA polymorphism associated with cardiac arrhythmia investigated in sudden infant death syndrome. Acta Paediatr 2007;96:206–10.
Wang DW, Desai RR, Crotti L, et al. Cardiac sodium channel dysfunction in sudden infant death syndrome. Circulation. 2007;115:368–76.
Acknowledgments
Financial support provided by the CJ Foundation for SIDS, First Candle/SIDS Alliance, the San Diego Guild for Infant Survival, and the Guild for Infant Survival of Orange County, as well as the generous donations from SIDS families, is gratefully acknowledged. The authors thank the staff of the Office of the Chief Medical Examiner of San Diego County, California. Drs. Ron Harper (UCLA School of Medicine), Ron Ariagno (Stanford University), and Thomas Keens (University of Southern California School of Medicine) provided helpful comments.
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Krous, H.F., Chadwick, A.E., Haas, E. et al. Sudden infant death while awake. Forensic Sci Med Pathol 4, 40–46 (2008). https://doi.org/10.1007/s12024-007-9003-y
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DOI: https://doi.org/10.1007/s12024-007-9003-y