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Sudden unexpected death in epilepsy (SUDEP) in New Zealand; a retrospective review.
Brennan, M, Scott, S, Bergin, P
The New Zealand medical journal. 2020;(1508):65-71
Abstract
AIM: Sudden unexpected death in epilepsy (SUDEP) is well recognised and widely reported but remains poorly understood. SUDEP in young adults is 27 times more common than sudden death in control populations. The incidence of SUDEP in New Zealand is not known but up to 40 people with epilepsy may die from SUDEP every year. A review of coroner's reports of SUDEP was undertaken to learn more about SUDEP in New Zealand. METHOD Coroner's reports of all cases of possible SUDEP in New Zealand from 2007-2016 (n=190) were obtained and post-mortem and toxicology results were reviewed. Cases were categorised using published criteria. RESULTS We obtained reports of 190 cases from the coroner's office. Of these 190 cases, we determined that 123 were definite SUDEP, 40 were definite SUDEP plus, three were probable SUDEP, seven were possible SUDEP and 17 were probably not SUDEP. The number of cases per year varied from 11-26 (2013). Cases were aged 1.5-67 years, with 63% aged 15-45 (mean 37 years). Sixty-one percent were male. Eighty-seven percent of the deaths occurred at home, with 74% found dead in their bed or bedroom. The majority were not employed, with only 33% working or retired at the time of death; 15% were children or students. Information regarding work status was not available for 11%. Toxicology results were available for 155 cases; antiepileptic drug (AED) use was detected in 67% of these cases, with a single AED detected in 44%, two AEDs in 21%, and three AEDs in 3% of samples taken at autopsy. Approximately half who took an AED were taking either sodium valproate or carbamazepine. CONCLUSION This study suggests that people with epilepsy who die from SUDEP in New Zealand are young and are often compliant with their medication. We plan to establish a nationwide SUDEP registry using the EpiNet database to determine the incidence of SUDEP in New Zealand, and to track changes in SUDEP rates. We are also planning to take part in an international case-control study of SUDEP in the hope that we might learn more about risk factors that predispose people with epilepsy to SUDEP, and factors that might reduce the risk.
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Sudden unexpected death in Parkinson's disease: why is drinking water important?
Menezes-Rodrigues, FS, Scorza, CS, Fiorini, AC, Caricati-Neto, A, Scorza, CA, Finsterer, J, Scorza, FA
Neurodegenerative disease management. 2019;(4):241-246
Abstract
Parkinson's disease (PD) is one of the most common age-related neurodegenerative disorders. Several studies over the last few years have shown that PD is accompanied by high rates of premature death compared with healthy controls. Death in PD patients is usually caused by determinant factors such as pneumonia, and cerebrovascular and cardiovascular diseases. During recent years it has emerged that dehydration may also contribute to mortality in PD. Interestingly, it has been documented that a substantial proportion of patients with PD die suddenly (known as sudden and unexpected death in PD). In this article, we focus on the magnitude of the problem of sudden and unexpected death in PD, with special reference to the daily water consumption of PD patients.
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3.
The Interplay Between Diabetes and Pancreatitis: Two Case Reports of Sudden, Natural Deaths and a Review of the Literature.
Butler, DC, Emanuel, AJ, Self, SE, Batalis, NI
Journal of forensic sciences. 2017;(2):519-524
Abstract
Diabetes mellitus (DM) is a common disease involving insulin resistance or deficit that, when left unchecked, may cause severe hyperglycemia and subsequent end-organ damage. Acute pancreatitis (AP) is inflammation of the pancreas that can lead to significant morbidity and mortality. AP and DM both account for a significant amount of sudden deaths, and rarely both disease processes may be present in the same decedent, causing some difficulty in wording the cause of death statement. Although much research has been directed at studying the causes and risk factors for AP and DM, there is a complex interplay between these diseases that is not fully understood. This study presents two autopsy cases of sudden, natural deaths that illustrate this interplay, along with a review of the literature. An algorithm for differentiating AP and DM is then discussed in the context of the presented cases as a proposed aid for forensic pathologists in the certification of such deaths.
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A sudden death risk score specifically for hypertension: based on 25 648 individual patient data from six randomized controlled trials.
Le, HH, Subtil, F, Cerou, M, Marchant, I, Al-Gobari, M, Fall, M, Mimouni, Y, Kassaï, B, Lindholm, L, Thijs, L, et al
Journal of hypertension. 2017;(11):2178-2184
Abstract
OBJECTIVE To construct a sudden death risk score specifically for hypertension (HYSUD) patients with or without cardiovascular history. METHODS Data were collected from six randomized controlled trials of antihypertensive treatments with 8044 women and 17 604 men differing in age ranges and blood pressure eligibility criteria. In total, 345 sudden deaths (1.35%) occurred during a mean follow-up of 5.16 years. Risk factors of sudden death were examined using a multivariable Cox proportional hazards model adjusted on trials. The model was transformed to an integer system, with points added for each factor according to its association with sudden death risk. RESULTS Antihypertensive treatment was not associated with a reduction of the sudden death risk and had no interaction with other factors, allowing model development on both treatment and placebo groups. A risk score of sudden death in 5 years was built with seven significant risk factors: age, sex, SBP, serum total cholesterol, cigarette smoking, diabetes, and history of myocardial infarction. In terms of discrimination performance, HYSUD model was adequate with areas under the receiver operating characteristic curve of 77.74% (confidence interval 95%, 74.13-81.35) for the derivation set, of 77.46% (74.09-80.83) for the validation set, and of 79.17% (75.94-82.40) for the whole population. CONCLUSION Our work provides a simple risk-scoring system for sudden death prediction in hypertension, using individual data from six randomized controlled trials of antihypertensive treatments. HYSUD score could help assessing a hypertensive individual's risk of sudden death and optimizing preventive therapeutic strategies for these patients.
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Sudden death due to aortic pathology.
Papagiannis, J
Cardiology in the young. 2017;(S1):S36-S42
Abstract
Sudden death from aortic dissection of an ascending aortic aneurysm is an uncommon but important finding in all series of sudden death in young, apparently healthy athletes. Individuals at risk include those having any of a variety of conditions in which structural weakness of the ascending aorta predisposes to pathological dilation under prolonged periods of increased wall stress. These conditions include Marfan syndrome, Loeys-Dietz syndrome, bicuspid aortic valve, and the vascular form of Ehlers-Danlos syndrome. Diagnostic criteria, surveillance strategies, medical management, and surgical indications are discussed. Finally, the current recommendations for sports participation are provided.
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[The Brugada Syndrome as a Cause of Sudden Death. Diagnostics and Clinical Manifestations in Children].
Miklashevich, IM, Shkolnikova, MA
Kardiologiia. 2016;(10):63-71
Abstract
The Brugada syndrome is a hereditary potentially arrhythmogenic disease related to the category of channelopathies. It is manifested as syncopal states and sudden death in young people in the absence of structural cardiac disease. The basis of the disease is genetically determined abnormality of function of ionic channels of cardiomyocytes (sodium, potassium, calcium) phenotypically manifesting as sustained or transitory segment-ST elevation and high risk of polymorphic ventricular tachycardia, fibrillation, and sudden death. The only proven method of prevention of fatal arrhythmia and sudden death is cardioverter defibrillator implantation.
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Sudden unexpected death in Dravet syndrome: respiratory and other physiological dysfunctions.
Kalume, F
Respiratory physiology & neurobiology. 2013;(2):324-8
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Abstract
Sudden unexpected deaths in epilepsy (SUDEP) occur at an alarming higher rate in patients with Dravet syndrome (DS) than in patients with most other forms of epilepsy. DS is a severe infantile-onset epilepsy caused by a heterozygote loss-of-function mutation in SCN1A, which encodes the voltage-gated-sodium channel NaV 1.1. The mechanisms leading to SUDEP in DS or other epilepsies are not completely understood. Understanding the pathophysiological mechanisms of SUDEP, common to most epilepsies and those specific to DS, may pave the way toward the discovery of effective preventive strategies for these epilepsy-related tragic events.
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[Metabolic acidosis--cause of sudden, unexplained death among chronic alcohol abusers].
Rybakowska, I, Waldman, W, Kaletha, K, Sein Anand, J
Przeglad lekarski. 2012;(8):552-4
Abstract
Metabolic acidosis (lactic and ketoacidosis) are frequent cause of sudden, unexplained deaths of alcohol abusers with negative results of pathomorphological and toxicological determinations. We described the pathomechanism leading to the acidosis.
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Changing times: DNA resequencing and the "nearly normal autopsy".
Karch, SB
Journal of forensic and legal medicine. 2007;(7):389-97
Abstract
No matter how meticulous the autopsy, non-traumatic deaths in the young go unexplained from 5-10% of the time. The percentage is higher in children and young adults. Advances in molecular biology and DNA technology now make it possible to explain many of those deaths. This development is not without irony. At the same time that many clinicians are expressing frustration about the lack of tangible gains provided by the Human Genome Project [Greenhalgh T. The Human Genome Project. J R Soc Med. Dec 2005;98(12):545], and pathologists are wondering about the viability of their field, DNA technology is about to reshape the field of forensic pathology. Emerging evidence suggests that the underlying cause of death in many is genetic, and that both the heart and liver abnormalities can both play a role. The problem is that death from a wide variety of genetic defects may leave no histological markers. The ability to identify these "invisible diseases" with postmortem genetic testing has become a reality far more quickly than anyone had ever imagined. The US Food and Drug Administration is about to place "black box" warnings on warfarin advising doctors screen potential recipients for the ability to metabolize that drug and the American Heart Association has recently editorialized that because of genetic-induced variations in electrical conduction that all newborns should have a screening electrocardiogram before they leave the hospital. The introduction of large-scale genetic screening will have an enormous effect on the practice of forensic pathology, far beyond anything seen in our lifetimes. It will also change the practice of medicine as we know it. This paper reviews the current status of the problem.
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[Mechanoelectric feedback and sudden death in heart failure].
Guadalajara Boo, JF
Archivos de cardiologia de Mexico. 2001;:S69-75
Abstract
Better knowledge of mechanisms which perpetuate heart failure and promote progression and death in patients with these sicknesses, has led to find a better medical treatment to improve the functional status, decrease mortality and improve life span, avoiding the progression of ventricular dysfunction. Mortality reduction due to the disease progression has led to evident arrhythmic mortality show by sudden death. Aspects involved in the genesis and pathophysiology of sudden death in patients with chronic-heart failure; are reviewed in this paper. Special reference to mechano-electrical feedback is considered.