scholarly journals Update on the Genetic Basis of Sudden Unexpected Death in Epilepsy

2019 ◽  
Vol 20 (8) ◽  
pp. 1979 ◽  
Author(s):  
Monica Coll ◽  
Antonio Oliva ◽  
Simone Grassi ◽  
Ramon Brugada ◽  
Oscar Campuzano
Keyword(s):  
Sudden Unexpected Death ◽  
Close Monitoring ◽  
Unexpected Death ◽  
Effective Prevention ◽  
Sleeping Position ◽  
Prone Sleeping Position ◽  
Incidence Risk ◽  
History Of ◽  
Antiepileptic Medications ◽  
Patients With Epilepsy

Epilepsy is a common neurological disorder associated with increased morbidity and mortality. Sudden unexpected death in epilepsy, also known as SUDEP, is the main cause of death in patients with epilepsy. SUDEP has an incidence of 1.2 per 1000 person-years in adults and 0.2 per 1000 person-years in children. SUDEP accounts for 8–17% of deaths in patients with epilepsy. It is commonly associated with a history of generalized tonic-clonic seizures, and its risk may be increased by other factors such as postictal electroencephalographic suppression, prone sleeping position, altered heart rate variability, conduction abnormalities, gender, or antiepileptic medications. Recently, electrocardiograms, electroencephalograms, and imaging markers have helped clinicians stratify SUDEP risk and identify patients in need of close monitoring. However, the pathophysiology of SUDEP is likely multifactorial and still unknown. Improving the knowledge of SUDEP incidence, risk factors, and biomarkers can help design and implement effective prevention strategies.

Sudden Unexpected Death of Two Infants in Baby Carriers

2021 ◽  
Author(s):  
Dennis Storz ◽  
Christof Dame ◽  
Anke Wendt ◽  
Alexander Gratopp ◽  
Christoph Bührer
Keyword(s):  
Risk Factors ◽  
Infant Development ◽  
Sudden Infant Death ◽  
Developed Countries ◽  
Bottle Feeding ◽  
Sudden Unexpected Death ◽  
Sudden Infant ◽  
Unexpected Death ◽  
Sleeping Position ◽  
Prone Sleeping Position

Sudden unexpected death in infancy (SUDI), previously termed sudden infant death syndrome (SIDS), is the second leading cause of death in infants beyond the neonatal period in Germany, and a major cause of infant mortality in economically well developed countries (OECD Health Statistics, 2019). The risk of SUDI peaks at the age of 2–4 months and then decreases continuously till the end of the first year. A complex multifactorial cause, rather than a single characteristic factor, may cause SUDI within a critical period of infant development (Guntheroth WG et al., Pediatrics 2002; 110: e64–e64). Risk factors include prematurity, male gender, bottle-feeding, prone sleeping position, overheating, as well as exposure to smoke amongst others (Carpenter RG et al., Lancet 2004; 363: 185–191). Thus, health professionals consistently advise and educate parents about avoidable risk factors of SUDI at routine well-baby examinations. Since the advent of SUDI prevention strategies in the 1980s, the incidence has decreased 10fold, from 1,55/1.000 live births in 1991 to 0,15/1000 in 2015. This number seems to have reached a steady state (Statistisches Bundesamt Germany, 2015).

Sudden, Unexpected Death Associated with Meningioangiomatosis: Case Report

2005 ◽  
Vol 8 (2) ◽  
pp. 240-244 ◽  
Author(s):  
Chris Wixom ◽  
Amy E. Chadwick ◽  
Henry F. Krous
Keyword(s):  
Case Report ◽  
Family History ◽  
Postmortem Examination ◽  
Sudden Unexpected Death ◽  
Healthy Male ◽  
Orbital Frontal Cortex ◽  
Unexpected Death ◽  
Microscopic Features ◽  
History Of ◽  
The Right

We report a case of sudden, unexpected death associated with meningioangiomatosis in a 13-year-old, previously healthy male without a history of seizures, neurologic deficits, or clinical stigmata of neurofibromatosis. There was no family history of neurofibromatosis. The postmortem examination showed a 5-cm mass involving the right posterior frontal and orbital frontal cortex that had microscopic features diagnostic of meningioangiomatosis. Because no other cause of death was found, we postulate that he likely died as a result of a seizure secondary to meningioangiomatosis.

Sudden Unexpected Death in Neonates: A Clinico-pathological Study

2018 ◽  
Vol 21 (6) ◽  
pp. 528-536 ◽  
Author(s):  
Jeanette A Reyes ◽  
Gino R Somers ◽  
David A Chiasson
Keyword(s):  
Physiological Stress ◽  
Growth Parameters ◽  
Wide Spectrum ◽  
Vital Signs ◽  
Clinical History ◽  
Sudden Unexpected Death ◽  
Unexpected Death ◽  
Inborn Errors ◽  
Ancillary Test ◽  
History Of

Clinico-pathological studies that focus on sudden unexpected death (SUD) in the neonatal period are rare. The objective of this study was to elucidate the frequency and pathological spectrum of anatomical causes of death (CODs), found in the setting of sudden unexpected death in neonates (SUD-N), and to correlate the COD with premortem circumstantial information. We conducted a detailed review of all autopsy reports on SUD-N cases at our institution from 1997 to 2015. Analyzed clinical data included obstetrical history, postpartum/neonatal medical course, and circumstances surrounding death. Evaluated autopsy data included growth parameters, pathological findings, ancillary test results, and COD. Data from decedents in which a COD was established (COD-E) were statistically compared with that from decedents in which the COD was undetermined (COD-U). Of 104 neonates (M: 49; F: 55) who fulfilled our inclusion criteria, a COD was established at autopsy in 46 cases (44%). Infections, congenital abnormalities, and inborn errors of metabolism were the most common CODs. Single variables statistically more likely to be found in COD-E neonates were clinical history of prodromal illness, witnessed loss of vital signs, and evidence of physiological stress in the thymus or the liver. A prodrome was statistically more common in the COD-E group, but the absence of a prodrome does not reliably exclude COD-E cases, since over 50% of these patients were asymptomatic prior to their demise. In COD-U neonates, the statistically significant factors were death during sleep, death during sleep while “bed”-sharing, “heavy” lungs, and petechial hemorrhages on the epicardium or pleura. Given the frequency and wide spectrum of underlying pathologies in COD-E neonates, referral of SUD-N cases to pathologists with specialized pediatric autopsy expertise is recommended.

scholarly journals Practice guideline summary: Sudden unexpected death in epilepsy incidence rates and risk factors

Neurology ◽  
2017 ◽  
Vol 88 (17) ◽  
pp. 1674-1680 ◽  
Author(s):  
Cynthia Harden ◽  
Torbjörn Tomson ◽  
David Gloss ◽  
Jeffrey Buchhalter ◽  
J. Helen Cross ◽  
...  
Keyword(s):  
Risk Factors ◽  
Evaluation Process ◽  
Incidence Rates ◽  
Sudden Unexpected Death ◽  
Seizure Freedom ◽  
Unexpected Death ◽  
New Approach ◽  
Assessment Development ◽  
Clonic Seizures ◽  
Patients With Epilepsy

Objective:To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified.Methods:Systematic review of evidence; modified Grading Recommendations Assessment, Development, and Evaluation process for developing conclusions; recommendations developed by consensus.Results:Findings for incidence rates based on 12 Class I studies include the following: SUDEP risk in children with epilepsy (aged 0–17 years) is 0.22/1,000 patient-years (95% confidence interval [CI] 0.16–0.31) (moderate confidence in evidence). SUDEP risk increases in adults to 1.2/1,000 patient-years (95% CI 0.64–2.32) (low confidence in evidence). The major risk factor for SUDEP is the occurrence of generalized tonic-clonic seizures (GTCS); the SUDEP risk increases in association with increasing frequency of GTCS occurrence (high confidence in evidence).Recommendations:Level B: Clinicians caring for young children with epilepsy should inform parents/guardians that in 1 year, SUDEP typically affects 1 in 4,500 children; therefore, 4,499 of 4,500 children will not be affected. Clinicians should inform adult patients with epilepsy that SUDEP typically affects 1 in 1,000 adults with epilepsy per year; therefore, annually 999 of 1,000 adults will not be affected. For persons with epilepsy who continue to experience GTCS, clinicians should continue to actively manage epilepsy therapies to reduce seizures and SUDEP risk while incorporating patient preferences and weighing the risks and benefits of any new approach. Clinicians should inform persons with epilepsy that seizure freedom, particularly freedom from GTCS, is strongly associated with decreased SUDEP risk.

Sudden unexpected death in patients with epilepsy receiving renal replacement therapy with dialysis: A 17-year experience at a single institution

2010 ◽  
Vol 14 (4) ◽  
pp. 364-369 ◽  
Author(s):  
Fulvio A. SCORZA ◽  
Marcello SCATTOLINI ◽  
Roberta M. CYSNEIROS ◽  
Ricardo M. ARIDA ◽  
Marly DE ALBUQUERQUE ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Sudden Unexpected Death ◽  
Single Institution ◽  
Unexpected Death ◽  
Patients With Epilepsy

scholarly journals SUDEP in adults and children

2022 ◽  
Vol 7 (4) ◽  
pp. 259-265
Author(s):  
Ajith Cherian ◽  
Divya K P
Keyword(s):  
Premature Death ◽  
Drug Level ◽  
Dravet Syndrome ◽  
Therapeutic Drug ◽  
Neurogenic Pulmonary Edema ◽  
Unexpected Death ◽  
Temporal Lobe Resection ◽  
Antiepileptic Medications ◽  
Drug Resistant Epilepsy ◽  
Patients With Epilepsy

Sudden unexpected death in epilepsy (SUDEP) represents an important cause of death in patients with epilepsy and it exceeds the expected rate of sudden death in the general population by nearly 24 times. We searched the electronic databases (Cochrane, EMBASE, Scopus, Medline, Pubmed) for studies related to etiology and risk stratification of SUDEP including data on Takotsubo cardiomyopathy (TKC) following seizures resulting in death or near death.: SUDEP is more common among males in the fourth decade of life. Risk for SUDEP is increased by early onset of seizures, low IQ, generalised tonic clonic seizures, nocturnal seizures and seizure frequency. Nonadherance to antiepileptic medications, absence of therapeutic drug level monitoring, presence of neuropathological lesions on imaging and certain subgroups like Dravet syndrome increase its risk. The risk for premature death in patients undergoing temporal lobe resection for drug resistant epilepsy decreased over time but remained above the standard population. Prolonged postictal electroencephalographic suppression was a risk factor for SUDEP in patients with generalised seizures which may indicate a cerebral electrical shutdown. Documented ictal/postictal hypoventilation, laryngeal spasm and cardiac rhythm abnormalities prior to SUDEP may suggest central apnea, neurogenic pulmonary edema, cardiac arrhythmia, or a combination of the above as a cause. Seizure triggered TKC does not seem to play a major role in the pathogenesis of SUDEP.

scholarly journals Reducing Sudden Unexpected Death in Epilepsy: Considering Risk Factors, Pathophysiology and Strategies

2021 ◽  
Vol 23 (11) ◽  
Author(s):  
Nathan A. Shlobin ◽  
Josemir W. Sander
Keyword(s):  
Risk Factors ◽  
Significant Proportion ◽  
Premature Mortality ◽  
Sudden Unexpected Death ◽  
Unexpected Death ◽  
Cardiorespiratory Function ◽  
Younger Age ◽  
History Of ◽  
Symptomatic Epilepsy ◽  
Convulsive Seizures

Abstract Purpose of Review Sudden Unexpected Death in Epilepsy (SUDEP) is the commonest cause of epilepsy-related premature mortality in people with chronic epilepsy. It is the most devastating epilepsy outcome. We describe and discuss risk factors and possible pathophysiological mechanisms to elucidate possible preventative strategies to avert SUDEP. Recent Findings Sudden death accounts for a significant proportion of premature mortality in people with epilepsy compared to the general population. Unmodifiable risk factors include a history of neurologic insult, younger age of seizure-onset, longer epilepsy duration, a history of convulsions, symptomatic epilepsy, intellectual disability, and non-ambulatory status. Modifiable risk factors include the presence of convulsive seizures, increased seizure frequency, timely and appropriate use of antiseizure medications, polytherapy, alcoholism, and supervision while sleeping. Pathophysiology is unclear, but several possible mechanisms such as direct alteration of cardiorespiratory function, pulmonary impairment, electrocerebral shutdown, adenosine dysfunction, and genetic susceptibility suggested. Summary Methods to prevent SUDEP include increasing awareness of SUDEP, augmenting knowledge of unmodifiable risk factors, obtaining full seizure remission, addressing lifestyle factors such as supervision and prone positioning, and enacting protocols to increase the detection of and intervention for SUDEP. Further studies are required to characterize precisely and comprehensively SUDEP risk factors and pathophysiological drivers and develop evidence-based algorithms to minimize SUDEP in people with epilepsy.

scholarly journals Awareness f Sudden Unexpected Death in Epilepsy Among Neurologists in Latvia / Neirologu Informētība par Pēkšņas, Negaidītas Nāves Epilepsijas Laikā Sindromu Latvijā

2015 ◽  
Vol 69 (5) ◽  
pp. 265-268
Author(s):  
Normunds Sūna ◽  
Madara Lazdāne ◽  
Guntis Karelis ◽  
Egils Vītols
Keyword(s):  
Risk Factors ◽  
Sudden Unexpected Death ◽  
Unexpected Death ◽  
Partial Explanation ◽  
Common Cause ◽  
Counselling Practice ◽  
Depth Analysis ◽  
Improve Compliance ◽  
Patients With Epilepsy

Abstract Sudden unexpected death in epilepsy (SUDEP) is a common cause of mortality in patients with epilepsy, but it is unknown how neurologists disclose this risk when counselling patients. This study was aimed at examining SUDEP discussion practices of neurologists in Latvia, as well as the awareness of the syndrome. Two hundred questionnaires were distributed, and we received 84 responses. We found that the majority of Latvian neurologists (79.0%) do not inform their patients of SUDEP, which is opposite to the findings in other countries. Despite the existing practice, 93.1% of neurologists believed that patients should be informed about SUDEP. A partial explanation for not discussing the negative aspects of epilepsy is that 75.3% of caregivers believe that being informed about SUDEP would cause permanent anxiety in patients, whereas 69.4% believe that it would improve compliance. This study revealed average awareness of SUDEP risk factors and warrants further studies for in-depth analysis of existing counselling practice.

Sudden Unexpected Death In Patients with Epilepsy

CNS Drugs ◽  
2000 ◽  
Vol 13 (5) ◽  
pp. 337-349 ◽  
Author(s):  
Yvonne Langan ◽  
Josemir W.A.S. Sander
Keyword(s):  
Sudden Unexpected Death ◽  
Unexpected Death ◽  
Patients With Epilepsy
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