scholarly journals Sudden death in a child with epilepsy: potential cerebellar mechanisms?

2011 ◽  
Vol 69 (4) ◽  
pp. 707-710 ◽  
Author(s):  
Fulvio A. Scorza ◽  
Vera C. Terra ◽  
Ricardo M. Arida ◽  
Américo C. Sakamoto ◽  
Ronald M. Harper

Epilepsy is the most common neurological disorder in humans. People with epilepsy are more likely to die prematurely than those without epilepsy, with the most common epilepsy-related category of death being sudden unexpected death in epilepsy (SUDEP). The central mechanisms underlying the fatal process remain unclear, but cardiac and respiratory mechanisms appear to be involved. Recently, cerebellar, thalamic, basal ganglia and limbic brain structures have been shown to be implicated in respiratory and cardiac rate regulation. We discuss here the potential mechanisms underlying the fatal process, with a description of cerebellar actions likely failing in that SUDEP process.

2012 ◽  
Vol 70 (3) ◽  
pp. 226-227 ◽  
Author(s):  
Fulvio Alexandre Scorza

People with epilepsy are more likely to die prematurely, and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Thus, the purpose of this article was to alert the scientific community about SUDEP.


2011 ◽  
Vol 17 (2) ◽  
pp. 77-77 ◽  
Author(s):  
Vera C. Terra ◽  
Marly de Albuquerque ◽  
Carla A. Scorza ◽  
Ricardo M. Arida ◽  
Fulvio A. Scorza

INTRODUCTION: Epilepsy is one of the most common chronic neurological disorder in the world and has a higher mortality rate than would be expected in a healthy population. One of the most related category of death is sudden unexpected death in epilepsy (SUDEP). Many risk factors have been related to SUDEP, but the mechanisms involved in its genesis is still unknown. OBJECTIVE: Here we describe one case of a patient with low serum magnesium levels that suffered of SUDEP. CONCLUSION: we discuss a possible cause-effect relation, suggesting that magnesium may be, in some cases, a biomarker of SUDEP.


2018 ◽  
Vol 52 (9) ◽  
pp. 868-875 ◽  
Author(s):  
Sonalie Patel ◽  
Mitchell M. Conover ◽  
Golsa Joodi ◽  
Sarah Chen ◽  
Ross J. Simpson ◽  
...  

Background: In Wake County, NC, sudden unexpected death accounts for 10% to 15% of all natural deaths in individuals 18 to 64 years old. Medications such as aspirin, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, statins, and β-blockers are recommended in guidelines to reduce cardiovascular events and even sudden death (β-blockers). However, guidelines are often underpracticed, even in high-risk patients, with noted disparities in women. Objective: We assessed the relation between prescription of evidence-based medications and sudden unexpected death in Wake County, NC. Methods: We analyzed 399 cases of sudden unexpected death for the time period March 1, 2013 to February 28, 2015 in Wake County, NC. Medications were assessed from available medical examiner reports and medical records and grouped using the third level of the Anatomical Therapeutic Chemical Classification System (ATC) codes. This study was reviewed and exempt by the University of North Carolina’s institutional review board. Results: Among 126 female and 273 male victims, women were prescribed more medications overall than men (6.5 vs 4.3, P = 0.001); however, the use of guideline-directed therapies was not different between genders in the chronic conditions associated with sudden death. Overall, there was remarkably low use of evidence-based medications. Conclusions: Our findings highlight the need to improve prescribing of evidence-based medications and to further explore the relationship between undertreatment and sudden unexpected death.


2009 ◽  
Vol 73 (1) ◽  
pp. 67-69 ◽  
Author(s):  
F.A. Scorza ◽  
R. de Albuquerque ◽  
R.M. Arida ◽  
B. Schmidt ◽  
Antonio-Carlos G. de Almeida ◽  
...  

2009 ◽  
Vol 67 (4) ◽  
pp. 1001-1002 ◽  
Author(s):  
Vera C. Terra ◽  
Fulvio A. Scorza ◽  
Américo C. Sakamoto ◽  
Kylvia G.F.D. Pinto ◽  
Regina M.F. Fernandes ◽  
...  

People with epilepsy are more likely to die prematurely and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Several studies have reported a moderate or high seizure frequency among SUDEP cases and SUDEP is considered rare in patients in remission. METHOD: We reviewed the occurrence of SUDEP in our epilepsy unit over an 8-year period to identify a potential association between seizure frequency and SUDEP occurrence in children with epilepsy. RESULTS: From 835 patients evaluated, 12 had suffered SUDEP and nearly all of the SUDEP cases in our children are related to chronic uncontrolled epilepsy (daily - 50.0%, two to four/week - 41.7%, monthly - 8.3%). CONCLUSION: SUDEP is not a rare event in children and increased mortality was recorded in those individuals who had not responded to pharmacologic treatment. Improved seizure control seems to be one of the most important measures to prevent SUDEP.


Author(s):  
C. Anwar A. Chahal ◽  
David J. Tester ◽  
Ahmed U. Fayyaz ◽  
Keerthi Jaliparthy ◽  
Nadeem A. Khan ◽  
...  

Background Sudden cardiac arrest is the leading mode of death in the United States. Epilepsy affects 1% of Americans; yet epidemiological data show a prevalence of 4% in cases of sudden cardiac arrest. Sudden unexpected death in epilepsy (SUDEP) may share features with sudden cardiac arrest. The objective of this study was to report autopsy and genomic findings in a large cohort of SUDEP cases. Methods and Results Mayo Clinic Sudden Death Registry containing cases (ages 0–90 years) of sudden unexpected and unexplained deaths 1960 to present was queried. Exome sequencing performed on decedent cases. From 13 687 cases of sudden death, 656 (4.8%) had a history of seizures, including 368 confirmed by electroencephalography, 96 classified as SUDEP, 58 as non‐SUDEP, and 214 as unknown (insufficient records). Mean age of death in SUDEP was 37 (±19.7) years; 56 (58.3%) were male; 65% of deaths occurred at night; 54% were found in bed; and 80.6% were prone. Autopsies were obtained in 83 cases; bystander coronary artery disease was frequently reported as cause of death; nonspecific fibrosis was seen in 32.6% of cases, in structurally normal hearts. There were 4 cases of Dravet syndrome with pathogenic variants in SCN1A gene. Using whole exome sequencing in 11 cases, 18 ultrarare nonsynonymous variants were identified in 6 cases including CACNB2, RYR2, CLNB, CACNA1H, and CLCN2 . Conclusions This study examined one of the largest single‐center US series of SUDEP cases. Several cases were reclassified as SUDEP, 15% had an ECG when alive, and 11 (11.4%) had blood for whole exome sequencing analysis. The most frequent antemortem genetic finding was pathogenic variants in SCN1A ; postmortem whole exome sequencing identified 18 ultrarare variants.


Epilepsia ◽  
2017 ◽  
Vol 59 (1) ◽  
pp. 92-105 ◽  
Author(s):  
Shruthi H. Iyer ◽  
Stephanie A. Matthews ◽  
Timothy A. Simeone ◽  
Rama Maganti ◽  
Kristina A. Simeone

Sign in / Sign up

Export Citation Format

Share Document