An electroclinical case‐control study of sudden unexpected death in epilepsy

Lhatoo, Samden D.; Faulkner, Howard J.; Dembny, Krystina; Trippick, Kathy; Johnson, Claire; Bird, Jonathan M.

doi:10.1002/ana.22101

Cited by 311 publications

(369 citation statements)

References 23 publications

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“…For both mice, recordings revealed central apnea, as there was no evidence for airway obstruction in the video recording, EMG, or plethysmography trace. Notably, the EEG rapidly became flat after the seizure, consistent with PGES (25). This same observation was reported previously after maximal electroshock (MES) induced seizures in Lmx1b f/f/p mice, which lack over 99% of 5-hydroxytryptamine (5-HT) neurons in the CNS (19).…”

Section: Severe Peri-ictal Respiratory Dysfunction Is Common In Dravesupporting

confidence: 87%

“…A variety of mechanisms have been proposed for SUDEP (1,2,(4)(5)(6), including cardiac arrhythmias (7)(8)(9)(10), dysfunction of autonomic control (11)(12)(13)(14)(15), apnea/hypoventilation (3,(16)(17)(18)(19)(20)(21), airway obstruction (22), pulmonary edema (23), brain stem spreading depolarization (BSD) (24), and postictal generalized EEG suppression (PGES) (25). Many investigators have focused on cardiac tachyarrhythmias as the cause of death, in part because of an association between SUDEP and mutations of genes expressed in the heart, such as those that underlie long QT syndrome (10,(26)(27)(28).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Severe peri-ictal respiratory dysfunction is common in Dravet syndrome

Kim¹,

Bravo²,

Thirnbeck³

et al. 2018

Journal of Clinical Investigation

117

168

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Section: Severe Peri-ictal Respiratory Dysfunction Is Common In Dravesupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Severe peri-ictal respiratory dysfunction is common in Dravet syndrome

Kim¹,

Bravo²,

Thirnbeck³

et al. 2018

Journal of Clinical Investigation

117

168

View full text Add to dashboard Cite

show abstract

“…However, potential mechanisms are speculative. Epidemiological studies have consistently pointed to the generalized tonic-clonic seizure as the seizure type most commonly associated with SUDEP (6,7), and almost all SUDEP reports of deaths during monitoring have occurred after generalized tonic-clonic seizures, (8)(9)(10)(11) and often during sleep (3,12). This seizure type is associated with more severe obtundation of sensorium in the postictal period and can be associated with cardiorespiratory dysfunction during this time.…”

mentioning

confidence: 99%

Seizures, Cerebral Shutdown, and SUDEP

Bozorgi

Lhatoo

2013

Epilepsy Curr

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In Clinical ResearchThere is strong evidence to suggest that sudden unexpected death in epilepsy (SUDEP) is a seizure-related phenomenon (1-5). The agonal pathophysiological processes in SUDEP are likely to begin during, or in the immediate aftermath of, a seizure. However, potential mechanisms are speculative. Epidemiological studies have consistently pointed to the generalized tonic-clonic seizure as the seizure type most commonly associated with SUDEP (6, 7), and almost all SUDEP reports of deaths during monitoring have occurred after generalized tonic-clonic seizures, (8-11) and often during sleep (3,12). This seizure type is associated with more severe obtundation of sensorium in the postictal period and can be associated with cardiorespiratory dysfunction during this time. Several questions arise: How do seizures terminate, and how do termination mechanisms relate to postictal arousal? Is the extent of obtundation and subsequent postictal arousal different in the frequent generalized tonic-clonic seizures of patients with chronic intractable epilepsy at high risk of SUDEP? Does postictal arousal differ according to the sleep-wake state at the time of seizure onset? Is postictal failure of arousal the same as the "cerebral shutdown" phenomenon seen on EEG? These questions remain unanswered. Seizure Termination and the Postictal StateThe generalized tonic-clonic seizure is usually characterized by generalized tonic posturing, a phase of tremulousness or "vibration" followed by a generalized clonic phase and, usually, seizure termination within the first 2 minutes of the clinical onset of generalization (13). In some genetic generalized epilepsies (e.g., juvenile myoclonic epilepsy), there may be generalized myoclonic jerking before the tonic phase; in secondarily generalized seizures, there may be a pretonic phase where version or "figure 4" posturing occurs. However, given this relative stereotypy, there is considerable clinical as well as electrographic heterogeneity in the postictal period (14, 15). Some patients recover quickly, while others have varying periods of postictal stupor. Some have brief periods of focal or generalized EEG slowing, while others have prolonged postictal generalized EEG suppression (PGES) and recovery to baseline can sometimes take many hours. Whether this postictal electroclinical heterogeneity aids SUDEP risk-stratification is debated (9, 16). Undoubtedly, there are a number of potential influences, including those of medication effects, but none have been systematically studied. Postictal Generalized EEG Suppression and "Cerebral Shutdown"PGES occurs in between 8% of pediatric seizure patients (17) to 65% or more of adult patients with generalized motor seizures (9) and has been reported in several monitored SUDEP/near SUDEP cases (8-11, 18, 19) where some authors have used the term "cerebral shutdown" (19). Prolonged PGES may be an independent risk factor for SUDEP, but whether it is a surrogate marker or a direct index of cerebral and brainstem dysregulation is unclear. It...

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“…[26] Postictal flattening of EEG after a convulsive seizure recorded during epilepsy monitoring was significantly longer among 10 patients who on follow-up suffered SUDEP compared with surviving epilepsy controls. This prolonged cerebral inhibition might contribute to postictal respiratory dysfunction.…”

Section: Risk Factorsmentioning

confidence: 93%

Sudden Unexpected Death in Epilepsy (SUDEP): an overview

Tomson¹,

Sveinsson²

2013

Epilepsi

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SummarySudden Unexpected Death in Epilepsy (SUDEP) has gained increased awareness over the last 2-3 decades. SUDEP is generally defined as the sudden, unexpected, witnessed or unwitnessed, non-traumatic, and non-drowning death in patients with epilepsy with or without evidence for a seizure, and excluding documented status epilepticus, in which postmortem examination does not reveal a structural or toxicologic cause for death. Sudden death is at least 20 times more common in people with epilepsy compared with the general population. The risk of SUDEP, however, varies widely within the epilepsy population, being the highest in patient with chronic treatment resistent epilepsy. There are probably a number of different mechanisms behind SUDEP, but most research has focused on seizure-related respiratory depression, cardiac arrhythmia, cerebral depression and autonomic dysfunction. Since the frequency of tonic-clonic seizures is the largest risk factor, effective treatment for epilepsy is the best prevention for SUDEP.Key words: Sudden unexpected death in epilepsy; SUDEP; tonic-clonic seizures. ÖzetSon 20-30 yılda epilepside ani beklenmedik ölüm (Sudden Unexpected Death in Epilepsy -SUDEP) artan bir farkındalık kazanmıştır. SUDEP, postmortem incelemenin ölümün yapısal ve toksikolojik nedenini ortaya çıkartamadığı, belgelenmiş status epilepticus dışında kasılma nö-betleri kanıtları olan veya olmayan, genellikle ani, beklenmedik, tanık olunmuş veya olunmamış, travma ve boğulmaya bağlı olmayan ölüm olarak tanımlanmaktadır. Toplum geneline göre epilepsili kişilerde ani ölüm en azından 20 kez daha sık görülmektedir. Ancak SUDEP riski epileptik popülasyonda geniş ölçüde değişmektedir. İnatçı epilepsi için kronik tedavi alanlarda bu risk en yüksek düzeydedir. Olasılıkla SUDEP'in altta yatan birçok farklı mekanizması olmasına rağmen araştırmaların çoğu nöbetlerle ilişkili solunum baskılanması, kalp ritim bozuklukları, serebral depresyon ve anatomik işlev bozukluğuna odaklanmıştır. Tonik-klonik konvülsiyonların sıklığı en buyük risk faktörü olduğu için SUDEP'i önlemenin en iyi yolu epilepsinin etkili tedavisi olmaktadır.

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An electroclinical case‐control study of sudden unexpected death in epilepsy

Cited by 311 publications

References 23 publications

Severe peri-ictal respiratory dysfunction is common in Dravet syndrome

Severe peri-ictal respiratory dysfunction is common in Dravet syndrome

Seizures, Cerebral Shutdown, and SUDEP

Sudden Unexpected Death in Epilepsy (SUDEP): an overview

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