Sudden Unexpected Death in Epilepsy

Abstract

Sudden unexpected death in epilepsy refers to sudden death of an individual with a clinical history of epilepsy, in whom a postmortem examination fails to uncover a gross anatomic, toxicologic, or environmental cause of death. Evidence of terminal seizure activity may not be present. One to two percent of natural deaths certified by the medicolegal death investigator are attributed to epilepsy. Detailed microscopic examination of the brain has increasingly afforded the identification of structural changes representative of epileptogenic foci. The authors present 70 cases of death attributed to sudden unexpected death in epilepsy. These cases were classified as follows: individuals who lacked a gross brain lesion, those who had a brain lesion demonstrable at autopsy, and those who lacked neuropathologic evaluation because of decomposition or because only an external examination was done. All of the subjects had a clinical history of seizures. The authors confirm that various microscopic findings, including neuronal clusters, increased perivascular oligodendroglia, gliosis, cystic gliotic lesions, decreased myelin, cerebellar Bergmann's gliosis, and folial atrophy, are present in a higher percentage of the brains of sudden unexpected death in epilepsy subjects than in the brains of age- and sex-matched control subjects.

Sudden unexpected death in epilepsy: a retrospective analysis of 24 adult cases

“…One case had a history of clinical developmental delay with no visible neuropathological lesions. The common neuropathological lesions noted in similar studies are contusion [7,16], gliotic scar [16], hydrocephalus [20], heterotopia [16], unilateral hippocampal sclerosis [7], and neoplasm [16]. We did not find a strong relationship between structural brain lesions and the incidence of SUDEP which supports some previously published studies [12,19,21], but contrasts with others [7,20].…”

“…We did not find a strong relationship between structural brain lesions and the incidence of SUDEP which supports some previously published studies [12,19,21], but contrasts with others [7,20]. Various microscopic neuropathological findings have been demonstrated by Shields et al [16] in epilepsy subjects, more so than in the brains of age and sex matched control subjects.…”

“…While it has been stated that ''poor compliance to AED treatments may also relate to risk of SUDEP'' [5,7,11,16,19,20,23,25], this was not the finding of Opeskin et al [26]. Our study revealed 15 cases in which at least one drug was subtheraputic or drug screen was negative (63%) and suggests poor compliance as a risk factor.…”

“…Retrospective [6][7][8][9], retrospective case control [10,11] prospective [4,5] and prospective case control [12,13] studies have been done in the past. The previous studies have suggested risk factors [11,12,14], analyzed circumstances [15], and demonstrated neuropathological findings [9,16] and potential pathologic mechanisms [17]. In this paper, we describe a retrospective review of 24 adult cases of SUDEP.…”

Sudden adult death

“…It has been postulated that the uncoordinated electrical discharge within the brain occurring during a seizure evokes a lethal cardiac arrhythmia or fatal autonomic disturbance [362][363][364][365][366][367]; alternatively, apnoea during the tonic phase of the fit may be a mechanism of death [368,369]. Examination of the brain by a neuropathologist is advised in all possible epilepsy related deaths [370] and a brain lesion responsible for seizures may be found in up to 60% of cases examined by a neuropathologist [371]; however, in this author's experience of practice without neuropathological support a structural lesion is rarely seen. It has also been noted that some patients diagnosed with epilepsy have long QT syndrome [372], which could account for sudden death.…”