Unexpected sudden death is a common event in otherwise healthy epilept
ics, though its etiology has remained unclear. Many authors have sugge
sted cardiac arrhythmias as the cause, and limited data in humans and
animal studies have supported this. However, autopsy series in humans
have shown pulmonary edema, a phenomenon not compatible with a sudden
arrhythmic death, as a possible cause. We developed a model of status
epilepticus in unanesthetized, chronically instrumented sheep in which
sudden death and pulmonary edema occur. Catecholamine levels and seiz
ure type and duration did not differ between animals dying suddenly an
d those surviving. Benign arrhythmias were generated in all animals; i
n no case did an arrhythmia account for the-death of an animal. Striki
ng hypoventilation was demonstrated in the sudden death group but not
in the surviving animals. Differences in peak left atrial and pulmonar
y artery pressures, and in extravascular lung water were also demonstr
ated; pulmonary edema did not account for the demise of the sudden dea
th animals. Thus, our model of epileptic sudden death supports a role
of central hypoventilation in the etiology of sudden unexpected death
and confirms the association with pulmonary edema. The importance of a
rrhythmia in its pathogenesis is not confirmed.