Department of Neurology, University of Pittsburgh, 3471 Fifth Avenue, Pittsburgh, PA 15213, U.S.A.
Epilepsia. 1997 Nov;38(11 Suppl):S35-7. doi: 10.1111/j.1528-1157.1997.tb06124.x.
The pathologic hallmark of sudden unexpected death in epilepsy (SUDEP) in humans is pulmonary edema. In an animal model of seizures, pulmonary vascular pressure, but not systemic pressure, increases in proportion to seizure duration. The induced pulmonary vascular hypertension drives fluid out of the vascular compartment into the lung parenchyma. Blocking intra-ictal pulmonary vascular pressure elevations prevents changes in the observed doubling of the pulmonary transcapillary fluid flux. In this animal model, the main difference between surviving animals and those that die during the seizure is apnea, with a precipitous fall in the partial pressure of oxygen (pO(2)) and a parallel elevation in the partial pressure of carbon dioxide (pCO(2)) recorded in nonsurvivors. Pulmonary artery and left atrial pressures in animals that die are double those of surviving animals, with a resultant increase in extra-vascular lung water at postmortem examination. The pulmonary edema is due to the combined effects of seizure- and hypoxia-induced pulmonary vascular hypertension. This animal model reproduces both death during epilepsy and pulmonary edema at postmortem examination. The etiology of the pulmonary edema appears to be that of pulmonary vascular hypertension, and the etiology of sudden death appears to be that of centrally induced apnea.
癫痫猝死（SUDEP）患者的病理特征是肺水肿。在癫痫发作的动物模型中，肺血管压力而不是全身压力与癫痫持续时间成比例增加。诱发的肺动脉高压将液体从血管腔驱入肺实质。阻断癫痫发作期间的肺血管压力升高可防止观察到的肺毛细血管跨膜液体通量增加一倍的变化。在该动物模型中，存活动物与癫痫发作期间死亡动物之间的主要区别是呼吸暂停，未存活动物的氧分压（pO2）急剧下降，二氧化碳分压（pCO2）平行升高。死亡动物的肺动脉和左心房压力是存活动物的两倍，尸检时肺外血管水增加。肺水肿是由癫痫发作和缺氧引起的肺血管高压共同作用引起的。该动物模型再现了癫痫发作期间的死亡和尸检时的肺水肿。肺水肿的病因似乎是肺血管高压，猝死的病因似乎是中枢性呼吸暂停。
