Aminoff M J, Simon R P
Am J Med. 1980 Nov;69(5):657-66. doi: 10.1016/0002-9343(80)90415-5.
The etiology, clinical features and outcome of generalized major motor status epilepticus in 98 patients over the age of 14 years have been reviewed. Approximately half of the patients had not had previous seizures. The most common single cause of the status was noncompliance with anticonvulsant drug regimens and this accounted for the status in 53 percent of the patients with previous seizures and in 28 percent of all the patients in our series. The other causes in our series were alcohol-withdrawal, cerebrovascular disease, cerebral tumors or trauma (involving especially the frontal lobe), intracranial infection, metabolic disorders, drug overdose and cardiac arrest. In 15 percent of the patients, however, no specific cause could be found. Status was never the initial manifestation of primary (constitutional) generalized epilepsy in our experience. The etiology of the status was sometime multifactorial, so patients must be screened as fully as possible even when a likely cause is readily apparent. The motor manifestations of the convulsions were frequently restricted in distribution (62 percent of the cases). Focal or lateralized convulsive activity, especially during the course of continued seizure activity, did not necessarily indicate that localized structural pathology was responsible for the status. The seizures were of the tonic variety in a few of our patients and in such circumstances were usually associated with cerebral anoxia. We found that a poor outcome of the status was more likely as its duration increased, and the morbidity rate from the status itself was 12.5 percent among our patients, with a mortality rate of 2.5 percent. The episode of status was usually accompanied by hyperthermia, and often by a brisk peripheral leukocytosis, and in some of our patients a status-induced cerebrospinal fluid pleocytosis also developed. These features may lead to diagnostic confusion if their basis is not recognized. In most of our patients a systemic acidosis developed during the course of the status, but this did not appear to greatly influence the outcome.
对98例14岁以上全身性大发作癫痫持续状态患者的病因、临床特征及转归进行了回顾性研究。约半数患者既往无癫痫发作史。癫痫持续状态最常见的单一病因是未遵医嘱服用抗惊厥药物，在既往有癫痫发作的患者中，该病因导致癫痫持续状态的占53%，在我们研究系列的所有患者中占28%。我们研究系列中的其他病因包括酒精戒断、脑血管疾病、脑肿瘤或创伤（尤其累及额叶）、颅内感染、代谢紊乱、药物过量及心脏骤停。然而，15%的患者未发现明确病因。根据我们的经验，癫痫持续状态从未是原发性（全身性）癫痫的首发表现。癫痫持续状态的病因有时是多因素的，因此即使明显存在可能的病因，也必须尽可能全面地对患者进行筛查。抽搐的运动表现通常局限于某一部位（62%的病例）。局灶性或局限性抽搐活动，尤其是在持续癫痫发作过程中，并不一定表明局限性结构病变是癫痫持续状态的病因。我们的一些患者癫痫发作呈强直型，在这种情况下通常与脑缺氧有关。我们发现，癫痫持续状态持续时间越长，预后越差，在我们的患者中，癫痫持续状态本身的发病率为12.5%，死亡率为2.5%。癫痫持续状态发作通常伴有高热，常伴有外周血白细胞计数迅速升高，在我们的一些患者中还出现了癫痫持续状态诱发的脑脊液淋巴细胞增多。如果不认识这些特征的本质，可能会导致诊断混淆。在我们的大多数患者中，癫痫持续状态过程中出现了全身性酸中毒，但这似乎对预后影响不大。
