Perucca E
Clinical Pharmacology Unit, Department of Internal Medicine and Therapeutics, University of Pavia, Piazza Botta 10, 27100 Pavia, Italy.
Epilepsia. 2001;42 Suppl 3:31-5. doi: 10.1046/j.1528-1157.2001.042suppl.3031.x.
Idiopathic epilepsies comprise a wide variety of partial and generalized syndromes that have in common a known or presumed genetic etiology and the lack of overt abnormalities other than the epilepsy itself. Most of these epilepsies have a benign natural history and/or show a favorable response to antiepileptic drug (AED) therapy, but pharmacoresistance does occur in some patients. In general, therapeutic algorithms in idiopathic partial epilepsies (IPEs) are similar to those used for symptomatic partial epilepsies, but aggressive pharmacologic therapy is rarely indicated in these patients. In self-limited conditions such as benign epilepsy of childhood with centrotemporal spikes or some forms of benign epilepsy with occipital paroxysms, AED treatment may not even be indicated unless seizures interfere significantly with quality of life. Valproate (VPA) is usually regarded as the drug of choice in idiopathic generalized epilepsies (IGEs). Most patients become rapidly seizure free, and poor compliance or prescription of an inappropriate AED because of misdiagnosis are the most common causes of treatment failure in IGEs. In those patients who did not respond well to VPA (or in whom VPA is considered contraindicated), the choice of alternative AEDs is guided by syndromic diagnosis and associated possible coexistence of multiple seizure types. Lamotrigine is establishing itself as a useful agent for many refractory IGEs, and might be considered for first-line use in selected patients. Topiramate (TPM) is another promising new agent in the management of refractory tonic-clonic seizures of nonfocal onset, but its potential efficacy against other primarily generalized seizure types has not been clearly established. Some of the older drugs, particularly ethosuximide (ESM), barbiturates, and benzodiazepines (BZDs), still have an important role in the management of refractory IGEs, especially in combination with VPA. Because carbamazepine (CBZ), phenytoin (PHT), tiagabine (TGB), vigabatrin (VGB), and gabapentin (GBP) may precipitate or aggravate absence and/or myoclonic jerks, their role in IGE syndromes associated with multiple seizure types is limited mostly to adjunctive use in patients unresponsive to first-line therapy.
特发性癫痫包括多种局灶性和全身性综合征，它们的共同特点是具有已知或推测的遗传病因，且除癫痫本身外无明显异常。这些癫痫大多具有良性自然病程和/或对抗癫痫药物（AED）治疗反应良好，但部分患者确实会出现药物抵抗。一般来说，特发性局灶性癫痫（IPE）的治疗方案与症状性局灶性癫痫相似，但这些患者很少需要积极的药物治疗。在自限性疾病中，如儿童中央颞区棘波良性癫痫或某些枕叶阵发性良性癫痫，除非癫痫发作严重影响生活质量，否则甚至可能不需要AED治疗。丙戊酸盐（VPA）通常被视为特发性全身性癫痫（IGE）的首选药物。大多数患者很快就不再发作，而依从性差或因误诊而开具不适当的AED是IGE治疗失败的最常见原因。对于那些对VPA反应不佳（或认为VPA禁忌） 的患者，替代AED的选择以综合征诊断和多种发作类型可能并存为指导。拉莫三嗪正成为许多难治性IGE的有效药物，在某些特定患者中可考虑作为一线用药。托吡酯（TPM）是治疗非局灶性发作的难治性强直阵挛发作的另一种有前景的新药，但其对其他主要全身性发作类型的潜在疗效尚未明确确立。一些较老的药物，特别是乙琥胺（ESM）、巴比妥类药物和苯二氮䓬类药物（BZD），在难治性IGE的治疗中仍发挥重要作用，尤其是与VPA联合使用时。由于卡马西平（CBZ）、苯妥英（PHT）、噻加宾（TGB）、氨己烯酸（VGB）和加巴喷丁（GBP）可能诱发或加重失神发作和/或肌阵挛抽搐，它们在与多种发作类型相关的IGE综合征中的作用主要限于对一线治疗无反应的患者的辅助使用。
