Guerrini R, Dravet C, Genton P, Belmonte A, Kaminska A, Dulac O
Institute of Child Neurology and Psychiatry, University of Pisa, Calambrone, Italy.
Epilepsia. 1998 May;39(5):508-12. doi: 10.1111/j.1528-1157.1998.tb01413.x.
In severe myoclonic epilepsy of infancy (SME), multiple drug-resistant focal and generalized seizure types occur. Lamotrigine (LTG), found effective in many generalized and partial seizures, has been little used in severe childhood epilepsy syndromes with multiple seizure types. We studied the effects of LTG in SME.
Twenty-one patients with SME, aged 2-18 years, were treated with LTG, 20 in add-on and one in monotherapy. LTG was started at 0.2-2.5 mg/kg/day and increased to 2.5-12.5 mg/kg/day. For each seizure type, excluding atypical absences, >50% variations compared with the 2 months preceding LTG were considered indicators of response, also taking into account the degree of disability each seizure type produced.
LTG induced worsening in 17 (80%) patients, no change in three, and improvement in one. There was >50% increase in convulsive seizures in eight (40%) of 20 patients. Myoclonic seizures worsened in six (33%) of 18 patients. Of five patients improving in at least one seizure type, four had concomitant worsening of more invalidating seizures. Clear-cut worsening appeared within 3 months in most patients but was insidious in some. LTG was suspended in 19 patients after 15 days-5 years (mean, 14 months) with consequent improvement in 18.
The pronounced seizure deterioration during LTG treatment was not attributable to the natural course of the disease and could be a direct effect of therapeutic LTG doses. LTG treatment seems inappropriate in SME.
在婴儿严重肌阵挛癫痫（SME）中，会出现多种耐药性局灶性和全身性癫痫发作类型。拉莫三嗪（LTG）在许多全身性和部分性癫痫发作中被证明有效，但在具有多种发作类型的严重儿童癫痫综合征中很少使用。我们研究了LTG在SME中的作用。
21例年龄在2至18岁的SME患者接受了LTG治疗，其中20例为添加治疗，1例为单药治疗。LTG起始剂量为0.2 - 2.5毫克/千克/天，随后增加至2.5 - 12.5毫克/千克/天。对于每种癫痫发作类型（不包括非典型失神发作），与开始使用LTG前2个月相比，发作次数变化>50%被视为反应指标，同时考虑每种发作类型导致的残疾程度。
LTG使17例（80%）患者病情恶化，3例无变化，1例改善。20例患者中有8例（40%）惊厥性发作增加>50%。18例患者中有6例（33%）肌阵挛发作恶化。在至少一种发作类型有所改善的5例患者中，有4例伴随更多致残性发作的恶化。大多数患者在3个月内出现明显恶化，但有些患者恶化较为隐匿。19例患者在15天至5年（平均14个月）后停用LTG，18例随后病情改善。
LTG治疗期间癫痫发作明显恶化并非疾病自然病程所致，可能是治疗剂量的LTG的直接作用。LTG治疗在SME中似乎不合适。
