Guerrini R, Genton P, Bureau M, Parmeggiani A, Salas-Puig X, Santucci M, Bonanni P, Ambrosetto G, Dravet C
Institute of Child Neurology and Psychiatry, University of Pisa, Italy.
Neurology. 1998 Aug;51(2):504-12. doi: 10.1212/wnl.51.2.504.
Patients with cortical malformations often have intractable seizures and are candidates for epilepsy surgery. Within an unselected series of patients with various forms of cortical malformation, nine patients with multilobar polymicrogyria had electrical status epilepticus during sleep (ESES) accompanied by infrequent focal motor seizures. Eight patients also had intractable atonic drop attack seizures. Because ESES usually is accompanied by a good long-term seizure prognosis, the objective of this study was to examine ESES outcome among patients with a structural lesion that is usually highly epileptogenic and has a low seizure remission trend.
The nine patients had follow-up periods lasting 4 to 19 years. All underwent brain MRI, serial sleep EEG recordings, and cognitive testing during and after ESES.
ESES and drop attack seizures appeared between the ages of 2 and 5 years (mean, 4 years) and ceased between the ages of 5 and 12 years (mean, 8 years). At the last visit patients were 8 to 23 years of age (mean, 14.5 years) and were either seizure free or had very infrequent focal motor seizures during sleep. Three patients were free from antiepileptic drugs. In no patient was definite cognitive deterioration apparent after ESES in comparison with earlier evaluations.
Age-related secondary bilateral synchrony underlying ESES may be facilitated in multilobar polymicrogyria. The good seizure outcome contrasts with that usually found in the presence of cortical malformations. For children with polymicrogyria and drop attack seizures, surgical treatment of the epilepsy should be considered cautiously, and sleep EEG recordings should be performed systematically.
皮质发育畸形患者常伴有难治性癫痫发作，是癫痫手术的候选对象。在一系列未经选择的各种形式皮质发育畸形患者中，9例多脑叶多微小脑回患者在睡眠中出现癫痫持续状态（ESES），伴有不频繁的局灶性运动性发作。8例患者还伴有难治性失张力性跌倒发作。由于ESES通常伴有良好的长期癫痫发作预后，本研究的目的是探讨在通常具有高度致痫性且癫痫发作缓解趋势较低的结构性病变患者中ESES的转归情况。
9例患者的随访期为4至19年。所有患者均接受了脑部MRI检查、ESES期间及之后的系列睡眠脑电图记录以及认知测试。
ESES和跌倒发作出现在2至5岁之间（平均4岁），并在5至12岁之间（平均8岁）停止。在最后一次随访时，患者年龄为8至23岁（平均14.5岁），要么无癫痫发作，要么在睡眠中仅有非常不频繁的局灶性运动性发作。3例患者停用了抗癫痫药物。与早期评估相比，没有患者在ESES后出现明确的认知功能恶化。
多脑叶多微小脑回中与年龄相关的ESES潜在继发性双侧同步化可能更容易出现。良好的癫痫发作转归与通常在皮质发育畸形情况下发现的情况形成对比。对于有多微小脑回和跌倒发作的儿童，癫痫的手术治疗应谨慎考虑，并且应系统地进行睡眠脑电图记录。
