Wyllie E, Comair Y G, Kotagal P, Raja S, Ruggieri P
Department of Neurology, Cleveland Clinic Foundation, OH 44195, USA.
Epilepsia. 1996 Jul;37(7):625-37. doi: 10.1111/j.1528-1157.1996.tb00626.x.
We report 12 infants who had frontal (n = 3), temporal (n = 2), or temporoparieto-occipital (n = 2) resection or functional hemispherectomies (n = 5) at age 2.5-29 (mean 15.3) months for catastrophic epilepsy due to focal cortical dysplasia (n = 5), Sturge-Weber syndrome (n = 3), ganglioglioma (n = 3), or hemimegalencephaly (n = 1). Seizures began at 1 day to 14 months (mean, 4.0 months) after birth, occurred frequently (often many times per day, and were refractory to antiepileptic drugs. Patients were evaluated for surgery at 2.5-24 (mean 12.4) months of age. Seven patients had hemiparesis and eight had slowed cognitive development. Seizures were characterized by arrest or marked reduction of behavioral motor activity with nuclear level of consciousness (n = 4, with temporal or temporoparietal EEG seizures), focal clonic activity (n = 3, with perirolandic EEG seizures), generalized tonic stiffening (n = 3, with temporoparieto-occipital, parietal, or frontal EEG seizures), or infantile spasms and hypsarrhythmia (n = 2, with a frontal tumor or temporoparieto-occipital cortical dysplasia).
Magnetic resonance imaging (MRI) revealed the epileptogenic lesion in all but two patients, both with cortical dysplasia localized by interictal positron-emission tomography (PET) and other clinical or EEG features and confirmed on histopathologic examination of resected tissue.
At follow-up 4-86 (mean 32) months after surgery, six patients were seizure free (Engel outcome class I), three had rare seizures with none in at least the previous 6 months (Engel class II), and two had worthwhile improvement (Engel class III). Except for the severely developmentally delayed infant with hemimegalencephaly, several patients had marked "catch-up" developmental progress after class I, II, or III outcome. Postoperative complications included subdural hematoma over the contralateral hemisphere (one patient) and entrapment and enlargement of the ipsilateral temporal horn (one patient) after hemispherectomy, both corrected uneventfully with a second surgical procedure. One patient died of unexplained causes several hours after frontal lobectomy. No patients had new neurologic deficits after surgery, and one patient had resolution of progressive fluctuating hemiparesis after resection of temporoparieto-occipital cortical dysplasia.
Our results agree with previous reports that epilepsy surgery can provide relief from catastrophic epilepsy in carefully selected infants.
我们报告了12例婴儿，他们在2.5至29（平均15.3）个月大时接受了额叶（n = 3）、颞叶（n = 2）或颞顶枕叶（n = 2）切除术或功能性大脑半球切除术（n = 5），病因是灾难性癫痫，分别由局灶性皮质发育不良（n = 5）、斯特奇-韦伯综合征（n = 3）、神经节胶质瘤（n = 3）或半侧巨脑症（n = 1）引起。癫痫发作始于出生后1天至14个月（平均4.0个月），发作频繁（通常每天多次），且对抗癫痫药物难治。患者在2.5至24（平均12.4）个月大时接受手术评估。7例患者有偏瘫，8例认知发育迟缓。癫痫发作的特征为行为运动活动停止或显著减少且意识水平正常（n = 4，伴有颞叶或颞顶叶脑电图癫痫发作）、局灶性阵挛活动（n = 3，伴有中央前回周围脑电图癫痫发作）、全身性强直僵硬（n = 3，伴有颞顶枕叶、顶叶或额叶脑电图癫痫发作）或婴儿痉挛和高度节律失调（n = 2，伴有额叶肿瘤或颞顶枕叶皮质发育不良）。
除2例患者外，磁共振成像（MRI）显示了所有患者的致痫病变，这2例患者的皮质发育不良通过发作间期正电子发射断层扫描（PET）及其他临床或脑电图特征定位，并在切除组织的组织病理学检查中得到证实。
术后4至86（平均32）个月的随访中，6例患者无癫痫发作（恩格尔结果分级I级），3例很少发作，至少在过去6个月内无发作（恩格尔分级II级），2例有明显改善（恩格尔分级III级）。除了患有半侧巨脑症且严重发育迟缓的婴儿外，几名患者在I、II或III级结果后有显著的“追赶”发育进展。术后并发症包括对侧半球硬膜下血肿（1例患者）和大脑半球切除术后同侧颞角受压及扩大（1例患者），两者均通过第二次手术顺利纠正。1例患者在额叶切除术后数小时死于不明原因。术后无患者出现新的神经功能缺损，1例患者在切除颞顶枕叶皮质发育不良后进行性波动偏瘫得到缓解。
我们的结果与先前的报告一致，即癫痫手术可以为精心挑选的婴儿缓解灾难性癫痫。
