Taha J M, Crone K R, Berger T S
Department of Neurosurgery, University of Cincinnati College of Medicine, Ohio.
J Neurosurg. 1994 Jul;81(1):37-42. doi: 10.3171/jns.1994.81.1.0037.
The role of hemispherectomy in treating holohemispheric hemimegaloencephaly, a unilateral brain malformation, is still not well defined. The authors describe the cases of five infants presenting with intractable seizures, progressive neurological deficits, and severe developmental delay. Electroencephalography (EEG) showed generalized polyspikes from the megaloencephalic hemisphere and progressive slowing on the opposite side in all children; contralateral seizure spikes occurred in three children. Three of the five children underwent hemispherectomy for intractable seizures before 2 years of age, after which the seizures subsided completely in two children and improved remarkably in the third. Preoperative Wada testing proved useful in evaluating pharmacologically the effect of hemispherectomy on contralateral polyspikes. Postoperative EEG revealed the absence of polyspikes in the operated hemisphere and decreased slowing on the contralateral side. Psychomotor development in the surgically treated infants exceeded that of the children not undergoing hemispherectomy. Of the two children treated medically, one died at 4 years of age in status epilepticus and the other (now 5 years old) has frequent seizures and severe developmental delay. Based on these results, hemispherectomy appears to be a useful procedure for controlling seizures and improving psychomotor development in children with hemimegaloencephaly involving the entire hemisphere. Surgery in infancy can prevent or minimize seizure foci and encephalopathic changes that may develop in the contralateral hemisphere. Staging the procedure and exercising meticulous hemostasis make surgery relatively safe in infants who otherwise may have significant blood loss associated with increased blood flow to the megaloencephalic hemisphere.
大脑半球切除术在治疗全大脑半球巨脑回畸形（一种单侧脑畸形）中的作用仍未明确界定。作者描述了5例婴儿的病例，这些婴儿表现为难治性癫痫、进行性神经功能缺损和严重发育迟缓。脑电图（EEG）显示，所有患儿的巨脑半球均出现广泛性多棘波，对侧则出现进行性减慢；3例患儿出现对侧癫痫棘波。5例患儿中有3例在2岁前因难治性癫痫接受了大脑半球切除术，术后2例患儿的癫痫完全缓解，第3例明显改善。术前Wada试验被证明有助于从药理学角度评估大脑半球切除术对对侧多棘波的影响。术后脑电图显示，手术侧半球无多棘波，对侧减慢程度减轻。接受手术治疗的婴儿的精神运动发育超过了未接受大脑半球切除术的儿童。在接受药物治疗的2例患儿中，1例在4岁时死于癫痫持续状态，另1例（现5岁）频繁发作癫痫且有严重发育迟缓。基于这些结果，大脑半球切除术似乎是控制涉及整个半球的巨脑回畸形患儿癫痫发作和改善精神运动发育的有效方法。婴儿期手术可预防或最小化对侧半球可能出现的癫痫病灶和脑病性改变。分阶段进行手术并细致止血，可使原本可能因巨脑半球血流量增加而有大量失血风险的婴儿手术相对安全。
