Shields W D, Shewmon D A, Peacock W J, LoPresti C M, Nakagawa J A, Yudovin S
Division of Pediatric Neurology, UCLA Children's Hospital, Los Angeles, California 90095-1752, USA.
Epilepsia. 1999 Sep;40(9):1305-8. doi: 10.1111/j.1528-1157.1999.tb00862.x.
The most appropriate time to consider cortical resection to treat medically intractable infantile spasms has not been clearly defined. The risks that need to be reconciled to make this decision are: What is the risk of loss of developmental potential if surgery is delayed too long versus what is the risk of unnecessary surgery if it is done too soon. We propose that, in addition to evaluation of seizures, developmental assessment is a key factor in the surgical decision. The case report illustrates this concept.
HC had onset of seizures at 9 days of age and developed infantile spasms due to mild right hemimegancephaly. At 19 months, she was having up to 50 seizures/day and was evaluated and approved for right hemispherectomy but surgery was delayed. Despite the seizures, her development had been much better than most patients with hemimegencephaly and infantile spasms. At 25 months her seizure control was much improved but she had several seizures/week. EcoG at the time of surgery did not demonstrate the usual abnormalities so no resection was performed. She has had only 5 seizures in the 2(1/2) year since.
(a) Hemimegalencephaly is not always associated with severe mental retardation; (b) normal or near-normal development may, in some cases, indicate the possibility of medical control of seizures as the child grows; (c) a localized developmental brain abnormality in a child with intractable seizures should not necessarily lead to cortical resection; and (d) when a child meets developmental milestones, it may be appropriate to delay surgical intervention.
目前尚未明确界定考虑进行皮质切除术治疗药物难治性婴儿痉挛症的最合适时机。做出这一决定需要权衡的风险是：如果手术延迟过长，发育潜能丧失的风险与如果手术进行得过早则进行不必要手术的风险相比如何。我们提出，除了评估癫痫发作外，发育评估是手术决策的关键因素。本病例报告说明了这一概念。
HC在9日龄时开始出现癫痫发作，并因轻度右侧半巨脑畸形而发展为婴儿痉挛症。19个月大时，她每天癫痫发作多达50次，并接受了评估，被批准进行右半球切除术，但手术被推迟。尽管有癫痫发作，但她的发育情况比大多数患有半巨脑畸形和婴儿痉挛症的患者要好得多。25个月大时，她的癫痫控制情况有了很大改善，但仍每周发作几次。手术时的脑电图未显示出通常的异常，因此未进行切除。自那以后的两年半时间里，她仅发作了5次。
（a）半巨脑畸形并不总是与严重智力发育迟缓相关；（b）在某些情况下，正常或接近正常的发育可能表明随着孩子成长癫痫发作有可能通过药物控制；（c）患有难治性癫痫发作的儿童出现局部发育性脑异常不一定需要进行皮质切除术；（d）当孩子达到发育里程碑时，推迟手术干预可能是合适的。
