Arzimanoglou A A, Andermann F, Aicardi J, Sainte-Rose C, Beaulieu M A, Villemure J G, Olivier A, Rasmussen T
Service of Child Neurology and Metabolic Disorders, University Hospital Robert Debré, University Hospital Enfants Malades, Paris, France.
Neurology. 2000 Nov 28;55(10):1472-9. doi: 10.1212/wnl.55.10.1472.
To discuss the indications and timing for resective surgery in patients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy.
SWS that causes epilepsy severe enough to merit surgery is rare. Because of the variable natural history of the disease, it is difficult to establish clear-cut indications for surgery and prospective studies are not feasible. Attitudes of clinicians and surgeons remain variable.
The authors assessed the presurgical epilepsy profile, criteria for surgery, monitoring techniques, and the postoperative outcome of epilepsy in all patients with SWS consecutively admitted between 1972 and 1990 to two referral centers (Paris and Montreal) and underwent surgery for intractable seizures.
All 20 patients had a minimal postoperative follow-up of 4 years and all but one are still followed by one of the authors. One patient had a callosotomy, five underwent hemispherectomy, and 14 had cortical resection. Despite variability in the age at onset of seizures (range: 2 months to 12 years), age at operation (range: 8 months to 34 years) and surgical methods, almost all patients benefited from surgery. Visually guided complete resection of the pial angioma and underlying cortex, whenever possible, seemed sufficient; results were no better with intraoperative corticography. In children with previous hemiparesis, hemispherectomy proved particularly effective: all five became seizure free. None of the patients showed any aggravation of cognitive impairment following surgery; none of those who were operated on early presented with severe mental retardation, and 13 of 20 became seizure free.
Although the natural history of SWS is imperfectly known, increasing duration of seizures and of postictal deficits, increase in atrophy or of calcified lesions or both, are indicative of its progressive nature. Despite the expected heterogeneity that renders formal comparison of the various approaches difficult, the current study provides new evidence to support early surgery in patients with SWS and drug-resistant epilepsy. The authors' results suggest that lesionectomy is a good approach, provided that the pial angioma is unilateral and the resection can be complete.
探讨患有斯特奇-韦伯综合征（SWS）且药物难治性癫痫患者的切除性手术适应症及时机。
导致严重到需要手术治疗的癫痫的SWS很罕见。由于该疾病自然史的多变性，难以确立明确的手术适应症，前瞻性研究也不可行。临床医生和外科医生的态度仍存在差异。
作者评估了1972年至1990年间连续入住两个转诊中心（巴黎和蒙特利尔）并因顽固性癫痫接受手术的所有SWS患者的术前癫痫情况、手术标准、监测技术及癫痫术后结局。
所有20例患者术后至少随访4年，除1例患者外，其余患者仍由作者之一进行随访。1例患者接受了胼胝体切开术，5例接受了大脑半球切除术，14例接受了皮质切除术。尽管癫痫发作起始年龄（范围：2个月至12岁）、手术年龄（范围：8个月至34岁）和手术方法存在差异，但几乎所有患者都从手术中获益。只要有可能，视觉引导下完全切除软脑膜血管瘤及下方皮质似乎就足够了；术中皮质电图检查效果并无更好。对于既往有偏瘫的儿童，大脑半球切除术证明特别有效：所有5例患者术后均无癫痫发作。术后没有患者出现认知障碍加重；早期接受手术的患者均未出现严重智力发育迟缓，20例患者中有13例术后无癫痫发作。
尽管SWS的自然史尚未完全明确，但癫痫发作持续时间和发作后缺陷增加、萎缩或钙化病变增加或两者均增加，表明其具有进行性。尽管预期的异质性使得难以对各种方法进行正式比较，但本研究提供了新的证据支持对患有SWS和药物难治性癫痫的患者进行早期手术。作者的结果表明，只要软脑膜血管瘤是单侧的且切除可以完全，病灶切除术是一种很好的方法。
