Sturge-Weber syndrome - Indications and results of surgery in 20 patients

被引:77
作者
Arzimanoglou, AA
Andermann, F
Aicardi, J
Sainte-Rose, C
Beaulieu, MA
Villemure, JG
Olivier, A
Rasmussen, T
机构
[1] Univ Hosp Robert Debre, Serv Neurol Pediat & Malad Metab, Epilepsy Program, F-75019 Paris, France
[2] Univ Hosp Enfants Malades, Neurosurg Serv, Paris, France
[3] CHU Vaudois, Serv Neurochirurg, CH-1011 Lausanne, Switzerland
[4] McGill Univ, Dept Neurol & Neurosurg, Montreal, PQ H3A 2T5, Canada
[5] Montreal Neurol Hosp & Inst, Quebec City, PQ, Canada
关键词
D O I
10.1212/WNL.55.10.1472
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective: To discuss the indications and timing for resective surgery in patients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy. Background: 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. Methods: 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. Results: 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. Conclusion: 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.
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页码:1472 / 1479
页数:8
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