Posterior quadrant disconnection surgery for Sturge-Weber syndrome

作者：Sugano Hidenori^*; Nakanishi Hajime; Nakajima Madoka; Higo Takuma; Iimura Yasushi; Tanaka Kyoko; Hosozawa Mariko; Niijima Shinichi; Arai Hajime

来源：Epilepsia, 2014, 55(5): 683-689.

DOI：10.1111/epi.12547

• Sturge-Weber syndrome
• Epilepsy surgery
• Posterior quadrantectomy
• Seizure outcome
• Psychomotor development

ObjectiveSome patients with Sturge-Weber syndrome (SWS) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. The majority of patients with SWS have leptomeningeal angioma located over the temporal, parietal, and occipital lobes. We applied posterior quadrant disconnection surgery for this type of SWS with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development. %26lt;br%26gt;MethodsTen patients who were surgically treated using the posterior quadrantectomy (PQT) were enrolled in this study. Surgical outcome was analyzed as seizure-free or not at 2years after surgery. Psychomotor development was evaluated by the scores of mental developmental index (MDI) and psychomotor developmental index (PDI) in the Bayley Scales of Infant Development II preoperatively, and at 6 and 12 months after the PQT. %26lt;br%26gt;ResultsEight of 10 patients were seizure-free. Patients without complete elimination of the angiomatous areas had residual seizures. Average MDI and PDI scores before the surgery were 64.8 and 71.6, respectively. Scores of MDI at 6 and 12 months after the PQT in seizure-free patients were 80.5 and 84.5, respectively (p%26lt;0.01). PDI scores at these postoperative intervals were 87.3 and 86.4, respectively (p%26lt;0.05). Patients with residual seizures did not improve in either MDI or PDI. %26lt;br%26gt;SignificanceThe PQT achieved good seizure control and improved psychomotor development in patients with SWS. The complete deafferentation of angiomatous areas is required for seizure-free results and psychomotor developmental improvement. A PowerPoint slide summarizing this article is available for download in the Supporting Information section .

• 出版日期2014-5