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下丘脑错构瘤:临床评估和诊断的最佳方法。

Hypothalamic hamartomas: optimal approach to clinical evaluation and diagnosis.

机构信息

Division of Pediatric Neurology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, U.S.A.

出版信息

Epilepsia. 2013 Dec;54 Suppl 9:109-14. doi: 10.1111/epi.12454.

Abstract

Hypothalamic hamartomas (HHs) present a difficult medical problem, manifested by gelastic seizures, which are often medically intractable. Although existing techniques offer modest surgical outcomes with the potential for significant morbidity, the relatively novel technique of magnetic resonance imaging (MRI)-guided stereotactic laser ablation (SLA) offers a potentially safer, minimally invasive method with high efficacy for the HH treatment. We report here on 14 patients with medically refractory gelastic epilepsy who underwent stereotactic frame-based placement of an MR-compatible laser catheter (1.6 mm diameter) through a 3.2-mm twist drill hole. A U.S. Food and Drug Administration (FDA)-cleared laser surgery system (Visualase, Inc.) was utilized to ablate the HH, using real-time MRI thermometry. Seizure freedom was obtained in 12 (86%) of 14 cases, with mean follow-up of 9 months. There were no permanent surgical complications, neurologic deficits, or neuroendocrine disturbances. One patient had a minor subarachnoid hemorrhage that was asymptomatic. Most patients were discharged home within 1 day. SLA was demonstrated to be a safe and effective minimally invasive tool in the ablation of epileptogenic HH. Because use of SLA for HH is being adopted by other medical centers, further data will be acquired to help treat this difficult disorder.

摘要

下丘脑错构瘤 (HHs) 表现为一种医学难题,其特征为痴笑发作,且往往难以用药物治疗。虽然现有的技术提供了适度的手术效果,但具有较高发病率的潜在风险,而相对新颖的磁共振成像 (MRI) 引导立体定向激光消融 (SLA) 技术为 HH 治疗提供了一种潜在更安全、微创、高效的方法。我们在此报告 14 例经药物难治性痴笑性癫痫患者,通过 3.2mm 麻花钻钻孔将可兼容 MRI 的激光导管(直径 1.6mm)通过立体定向框架放置。使用经美国食品和药物管理局 (FDA) 批准的激光手术系统 (Visualase, Inc.),利用实时 MRI 测温来消融 HH。14 例中有 12 例(86%)获得了无癫痫发作,平均随访 9 个月。无永久性手术并发症、神经功能缺损或神经内分泌紊乱。1 例患者发生无症状性轻微蛛网膜下腔出血。大多数患者在 1 天内出院。SLA 被证明是一种安全有效的微创工具,可用于消融致痫性 HH。由于其他医疗中心也在采用 SLA 治疗 HH,因此将获得更多数据来帮助治疗这种难治性疾病。

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