Kameyama Shigeki, Murakami Hiroatsu, Masuda Hiroshi, Sugiyama Ichiro
Department of Functional Neurosurgery, Epilepsy Center, Nishi-Niigata Chuo National Hospital, Niigata, Japan.
Neurosurgery. 2009 Sep;65(3):438-49; discussion 449. doi: 10.1227/01.NEU.0000348292.39252.B5.
To validate the safety and efficacy of magnetic resonance imaging (MRI)-guided stereotactic radiofrequency thermocoagulation (SRT) for epileptogenic hypothalamic hamartoma (HH), we evaluated surgical outcomes and revised the MRI classification.
We retrospectively reviewed 25 consecutive patients with HH (age range, 2-36 years; mean age, 14.8 years) with gelastic seizures. Other seizure types were exhibited in 22 patients (88.0%), precocious puberty in 8 (32.0%), behavioral disorder in 10 (40.0%), and mental retardation in 14 (56.0%). We classified HH into 3 subtypes according to coronal MRI: intrahypothalamic, parahypothalamic, and mixed hypothalamic type. Maximum diameter ranged from 8 to 30 mm (mean, 15.3 mm). All patients underwent SRT (74 degrees C, 60 seconds) for HH.
HH subtype and size were correlated with precocious puberty, mental retardation, and behavioral disorder. Thirty-one SRT procedures were performed, requiring 1 to 8 tracks (mean, 3.8 tracks) and involving 1 to 18 lesions (mean, 7.2 lesions). There were no adaptive limitations, regardless of size or subtype. Mixed-type HHs needed more tracks and more lesions. No permanent complications persisted after SRT, and gelastic seizures disappeared in all but 2 patients. Complete seizure freedom was achieved in 19 patients (76.0%). These patients had not only disappearance of all seizure types and behavioral disorder but also intellectual improvement.
The present SRT procedure has favorable efficacy and invasiveness and has no adaptive limitations. SRT should therefore be considered before adulthood. The new HH classification is useful to understand clinical symptoms and to determine surgical strategies.
为验证磁共振成像（MRI）引导下立体定向射频热凝术（SRT）治疗致痫性下丘脑错构瘤（HH）的安全性和有效性，我们评估了手术结果并修订了MRI分类。
我们回顾性分析了25例连续性HH患者（年龄范围2至36岁，平均年龄14.8岁），均有痴笑发作。22例患者（88.0%）还表现出其他发作类型，8例（32.0%）有性早熟，10例（40.0%）有行为障碍，14例（56.0%）有智力障碍。根据冠状位MRI将HH分为3种亚型：下丘脑内型、下丘脑旁型和混合型。最大直径为8至30毫米（平均15.3毫米）。所有患者均接受针对HH的SRT治疗（74℃，60秒）。
HH的亚型和大小与性早熟、智力障碍和行为障碍相关。共进行了31次SRT手术，需要1至8条轨迹（平均3.8条轨迹），涉及1至18个病灶（平均7.2个病灶）。无论大小或亚型如何，均无适应性限制。混合型HH需要更多的轨迹和更多的病灶。SRT后无永久性并发症，除2例患者外，所有患者的痴笑发作均消失。19例患者（76.0%）实现了完全无发作。这些患者不仅所有发作类型和行为障碍消失，而且智力有所改善。
目前的SRT手术具有良好的疗效和微创性，且无适应性限制。因此，应在成年前考虑SRT。新的HH分类有助于理解临床症状并确定手术策略。
