Abstract：
OBJECTIVE: Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications.
METHODS: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®.
RESULTS: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported.
CONCLUSIONS: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.
METHODS: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®.
RESULTS: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported.
CONCLUSIONS: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.
摘要：
目的：下丘脑错构瘤是先天性病变，通常表现为癫痫发作，难治性癫痫,神经发育迟缓,和严重的认知障碍。据报道，外科手术可以有效去除错构瘤，然而,它们与显著的发病率有关。因此,它不被认为是一种安全的治疗方式。图像引导的机器人放射外科（CyberKnife®放射外科系统）已被证明可以提供良好的结果，而不会出现持久的并发症。
方法：这一系列病例描述了临床，放射学,放射治疗,5例下丘脑错构瘤继发癫痫性脑病患者接受Cyberknife®治疗的术后结局。
结果：所有患者均表现为难治性癫痫，伴有弹性癫痫发作，不适合手术切除。处方剂量在16至25Gy之间，在严格遵守视觉通路限制的情况下，对四名患者进行单次递送，对一名患者进行五次递送。放射手术后,四名患者保持癫痫发作控制(一名患有EngelIa级，三个有恩格尔1d级)，另一个提出了零星的，非致残性癫痫发作（EngelIIa级）。经过24-26个月的随访，在三个病人中，他们的智商得分提高了。无并发症报告。
结论：该报告表明，Cyberknife可能是治疗下丘脑错构瘤的良好选择，特别是在没有其他非侵入性替代方案的情况下。然而，为了评估该技术在这些情况下的有效性，额外的研究是必不可少的。
方法：这一系列病例描述了临床，放射学,放射治疗,5例下丘脑错构瘤继发癫痫性脑病患者接受Cyberknife®治疗的术后结局。
结果：所有患者均表现为难治性癫痫，伴有弹性癫痫发作，不适合手术切除。处方剂量在16至25Gy之间，在严格遵守视觉通路限制的情况下，对四名患者进行单次递送，对一名患者进行五次递送。放射手术后,四名患者保持癫痫发作控制(一名患有EngelIa级，三个有恩格尔1d级)，另一个提出了零星的，非致残性癫痫发作（EngelIIa级）。经过24-26个月的随访，在三个病人中，他们的智商得分提高了。无并发症报告。
结论：该报告表明，Cyberknife可能是治疗下丘脑错构瘤的良好选择，特别是在没有其他非侵入性替代方案的情况下。然而，为了评估该技术在这些情况下的有效性，额外的研究是必不可少的。
