立体定向射频毁损治疗表现为痴笑性癫癎的下丘脑错构瘤

Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with gelastic seizures

目的总结采用立体定向热凝毁损手术方法治疗引起痴笑性癫癎的下丘脑微小错构瘤的经验。方法报告1例病人,男性,22岁,表现为痴笑性癫癎21年,继发复杂部分性发作7年,发作3～4次/d,药物治疗无效。MRI诊断为第三脑室内错构瘤,大小6mm×6mm×7mm。局麻下采用有框架立体定向技术,在病变内插入深部电极,记录并刺激后行射频毁损,制作4个靶点。结果深部电极在病灶内记录到棘波和慢波,刺激未诱发出痴笑性癫癎。术中行可逆毁损(45℃)和毁损(70℃)时,病人出现一过性中枢性高热、高血压和心动过速。术后随访6个月,痴笑性癫癎和复杂部分性发作完全消失,无手术并发症发生。结论下丘脑受到直接热刺激时可产生中枢性高热。对于较小的下丘脑错构瘤,立体定向热凝毁损术是一种安全有效的手术方法。

Objective To review the experience in the treatment of small hypothalamic hamartoma complicated with gelastic seizures by stereotactic radiofrequency thermocoagulation. Methods A 22-year-old male presented with intractable gelastic seizures for 21 years with complex partial seizure for 7 years, who had such seizures occurring 3-4 times a day and no efficacy with drug treatment. MRI revealed a small hypothalamic hamartoma （6 mm ×6 mm × 7 mm） in the third ventricle. Under local anesthesia, four lesions were created in the hamartoma with stereotactic radiofrequency thermocoagulation after depth electrode recording and stimulation. Results Intra-hamartoma depth electrode recordings showed spikes and slow waves. No gelastic seizure was induced by deep stimulation. Transient central hyperthermia, hypertension and tachycardia were observed during the reversible （45 ℃） and iureversibel （70 ℃） thermocoagulation. The patients were seizure free during 6-month follow up without permanent surgical complications. Conclusion High temperature stimulation of hypothalamus could induce central hyperthermia. Stereotactic radiofrequency thermocoagulation is an effective and safe treatment for small hypothalamic hamartoma.