Berdinov F B, Levov A V, Solovev V B, Kuznetsova A A, Dorofeeva M Yu, Kislyakov A N, Umerenkov V N, Livshits M I, Chmutin G E
Patrice Lumumba Peoples' Friendship University of Russia, Moscow, Russia.
Morozov Children's Clinical Hospital, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2025;89(3):17-27. doi: 10.17116/neiro20258903117.
The most common symptom of CNS damage in tuberous sclerosis is epilepsy. The last one is drug-resistant in most cases. Surgical treatment should be considered in these patients.
To analyze the results of surgical treatment of drug-resistant epilepsy in children with tuberous sclerosis, to identify predictors of successful treatment.
A retrospective study included 45 children between 2017 and 2023. In addition to standard non-invasive procedures, preoperative management included stereo-EEG in 19 cases, subdural EEG in 2 cases and staged subdural and stereo-EEG in 1 case. Selective tuberectomy was performed in 15 patients, tuberectomy (+) - in 16 children, lobectomy - in 6, multilobar resection - in 4, posterior quadrant disconnection - in 2, hemisphrotomy - in 2, repeated surgeries - in 6 children.
The median follow-up was 27 months. Postoperative outcomes ILAE grade I, IV, V and VI were observed in 62%, 25%, 11% and 2% of cases, respectively. Antiepileptic therapy was discontinued in 4.4% of cases. In 22.2% of patients, doses of drugs were reduced. Epileptogenic zone in frontal lobe favorably affected postoperative outcome (=0.027). Transient hemiparesis occurred in 5 (11.1%) cases, persistent expected hemiparesis - in 1 (2.2%) case.
Surgical treatment is effective for epilepsy in children with tuberous sclerosis. It is advisable to refer children with epilepsy and tuberous sclerosis to specialized centers for preoperative examination if therapy with two drugs is ineffective.
结节性硬化症中枢神经系统损伤最常见的症状是癫痫。在大多数情况下，最后一种是耐药性的。这些患者应考虑手术治疗。
分析结节性硬化症患儿耐药性癫痫的手术治疗结果，确定治疗成功的预测因素。
一项回顾性研究纳入了2017年至2023年间的45名儿童。除标准的非侵入性检查外，术前管理包括19例立体脑电图、2例硬膜下脑电图和1例分期硬膜下和立体脑电图。15例患者进行了选择性结节切除术，16例儿童进行了结节切除术（+），6例进行了叶切除术，4例进行了多叶切除术，2例进行了后象限离断术，2例进行了大脑半球切开术，6例儿童进行了再次手术。
中位随访时间为27个月。术后结果分别在62%、25%、11%和2%的病例中观察到国际抗癫痫联盟（ILAE）I级、IV级、V级和VI级。4.4%的病例停用了抗癫痫治疗。22.2%的患者药物剂量减少。额叶致痫区对术后结果有有利影响（P=0.027）。5例（11.1%）出现短暂性偏瘫，1例（2.2%）出现持续性预期偏瘫。
手术治疗对结节性硬化症患儿的癫痫有效。如果两种药物治疗无效，建议将患有癫痫和结节性硬化症的儿童转诊至专业中心进行术前检查。
